BACKGROUND Langerhans cell histiocytosis(LCH)is a rare clonal proliferative disease of Langerhans cells with unknown pathogenesis.An increasing number of clinicians recognize that LCH has a wide clinical spectrum and ...BACKGROUND Langerhans cell histiocytosis(LCH)is a rare clonal proliferative disease of Langerhans cells with unknown pathogenesis.An increasing number of clinicians recognize that LCH has a wide clinical spectrum and a highly varied course.Adults rarely develop LCH.Here,we report a case of adult localized LCH.CASE SUMMARY A 32-year-old woman presented with plaques and ulcers on the vulva and crissum,accompanied by pain that persisted for more than one year.Physical examination revealed a red-infiltrating plaque with ulcerations and exudates in the vulva and crissum.Pathological examination revealed a diffuse infiltration of lymphocytes,eosinophilic granulocytes,and histiocytoid cells in the superficial dermis.Proliferative histiocytoid cells showed mild atypia,partly with kidneyshaped nuclei.Immunohistochemical examination showed that the histiocytoid cells were positive for S100 protein and CD1 and weakly positive for CD68(20%+),with a Ki-67 index of 30%.Laboratory tests did not reveal any other systemic damage.The patient was diagnosed with adult localized LCH and was prescribed oral prednisone(20 mg)once daily.The skin lesions gradually improved and are still being followed-up.CONCLUSION Adult localized LCH is rare and must be differentiated from other common conditions.展开更多
The brain parenchymal Langerhans cell histiocytosis (LCH) without systemic disease or lytic skull lesions is extremely rare. We report a 23-year-old male presenting with new onset 1 hour seizure with loss of conscio...The brain parenchymal Langerhans cell histiocytosis (LCH) without systemic disease or lytic skull lesions is extremely rare. We report a 23-year-old male presenting with new onset 1 hour seizure with loss of consciousness 20 days prior to admission, and recurrent seizure 2 weeks later. Brain magnetic resonance imaging (MRI) showed an irregularly mass with enhancement involving the right frontal lobe. Microscopically, the lesion was characterized by sheets of Langerhans cells in addition to reactive inflammatory elements. Immunohistochemically, Langerhans cells were positive for Langerin, CDla and S-100 protein. The patient received no chemotherapy or radiotherapy after surgery. After 24 months of follow-up, no recurrence or other systemic lesions were observed. Although there is no standard treatment for solitary cerebral LCH, the prognosis generally appears to be good.展开更多
BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a rare cystic lung disease usually affecting young adults.It is predicted that PLCH is a lung tumor precursor associated with dysfunction of the myeloid dendr...BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a rare cystic lung disease usually affecting young adults.It is predicted that PLCH is a lung tumor precursor associated with dysfunction of the myeloid dendritic cells in the lung.CASE SUMMARY A 70-year-old male patient presented with chronic cough and sputum.He had symptoms for 5 years and described shortness of breath on exertion for the previous 3 years.He had a 60 packs/year smoking history.Computerized tomography of the thorax revealed an 11-mm nodule in the right lung lower lobe superior segment and a 7-mm nodule in the right lung lower lobe poster basal segment.Those two nodules were resected by means of right thoracoscopic surgery.Pathological evaluation revealed a squamous cell carcinoma and PLCH.CONCLUSION Coexistent squamous cell carcinoma and PLCH suggest possible association between PLCH and lung cancer.展开更多
BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a relatively rare type of lung disease,common in middle-aged smoking men.It is characterized by proliferation and infiltration of Langerhans cells,and the for...BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a relatively rare type of lung disease,common in middle-aged smoking men.It is characterized by proliferation and infiltration of Langerhans cells,and the formation of multiple parabronchial mesenchymal nodules in lung tissue,and may lead to organ dysfunction.There are no typical symptoms and signs,and it is easily misdiagnosed or missed,and therefore deserves clinical attention and further discussion.CASE SUMMARY We describe the case of a nonsmoking 46-year-old man with PLCH diagnosed based on clinical manifestations of fever and dry cough,with a history of hypothyroidism and diabetes insipidus for 9 years.Computed tomography(CT)-and CT-guided puncture examinations revealed no abnormalities,and he ultimately underwent thoracoscopic biopsy to confirm the diagnosis.The pathological diagnosis was PLCH.Thyroid function was maintained by medication.Pituitary magnetic resonance imaging showed that the pituitary stalk had become thinner.CONCLUSION LCH often involves multiple systems.Moreover,the pathogenesis is not clear,clinical manifestations lack specificity,and diagnosis requires special attention.Diagnosis of PLCH can significantly benefit from comprehensive multidisciplinary analysis.展开更多
BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report...BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult. CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea. CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thinwalled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.展开更多
BACKGROUND The common computed tomography findings of pulmonary Langerhans cell histiocytosis (PLCH) are multiple cysts and micronodules predominantly in middle to upper lung lobes.Non-cystic nodules and large nodules...BACKGROUND The common computed tomography findings of pulmonary Langerhans cell histiocytosis (PLCH) are multiple cysts and micronodules predominantly in middle to upper lung lobes.Non-cystic nodules and large nodules are atypical findings of PLCH.CASE SUMMARY The patient was a 48-year-old Japanese man with a smoking history (20 cigarettes/d,28 years) and no symptoms.Multiple nodules existed in all lung lobes,predominantly in the right lower lobe.Some nodules seemed to be distributed randomly,and others were adjacent to bronchus.Most nodules were solid;some small ones were cystic.The largest nodule was 22 mm in diameter.Although metastatic lung tumors were suspected,thoracoscopic lung biopsy led to the diagnosis of PLCH.At 6 months after he quit smoking,all nodules had almost disappeared.We investigated the characteristics of nodules at diagnosis in detail.Of 349 nodules in total,116 were in upper and 199 were in lower lobes.Ninety-six (27.5%) were cystic;the remaining 253 (72.5%) were non-cystic.The prevalence of cystic nodules was higher in upper lobes than in lower lobes (right upper 37.5% vs lower 18.2%,P = 0.0068;left upper 48.1% vs lower 24.4%,P = 0.0078).The average size (dia.) of cystic nodules was smaller than that of noncystic nodules (5.03 mm vs 7.40 mm,respectively,P < 0.0001).CONCLUSION Although multiple non-cystic nodules including large nodules (over 20 mm) are atypical,PLCH should be included in differential diagnoses.The presence of small cystic nodules predominantly in upper lobes and asymptomatic situation are also important for differential diagnoses to distinguish from metastatic cancers.展开更多
Objective: Langerhans cell histiocytosis (LCH) has been well described only in children. We analyzed the characteristics, reactivation, and outcome of LCH in a cohort of 55 patients across all ages. Methods: We review...Objective: Langerhans cell histiocytosis (LCH) has been well described only in children. We analyzed the characteristics, reactivation, and outcome of LCH in a cohort of 55 patients across all ages. Methods: We reviewed the records of all patients with LCH treated at a single institute between Jan. 1974 and May 1998. Results: The 55 patients were 2 to 67 years of age (median, 31 years) at the time of diagnosis, and 85.5% were male. Forty patients (72.7%) had single-system LCH; Fifteen (27.3%) had multisystem disease. The head and neck was the most frequent tumor site (63.6%). LCH was not found in organs at risk of involvement (liver, spleen, bone marrow, and lungs). The frequency of bony invasion (23.6% overall) differed significantly according to age ≤15 years (66.7%) vs. age >15 years (11.6%) (P=0.0005). At a median follow-up of 12 years, no patient died of LCH. The 5, 10-year survival estimates were 100%. The 5, 10-year disease-free survival estimates were 70.9% and 58.4%. The 5-year disease-free survival estimate was 58.3% for age ≤ 15 years vs. 74.4% for age >15 years (P=0.83) and 75% for single-system disease vs. 60% for multisystem disease (P=0.13). LCH was reactivated in 43.6% of patients, with a median of 14 months (range, 2-180 months). Three patients with recurrent disease experienced spontaneous remission. At the time of the most recent follow-up, 23.6% of survivors had active disease. Conclusion: LCH is not found exclusively in children and adolescents. The frequency of bone invasion is inversely related to age. Reactivation is very common regardless of the type of treatment, but the prognosis is generally good.展开更多
BACKGROUND Langerhans cell histiocytosis(LCH)is a rare condition wherein Langerhans cells proliferate abnormally,adversely impacting organs including lymph nodes,bones,skin,lungs,and pituitary gland.The LCH disease co...BACKGROUND Langerhans cell histiocytosis(LCH)is a rare condition wherein Langerhans cells proliferate abnormally,adversely impacting organs including lymph nodes,bones,skin,lungs,and pituitary gland.The LCH disease course varies widely among patients from a self-limiting condition to one that progresses rapidly and culminates in death.It is uncommon for multisystem LCH to be observed in adults.Herein we describe a woman suffering from multi-system LCH involvement.CASE SUMMARY A 37-year old Chinese woman was admitted to the hospital in June 2019 suffering from dyspnea that had progressed over the course of 5 years.Her medical history included:central diabetes insipidus(DI)that had been treated via radiotherapy,desmopressin acetate,and bromocriptine;bilateral pneumothorax with two surgeries having been performed to remove bullae;and autoimmune hepatitis that had been unsuccessfully treated using a combination of methylprednisolone and mycophenolate mofetil.A chest computed tomography(CT)scan revealed the presence of multiple pulmonary cysts of varying sizes.We re-analyzed right pulmonary bullae samples that had been removed in 2014,performed a systematic 18 F-FDG PET/CT analysis,and convened a multidisciplinary medical team to diagnose and treat this patient.As a result,we were able to eventually diagnose this patient with LCH that was not associated with BRAF-V600 E mutations.CONCLUSION We hope to emphasize the importance of systemic evaluation and of cooperation between multidisciplinary physicians with the goal of improving awareness and detection of this orphan disease.展开更多
Langerhans cell histiocytosis(LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma(EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many re...Langerhans cell histiocytosis(LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma(EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. We report here a new observation of atlantoaxial LCH in a 4-year-old boy revealed by persistent torticollis. He was successfully treated with systemic chemotherapy and surgery. Inter-body fusion packed by autologous iliac bone was performed with resolution of his symptoms. It is known that conservative treatment is usually sufficient and surgery should be reserved for major neurologic defects in spine EG. In atlantoaxial lesion, surgical treatment should be frequently considered.展开更多
Langerhans cell histiocytosis(LCH)is a malignant disease of the histiocytes involving various organ systems.The spectrum of liver involvement in LCH ranges from mild transaminitis to end-stage liver disease.The hallma...Langerhans cell histiocytosis(LCH)is a malignant disease of the histiocytes involving various organ systems.The spectrum of liver involvement in LCH ranges from mild transaminitis to end-stage liver disease.The hallmark of hepatic LCH is secondary sclerosing cholangitis,which manifests due to a progressive destruction of the biliary tree by malignant histiocytes.Chemotherapy remains the mainstay of treatment for active LCH.Early recognition,diagnosis and a systematic approach to the management of LCH can ameliorate the disease process.Nonetheless,the liver involvement in these patients may progress despite the LCH being in remission.Liver transplantation(LT)remains central in the management of such patients.Various facets of the management of LCH,especially those with liver involvement remain unclear.Furthermore,aspects of LT in LCH with regards to the indication,timing and post-LT management,including immunosuppression and adjuvant therapy,remain undefined.This review summarises the current evidence and discusses the practical aspects of the role of LT in the management of LCH.展开更多
Introduction: Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils, resulting in so...Introduction: Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils, resulting in solitary or few, indolent and chronic, lesions of bone or other organs called eosinophilic granulomas. Calvarial LCH is quite rare and an underappreciated differential etiology of skull lesions. We present a most unusual case of a young child with hyperacutely symptomatic langerhans histiocytosis of the skull. Method: A 7-year-old male presented with a history of increasing (progressive) frontal headaches of 8 days duration, unaccompanied by associated nausea, vomiting, or diplopia. His only additional complaint was a hard bump on his forehead. MRI and CT done in the ER identified a right fronto-parietal lesion with associated skull erosion. Nuclear medicine and SPECT studies confirmed an erosive skull lesion without significant metabolic activity. A right frontal craniectomy and excision was performed. Results: A soft, rubbery well-circumcised mass coming from the diploic layer of the skull with involvement of bone was identified. The mass had eroded both the outer and inner table of the skull, and the involved area of the right frontal bone was resected. Intra-operative histo-pathologic analysis of the lesion revealed Langerhans cell histiocytosis without involvement of the dura. The patient experienced no neurological worsening as a result of the resection. He was discharged home in stable condition. Conclusion: LCH lesions of the skull are common findings, however, this focal hyperacute symptomatic presentation is most rare and should not deter us from anticipating an erosive bony tumor and planning timely surgical management.展开更多
Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. Clinical presentation is variable, ranging from a single location in the bone to se...Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. Clinical presentation is variable, ranging from a single location in the bone to severe multivisceral involvement. Moreover, spinal involvement causing myelopathy is even rare and unusual. We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution.展开更多
To find out the most appropriate management,clinical features of 18 cases of adult multisystem langerhans cell histiocytosis(LCH) have been analyzed. The patients comprising of 9 males and 9 females were median age of...To find out the most appropriate management,clinical features of 18 cases of adult multisystem langerhans cell histiocytosis(LCH) have been analyzed. The patients comprising of 9 males and 9 females were median age of 36 years,ranging from 18-53 years at diagnosis. Regarding the initial symptoms,7 patients(2 males and 5 females) showed central diabetes insipidus(CDI) and other endocrine symptoms with thickened pituitary stalk or a mass at the hypothalamic region. Additional 2 patients initiated the disease with CDI with no immediate diagnosis. In the remaining patients,the diseasebegun with single(n = 3) or multiple(n = 1) spinal bone lesion(s) in 4 patients(all males),with multiple bone lesions in 3 patients(1 male and 2 females),with single skull lesion in one female patient and with ambiguous symptoms including hypothyroidism in the remaining one male patient. We also recognized the correlation between pregnancy/childbirth and LCH in 4 patients. In terms of treatment,9 patients received systemic immuno-chemotherapy alone,of which the majority received vinblastine-based chemotherapy while 4 received 2-chlorodeoxyadenosine. Five had a combination of immuno-chemotherapy with surgical resection or radiotherapy,2 had immunotherapy alone,2 had surgical resection followed by observation alone to date. Three patients received hematopoietic stem cell transplantation after extensive chemotherapy. In terms of outcome,15 patients are alive(9 with active disease,6 without active disease),with a median of 66 mo(range 17-166 mo),two died of disease while the remaining 1 lost to follow-up. Based on these results,we think that early diagnosis and rapid introduction of appropriate treatment are essential,in order to overcome the problems relevant to adult LCH.展开更多
Langerhans cell histiocytosis(LCH)is a rare neoplastic disease in dendritic cells.LCH is classified as either a single-system(SS)or multisystem(MS)disease.There is not a standard first-line treatment for LCH in adults...Langerhans cell histiocytosis(LCH)is a rare neoplastic disease in dendritic cells.LCH is classified as either a single-system(SS)or multisystem(MS)disease.There is not a standard first-line treatment for LCH in adults.We analyzed the efficacy and safety of immunomodulatory drugs(IMiDs)by searching PubMed/MEDLINE for case reports previously published.The clinical response(nonactive disease or active disease that regressed)was 94%in SS and 53%in MS.IMiDs should only be considered for adults with cutaneous SS involvement;in MS,they should be used only for patients not eligible for more aggressive treatments.展开更多
Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of ...Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of the disease involving the bone, the skin or the pituitary gland have been well described whereas others, such as thymic histiocytosis, are still poorly understood. Here, we report a case of isolated Langerhans cell histiocytosis of the thymus in an infant with non-specific symptoms. We provide a description of the disease and discuss patient management.展开更多
Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an...Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-yearold woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increas-ing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.展开更多
Hepatic Langerhans cell histiocytosis(LCH)is characterized by proliferation and accumulation of Langerhans cells in the liver,causing liver dysfunction or forming a mass lesion.The liver can be involved in isolation,o...Hepatic Langerhans cell histiocytosis(LCH)is characterized by proliferation and accumulation of Langerhans cells in the liver,causing liver dysfunction or forming a mass lesion.The liver can be involved in isolation,or be affected along with other organs.A common clinical hepatic presentation is cholestasis with pruritis,fatigue and direct hyperbilirubinemia.In late stages,there may be hypoalbuminemia.Liver biopsy may be required for the diagnosis of hepatic LCH.Histologic finding may be diverse,including lobular Langerhans cell infiltrate with mixed inflammatory background,primary biliary cholangitis-like pattern,sclerosing cholangitis-like pattern,and even cirrhosis at later stages.Because of its non-specific injury patterns with broad differential diagnosis,establishing a diagnosis of hepatic LCH can be challenging.Hepatic LCH can easily be missed unless this diagnosis is considered at the time of biopsy interpretation.A definitive diagnosis relies on positive staining with CD1a and S100 antigen.Liver involvement is a high risk feature in LCH.The overall prognosis of hepatic LCH is poor.Treating at an early stage may improve the outcome.Systemic chemotherapy is the mainstay of treatment and liver transplantation may be offered.New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy.However,further studies are needed to improve outcome.展开更多
Purpose:Ocular trauma occurs disproportionately in children on an annual basis.However,because of this frequency,other diagnoses,.such as orbital neoplasms,.can easily be ignored.Methods:We report on a two-year-old bo...Purpose:Ocular trauma occurs disproportionately in children on an annual basis.However,because of this frequency,other diagnoses,.such as orbital neoplasms,.can easily be ignored.Methods:We report on a two-year-old boy who presented with a dark purple,irregularly shaped lesion on his lower left eyelid.The patient had suffered injuries twice to that area.Results:Axial computed tomography images demonstrated an ill-defined and inhomogeneous soft tissue mass in the lateral and posterior region of the left orbit,.with bony destruction and absorption of the adjacent orbital wall..Magnetic resonance imaging of the orbit showed a lesion involving the greater sphenoidal wing,the supraorbital wall,and the frontal area.Enlarged nuchal and inguinal lymph nodes were detected by sonographic examination.Histopathological examination of a surgical biopsy showed an accumulation of characteristic Langerhans cells.The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemical examinations positive for CD1a and SP-100.Conclusion:Orbital Langerhans cell histiocytosis is rarely encountered in ophthalmic practice,.so the ophthalmologist needs to be familiar with its presentation and work-up,.and has to be aware of possible OLCH diagnosis when a child(or even an adult)presents with prolonged and persistent eyelid edema,.even with a history of ocular trauma.展开更多
LANGERHANS cell histiocytosis(LCH)is a rare disease,mainly involving the bone,skin,lung,liver,spleen,and skin.1 The heterogenenous nature of LCH makes it difficult to diagnose.Not only do the involved organs vary from...LANGERHANS cell histiocytosis(LCH)is a rare disease,mainly involving the bone,skin,lung,liver,spleen,and skin.1 The heterogenenous nature of LCH makes it difficult to diagnose.Not only do the involved organs vary from case to case,but also its natural history.Herein,we describe a rare case of conjuctival LCH in an Asian woman.展开更多
Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells, characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body...Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells, characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body, including the temporal bone which localization is rare in children. The objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare our case with previously reported series. We report the case of a young 4-year-old patient with a history of otorrhea and recurrent otalgia, with left axillary non-blowing exophthalmia progressive since 5 months associated with indurated left temporal swelling and also an indurated swelling of posterior face of the left shoulder on pathological fracture. A cerebro-orbital MRI performed at home showed an extra-conical process with extension at the temporal fossa. The patient, then, underwent an open-air biopsy of the temporal mass and the pathological analysis returned to favor Langerhans cell histiocytosis. The diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The child was treated by chemotherapy with a good outcome. Our results concord with literature. The prognosis is different and better in children with unifocal bone disease than those with a multifocal disease with a survival rate of 65% - 100%.展开更多
基金Traditional Chinese Medicine Research Program of Hebei Provincial Administration of Traditional Chinese Medicine,No.2022465.
文摘BACKGROUND Langerhans cell histiocytosis(LCH)is a rare clonal proliferative disease of Langerhans cells with unknown pathogenesis.An increasing number of clinicians recognize that LCH has a wide clinical spectrum and a highly varied course.Adults rarely develop LCH.Here,we report a case of adult localized LCH.CASE SUMMARY A 32-year-old woman presented with plaques and ulcers on the vulva and crissum,accompanied by pain that persisted for more than one year.Physical examination revealed a red-infiltrating plaque with ulcerations and exudates in the vulva and crissum.Pathological examination revealed a diffuse infiltration of lymphocytes,eosinophilic granulocytes,and histiocytoid cells in the superficial dermis.Proliferative histiocytoid cells showed mild atypia,partly with kidneyshaped nuclei.Immunohistochemical examination showed that the histiocytoid cells were positive for S100 protein and CD1 and weakly positive for CD68(20%+),with a Ki-67 index of 30%.Laboratory tests did not reveal any other systemic damage.The patient was diagnosed with adult localized LCH and was prescribed oral prednisone(20 mg)once daily.The skin lesions gradually improved and are still being followed-up.CONCLUSION Adult localized LCH is rare and must be differentiated from other common conditions.
文摘The brain parenchymal Langerhans cell histiocytosis (LCH) without systemic disease or lytic skull lesions is extremely rare. We report a 23-year-old male presenting with new onset 1 hour seizure with loss of consciousness 20 days prior to admission, and recurrent seizure 2 weeks later. Brain magnetic resonance imaging (MRI) showed an irregularly mass with enhancement involving the right frontal lobe. Microscopically, the lesion was characterized by sheets of Langerhans cells in addition to reactive inflammatory elements. Immunohistochemically, Langerhans cells were positive for Langerin, CDla and S-100 protein. The patient received no chemotherapy or radiotherapy after surgery. After 24 months of follow-up, no recurrence or other systemic lesions were observed. Although there is no standard treatment for solitary cerebral LCH, the prognosis generally appears to be good.
文摘BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a rare cystic lung disease usually affecting young adults.It is predicted that PLCH is a lung tumor precursor associated with dysfunction of the myeloid dendritic cells in the lung.CASE SUMMARY A 70-year-old male patient presented with chronic cough and sputum.He had symptoms for 5 years and described shortness of breath on exertion for the previous 3 years.He had a 60 packs/year smoking history.Computerized tomography of the thorax revealed an 11-mm nodule in the right lung lower lobe superior segment and a 7-mm nodule in the right lung lower lobe poster basal segment.Those two nodules were resected by means of right thoracoscopic surgery.Pathological evaluation revealed a squamous cell carcinoma and PLCH.CONCLUSION Coexistent squamous cell carcinoma and PLCH suggest possible association between PLCH and lung cancer.
文摘BACKGROUND Pulmonary Langerhans cell histiocytosis(PLCH)is a relatively rare type of lung disease,common in middle-aged smoking men.It is characterized by proliferation and infiltration of Langerhans cells,and the formation of multiple parabronchial mesenchymal nodules in lung tissue,and may lead to organ dysfunction.There are no typical symptoms and signs,and it is easily misdiagnosed or missed,and therefore deserves clinical attention and further discussion.CASE SUMMARY We describe the case of a nonsmoking 46-year-old man with PLCH diagnosed based on clinical manifestations of fever and dry cough,with a history of hypothyroidism and diabetes insipidus for 9 years.Computed tomography(CT)-and CT-guided puncture examinations revealed no abnormalities,and he ultimately underwent thoracoscopic biopsy to confirm the diagnosis.The pathological diagnosis was PLCH.Thyroid function was maintained by medication.Pituitary magnetic resonance imaging showed that the pituitary stalk had become thinner.CONCLUSION LCH often involves multiple systems.Moreover,the pathogenesis is not clear,clinical manifestations lack specificity,and diagnosis requires special attention.Diagnosis of PLCH can significantly benefit from comprehensive multidisciplinary analysis.
文摘BACKGROUND Langerhans cell histiocytosis (LCH) is a rare disease of unknown aetiology. While it may affect any organ of the body, few cases of solitary lung involvement are published in the literature. Here, we report a rare case of pulmonary LCH (PLCH) in an adult. CASE SUMMARY A 52-year-old male presented to hospital in July 2018 with complaints of progressively worsening cough with sputum, breathlessness, easy fatigability, and loss of appetite since 2016, and a 32-year history of heavy cigarette smoking (average 30 cigarettes/d). Physical examination showed only weakened breathing sounds and wheezing during lung auscultation. Chest computed tomography (CT) showed irregular micronodules and multiple thin-walled small holes. Respiratory function tests showed a slight decrease. Ultrasonic cardiogram showed mild tricuspid regurgitation and no pulmonary hypertension. Fibreoptic bronchoscopy was performed with transbronchial biopsies from the basal segment of right lower lobe. LCH was confirmed by immunohistochemistry. The final diagnosis was PLCH without extra-pulmonary involvement. We suggested smoking cessation treatment. A 3-mo follow-up chest CT scan showed clear absorption of the nodule and thin-walled small holes. The symptoms of cough and phlegm had improved markedly and appetite had improved. There was no obvious dyspnoea. CONCLUSION Imaging manifestations of nodules, cavitating nodules, and thick-walled or thinwalled cysts prompted suspicion of PLCH and lung biopsy for diagnosis.
文摘BACKGROUND The common computed tomography findings of pulmonary Langerhans cell histiocytosis (PLCH) are multiple cysts and micronodules predominantly in middle to upper lung lobes.Non-cystic nodules and large nodules are atypical findings of PLCH.CASE SUMMARY The patient was a 48-year-old Japanese man with a smoking history (20 cigarettes/d,28 years) and no symptoms.Multiple nodules existed in all lung lobes,predominantly in the right lower lobe.Some nodules seemed to be distributed randomly,and others were adjacent to bronchus.Most nodules were solid;some small ones were cystic.The largest nodule was 22 mm in diameter.Although metastatic lung tumors were suspected,thoracoscopic lung biopsy led to the diagnosis of PLCH.At 6 months after he quit smoking,all nodules had almost disappeared.We investigated the characteristics of nodules at diagnosis in detail.Of 349 nodules in total,116 were in upper and 199 were in lower lobes.Ninety-six (27.5%) were cystic;the remaining 253 (72.5%) were non-cystic.The prevalence of cystic nodules was higher in upper lobes than in lower lobes (right upper 37.5% vs lower 18.2%,P = 0.0068;left upper 48.1% vs lower 24.4%,P = 0.0078).The average size (dia.) of cystic nodules was smaller than that of noncystic nodules (5.03 mm vs 7.40 mm,respectively,P < 0.0001).CONCLUSION Although multiple non-cystic nodules including large nodules (over 20 mm) are atypical,PLCH should be included in differential diagnoses.The presence of small cystic nodules predominantly in upper lobes and asymptomatic situation are also important for differential diagnoses to distinguish from metastatic cancers.
文摘Objective: Langerhans cell histiocytosis (LCH) has been well described only in children. We analyzed the characteristics, reactivation, and outcome of LCH in a cohort of 55 patients across all ages. Methods: We reviewed the records of all patients with LCH treated at a single institute between Jan. 1974 and May 1998. Results: The 55 patients were 2 to 67 years of age (median, 31 years) at the time of diagnosis, and 85.5% were male. Forty patients (72.7%) had single-system LCH; Fifteen (27.3%) had multisystem disease. The head and neck was the most frequent tumor site (63.6%). LCH was not found in organs at risk of involvement (liver, spleen, bone marrow, and lungs). The frequency of bony invasion (23.6% overall) differed significantly according to age ≤15 years (66.7%) vs. age >15 years (11.6%) (P=0.0005). At a median follow-up of 12 years, no patient died of LCH. The 5, 10-year survival estimates were 100%. The 5, 10-year disease-free survival estimates were 70.9% and 58.4%. The 5-year disease-free survival estimate was 58.3% for age ≤ 15 years vs. 74.4% for age >15 years (P=0.83) and 75% for single-system disease vs. 60% for multisystem disease (P=0.13). LCH was reactivated in 43.6% of patients, with a median of 14 months (range, 2-180 months). Three patients with recurrent disease experienced spontaneous remission. At the time of the most recent follow-up, 23.6% of survivors had active disease. Conclusion: LCH is not found exclusively in children and adolescents. The frequency of bone invasion is inversely related to age. Reactivation is very common regardless of the type of treatment, but the prognosis is generally good.
文摘BACKGROUND Langerhans cell histiocytosis(LCH)is a rare condition wherein Langerhans cells proliferate abnormally,adversely impacting organs including lymph nodes,bones,skin,lungs,and pituitary gland.The LCH disease course varies widely among patients from a self-limiting condition to one that progresses rapidly and culminates in death.It is uncommon for multisystem LCH to be observed in adults.Herein we describe a woman suffering from multi-system LCH involvement.CASE SUMMARY A 37-year old Chinese woman was admitted to the hospital in June 2019 suffering from dyspnea that had progressed over the course of 5 years.Her medical history included:central diabetes insipidus(DI)that had been treated via radiotherapy,desmopressin acetate,and bromocriptine;bilateral pneumothorax with two surgeries having been performed to remove bullae;and autoimmune hepatitis that had been unsuccessfully treated using a combination of methylprednisolone and mycophenolate mofetil.A chest computed tomography(CT)scan revealed the presence of multiple pulmonary cysts of varying sizes.We re-analyzed right pulmonary bullae samples that had been removed in 2014,performed a systematic 18 F-FDG PET/CT analysis,and convened a multidisciplinary medical team to diagnose and treat this patient.As a result,we were able to eventually diagnose this patient with LCH that was not associated with BRAF-V600 E mutations.CONCLUSION We hope to emphasize the importance of systemic evaluation and of cooperation between multidisciplinary physicians with the goal of improving awareness and detection of this orphan disease.
文摘Langerhans cell histiocytosis(LCH) is a rare condition mostly seen in children and adolescents. Eosinophilic granuloma(EG) is one of its three clinical entities and is considered as a benign osteolytic lesion. Many reports of patients with spine histiocytosis are well documented in the literature but it is not the case of atlantoaxial localization. We report here a new observation of atlantoaxial LCH in a 4-year-old boy revealed by persistent torticollis. He was successfully treated with systemic chemotherapy and surgery. Inter-body fusion packed by autologous iliac bone was performed with resolution of his symptoms. It is known that conservative treatment is usually sufficient and surgery should be reserved for major neurologic defects in spine EG. In atlantoaxial lesion, surgical treatment should be frequently considered.
文摘Langerhans cell histiocytosis(LCH)is a malignant disease of the histiocytes involving various organ systems.The spectrum of liver involvement in LCH ranges from mild transaminitis to end-stage liver disease.The hallmark of hepatic LCH is secondary sclerosing cholangitis,which manifests due to a progressive destruction of the biliary tree by malignant histiocytes.Chemotherapy remains the mainstay of treatment for active LCH.Early recognition,diagnosis and a systematic approach to the management of LCH can ameliorate the disease process.Nonetheless,the liver involvement in these patients may progress despite the LCH being in remission.Liver transplantation(LT)remains central in the management of such patients.Various facets of the management of LCH,especially those with liver involvement remain unclear.Furthermore,aspects of LT in LCH with regards to the indication,timing and post-LT management,including immunosuppression and adjuvant therapy,remain undefined.This review summarises the current evidence and discusses the practical aspects of the role of LT in the management of LCH.
文摘Introduction: Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells (LCs) and mature eosinophils, resulting in solitary or few, indolent and chronic, lesions of bone or other organs called eosinophilic granulomas. Calvarial LCH is quite rare and an underappreciated differential etiology of skull lesions. We present a most unusual case of a young child with hyperacutely symptomatic langerhans histiocytosis of the skull. Method: A 7-year-old male presented with a history of increasing (progressive) frontal headaches of 8 days duration, unaccompanied by associated nausea, vomiting, or diplopia. His only additional complaint was a hard bump on his forehead. MRI and CT done in the ER identified a right fronto-parietal lesion with associated skull erosion. Nuclear medicine and SPECT studies confirmed an erosive skull lesion without significant metabolic activity. A right frontal craniectomy and excision was performed. Results: A soft, rubbery well-circumcised mass coming from the diploic layer of the skull with involvement of bone was identified. The mass had eroded both the outer and inner table of the skull, and the involved area of the right frontal bone was resected. Intra-operative histo-pathologic analysis of the lesion revealed Langerhans cell histiocytosis without involvement of the dura. The patient experienced no neurological worsening as a result of the resection. He was discharged home in stable condition. Conclusion: LCH lesions of the skull are common findings, however, this focal hyperacute symptomatic presentation is most rare and should not deter us from anticipating an erosive bony tumor and planning timely surgical management.
文摘Langerhans cell histiocytosis is a rare disease involving clonal proliferation of langerhans cells seen in children and young adults. Clinical presentation is variable, ranging from a single location in the bone to severe multivisceral involvement. Moreover, spinal involvement causing myelopathy is even rare and unusual. We report a rare case of adult Langerhans cell histiocytosis in the dorsal spine causing a spinal cord compression associated with a pulmonary process treated by surgery, radiotherapy and systemic therapy with good evolution.
文摘To find out the most appropriate management,clinical features of 18 cases of adult multisystem langerhans cell histiocytosis(LCH) have been analyzed. The patients comprising of 9 males and 9 females were median age of 36 years,ranging from 18-53 years at diagnosis. Regarding the initial symptoms,7 patients(2 males and 5 females) showed central diabetes insipidus(CDI) and other endocrine symptoms with thickened pituitary stalk or a mass at the hypothalamic region. Additional 2 patients initiated the disease with CDI with no immediate diagnosis. In the remaining patients,the diseasebegun with single(n = 3) or multiple(n = 1) spinal bone lesion(s) in 4 patients(all males),with multiple bone lesions in 3 patients(1 male and 2 females),with single skull lesion in one female patient and with ambiguous symptoms including hypothyroidism in the remaining one male patient. We also recognized the correlation between pregnancy/childbirth and LCH in 4 patients. In terms of treatment,9 patients received systemic immuno-chemotherapy alone,of which the majority received vinblastine-based chemotherapy while 4 received 2-chlorodeoxyadenosine. Five had a combination of immuno-chemotherapy with surgical resection or radiotherapy,2 had immunotherapy alone,2 had surgical resection followed by observation alone to date. Three patients received hematopoietic stem cell transplantation after extensive chemotherapy. In terms of outcome,15 patients are alive(9 with active disease,6 without active disease),with a median of 66 mo(range 17-166 mo),two died of disease while the remaining 1 lost to follow-up. Based on these results,we think that early diagnosis and rapid introduction of appropriate treatment are essential,in order to overcome the problems relevant to adult LCH.
文摘Langerhans cell histiocytosis(LCH)is a rare neoplastic disease in dendritic cells.LCH is classified as either a single-system(SS)or multisystem(MS)disease.There is not a standard first-line treatment for LCH in adults.We analyzed the efficacy and safety of immunomodulatory drugs(IMiDs)by searching PubMed/MEDLINE for case reports previously published.The clinical response(nonactive disease or active disease that regressed)was 94%in SS and 53%in MS.IMiDs should only be considered for adults with cutaneous SS involvement;in MS,they should be used only for patients not eligible for more aggressive treatments.
文摘Langerhans cell histiocytosis has long been described as a rare systemic disorder involving the proliferation of Langerhans cells with formation of granuloma. The disease may be localised or diffuse. Typical forms of the disease involving the bone, the skin or the pituitary gland have been well described whereas others, such as thymic histiocytosis, are still poorly understood. Here, we report a case of isolated Langerhans cell histiocytosis of the thymus in an infant with non-specific symptoms. We provide a description of the disease and discuss patient management.
文摘Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-yearold woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increas-ing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.
文摘Hepatic Langerhans cell histiocytosis(LCH)is characterized by proliferation and accumulation of Langerhans cells in the liver,causing liver dysfunction or forming a mass lesion.The liver can be involved in isolation,or be affected along with other organs.A common clinical hepatic presentation is cholestasis with pruritis,fatigue and direct hyperbilirubinemia.In late stages,there may be hypoalbuminemia.Liver biopsy may be required for the diagnosis of hepatic LCH.Histologic finding may be diverse,including lobular Langerhans cell infiltrate with mixed inflammatory background,primary biliary cholangitis-like pattern,sclerosing cholangitis-like pattern,and even cirrhosis at later stages.Because of its non-specific injury patterns with broad differential diagnosis,establishing a diagnosis of hepatic LCH can be challenging.Hepatic LCH can easily be missed unless this diagnosis is considered at the time of biopsy interpretation.A definitive diagnosis relies on positive staining with CD1a and S100 antigen.Liver involvement is a high risk feature in LCH.The overall prognosis of hepatic LCH is poor.Treating at an early stage may improve the outcome.Systemic chemotherapy is the mainstay of treatment and liver transplantation may be offered.New molecular markers involved in pathogenesis of LCH are being explored with a potential for targeted therapy.However,further studies are needed to improve outcome.
文摘Purpose:Ocular trauma occurs disproportionately in children on an annual basis.However,because of this frequency,other diagnoses,.such as orbital neoplasms,.can easily be ignored.Methods:We report on a two-year-old boy who presented with a dark purple,irregularly shaped lesion on his lower left eyelid.The patient had suffered injuries twice to that area.Results:Axial computed tomography images demonstrated an ill-defined and inhomogeneous soft tissue mass in the lateral and posterior region of the left orbit,.with bony destruction and absorption of the adjacent orbital wall..Magnetic resonance imaging of the orbit showed a lesion involving the greater sphenoidal wing,the supraorbital wall,and the frontal area.Enlarged nuchal and inguinal lymph nodes were detected by sonographic examination.Histopathological examination of a surgical biopsy showed an accumulation of characteristic Langerhans cells.The diagnosis of Langerhans cell histiocytosis was confirmed by immunohistochemical examinations positive for CD1a and SP-100.Conclusion:Orbital Langerhans cell histiocytosis is rarely encountered in ophthalmic practice,.so the ophthalmologist needs to be familiar with its presentation and work-up,.and has to be aware of possible OLCH diagnosis when a child(or even an adult)presents with prolonged and persistent eyelid edema,.even with a history of ocular trauma.
文摘LANGERHANS cell histiocytosis(LCH)is a rare disease,mainly involving the bone,skin,lung,liver,spleen,and skin.1 The heterogenenous nature of LCH makes it difficult to diagnose.Not only do the involved organs vary from case to case,but also its natural history.Herein,we describe a rare case of conjuctival LCH in an Asian woman.
文摘Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells with inflammatory cells, characterized by the activation of histiocytic cells or their proliferation that affects structures of the human body, including the temporal bone which localization is rare in children. The objective of this study is to illustrate the clinical presentation, management and prognosis of this disease, and to compare our case with previously reported series. We report the case of a young 4-year-old patient with a history of otorrhea and recurrent otalgia, with left axillary non-blowing exophthalmia progressive since 5 months associated with indurated left temporal swelling and also an indurated swelling of posterior face of the left shoulder on pathological fracture. A cerebro-orbital MRI performed at home showed an extra-conical process with extension at the temporal fossa. The patient, then, underwent an open-air biopsy of the temporal mass and the pathological analysis returned to favor Langerhans cell histiocytosis. The diagnosis was made by immunohistochemical findings of the S-100 protein and/or the CD1 antigen. The child was treated by chemotherapy with a good outcome. Our results concord with literature. The prognosis is different and better in children with unifocal bone disease than those with a multifocal disease with a survival rate of 65% - 100%.