Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease...Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.展开更多
Lymphomatoid granulomatosis, currently called as extranodal angiocentric and angiodestructive immunoproliferative disorder, is a rare entity of unclear etiology. It involves most frequently lungs, central nervous syst...Lymphomatoid granulomatosis, currently called as extranodal angiocentric and angiodestructive immunoproliferative disorder, is a rare entity of unclear etiology. It involves most frequently lungs, central nervous system and skin. The clinical course is variable, but mortality is high. Today, it represents a diagnostic challenge because it can emulate autoimmunity, infection, and malignancy processes. Optimal therapy scheme is still unknown. We report the case of a 20 year-old man presenting with fever, weight loss, sweating, multiple bilateral lung nodules on the chest X-ray and cutaneous involvement.展开更多
BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulm...BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulmonary nodules.Its diagnosis depends on lung biopsy findings.Most patients are immunodeficient,and it commonly presents in children undergoing chemotherapy for leukemia.We report the case of a child with PLG caused by a mutation in the macrophageexpressed gene 1(MPEG1),suggesting possible PLG occurrence in children undergoing treatment for pulmonary nodular lesions.CASE SUMMARY This study reports a case of PLG without apparent immunodeficiency,suggesting the possibility of this disease occurrence during the treatment of pulmonary nodular lesions in children.Initially,the cause was assumed to be an atypical pathogen.Following conventional anti-infective treatment,chest computed tomography findings revealed that there were still multiple nodules in the lungs.Additionally,the patient was found to be infected with the Epstein-Barr virus.Histopathological examination of the resected lung revealed lymphoproliferative lesions with necrosis.Small lymphocytes,plasma cells,and histiocytes were observed in the background,although Reed-Sternberg cells were absent.Immunohistochemical staining[CD20(+),CD30(+),and CD3(+)]and EBV-encoded small RNA1/2 in situ hybridization of small lymphocytes revealed approximately 200 cells/high-power field.Whole exon sequencing of the patient revealed a mutation in the MPEG1.The patient was eventually diagnosed with PLG and transferred to the Department of Pediatric Oncology for bone marrow transplantation.CONCLUSION As PLG is rare and fatal,it should be suspected in clinical settings when treatment of initial diagnosis is ineffective.展开更多
Since X-linked chronic granulomatosis disease(X-CGD)exhibits no specific clinical symptoms at an early stage,early diagnosis is difficult and depends predominantly on neonatal screening.Therefore,the aim of this study...Since X-linked chronic granulomatosis disease(X-CGD)exhibits no specific clinical symptoms at an early stage,early diagnosis is difficult and depends predominantly on neonatal screening.Therefore,the aim of this study was to explore routine biomarkers for X-CGD in children and provide clues for early diagnosis.The cases of 10 children with X-CGD diagnosed at Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology from 2013 to 2016 and 122 Chinese children with X-CGD reported in the literature were summarized.Serum biomarkers and clinical symptoms at acute infection were organized.A total of 132 children with X-CGD were enrolled in this study.For 55.8%of the patients,the diagnosis was delayed more than one year after the onset of the first symptoms because no typical clinical symptoms manifested.Children with X-CGD at an acute infection stage showed three recurrent signs in temis of serum biomarkers:(1)the total number of white blood cells(especially N%)was in creased significantly,accompanied by anemia in some cases;(2)C-reactive protein(CRP)levels were increased significantly;and(3)most of the patients exhibited very high serum IgG levels(>12 g/L).Diagnosis of X-CGD at an early age is difficult because of its nonspecific clinical features.Our study suggested children with X-CGD suffering acute infection show increases in three typical serum biomarkers,which can provide clues for early diagnosis.展开更多
Rationale:American tegumentary leishmaniasis comprises cutaneous and mucocutaneous manifestations caused by parasitic infections by various Leishmania species.This report details the clinical interventions for a patie...Rationale:American tegumentary leishmaniasis comprises cutaneous and mucocutaneous manifestations caused by parasitic infections by various Leishmania species.This report details the clinical interventions for a patient with American tegumentary leishmaniasis in Mendoza,Argentina,a non-endemic region.Patient concerns:A 43-year-old male was admitted to a tertiary care hospital in Mendoza,Argentina Republic with a history of progressive nasal discharge,septal perforation,facial pain,and pruritus.Despite treatment for presumed nasal myiasis and vasculitis with granulomatosis,symptoms persisted.Diagnosis:American tegumentary leishmaniasis.Interventions:Intravenous liposomal amphotericin B.Outcomes:Follow-up at 30 days showed no recurrence of symptoms with a remarkable clinical improvement of the nasal lesion.Lessons:This case sheds light on the necessity of accurate identification for timely intervention and the need to recognize the diverse manifestations of American tegumentary leishmaniasis to avoid misdiagnosis.展开更多
Lymphomatoid granulomatosis （LG） is a rare EpsteinBarr virus （EBV）-associated systemic granulomatous disease that is characterized by an angiocentric and occasionally angiodestructive polymorphic cellular infiltra...Lymphomatoid granulomatosis （LG） is a rare EpsteinBarr virus （EBV）-associated systemic granulomatous disease that is characterized by an angiocentric and occasionally angiodestructive polymorphic cellular infiltrate. It most frequently affects the lungs, kidneys,and brain.1,2 Usually the lesions are multi-systemic and multifocal. Central nervous system （CNS） involvement occurs in approximately 30% of affected patients, and patients may present with nonspecific neurological symptoms.3 Although CNS lesions usually appear as a secondary metastasis of pulmonary lesions, in rare conditions,展开更多
目的 总结淋巴瘤样肉芽肿病(lym phom atoid granulom atosis,LG)与韦格内肉芽肿( egener granulo Wm atosis,W G)的临床特点,从临床上加以鉴别。 方法 回顾性总结我院与北京协和医院 1989 年以来收治的 6 例 LG和20 例 W G 患者的临床...目的 总结淋巴瘤样肉芽肿病(lym phom atoid granulom atosis,LG)与韦格内肉芽肿( egener granulo Wm atosis,W G)的临床特点,从临床上加以鉴别。 方法 回顾性总结我院与北京协和医院 1989 年以来收治的 6 例 LG和20 例 W G 患者的临床资料,并进行比较。 结果 发热、咳嗽及呼吸困难在 LG 中较常见,流涕、鼻衄及关节痛以W G 为多,肺受累以 LG 为多,耳鼻喉、眼及肾病变在 W G 中明显增多,皮肤受损两者相似。W G 患者常有 c-ANCA 阳性及尿沉渣异常。 LG X 线胸片为双侧多发结节,边界不清,多无肺门、纵隔淋巴结肿大; G 则为双侧多发边界清晰W的结节, 易形成空洞, 肺门和纵隔淋巴结可肿大。LG 病理改变为血管中心性淋巴增生性病变, 浸润的细胞主要是小淋巴细胞和不同数量大的不典型淋巴细胞; 而在 W G 中可见坏死性血管炎及大量中性白细胞、浆细胞及少量嗜酸细胞浸润形成的肉芽肿, 部分有多核巨细胞。W G 经治疗后, 多数患者病情缓解; 则疗效不佳。 结论 LG 和 W G 在临 LG床表现、实验室检查、 影像及病理诸方面的差异有利于两者的鉴别诊断。展开更多
文摘Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.
文摘Lymphomatoid granulomatosis, currently called as extranodal angiocentric and angiodestructive immunoproliferative disorder, is a rare entity of unclear etiology. It involves most frequently lungs, central nervous system and skin. The clinical course is variable, but mortality is high. Today, it represents a diagnostic challenge because it can emulate autoimmunity, infection, and malignancy processes. Optimal therapy scheme is still unknown. We report the case of a 20 year-old man presenting with fever, weight loss, sweating, multiple bilateral lung nodules on the chest X-ray and cutaneous involvement.
基金Supported by Science and Technology department of Sichuan Province,No.2020YFS0105West China Second University Hospital of Sichuan University,No.KL036.
文摘BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulmonary nodules.Its diagnosis depends on lung biopsy findings.Most patients are immunodeficient,and it commonly presents in children undergoing chemotherapy for leukemia.We report the case of a child with PLG caused by a mutation in the macrophageexpressed gene 1(MPEG1),suggesting possible PLG occurrence in children undergoing treatment for pulmonary nodular lesions.CASE SUMMARY This study reports a case of PLG without apparent immunodeficiency,suggesting the possibility of this disease occurrence during the treatment of pulmonary nodular lesions in children.Initially,the cause was assumed to be an atypical pathogen.Following conventional anti-infective treatment,chest computed tomography findings revealed that there were still multiple nodules in the lungs.Additionally,the patient was found to be infected with the Epstein-Barr virus.Histopathological examination of the resected lung revealed lymphoproliferative lesions with necrosis.Small lymphocytes,plasma cells,and histiocytes were observed in the background,although Reed-Sternberg cells were absent.Immunohistochemical staining[CD20(+),CD30(+),and CD3(+)]and EBV-encoded small RNA1/2 in situ hybridization of small lymphocytes revealed approximately 200 cells/high-power field.Whole exon sequencing of the patient revealed a mutation in the MPEG1.The patient was eventually diagnosed with PLG and transferred to the Department of Pediatric Oncology for bone marrow transplantation.CONCLUSION As PLG is rare and fatal,it should be suspected in clinical settings when treatment of initial diagnosis is ineffective.
基金Natural Science Foundation of Hubei,China(No.2017CFB645)JinLei Research Fund for Pediatric Endocrine Young Physician,China(No.PEGRF201304006)the Ministry of Science and Technology(No.2017ZX09304022).
文摘Since X-linked chronic granulomatosis disease(X-CGD)exhibits no specific clinical symptoms at an early stage,early diagnosis is difficult and depends predominantly on neonatal screening.Therefore,the aim of this study was to explore routine biomarkers for X-CGD in children and provide clues for early diagnosis.The cases of 10 children with X-CGD diagnosed at Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology from 2013 to 2016 and 122 Chinese children with X-CGD reported in the literature were summarized.Serum biomarkers and clinical symptoms at acute infection were organized.A total of 132 children with X-CGD were enrolled in this study.For 55.8%of the patients,the diagnosis was delayed more than one year after the onset of the first symptoms because no typical clinical symptoms manifested.Children with X-CGD at an acute infection stage showed three recurrent signs in temis of serum biomarkers:(1)the total number of white blood cells(especially N%)was in creased significantly,accompanied by anemia in some cases;(2)C-reactive protein(CRP)levels were increased significantly;and(3)most of the patients exhibited very high serum IgG levels(>12 g/L).Diagnosis of X-CGD at an early age is difficult because of its nonspecific clinical features.Our study suggested children with X-CGD suffering acute infection show increases in three typical serum biomarkers,which can provide clues for early diagnosis.
基金supported by the Consejo Nacional de Investigaciones Cientıfícas y Técnicas(CONICET)(PIP 2021-2023/11220200102779CO).
文摘Rationale:American tegumentary leishmaniasis comprises cutaneous and mucocutaneous manifestations caused by parasitic infections by various Leishmania species.This report details the clinical interventions for a patient with American tegumentary leishmaniasis in Mendoza,Argentina,a non-endemic region.Patient concerns:A 43-year-old male was admitted to a tertiary care hospital in Mendoza,Argentina Republic with a history of progressive nasal discharge,septal perforation,facial pain,and pruritus.Despite treatment for presumed nasal myiasis and vasculitis with granulomatosis,symptoms persisted.Diagnosis:American tegumentary leishmaniasis.Interventions:Intravenous liposomal amphotericin B.Outcomes:Follow-up at 30 days showed no recurrence of symptoms with a remarkable clinical improvement of the nasal lesion.Lessons:This case sheds light on the necessity of accurate identification for timely intervention and the need to recognize the diverse manifestations of American tegumentary leishmaniasis to avoid misdiagnosis.
文摘Lymphomatoid granulomatosis （LG） is a rare EpsteinBarr virus （EBV）-associated systemic granulomatous disease that is characterized by an angiocentric and occasionally angiodestructive polymorphic cellular infiltrate. It most frequently affects the lungs, kidneys,and brain.1,2 Usually the lesions are multi-systemic and multifocal. Central nervous system （CNS） involvement occurs in approximately 30% of affected patients, and patients may present with nonspecific neurological symptoms.3 Although CNS lesions usually appear as a secondary metastasis of pulmonary lesions, in rare conditions,
文摘目的 总结淋巴瘤样肉芽肿病(lym phom atoid granulom atosis,LG)与韦格内肉芽肿( egener granulo Wm atosis,W G)的临床特点,从临床上加以鉴别。 方法 回顾性总结我院与北京协和医院 1989 年以来收治的 6 例 LG和20 例 W G 患者的临床资料,并进行比较。 结果 发热、咳嗽及呼吸困难在 LG 中较常见,流涕、鼻衄及关节痛以W G 为多,肺受累以 LG 为多,耳鼻喉、眼及肾病变在 W G 中明显增多,皮肤受损两者相似。W G 患者常有 c-ANCA 阳性及尿沉渣异常。 LG X 线胸片为双侧多发结节,边界不清,多无肺门、纵隔淋巴结肿大; G 则为双侧多发边界清晰W的结节, 易形成空洞, 肺门和纵隔淋巴结可肿大。LG 病理改变为血管中心性淋巴增生性病变, 浸润的细胞主要是小淋巴细胞和不同数量大的不典型淋巴细胞; 而在 W G 中可见坏死性血管炎及大量中性白细胞、浆细胞及少量嗜酸细胞浸润形成的肉芽肿, 部分有多核巨细胞。W G 经治疗后, 多数患者病情缓解; 则疗效不佳。 结论 LG 和 W G 在临 LG床表现、实验室检查、 影像及病理诸方面的差异有利于两者的鉴别诊断。
