Diagnostic and management challenges in primary cutaneous anaplastic large cell lymphoma with necrosis,inflammation,and surgical intervention:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)poses significant diagnostic difficulties due to its similarity in the appearance of skin lesions with chronic inflammatory disorders and other dermatological conditions.This study aims to investigate these challenges by conducting a comprehensive analysis of a case presenting with PC-ALCL,emphasizing the necessity of accurate differentiation for appropriate management.CASE SUMMARY An 89-year-old female patient with diabetes and hypertension presented with arm and abdominal ulcerated mass lesions.Diagnostic procedures included skin biopsies,histopathological assessments,and immunohistochemistry,complemented by advanced imaging techniques to confirm the diagnosis.The patient’s lesions were determined as PC-ALCL,characterized by necrosis,chronic inflammation,and a distinct immunophenotypic profile,including CD30,CD3,CD4,and EBER,CD56,MUM-1,Ki 67-positive in>80%of tumor cells,CD10,but negative for anaplastic lymphoma kinase,CD5,CD20,PAX-5,Bcl-2,Bcl-6,CD8,and CD15.Recurrence was not reported at the 6-month follow-up.CONCLUSION Accurate PC-ALCL differentiation from similar conditions is crucial for effective management and requires a multidisciplinary approach.

Primary cutaneous anaplastic large cell lymphoma with overexpressed Ki-67 transitioning into systemic anaplastic large cell lymphoma:A case report

BACKGROUND Primary cutaneous anaplastic large cell lymphoma(PC-ALCL)differs from systemic anaplastic large cell lymphoma(sALCL)in cell biological behavior,clinical features,treatment,and outcome.PC-ALCL has been reported to rarely transition into sALCL,but the underlying mechanism is not clear.Here we report such a case with certain characteristics that shed light on this.CASE SUMMARY Herein,we report a 43-year-old male with symptoms of a skin nodule and histologically confirmed PC-ALCL with high expression of Ki-67.After three months of observation,two skin nodules re-appeared with muscle layer involvement and was histologically confirmed as sALCL.Seventeen months after receiving six cycles of CHOP regimen,the patient had pain in the chest and back,cough,shortness of breath,and night sweats.This was confirmed as relapse of sALCL by immunohistochemistry and several organs,such as the lung were involved as shown by positron emission tomography/computed tomography.After four cycles of DICE plus chidamide regimens followed by auto-hematopoietic stem cell transplantation(ASCT),complete remission(CR)duration was achieved for twelve months while the patient was on maintenance with chidamide(20 mg)pills.CONCLUSION This case had significantly high expression of Ki-67 when diagnosed as PC-ALCL initially and then transitioned into sALCL,which is rare.Auto-ASCT combined with demethylation drugs effectively maintained CR and prolonged progression free survival.

Primary pancreatic anaplastic large cell lymphoma, ALK negative: A case report

We present the fourth case of a primary pancreatic anaplastic large cell lymphoma (ALCL), ALK-. An 80-year-old man was admitted to our clinic for further investigation of a fever of unknown origin. He noted anorexia, weight loss and fatigue. His laboratory tests showed anemia and a great elevation of ESR, LDH, and β2 microglobulin. In CT and MRI scan, a soft tissue mass in the pancreas was observed. A repeated endoscopy after his admission revealed an ulcerated mass-like deformity of the duodenal bulb. Explorative laparotomy confirmed a diffuse spread of an unresectable malignant pancreatic mass extending to the adjacent organs. Duodenal and surgical biopsies identified an ALCL of T-cell lineage, ALK-. The patient died in the Intensive Care Unit due to hemodynamic instability.Our case is the first one indicating that primary pancreatic lymphoma should be suspected in a patient with pancreatic mass and elevated serum LDH and β2 microglobulin.

Primary cutaneous anaplastic large cell lymphoma with subsequent leg involvement

Primary cutaneous anaplastic large cell lymphoma(CALCL)is regarded as an indolent type of cutaneous T-cell lymphoma.However,a few recent publications revealed that C-ALCL patients with initial leg involvement had significantly worse survival than those without initial leg involvement.Herein,we report a case of C-ALCL with subsequent leg involvement,which led to death after chemoradiation therapy.A 75 years old Japanese man presented with multiple erythematous nodules in his left arm and the side of his left chest.Histopathological and immunohistochemical studies led to the diagnosis of primary C-ALCL.At the initial diagnosis,no leg lesion was found.One year after the initial diagnosis,C-ALCL appeared in his right lower thigh and left hip.Radiation therapy,low-dose etoposide and CHOP therapy were performed;however,the patient died of malignant lymphoma 4 years after the initial diagnosis.We speculated that the occurrence of subsequent leg involvement may also be indicative of a worse prognosis,as in the case with initial leg involvement in C-ALCL.Therefore,we propose that C-ALCL patients with initial or subsequentleg involvement should be classified as a distinct clinicopathological variant of C-ALCL("leg-type"involvement)and that they may require intense therapy.

Acute leukemic phase of anaplastic lymphoma kinase-anaplastic large cell lymphoma: A case report and review of the literature

BACKGROUND Anaplastic large cell lymphoma(ALCL)is a rare and heterogeneous malignant tumor,which is classi
ed as anaplastic lymphoma kinase(ALK)positive ALCL and ALK-ALCL.Many patients are diagnosed with ALCL at the stage of bone marrow involvement.However,ALCL patients with clinical manifestations consistent with acute leukemia are relatively rare.CASE SUMMARY In this report,the patient did not receive appropriate diagnosis and treatment despite a two-year history of lymph node enlargement.Hereafter,she was admitted for B symptoms and was diagnosed as ALK-ALCL by lymph node biopsy.Then,the disease progressed to leukemia without any treatment after 2 mo.The proportion of lymphoma cells in bone marrow was as high as 96%,and the proportion of peripheral blood was 84%.She also had clinical manifestations similar to acute leukemia.After completion of chemotherapy,she developed granulocytopenia and fever and died from septicemia.CONCLUSION ALCL with leukemic presentation is a late manifestation of lymphoma with low chemotherapy tolerance and poor prognosis.

Primary bone anaplastic lymphoma kinase positive anaplastic largecell lymphoma: A case report and review of the literature

BACKGROUND Primary bone lymphoma(PBL)is an uncommon extranodal disease that represents approximately 1%-3%of lymphomas.Anaplastic lymphoma kinase(ALK)positive anaplastic large-cell lymphoma(ALCL)is an extremely rare type of PBL.The aim of this report is describe the symptoms,diagnosis,and treatment of primary bone ALK-positive ALCL.CASE SUMMARY A 66-year-old man presented to our hospital with neck and shoulder pain and intermittent fever that lasted for 1 mo.After extensive evaluation,positron emission tomography-computed tomography(CT)examination showed multiple osteolytic bone lesions without other sites lesions.CT-guided biopsy of the T10 vertebral body was performed,and the pathology results showed that neoplastic cells were positive for ALK-1,CD30,and CD3.A diagnosis of primary bone ALK positive ALCL was ultimately made.The patient was in partial response after four cycle soft cyclophosphamide,doxorubicin,vincristine,and prednisone chemotherapy,and we planned to repeat the biopsy and radiological examination after completion of the fifth cycle of therapy.CONCLUSION Primary bone ALK positive ALCL is a rare disease and physicians should keep in mind that ALCL can present with isolated osseous involvement without nodal involvement,and lymphoma should be considered in the differential diagnosis of primary bone lesions.

Single Abscess as the First Manifestation of Breast ALK-Positive Anaplastic Large Cell Lymphoma in Pregnancy Without Breast Implants:A Case Report

Primary lymphoma involving the breast accounts for only 0.04-0.5%of malignant breast tumors,constituting 1-2%of all non-Hodgkin lymphomas(NHL).Here,we report a case of breast ALK-positive ALCL arising in a 21-year-old woman in pregnancy with single abscess as the first clinical manifestation.

CD30-Positive Anaplastic Large Cell Lymphoma with Abundant Neutrophils (Report of a Case)

ObjectiveTo report a rare case of ALCL (Anaplastic Large Cell Lymphoma) with abundant neutrophils and necrosis and to analyse the histomorphologic subtypes and immunohistologic characteristics of ALCL. MethodsA biopsy of a painless tumor on a 26 year old male′s neck was taken for morphologic and immunohistochemical analyse. ResultsJudging from the morphologic feature of ALCL,the prognosis of the patient is good. ConclusionIt is necessary to do immunohistochemical staining in diagnosing an ALCL since p80 NPM/ALK or ALK is the best antibody to distinguish ALCL from other lymphomas.

ALK-positive anaplastic large cell lymphoma presenting multiple lymphomatous polyposis: A case report and literature review

BACKGROUND Anaplastic large cell lymphoma(ALCL)is a type of T-cell lymphoma that can be divided into two categories:anaplastic lymphoma kinase-positive(ALK+)and ALK-negative.Gastrointestinal ALK+ALCL is rare.Multiple lymphomatous polyposis(MLP)is thought to be a representative form of gastrointestinal lesion in mantle cell lymphoma,and T-cell lymphomas seldom show this feature.Here,we report the first known case of ALK+ALCL with gastroduodenal involvement to present with MLP.CASE SUMMARY The patient was a 43-year-old man who was complained of a mass in the left inguinal area and was performed open biopsy.ALK+ALCL was diagnosed pathologically.Computed tomography scan demonstrated multiple lymph node lesions in the abdomen-pelvis/inguinal region,and scattered nodular lesions in both lung fields.He did not complain of gastrointestinal symptoms.While,esophagogastroduodenoscopy identified MLP lesions from the antrum of the stomach to the descending portion of the duodenum and mild thickened folds on the corpus of the stomach,and biopsy showed invasion of ALK+ALCL.We treated this patient with six cycles of CHOEP(Cyclophosphamide,Doxorubicin,Vincristine,Etoposide,and Prednisone)chemotherapy.At the conclusion of treatment,there was complete remission.Numerous white scars were found on the stomach,endoscopically consistent with a remission image of lymphoma.The endoscopic features of this case were thought to be similar to those of MCL.CONCLUSION The macroscopic/endoscopic features of gastrointestinal ALK+ALCL may be more similar to those of B-cell lymphomas rather than T-cell lymphomas.

Anaplastic lymphoma kinase-negative anaplastic large cell lymphoma masquerading as Behcet’s disease: A case report and review of literature

BACKGROUND Anaplastic large cell lymphoma(ALCL)is a CD30-positive T cell lymphoma,a rare type of non-Hodgkin lymphoma.The current World Health Organization classification system divides ALCLs into anaplastic lymphoma kinase(ALK)-positive and ALK-negative groups.ALCL rarely presents in the gastrointestinal tract.CASE SUMMARY A 54-year-old male was admitted to the department of gastroenterology for abdominal pain.He presented with lower abdominal pain,diarrhea and recurrent oral and penile ulcers.He was misdiagnosed with Behcet's disease and treated with prednisone.But after one month,he was hospitalized in another hospital for reexamination.This time,the lesion on the penis was biopsied for histological examination.The final pathological diagnosis was ALCL,ALKnegative.The patient was treated with cyclophosphamide,doxorubicin,vincristine,prednisolone chemotherapy.However,he died within one month.CONCLUSION Gastrointestinal ALCL needs to be considered in the differential diagnosis to avoid delaying treatment.Repeated biopsy is the most important for early diagnosis and treatment.

Kawasaki disease, <i>Mycoplasma pneumoniae</i>infection and anaplastic large cell lymphoma: An uncommon association

Kawasaki disease (KD) is an acute febrile systemic vasculitis occurring predominantly in young children less than 5 years of age. Although imperfectly known, the aetiopathogenesis of KD would be secondary to immunological abnormalities that could constitute a favourable terrain for neoplasms. We report on a case in a 2-year-old girl who presented clinical manifestations compatible with Kawasaki disease complicated by coronary aneurysm. Aetiopathological investigations revealed M. pneumoniae infection as specific IgM were present in the serum (Elisa). The patient was initially treated by intravenous immunoglobulins (IVIG) and aspirin to anti-inflammatory dose. Following a few days of desquamation, resolution of the symptomatology occurred. Four weeks later she developed disseminated tumorous syndrome. Lymph node biopsy revealed massive infiltration by large cells lymphomatous proliferation. Histologic and immunophenotypic findings were characteristic of ALK-1+ anaplastic large cell lymphoma. Four weeks later, the patient died from a severe nosocomial infection complicated by septic shock. Our observation is the first cases describing the association between anaplastic large cell lymphoma, KD and M. pneumoniae. Immunologic disorder due to KD and M. pneumoniae infection may play probably a central role for malignancy.

ALK-positive anaplastic large cell lymphoma of the thoracic spine occurring in pregnancy: A case report

BACKGROUND Anaplastic large cell lymphoma (ALCL) is a type of non-Hodgkin’s lymphoma (NHL). ALCL is rare as a bone lesion and in pregnancy. CASE SUMMARY We present the first case of anaplastic lymphoma kinase (ALK)+ ALCL of the thoracic spine during pregnancy. A 25-year-old pregnant woman was presented to us at 24 wk’ gestation with severe back pain and weakness in the left lower limb. Imaging examination showed lesions at T10 and T11. She underwent posterior pedicle screw fixation and vertebroplasty. Pathological examination showed ALK+ ALCL. The patient chose to have therapeutic abortion after surgery and received chemotherapy in the hematology department. She now remains disease free with no neurological deficit after 30 mo’ follow-up. CONCLUSION ALK+ ALCL with the thoracic spine involvement is uncommon, especially in pregnancy. Many symptoms can be misunderstood during pregnancy;therefore, when a pregnant patient has persistent back pain or lower limb neurological symptoms, imaging examinations should be performed.

Long Lasting Efficiency of Monthly Vinblastine in a Case of Relapsed Anaplastic Large Cell Lymphoma

Nearly 40% of children with Anaplastic Large cell lymphomas will relapse after a first-line strategy with short-pulse chemotherapy and reach a second remission in 30% to 60% with second line therapies including maintenance treatment with vinblastine or allogeneic hematopoietic stem-cell transplantation. The authors report a heavily pretreated case in second relapse who was maintained in third remission for 8 years with monthly vinblastine. He relapsed 16 weeks after discontinuation. This case demonstrates that monthly treatment with vinblastine may be sufficient to maintain a minimal disease. Oral compounds are now available and should be discussed in such situations.

Brentuximab Vedotin Monotherapy and Combined with Low Dose Donor Lymphocyte Infusion to Control Minimal Residual Disease and Sustain Clinical Remission in a Child with Relapsed Anaplastic Large Cell Lymphoma

Minimal residual disease (MRD) appears to have a strong negative predictive value for disease recurrence in children with anaplastic large cell lymphoma (ALCL). Brentuximab vedotin (BV) can be a therapeutic option for MRD-positive patients to achieve molecular remission and to decrease risk of subsequent relapse. We here report a 4-year-old child with ALCL progression during relapse treatment who received BV as a bridging therapy before haploidentical hematopoietic stem-cell transplantation, and as a maintenance therapy post-transplant alone or combined with simultaneous low dose donor-lymphocyte infusions. MRD monitoring showed a complete molecular response and reflected both BV efficiency and graft-versus-lymphoma effect.

Anaplastic Large Cell Lymphoma, ALK-Negative Presenting in the Rectum: A Case Report and Review of the Literature

ALK-negative anaplastic large cell lymphoma (ALCL, ALK-) is a CD30+ T-cell neoplasm composed of large lymphoid cells with abundant cytoplasm and pleomorphic nuclei that lacks expression of ALK protein. We describe a case of ALCL, ALK-with primary involvement of the rectum in a 37 year old man, where the original diagnosis was established based on a colonoscopic biopsy. T-cell lymphomas are rare in the colorectal area and besides ALCL, their differential diagnosis includes enteropathic T-cell lymphoma, peripheral T-cell lymphoma, not otherwise specified, NK/T cell lymphoma, or NK-cell enteropathy. In addition, syncytial variant of classical Hodgkin lymphoma or a pleomorphic CD30-positive diffuse large B-cell lymphoma should also be ruled out. We discuss pitfalls of the differential diagnosis and review the literature of anaplastic large cell lymphoma in the gastrointestinal tract. Correct diagnosis of ALCL in the colon is important to avoid a colorectal surgery for an assumed adenocarcinoma, and to open the possibility for lymphoma-directed chemotherapy.

Diagnosis and treatment of pediatric anaplastic lymphoma kinasepositive large B-cell lymphoma:A case report

BACKGROUND Anaplastic lymphoma kinase-positive(ALK+)large B-cell lymphoma(LBCL)is a rare type of lymphoma with high invasiveness and rapid progression.It occurs in all age groups,but is extremely rare in children.The lesions mainly involve the lymph nodes and may present with extra-nodal involvement.Response to conventional chemotherapies and local radiotherapy is poor,with a 5-year overall survival of less than 40%.Recently,the use of ALK inhibitors for the treatment of this disease has been reported.CASE SUMMARY We present a case of a 12-year-old boy diagnosed with ALK+LBCL.The patient had a 2-mo medical history of a calvarial mass,extensive systemic involvement,and positive bone marrow clathrin heavy chain(CLTC)-ALK fusion gene.Complete remission 1(CR1)was achieved using the modified LMB89 Group C regimen followed by autologous stem cell transplantation.The patient relapsed 3 mo later.He then achieved CR2 with three short courses of chemotherapy(COP,reduceddose ICE,low-dose Ara-c+VP16)and continuous alectinib targeted therapy.Afterward,allogeneic hematopoietic stem cell transplantation(allo-HSCT)was performed.At 16 mo after the allo-HSCT,the patient was still in CR2.CONCLUSION The modified LMB89 Group C regimen and ALK inhibitors are effective.Allo-HSCT should be performed after remission.

Intravascular proliferating anaplastic lymphoma kinasepositive anaplastic large-cell lymphoma

An 82-year-old Japanese man visited our emergency unit complaining of dyspnea. Laboratory data showed 15% atypical lymphocytes in peripheral blood which expressed the T-cell phenotype. Chest/abdominal computed tomography depicted hepatosplenomegaly and swelling of systemic lymph nodes. The patient died of advanced respiratory failure 5 d after the first occurrence of his dyspnea. At autopsy, the pathological features revealed a diffuse infiltration of large atypical lymphocytes to systemic organs including the spleen and lung. In immunohistochemical staining, these cells expressed CD30, TIA-1, anaplastic lymphoma kinase(ALK), CD5 and CD3. An advanced surface molecule analysis revealed a lack of CD54(intercellular cell adhesion molecule-1) and CD56(neural cell adhesion molecule). We observed the proliferation and infiltration of these lymphoma cells specifically at the intravascular lesions similar to intravascular lymphoma(IVL). T-cell IVL is not established as an independent clinical entity in the World Health Organization classification, and our patient's ALK-positive T-IVL in lung appears to be the first reported case.

Primary tracheobronchial anaplastic large cell lymphoma in an 8-year-old child presenting with asthma-like symptoms

Primary tracheobronchial anaplastic large cell lymphoma （ALCL） is very rare.In spite of its rarity,tracheobronchial tumor can cause life-threatening airway obstruction because of its growing site and biological characteristics.Here we represented the clinical and pathological features of a patient with ALCL in the respiratory tract.

Efficacy of peripheral blood stem cell transplantation versus conventional chemotherapy on anaplastic large-cell lymphoma:a retrospective study of 64 patients from a single center

Anaplastic large-cell lymphoma(ALCL) is characterized by frequently presenting adverse factors at diagnosis.Many groups believed aggressive treatment strategies such as autologous stem cell transplantation brought survival benefit for ALCL patients.However,few compared these approaches with conventional chemotherapy to validate their superiority.Here,we report a study comparing the efficacy of peripheral blood stem cell transplantation(PBSCT) and conventional chemotherapy on ALCL.A total of 64 patients with primary systemic ALCL were studied retrospectively.The median follow-up period was 51 months(range,1-167 months).For 48 patients undergoing conventional chemotherapy only,the 4-year event-free survival(EFS) and overall survival(OS) rates were 70.7% and 88.3%,respectively.Altogether,16 patients underwent PBSCT,including 11 at first remission(CR1/PR1),3 at second remission,and 2 with disease progression during first-line chemotherapy.The 4-year EFS and OS rates for patients underwent PBSCT at first remission were 81.8% and 90.9%,respectively.Compared with conventional chemotherapy,PBSCT did not show superiority either in EFS(P = 0.240) or in OS(P = 0.580) when applied at first remission.Univariate analysis showed that patients with B symptoms(P = 0.001),stage III/IV disease(P = 0.008),bulky disease(P = 0.075),negative anaplastic lymphoma kinase(ALK) expression(P = 0.059),and age ≤ 60 years(P = 0.054) had lower EFS.Furthermore,PBSCT significantly improved EFS in patients with B symptoms(100% vs.50.8%,P = 0.027) or bulky disease(100% vs.52.8%,P = 0.045) when applied as an up-front strategy.Based on these results,we conclude that,for patients with specific adverse factors such as B symptoms and bulky disease,PBSCT was superior to conventional chemotherapy in terms of EFS.

Research progresses in the pathogenesis of anaplastic largecell lymphoma

Anaplastic large cell lymphoma(ALCL) is a distinct subset of T-cell non-Hodgkin's lymphoma.As a consequence of its low incidence,general pathogenic consideration of ALCL is lacking.In this review,we summarize the pathogenesis,epidemiology,clinical manifestations,and treatment of ALCL,so as to better understand key stages of the development of this disease and provide valuable information for future treatment.