BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system.Composite lymphoma is defined as two o...BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system.Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphomas that occur in a single patient.Here,we report two cases of HLH secondary to composite lymphoma with mixed lineage features of T-and B-cell marker expression both in the bone marrow and lymph nodes in adult patients.CASE SUMMARY Two patients were diagnosed with HLH based on the occurrence of fever,pancytopenia,lymphadenopathy,splenomegaly,hemophagocytosis and hyperferritinemia.Immunohistochemical staining of the axillary lymph node and bone marrow in case 1 showed typical features of combined B-cell and T-cell lymphoma.In addition,a lymph node gene study revealed rearrangement of the T-cell receptor chain and the immunoglobulin gene.Morphology and immunohistochemistry studies of a lymph node biopsy in case 2 showed typical features of T cell lymphoma,but immunophenotyping by flow cytometry analysis of bone marrow aspirate showed B cell lymphoma involvement.The patients were treated with high-dose methylprednisolone combined with etoposide to control aggressive HLH progression.The patients also received immunochemotherapy with the R-CHOP(rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone)regimen immediately after diagnosis.Both patients presented with highly aggressive lymphoma,and died of severe infection or uncontrolled HLH.CONCLUSION We present two rare cases with overwhelming hemophagocytosis along with composite T-and B-cell lymphoma,which posed a diagnostic dilemma.HLH caused by composite lymphoma was characterized by poor clinical outcomes.展开更多
BACKGROUND We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma(MCL),an extremely rare and controversial condition that is not included in the World Health Organization-Europ...BACKGROUND We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma(MCL),an extremely rare and controversial condition that is not included in the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.CASE SUMMARY The patient presented diffuse cutaneous erythematous plaques and nodules throughout the body.Skin lesions were biopsied and histopathological examination showed diffuse monomorphic lymphocyte infiltration in the dermal and subcutaneous layers,sparing the epidermis.Immunohistochemical staining revealed CD20,cyclin-D1,CD5,and SOX-11 expression.Fluorescence in situ hybridization showed CCND1/IGH gene rearrangement.Correct diagnosis of primary cutaneous MCL requires ensuring that no other parts are involved;these cases require close follow-up to monitor their possible progression to systemic disease and for treating relapsed cutaneous disease.In this case,positron emission tomography scanning and clinical staging revealed no systemic involvement,and follow-up examination at 20 mo after diagnosis showed no evidence of systemic disease.The prognosis of primary cutaneous MCL is relatively good.Our patient received six cycles of chemotherapy,and the cutaneous manifestations presented almost complete remission.CONCLUSION Primary cutaneous MCL is rare,and its prognosis is relatively favorable.However,correct diagnosis is a prerequisite for proper treatment.展开更多
文摘BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare and life-threatening disease caused by inherited pathogenic mutations and acquired dysregulations of the immune system.Composite lymphoma is defined as two or more morphologically and immunophenotypically distinct lymphomas that occur in a single patient.Here,we report two cases of HLH secondary to composite lymphoma with mixed lineage features of T-and B-cell marker expression both in the bone marrow and lymph nodes in adult patients.CASE SUMMARY Two patients were diagnosed with HLH based on the occurrence of fever,pancytopenia,lymphadenopathy,splenomegaly,hemophagocytosis and hyperferritinemia.Immunohistochemical staining of the axillary lymph node and bone marrow in case 1 showed typical features of combined B-cell and T-cell lymphoma.In addition,a lymph node gene study revealed rearrangement of the T-cell receptor chain and the immunoglobulin gene.Morphology and immunohistochemistry studies of a lymph node biopsy in case 2 showed typical features of T cell lymphoma,but immunophenotyping by flow cytometry analysis of bone marrow aspirate showed B cell lymphoma involvement.The patients were treated with high-dose methylprednisolone combined with etoposide to control aggressive HLH progression.The patients also received immunochemotherapy with the R-CHOP(rituximab,cyclophosphamide,doxorubicin,vincristine,and prednisone)regimen immediately after diagnosis.Both patients presented with highly aggressive lymphoma,and died of severe infection or uncontrolled HLH.CONCLUSION We present two rare cases with overwhelming hemophagocytosis along with composite T-and B-cell lymphoma,which posed a diagnostic dilemma.HLH caused by composite lymphoma was characterized by poor clinical outcomes.
文摘BACKGROUND We describe the case of a 74-year-old man diagnosed with primary cutaneous mantle cell lymphoma(MCL),an extremely rare and controversial condition that is not included in the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas.CASE SUMMARY The patient presented diffuse cutaneous erythematous plaques and nodules throughout the body.Skin lesions were biopsied and histopathological examination showed diffuse monomorphic lymphocyte infiltration in the dermal and subcutaneous layers,sparing the epidermis.Immunohistochemical staining revealed CD20,cyclin-D1,CD5,and SOX-11 expression.Fluorescence in situ hybridization showed CCND1/IGH gene rearrangement.Correct diagnosis of primary cutaneous MCL requires ensuring that no other parts are involved;these cases require close follow-up to monitor their possible progression to systemic disease and for treating relapsed cutaneous disease.In this case,positron emission tomography scanning and clinical staging revealed no systemic involvement,and follow-up examination at 20 mo after diagnosis showed no evidence of systemic disease.The prognosis of primary cutaneous MCL is relatively good.Our patient received six cycles of chemotherapy,and the cutaneous manifestations presented almost complete remission.CONCLUSION Primary cutaneous MCL is rare,and its prognosis is relatively favorable.However,correct diagnosis is a prerequisite for proper treatment.
