Hepatosplenic γδ T-cell lymphoma after allogeneic bone marrow transplantation

Dear Editor, Hepatosplenic γδ T-cell lymphoma (HSγδTCL) is a rare extranodal T-cell non-Hodgkin lymphoma (NHL) that arises from a small subset of γδ T-cell receptor-expressing lymphocytes. It is characterized by an aggressive clinical course and poor response to conventional chemotherapy. HSγδTCL occurs in both immunocompetent and immunocompromised patients. To the best of our knowledge, it has never been observed after allogeneic bone marrow transplantation (alloSCT). Here, we report the case of a 47-year-old man who developed a HSγδTCL 10 years after an allo-SCT. The patient presented with fever, fatigue, and abdominal discomfort, 10 years after a familial allo-SCT performed for primary myelofibrosis. Physical examination showed marked hepatosplenomegaly in the absence of lymphadenopathy. Laboratory findings included mild thrombocytopenia (127, 000/mm) and leucopenia (2420/mm), but normal LDH and liver enzymes. Virological tests were negative for EBV, CMV, HIV, HBV, and HCV. The first bone marrow aspirate was normal. Liver biopsy disclosed sinusoidal infiltration by medium-sized lymphocytes with irregular nucleus that expressed a γδ T-cell phenotype (CD2+, CD3+, CD5−, CD4−, CD8−, CD7+, CD56+/−, γδTCR) and some cytotoxic granule-associated proteins (TIA1+, granzyme B−) (Fig. 1a–d). Molecular analysis showed a monoclonal TCRγ gene rearrangement and in situ hybridization (EBER) did not reveal the presence of EBV. FISH analysis demonstrated an isochromosome 7. The malignant T-cells were of host origin. The second marrow aspirate showed infiltration by phenotypically identical T-cells. The patient received three courses of cladribine with poor response, followed by myeloablative chemotherapy with busulfan-cyclophosphamide and a second allo-SCT from another sibling donor. He remained in complete remission for 61 months and relapsed. He failed to respond to belinostat, a histone deacetylase inhibitor, and died of refractory disease. Post-transplant lymphoproliferative disorders (PTLDs) are a common complication of immunosuppression after transplantation. The majority is of B-cell origin, with a strong association with EBV infection. The incidence is directly related to the intensity of immunosuppression. Reduction of immunosuppressive therapy may induce complete remission of the disease. In contrast, T-cell lineage PTLDs are very rare; they exhibit either a T-helper (CD4+) or a T-cytotoxic (CD8+) phenotype, and most of them express the αβ TCR. EBV is only detected in a minority of cases [1]. HSγδTCL accounts for less than 1 % of all NHL [2] and usually occurs in immunocompetent young adult males. Up to 20 % arise in the context of chronic immunosuppression, most commonly after solid organ transplantation, or neoplasms such as Hodgkin lymphoma and acute myeloblastic leukemia, but autoimmune disorders such as inflammatory bowel diseases, idiopathic thrombocytopenic purpura, and prolonged antigenic M.

Keywords:

• Bone marrow
• Lymphoma
• Lymphoproliferative disorders
• Hematology
• Immunology
• Transplantation
• T-cell lymphoma
• Immunosuppression
• Medicine
• Myelofibrosis
• Internal medicine
• gene rearrangement

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