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Clinical and Hematological Characteristics of Hepatosplenic T γ/δ Lymphoma with Isochromosome for Long Arm of Chromosome 7

1996; Taylor & Francis; Volume: 22; Issue: 5-6 Linguagem: Inglês

10.3109/10428199609054788

1042-8194

Ming Yao, Hwei‐Fang Tien, Ming-Tseh Lin, Ih‐Jen Su, C H Wang, Yao‐Chang Chen, Ming‐Ching Shen, Chiu‐Hwa Wang,

Acute Myeloid Leukemia Research

AbstractHepatosplenic T γ/δ lymphoma is a rare entity of peripheral T cell lymphoma. Three of 386 patients with non-Hodgkin's lymphoma in our institute were found to have this subtype of lymphoma. All had chromosomal abnormalities of isochromosome 7q and trisomy 8. The clinical and hematological features of these three patients are reported. All were males with ages ranging from 23 to 29 years. Initial presentation comprised purpura and variable degree of hepatosplenomegaly. None had superficial lymphadenopathy. Hematologically, they showed pictures resembling immune related thrombocytopenia and/or hemolytic anemia. Examination of the bone marrows revealed hypercellularity with increased number of megakaryocytes and erythroid cells and various degrees of abnormal lymphoid cell infiltration. The histopathologic section of the spleen from one patient who underwent splenectomy revealed abnormal cell infiltration in the sinusoids of the red pulp. Lymphoma cells showed T γ/δ lymphoid immunophenotype (CD3+ CD2+ CD4- CD8-, TCR δ -1+, and β F1-). The platelet counts were elevated transiently after initial treatment with corticosteroids, but the condition soon deteriorated. All died of refractory lymphoma five to nine months after diagnosis. Review of the literature, showed that only four other cases have been reported until now and although no cytogenetic data were available for these patients, they had very similar clinical pictures as those in this series. It is suggested that hepatosplenic T y/8 lymphoma represents a rare, but distinct, clinicopathological and cytogenetic entity.Key Words: Peripheral T-cell lymphomaT-cell receptor γ/δ