Hepato-splenic ?? T-cell lymphoma: A rare entity mimicking the hemophagocytic syndrome

Abstract

Hepatosplenic T-cell lymphoma is a rare histologic type of the peripheral T-cell lym-phomas, clinically characterized by predominant involvement of liver and spleen, no or little adenopathy, and an often aggressive course; it affects mainly adolescents and young adults, with a male predominance. Postthymic T-cell malignancies are heterogeneous in their clinical and laboratory features. Among the postthymic T-cell lympho-mas, two distinct entities (cutaneous and hepatosplenic, respectively) are reported in the literature. The former shows predominant multiple involvement of the skin and subcuta-neous tissue; it occurs mostly in older patients and the phenotype is CD3−, CD4−, CD8−. Because of the small number of reports, the course of the disease was unknown. The latter shows a clinical picture characterized by hepatosplenomegaly, no or little adenop-athy, and sometimes systemic symptoms (fever, cytopenias likely due to hypersplenism); it presents a peculiar sinusoidal involvement of liver and spleen. The bone marrow his-tologic feature often reveals a little infiltration, especially sinusoidal and easily underestimated phenotype: CD2+, CD3+, CD7+, CD5−, CD4−, CD8−, CD44+. Few cases of this lymphoma associated by hemophagocytic syndrome are described (Sun, 1990; Kadin, 1981; Jaffe, 1983). We report a case of a young man with a rapid and fatal course in which the more important clinical feature was hemophagocytosis. The diagnosis of lymphoma was very difficult because of paucity of histologic involvement, and only the rearrangement of TCR chain gene by polymerase chain reaction on paraffin sections confirmed a clonal T-cell proliferation. Am. J. Hematol. 60:61-65, 1999.

Hepatosplenic T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients

“…46 The latter might also explain our observation in spleen and bone marrow of numerous histiocytes admixed within the neoplastic ␥␦ T cells, resulting in features of hemophagocytosis in occasional patients, as also reported by others. 17 For clinical purposes, the presence of circulating myeloid precursor cells, monocytosis, or both in patients with splenomegaly may be confusing. This was illustrated in 1 of our patients initially given a misdiagnosis of chronic myelomonocytic disorder.…”

Hepatosplenic T cell lymphoma. A review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990

“…For example, isolated thrombocytopenia which disappeared after splenectomy has been misinterpreted as idiopathic thrombocytopenic purpura, 15,16 circulating blast-like lymphoma cells were erroneously interpreted as acute lymphoblastic leukemia blasts, 18 or presentation with hepatosplenomegaly, jaundice, elevated liver parameters, thrombocytopenia, and fever have led to the assumption of a virus infection, which has usually not been confirmed. 5,21,26,29 Even more difficulties regarding the diagnosis of hepatosplenic ␥␦ T cell lymphoma may result from not considering this rare entity as a differential diagnosis. In most of the cases splenectomy or liver biopsy should be sufficient to establish diagnosis.…”

“…15 A known phenomenon in peripheral T cell lymphomas and also documented in areas invaded by malignant hepatosplenic ␥␦ T cells is erythrophagocytosis. [1][2][3]8,18,22,23,26 Besides peripheral cytopenia, this feature may be another indicator for active cytokine production by the tumor. 18 Tumor necrosis factor ␣ has been presumed to be responsible for histiocyte activation and subsequent erythrophagocytosis in EBV transformed T cell lymphomas.…”

Hemophagocytic lymphohistiocytosis associated with hepatosplenic T-cell lymphoma: case report

“…Among children this syndrome may be triggered by a viral infection or may be related to genetic abnormalities 5 , 6. However, in adults, the main triggering event is represented by lymphoproliferative disorders and sometimes is the first presentation 3 , 7 , 8. The diagnosis is based on clinical and laboratory data (Table 1).…”