Hepatosplenic T cell lymphoma: a rare case report

Abstract

Hepatosplenic T cell lymphoma is a rare form of extra nodal and systemic neoplasm derived from cytotoxic T cells and represents less than 1 % of all non Hodgkins lymphoma. Here we present a case of hepatosplenic T cell lymphoma in a 30 year old male who presented with icterus, pancytopenia and hepatosplenomegaly, highlighting the diagnostic conundrum and pointers towards an accurate diagnosis. Histologically the sinusoids of liver and splenic red pulp were infiltration by CD 3 positive neoplastic lymphoid cells. The pattern of marrow involvement was very subtle and diagnosis was difficult to establish without the help of immunohistochemistry. This case highlights the importance of considering hepatosplenic T cell lymphoma as differential diagnosis in the clinical setting of pancytopenia with hepatosplenomegaly to allow timely diagnosis of these aggressive neoplasms.

Keywords: Hepatosplenomegaly; Lymphoma; Pancytopenia; T cell.

Fig. 1

a Neoplastic cells infiltrates the cord and sinuses of the splenic red pulp (Hematoxylin and eosin; ×100). b CD3 highlights the neoplastic cells (×100). c CD20 is negative in the neoplastic cells (×100)

Fig. 2

a and b Hepatic sinusoids are dilated and infiltrated by neoplastic cells (Hematoxylin and eosin; a ×100, b ×400). c Immunohistochemical reactivity of neoplastic cells in hepatic sinusoids by CD3 (×100). d Neoplastic cells are negative for CD20 (×100)

Fig. 3

Bone marrow biopsy. a The marrow is hypercellular (Hematoxylin and eosin; ×100); b Clusters of CD3 positive neoplastic cells are highlighted in the interstitium (black arrows) (×100)