ABSTRACT:
Introduction: Hepatosplenic T-cell lymphoma (HSTCL) is a highly aggressive form of primary extra nodal Non-Hodgkin Lymphoma (NHL) marked by the presence of hepatosplenomegaly without lymphadenopathy. The incidence of HSTCL was 3% in the United States and 2.3% in Europe of the total cases of T-cell lymphomas. Whereas in East and Southeast Asia, it is less common at only 0.2%. This is the first case reported within five years at Dr. Soetomo Hospital in Surabaya, Indonesia. Case: A male aged 35 presented with the main complaint of an enlarged abdomen in the past 2 months. Physical examination showed hepatosplenomegaly. The CBC result demonstrated pancytopenia which consisted of hemoglobin reading of 9.7g/dL, white blood cells of 3,140/µL, and platelets of 99 x 103/µL. In the bone marrow aspiration, the smears revealed an increase in lymphocytic cell activity with an abnormal morphology of lymphocytes (prominent cytoplasmic projection) with a proportion of 45%. Based on bone marrow aspiration conducted with lymphocytic infiltration with hairy cell morphology. The bone marrow aspiration immunophenotyping examination showed an abnormal population in the lymphocytic gate (positive CD3, CD7, and negative CD5) conducted with T cell phenotype. Discussion: The HSTCL is most frequent in young adults, with a male-to-female ratio of approximately 9:1. The median age at early diagnosis is 35 years old. Based on the clinical, hematology profile, bone marrow aspiration and immunophenotyping, the pattern was consistent with Hepatosplenic T-cell lymphoma (HSTCL). The HSTCL is an aggressive case. Most patients experience an initial positive response to chemotherapy, but relapses are commonly observed, with survival of less than 2 years. Applying high-dose therapy following hematopoietic stem cell transplantation at an early stage may enhance the chances survival. This patient was diagnosed when he was 35 years old and is currently undergoing chemotherapy. Conclusion: The case of Hepatosplenic T-cell lymphoma is very rare and challenging. The pattern of marrow involvement was difficult to establish diagnosis without immunohistochemistry. It is important to consider diagnosing Hepatosplenic T-cell lymphoma in case of pancytopenia with hepatosplenomegaly. An accurate and timely diagnosis is essential for effective management of these aggressive neoplasms.