Hui Monalisa, Uppin Shantveer G, Paul Tara Roshni, Beerappa N
Department of Pathology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, 500082 Andhra Pradesh India.
Department of Surgical Gastroenterology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, 500082 Andhra Pradesh India.
Indian J Hematol Blood Transfus. 2014 Sep;30(Suppl 1):17-20. doi: 10.1007/s12288-012-0219-y. Epub 2012 Dec 18.
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.
肝脾T细胞淋巴瘤是一种罕见的结外和全身性肿瘤,起源于细胞毒性T细胞,占所有非霍奇金淋巴瘤的比例不到1%。本文报告一例30岁男性肝脾T细胞淋巴瘤病例,该患者表现为黄疸、全血细胞减少和肝脾肿大,强调了诊断难题以及准确诊断的要点。组织学上,肝血窦和脾红髓被CD3阳性的肿瘤性淋巴细胞浸润。骨髓受累模式非常细微,若无免疫组化帮助,诊断很难确立。该病例强调了在全血细胞减少伴肝脾肿大的临床背景下,将肝脾T细胞淋巴瘤作为鉴别诊断的重要性,以便及时诊断这些侵袭性肿瘤。