Gupta Nishit, Mittal Aditi, Duggal Rajan, Dadu Tina, Agarwal Amit, Handoo Anil
Department of Haematology, BLK Superspeciality Hospital, Delhi, India, 110005.
Department of Histopathology, BLK Superspeciality Hospital, Delhi, India, 110005.
Int J Hematol Oncol Stem Cell Res. 2021 Oct 1;15(4):249-254. doi: 10.18502/ijhoscr.v15i4.7480.
Hodgkin lymphoma variant of Richter's transformation (HL-RT) is a rare event, occurring in < 1% chronic lymphocytic leukemia (CLL) cases, of which, in < 10% cases, HL is the first finding leading to a diagnosis of CLL that co-exists simultaneously. Here we report a 60 years old male patient who presented with an outside diagnosis of lymphocyte-rich classical HL. On evaluation, he had only B-symptoms in the form of low-grade fever and weight loss. Peripheral smear revealed mild leukocytosis with an absolute lymphocytosis and a few smudge cells. Bone marrow (BM) aspirate and biopsy exhibited diffuse infiltration by a small cell, low grade, Non-Hodgkin's lymphoma with no immunohistochemical evidence of HL. Flow cytometry performed on BM was consistent with classical immunoprofile of CLL. Meanwhile the lymph node received for review revealed diffuse effacement of nodal architecture by small mature lymphocytes with immunoprofile of CLL expressing CD20, CD5, and CD23. Interspersed between these cells, were a few eosinophils along with classical Reed Sternberg cells, expressing CD30, MUM-1, CD15, and dim PAX-5, with a surrounding rosette of T-Cells highlighted by CD3 and PD-1 and negative for CD45, CD20, and EBV immunohistochemistry. Fluorodeoxyglucose positron emission tomography (FDG-PET) scan revealed hepatosplenomegaly with multiple supra/infra diaphragmatic lymph nodes. So, a final diagnosis of HL-RT in CLL was considered. The patient is currently doing well after the first cycle of ABVD chemotherapy. HL-RT occurring in CLL is a rare event with heterogeneous clinical presentation, morphology, clonal origin, disease course, prognostic features, and survival.
里氏转化的霍奇金淋巴瘤变体（HL-RT）是一种罕见事件，发生于不到1%的慢性淋巴细胞白血病（CLL）病例中，其中不到10%的病例中，HL是导致CLL诊断同时并存的首发发现。在此，我们报告一名60岁男性患者，其最初被诊断为富于淋巴细胞的经典型HL。经评估，他仅表现为低热和体重减轻形式的B症状。外周血涂片显示轻度白细胞增多伴绝对淋巴细胞增多及少量涂抹细胞。骨髓穿刺和活检显示有小细胞、低级别非霍奇金淋巴瘤的弥漫性浸润，无HL的免疫组化证据。对骨髓进行的流式细胞术检测结果与CLL的经典免疫表型一致。同时，送检复查的淋巴结显示小成熟淋巴细胞弥漫性取代淋巴结结构，其免疫表型为表达CD20、CD5和CD23的CLL。在这些细胞之间散在分布着一些嗜酸性粒细胞以及经典的里德·斯腾伯格细胞，这些细胞表达CD30、MUM-1、CD15和弱阳性PAX-5，周围有CD3和PD-1标记的T细胞玫瑰花结，且CD45、CD20和EBV免疫组化呈阴性。氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）显示肝脾肿大伴多个膈上/膈下淋巴结。因此，考虑最终诊断为CLL中的HL-RT。该患者在接受ABVD化疗的第一个周期后目前情况良好。CLL中发生的HL-RT是一种罕见事件，具有异质性的临床表现、形态学、克隆起源、病程、预后特征和生存率。
