Ibrahim Uroosa, Parylo Sara, Kedia Shiksha, Hussein Shafinaz, Atallah Jean Paul
Department of Hematology/Oncology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA.
Department of Medicine, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA.
Case Rep Hematol. 2017;2017:7506542. doi: 10.1155/2017/7506542. Epub 2017 Jul 18.
Large granular lymphocytic (LGL) leukemia is a rare form of low grade leukemia characterized by large cytotoxic T cells or natural killer cells on morphological examination. Immunosuppressive therapy is employed as first-line therapy. Treatment options in refractory cases include the anti-CD52 antibody alemtuzumab and purine analogues. We report a rare case that responded to the anti-CD20 monoclonal antibody rituximab. A 77-year-old female presented with complaints of fatigue, fever, and chills of 3 months' duration. A CBC showed that pancytopenia with an absolute neutrophil count (ANC) was 0. Peripheral blood flow cytometry detected increased number of T cell large granular lymphocytes and T cell receptor rearrangement study detected a clonal T cell population. Bone marrow biopsy showed peripheral T cell lymphoma, most consistent with T-large granulocytic leukemia. The patient was treated with prednisone and oral cyclophosphamide for four months with no response. Thereafter, she received four weekly infusions of rituximab with improvement in her blood counts. A response to rituximab in refractory cases such as ours has been reported and may guide us towards exploring other immune-based therapeutics in this rare disease.
大颗粒淋巴细胞（LGL）白血病是一种罕见的低度白血病，形态学检查可见大量细胞毒性T细胞或自然杀伤细胞。免疫抑制治疗被用作一线治疗。难治性病例的治疗选择包括抗CD52抗体阿仑单抗和嘌呤类似物。我们报告了一例对抗CD20单克隆抗体利妥昔单抗有反应的罕见病例。一名77岁女性主诉疲劳、发热和寒战3个月。全血细胞计数显示全血细胞减少，绝对中性粒细胞计数（ANC）为0。外周血流式细胞术检测到T细胞大颗粒淋巴细胞数量增加，T细胞受体重排研究检测到克隆性T细胞群体。骨髓活检显示外周T细胞淋巴瘤，最符合T大粒细胞白血病。患者接受泼尼松和口服环磷酰胺治疗4个月无反应。此后，她接受了4次每周一次的利妥昔单抗输注，血细胞计数有所改善。已有报道称像我们这样的难治性病例对利妥昔单抗有反应，这可能会引导我们探索这种罕见疾病的其他基于免疫的治疗方法。
