Warde Medical Laboratory, Ann Arbor, Michigan.
Department of Hematology/Oncology, Marshfield Clinic Health System, Marshfield, Wisconsin.
Clin Med Res. 2023 Sep;21(3):155-158. doi: 10.3121/cmr.2023.1804.
Here we report development of hemophagocytic lymphohistiocytosis (HLH), along with unmasking of a TET2-mutated myeloid neoplasm, after initial doses of bendamustine and rituximab for longstanding chronic lymphocytic leukemia (CLL). After many years of CLL showing minimally progressive lymphocytosis, the patient's white blood cell count began to decline in parallel with neutrophil count, hemoglobin, and platelet count. Bone marrow biopsy showed partial CLL involvement; bendamustine+rituximab therapy was augmented with granulocyte colony-stimulating factor (g-CSF) and romiplostim to mitigate worsening pancytopenia, without response. Laboratory evaluation revealed a pattern supportive of the clinical impression of HLH, while bone marrow biopsy showed persistent CLL, new reticulin fibrosis, megakaryocytic proliferation, and 32% mutated TET2, but no compelling morphologic evidence of hemophagocytosis. The patient recovered with dexamethasone and g-CSF support.
在这里，我们报告了一例在接受苯达莫司汀和利妥昔单抗初始剂量治疗慢性淋巴细胞白血病（CLL）多年后，出现噬血细胞性淋巴组织细胞增生症（HLH），同时揭示了 TET2 突变的髓系肿瘤。在 CLL 表现为轻度进行性淋巴细胞增多多年后，患者的白细胞计数开始与中性粒细胞计数、血红蛋白和血小板计数平行下降。骨髓活检显示部分 CLL 受累；为缓解不断恶化的全血细胞减少症，在苯达莫司汀+利妥昔单抗治疗的基础上增加了粒细胞集落刺激因子（g-CSF）和罗米司亭，但无反应。实验室评估显示支持 HLH 的临床印象，而骨髓活检显示持续存在的 CLL、新的网状纤维纤维化、巨核细胞增殖和 32% 突变的 TET2，但没有吞噬细胞的形态学证据。患者在接受地塞米松和 g-CSF 支持后康复。
