Department of Hematology, AO Sant'Andrea, La Sapienza University, Rome, Italy.
Eur J Haematol. 2013 Aug;91(2):183-6. doi: 10.1111/ejh.12141. Epub 2013 Jun 28.
Large granular lymphocyte leukemias (LGLLs) represent a spectrum of biologically distinct lymphoproliferative diseases originating from either mature T cells (CD3+) or natural killer (NK) cells (CD3-). Both T-cell and NK-cell LGL leukemia can manifest as indolent or aggressive neoplasia. These rare lymphoproliferative disorders are often associated with autoimmune diseases and impaired hematopoiesis. Symptomatic patients are treated with immunosuppressive drugs. The co-association of T-LGLL with clonal B-cell disorders is reported in more than 10% of patients.
We describe the case of a 57-yr-old white male patient with no history of autoimmune disorders, with refractory T-LGLL and myeloma who was treated with bortezomib and subsequently with lenalidomide. After 30 months of on-going lenalidomide therapy, the patient is in partial remission from myeloma and in continuous complete hematological remission from T-LGLL.
As far as we know, this is the first report of a patient with refractory T-LGLL treated with bortezomib and lenalidomide. As refractory T-LGLL is a challenging condition, we think that lenalidomide and bortezomib deserve further investigation.
大颗粒淋巴细胞白血病（LGLL）代表了一组生物学上不同的淋巴增殖性疾病，起源于成熟 T 细胞（CD3+）或自然杀伤（NK）细胞（CD3-）。T 细胞和 NK 细胞 LGL 白血病均可表现为惰性或侵袭性肿瘤。这些罕见的淋巴增殖性疾病常与自身免疫性疾病和造血功能受损有关。有症状的患者采用免疫抑制药物进行治疗。T-LGLL 与克隆性 B 细胞疾病的共同发生在超过 10%的患者中有所报道。
我们描述了一例 57 岁白人男性患者的病例，该患者无自身免疫性疾病史，患有难治性 T-LGLL 和骨髓瘤，曾接受硼替佐米治疗，随后接受来那度胺治疗。在持续接受来那度胺治疗 30 个月后，患者骨髓瘤部分缓解，T-LGLL 持续完全血液学缓解。
据我们所知，这是首例难治性 T-LGLL 患者接受硼替佐米和来那度胺治疗的报告。鉴于难治性 T-LGLL 是一种具有挑战性的疾病，我们认为来那度胺和硼替佐米值得进一步研究。
