Center of Hematology, The First Affiliated Hospital of Nanchang University, Nanchang, China.
Jiangxi Clinical Research Center for Hematologic Disease, Nanchang, China.
Ann Hematol. 2024 Oct;103(10):4319-4323. doi: 10.1007/s00277-024-05759-z. Epub 2024 Aug 6.
Chronic neutrophil leukemia (CNL) is a rare and life-threatening disease. Cases of CNL combined with lymphoma are rare. Here, we report a case of CNL with T-acute lymphoblastic leukemia/lymphoma (T-ALL/LBL) in a 28-year-old male. After a regimen of ruxolitinib, VICLP (Vincristine, Idarubicin, Cyclophosphamide, Prednisone, Peg-asparaginase) regimen, high-dose cytarabine, and methotrexate regimens, the patient's bone marrow condition partially resolved. However, when the disease relapsed four months later, despite attempts with selinexor, venetoclax, and CAG(aclarubicin hydrochloride, Algocytidine, Granulocyte Stimulating Factor) chemotherapy, the leukocytes and peripheral blood primitive cells reduced, but the bone marrow did not achieve remission. This pathogenesis may be related to microenvironmental immune escape under prolonged inflammatory stimulation and gene disruption affecting protein function due to colony-stimulating factor 3 receptor gene (CSF3R) mutations. For this type of disease, early intervention may delay disease progression.
慢性中性粒细胞白血病(CNL)是一种罕见且危及生命的疾病。CNL 合并淋巴瘤的病例较为罕见。本文报道了 1 例 28 岁男性 CNL 合并 T 急性淋巴细胞白血病/淋巴瘤(T-ALL/LBL)病例。患者接受了 ruxolitinib、VICLP(长春新碱、伊达比星、环磷酰胺、泼尼松、培门冬酰胺酶)方案、高剂量阿糖胞苷和甲氨蝶呤方案治疗后,骨髓情况部分缓解。然而,疾病在 4 个月后复发,尽管尝试了 selinexor、venetoclax 和 CAG(盐酸阿克拉霉素、阿糖胞苷、粒细胞集落刺激因子)化疗,但白细胞和外周血原始细胞减少,骨髓未达到缓解。这种发病机制可能与长期炎症刺激下微环境免疫逃逸以及集落刺激因子 3 受体基因(CSF3R)突变导致的蛋白功能障碍有关。对于此类疾病,早期干预可能会延缓疾病进展。