■慢性中性粒细胞白血病是一种罕见的疾病,预后不良。由于缺乏遗传工具,其诊断具有挑战性。它很少与自身免疫性溶血性贫血相关。
■慢性中性粒细胞性白血病是一种罕见疾病,预后不良,特征是在缺乏单核细胞增多症或嗜碱性粒细胞的情况下持续的成熟嗜中性白细胞增多症,很少或没有循环的未成熟粒细胞,肝脾肿大,和骨髓的粒细胞增生。此外,未检测到其他骨髓增殖性肿瘤的分子标志物.2016年WHO分类将CSF3R突变的存在作为该疾病的关键诊断标准。尽管诊断时可能存在贫血,溶血性肿瘤很少使骨髓增殖性肿瘤复杂化。治疗主要基于细胞还原剂,但骨髓移植仍然是唯一的治疗选择。我们报告了一例与自身免疫性溶血性贫血相关的慢性中性粒细胞白血病患者。我们描述了流行病学,临床,预后,以及该疾病的治疗特征,以及突尼斯的诊断和管理困难。
UNASSIGNED: Chronic neutrophilic leukemia is a rare disease with a poor prognosis. Its diagnosis is challenging in the lack of genetic tools. It can infrequently be associated with autoimmune hemolytic anemia.
UNASSIGNED: Chronic neutrophilic leukemia is a rare disease with poor prognosis, characterized by a sustained mature neutrophilic leukocytosis in the absence of monocytosis or basophilia with few or no circulating immature granulocytes, hepatosplenomegaly, and granulocytic hyperplasia of the bone marrow. In addition, no molecular markers for other myeloproliferative neoplasms are detected. The 2016 WHO classification included the presence of the CSF3R mutation as a key diagnostic criterion for this disease. Although anemia may be present at diagnosis, hemolytic one rarely complicates myeloproliferative neoplasms. Treatment is largely based on cytoreductive agents, but bone marrow allograft remains the only curative option. We report the
case of a patient with chronic neutrophilic leukemia associated with autoimmune hemolytic anemia. We describe the epidemiological, clinical, prognostic, and therapeutic features of this disease in addition to the difficulties of its diagnosis and management in Tunisia.