Juvenile Myelomonocytic Leukemia: Report of Seven Cases and Review of Literature
Juvenile Myelomonocytic Leukemia: Report of Seven Cases and Review of Literature
复制标题
DOI：
10.2350/08-04-0456.1
复制
发表时间：
2009-03-01
影响因子：
1.9
通讯作者：
Kahwash, Samir B.
中科院分区：
文献类型：
--
作者：
Urs, Latha;Qualman, Stephen J.;Kahwash, Samir B.
青少年髓单细胞白血病（JMML）是一种罕见的、侵袭性的、儿童期的克隆性造血疾病，以骨髓发育不良（血小板减少、贫血）和骨髓增生（白细胞增多、单核细胞增多）为特征。多数病例有骨髓细胞增多、脾肿大和髓外受累。1997年，就术语达成了国际共识，并提出了诊断指南/标准。世界卫生组织最近的分类描述了JMML的当前诊断标准。虽然JMML的诊断已经得到了促进，但它可能具有挑战性，特别是在早期阶段或当它首次表现为髓外肿瘤时。我们报告了在10年间（从1996年1月1日到2005年12月31日）确诊的7例病例。两个病例有有趣的相关发现，可能导致诊断延误或误诊。另外两例有髓外受累，症状可指受累器官。本文对临床和病理表现进行了总结，并对相关文献进行了回顾。
Juvenile myelomonocytic leukemia (JMML) is a rare, aggressive, clonal hematopoietic disorder of childhood with features of both myelodysplasia (thrombocytopenia, anemia) and myeloproliferation (leukocytosis, monocytosis). In most cases there is marrow hypercellularity, splenomegaly, and extramedullary involvement. In 1997 an international consensus on terminology was reached and guidelines/criteria for diagnosis were proposed. A recent World Health Organization classification described the current diagnostic criteria of JMML. Although the diagnosis of JMML has been facilitated, it can be challenging, especially in the early stages or when it 1st presents as an extramedullary tumor. We report a series of 7 cases diagnosed over a period of 10 years (from January 1, 1996, to December 31, 2005). Two cases had interesting associated findings that would potentially lead to delay in diagnosis or misdiagnosis. Two other cases had extramedullary involvement with symptoms referable to the organs of involvement at presentation. Clinical and pathologic findings are summarized with a review of relevant literature.