Azma R Z, Zarina A L, Hamidah A, Jamal R, Sharifah N A, Ainoon O, Hamidah N H
Department of Pathology, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Malays J Pathol. 2009 Dec;31(2):121-8.
Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic - myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory findings, and discuss the difficulty in the classification and treatment of the disease.
青少年粒单核细胞白血病(JMML),以前称为青少年慢性髓细胞白血病(JCML),是一种罕见的骨髓增生异常-骨髓增殖性疾病,通常在儿童早期出现。由于其临床和骨髓表现相似,这种疾病很难与其他骨髓增殖综合征如慢性髓细胞白血病(CML)区分开来。然而,由于其独特的生物学特征,如伴有发育异常的绝对单核细胞增多、不存在费城染色体或BCR-ABL融合蛋白、高球蛋白血症和胎儿血红蛋白水平升高,这种疾病不符合纳入成人患者中CML或慢性粒单核细胞白血病(CMML)组的标准。我们描述了一系列三名患有JMML的患者,他们具有几乎相似的临床和实验室检查结果,并讨论了该疾病在分类和治疗方面的困难。