Acute Leukemia with M3 Morphology Without Cytogenetic Abnormalities Related to Acute Promyelocytic Leukemia: Description of a Refractory Pediatric Case

Clinical Leukemia Pub Date : 2009-08-01 DOI:10.3816/CLK.2009.n.012

Morgani Rodrigues , José Mauro Kutner , Andreza Alice Feitosa Ribeiro , Luci Tabacow Hidal , Adalberto Stape , Nydia Bacal , Nelson Hamerschlak

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Abstract

Acute promyelocytic leukemia (APL) is a distinct subtype of acute myeloid leukemia (AML). APL is characterized by specific genetic abnormality t(15;17), which results in fusion between the promyelocytic leukemia (PML) gene and the retinoic acid receptor-α (RARα). We describe the case of a 4-year-old boy who was admitted to hospital with severe infection of the oropharynx due to a peritonsillar abscess, along with hepatomegaly and splenomegaly. The initial laboratory tests showed a condition compatible with AML. The cytologic morphology, cytochemistry, and immunophenotyping were compatible with the AML M3 variant but with normal karyotype, fluorescence in situ hybridization and polymerase chain reaction (PCR) negative for t(15;17), and PCR negative for t(11;17). There was resistance to the initial chemotherapy, but the patient experienced an excellent result from nonrelative umbilical cord transplantation. The case represents an atypical situation of AML with promyelocytic characteristics and normal cytogenetics showing a poor prognosis that responded only to bone marrow transplantation.

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急性早幼粒细胞白血病伴M3形态无细胞遗传学异常的急性白血病:1例难治性儿童病例描述

急性早幼粒细胞白血病(APL)是急性髓性白血病(AML)的一个独特亚型。APL的特点是特异性遗传异常t(15;17)，导致早幼粒细胞白血病(PML)基因与视黄酸受体-α (RARα)融合。我们描述的情况下，一个4岁的男孩谁是入院严重感染口咽由于腹膜周围脓肿，以及肝和脾肿大。最初的实验室测试显示与急性髓性白血病相符。细胞学形态学、细胞化学和免疫表型与AML M3变异相一致，但核型正常，荧光原位杂交和聚合酶链反应(PCR) t阴性(15;17)，PCR阴性t(11;17)。对最初的化疗有抵抗，但患者经历了非亲属脐带移植的良好结果。该病例是非典型的AML，具有早幼粒细胞特征和正常的细胞遗传学，预后差，仅对骨髓移植有反应。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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Clinical Leukemia

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