Ganessan K, Goel Ruchika, Kumar Kamlesh, Bakhshi Sameer
Department of Medical Oncology, Dr. B. R. A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
Pediatr Hematol Oncol. 2007 Apr-May;24(3):195-8. doi: 10.1080/08880010701198787.
A 14-year-old child presented with generalized lymphadenopathy and massive hepatosplenomegaly. Peripheral smear and bone marrow examination were suggestive of Ph' positive chronic myeloid leukemia (CML) in chronic phase. However, lymph node biopsy showed extramedullary blast crisis with evidence of myeloid and T cell markers in blasts. Reverse transcriptase-polymerase chain reaction from lymph node aspirate revealed transcript for bcr-abl p210. Thus, we present here a unique case of childhood CML with extramedullary biphenotypic blast crisis (myeloid/T cell type) at initial presentation with bone marrow remaining in chronic phase. This case provides further evidence to the highly heterogeneous presentation of CML.
一名14岁儿童出现全身淋巴结肿大及巨脾。外周血涂片和骨髓检查提示慢性期Ph'阳性慢性髓性白血病（CML）。然而，淋巴结活检显示髓外原始细胞危象，原始细胞中有髓系和T细胞标志物的证据。淋巴结穿刺液的逆转录聚合酶链反应显示有bcr-abl p210转录本。因此，我们在此报告一例独特的儿童CML病例，初诊时即出现髓外双表型原始细胞危象（髓系/T细胞型），而骨髓仍处于慢性期。该病例为CML高度异质性的表现提供了进一步证据。
