Mariańska B, Apel D, Seferyńska I, Maj S
Kliniki Hematologicznej Instytutu Hematologii i Transfuzjologii w Warszawie.
Acta Haematol Pol. 1996;27(1):85-8.
We present a case of a 17-year old patient with extreme hepatosplenomegaly, hyperthrombocytosis, hyperleucocytosis and the presence of myelo- and megakaryoblasts in the peripheral blood film. Numerous complications that occurred in the course of the disease made cytostatic treatment difficult. Since Ph chromosome and hybrid gene bcr/abl were absent, the diagnosis of unclassified chronic myeloproliferative syndrome in the phase of blast crisis was established. Immunophenotyping confirmed a mixed myelo- megakaryoblastic character of the crisis. In the differential diagnosis other myeloproliferative syndromes were taken into account including i(17q) syndrome. The patient died after a 13-month observation due to neoplasm progression and sepsis.
我们报告一例17岁患者,有极度肝脾肿大、血小板增多、白细胞增多,且外周血涂片中有成髓细胞和原巨核细胞。疾病过程中出现的众多并发症使细胞抑制治疗变得困难。由于不存在费城染色体和bcr/abl融合基因,确诊为处于原始细胞危象期的未分类慢性骨髓增殖性综合征。免疫表型分析证实危象具有混合性成髓-原巨核细胞特征。鉴别诊断时考虑了其他骨髓增殖性综合征,包括i(17q)综合征。患者在13个月的观察期后因肿瘤进展和败血症死亡。