Demırkan Fatih, Özsan G Hayri, Özcan Mehmet Ali, Vural Filiz, Öztop İlhan, Yüksel Erdinç, Ündar Bülent
Turk J Haematol. 2003 Mar 5;20(1):25-30.
Thrombocytopenia may be the presenting cytopenia of myelodysplastic syndrome (MDS) and is named as refractory thrombocytopenia (RT) and categorized in the refractory cytopenia with multilineage dysplasia (RCMD) group according to the recent World Health Organization (WHO) classification of the acute leukemias and MDS. Abnormal cytogenetics can be found in 60% to 80% of patients with MDS. Most common cytogenetic abnormalities include monosomy 5, 5q-, monosomy 7, trisomy 8, deletion 20q and loss of X or Y chromosome. Here we report clinical features and outcomes of nine patients with RT. Cytogenetic abnormalities were detected in seven. Among two patients who have a normal karyotype at diagnosis, one of them transformed to acute myeloid leukemia (AML). During a median follow-up of 29 months, two patients died of hemorrhagia and one of AML. The features and prognosis of patients with RT needs to be determined by larger series.
血小板减少症可能是骨髓增生异常综合征(MDS)最初出现的血细胞减少症,被命名为难治性血小板减少症(RT),并根据世界卫生组织(WHO)最近对急性白血病和MDS的分类,归类于伴有多系发育异常的难治性血细胞减少症(RCMD)组。60%至80%的MDS患者可发现细胞遗传学异常。最常见的细胞遗传学异常包括5号染色体单体、5q-、7号染色体单体、8号染色体三体、20q缺失以及X或Y染色体丢失。在此,我们报告9例RT患者的临床特征及转归。7例检测到细胞遗传学异常。在诊断时核型正常的2例患者中,其中1例转化为急性髓系白血病(AML)。在中位随访29个月期间,2例患者死于出血,1例死于AML。RT患者的特征和预后需要通过更大规模的系列研究来确定。