MYELODYSPLASTIC SYNDROMES WITH BONE-MARROW FIBROSIS - A MYELODYSPLASTIC DISORDER WITH PROLIFERATIVE FEATURES
MYELODYSPLASTIC SYNDROMES WITH BONE-MARROW FIBROSIS - A MYELODYSPLASTIC DISORDER WITH PROLIFERATIVE FEATURES
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DOI：
10.1007/bf01698371
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发表时间：
1991-11-01
影响因子：
3.5
通讯作者：
BOOGAERTS, MA
中科院分区：
文献类型：
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作者：
VERHOEF, GEG;DEWOLFPEETERS, C;BOOGAERTS, MA
本文报告22例骨髓增生异常综合征（MDS）患者，均表现为明显的骨髓纤维化。诊断时血细胞计数变化不定，常伴有泪滴状异形红细胞增多和成白细胞红细胞样改变。内脏肿大17例，脾肿大7例。所有病例均表现出至少两种细胞谱系的发育异常。这组患者似乎无特异性细胞遗传学异常。Southern印迹分析显示，没有观察到的经典慢性粒细胞白血病的断点簇区域重排。铁动力学研究显示，除两例外，所有病例的红细胞生成量不足，无效红细胞生成的比例异常高。脾红细胞生成8例。中位生存期为18个月。在本报告撰写时，已有12名患者死亡。死亡原因为疾病进展（7例患者）和感染（5例患者）。人们可能会推测，目前的一系列案件代表了MDS和骨髓增生性疾病之间的过渡，从而显示两组疾病的特点。
We report on 22 patients with myelodysplastic syndrome (MDS), all of whom showed striking marrow fibrosis. Variable blood counts, often with teardrop poikilocytosis and a leukoerythroblastic picture, were present at diagnosis. Visceral enlargement was detected in 17 patients with a distinct splenomegaly in seven cases. All cases demonstrated dysplasia in at least two cell lineages. No specific cytogenetic abnormality seems to characterize this group of patients. Southern blot analysis showed no breakpoint cluster region rearrangement as observed in classical chronic myeloid leukemia. Ferrokinetic studies revealed quantitatively deficient erythropoiesis in all except two cases and an abnormally high fraction of ineffective erythropoiesis in all. Splenic erythropoiesis was present in eight patients. The median survival was 18 months. At the time of this report, 12 patients had died. The causes of death were disease progression (7 patients) and infection (5 patients). One might speculate that the present series of cases represents a transition between MDS and myeloproliferative disease, thereby displaying characteristics of both groups of diseases.