Bourgeois E, Caulier M T, Rose C, Dupriez B, Bauters F, Fenaux P
Service des Maladies du Sang CHU Lille, France.
Leukemia. 2001 Jun;15(6):950-3. doi: 10.1038/sj.leu.2402129.
Thrombocytopenia is generally of central origin in MDS, but can be due to peripheral platelet destruction in some cases. We studied platelet lifespan in 61 MDS cases with platelets < 70,000/mm3 and marrow blasts < 10%. Nine of them (15%) had a major platelet lifespan reduction (< 3.5 days), and were considered for splenectomy. Three of them were not splenectomized due to rapid death, patient refusal and older age plus liver predominance of platelet sequestration, respectively. The remaining six patients (two females and four males, median age 50 years, range 32 to 65) were splenectomized 3 to 21 months after diagnosis. Before splenectomy, five of them had RA and one had CMML. Platelets counts ranged from 5000 to 30,000/mm3 and did not durably respond to other treatments. Three of the patients has a relapse of platelet counts, concomitantly required platelet transfusion due to recurrent blending, whereas three had anemia (two required erythrocyte transfusion) and four had neutropenia. Three months after surgery, platelet counts ranged from 55,000 to 160,000/mm3 (> 100,000/mm3 in four cases), no patient required platelet or erythrocyte transfusion, but there was no effect on neutrophil counts. Three patients had a relapse of platelet counts, concomitant with progression to AML in two of them, whereas the third relapsing case achieved normal platelet counts with further danazol. One patient died with normal platelet counts 12 months after splenectomy (from sepsis, probably related to neutropenia rather than splenectomy). Two patients remained with normal platelet counts 10 and 52 months after surgery. Our findings suggest that the mechanism of thrombocytopenia should be studied more often in 'low risk' MDS (i.e. with low bone marrow blast counts) with thrombocytopenia, as about 15% of them appear to have peripheral platelet destruction. Some of those patients may benefit from splenectomy.
血小板减少症在骨髓增生异常综合征（MDS）中通常源于中枢，但在某些情况下可能是由于外周血小板破坏所致。我们研究了61例血小板计数<70,000/mm³且骨髓原始细胞<10%的MDS患者的血小板寿命。其中9例（15%）血小板寿命显著缩短（<3.5天），并考虑行脾切除术。其中3例分别因迅速死亡、患者拒绝以及年龄较大且以肝脏为主的血小板扣押而未行脾切除术。其余6例患者（2例女性，4例男性，中位年龄50岁，范围32至65岁）在诊断后3至21个月行脾切除术。脾切除术前，其中5例为难治性贫血（RA），1例为慢性粒单核细胞白血病（CMML）。血小板计数范围为5000至30,000/mm³，对其他治疗无持久反应。3例患者血小板计数复发，因反复出血而同时需要血小板输注，3例有贫血（2例需要红细胞输注），4例有中性粒细胞减少。术后3个月，血小板计数范围为55,000至160,000/mm³（4例>100,000/mm³），无患者需要血小板或红细胞输注，但对中性粒细胞计数无影响。3例患者血小板计数复发，其中2例进展为急性髓系白血病（AML），而第3例复发患者使用达那唑后血小板计数恢复正常。1例患者在脾切除术后12个月血小板计数正常时死亡（死于败血症，可能与中性粒细胞减少而非脾切除术有关）。2例患者术后10个月和52个月血小板计数仍正常。我们的研究结果表明，对于血小板减少的“低危”MDS（即骨髓原始细胞计数低），应更频繁地研究血小板减少的机制，因为其中约15%似乎存在外周血小板破坏。其中一些患者可能从脾切除术中获益。
