Elghetany M T, Hudnall S D, Gardner F H
Department of Pathology, University of Texas Medical Branch, Galveston 77555-0743, USA.
Haematologica. 1997 Jan-Feb;82(1):21-4.
Hypoplastic myelodysplastic syndromes (MDS) are being reported with increasing frequency. Aplastic anemia (AA) needs to be differentiated from hypoplastic MDS particularly primary hypoplastic refractory anemia (PHRA) because of the impact on management and prognosis. This distinction may be morphologically difficult even with careful marrow examination which may provide insufficient material due to extreme hypocellularity. The value of peripheral blood (PB) parameters in making the distinction between AA and PHRA is not well studied. In this work, we attempt to examine peripheral blood findings as an additional tool for differentiating PHRA from acquired idiopathic AA.
PB findings in ten cases of PHRA, which are selected based on the following: less than 30% cellularity, multilineage dysplasia and/or clonal cytogenetic abnormality, are compared to ten cases of classic AA. The PB is examined for automated parameters, differential white cell count, morphologic changes in red cells, white cells, platelets, and the presence of circulating blasts, megakaryocytic fragments and micromegakaryocytes.
AA patients tend to have lower platelet and monocytic counts and higher lymphocytic percentages. The following morphologic findings are seen only in PHRA but not in AA: hypochromic red cells, left shift, circulating blasts, hypersegmentation with long filaments, hypogranular, ring, and pelgeroid neutrophils, Dohle bodies, circulating micromegakaryocytes and megakaryocytic fragments.
We conclude that careful examination of peripheral blood may provide sufficient information to allow for the distinction between PHRA and AA early in the course of the disease. Similarly, patients with classic AA who subsequently develop unusual blood findings during routine follow up should be suspected of having a clonal evolution which needs to be confirmed by marrow examination and cytogenetic analysis.
低增生性骨髓增生异常综合征（MDS）的报道频率日益增加。再生障碍性贫血（AA）需要与低增生性MDS，尤其是原发性低增生性难治性贫血（PHRA）相鉴别，因为这会影响治疗和预后。即使进行仔细的骨髓检查，这种区分在形态学上也可能很困难，由于极度细胞减少，骨髓检查可能提供的样本不足。外周血（PB）参数在区分AA和PHRA方面的价值尚未得到充分研究。在这项研究中，我们试图检查外周血结果，作为区分PHRA与获得性特发性AA的辅助工具。
选取10例PHRA患者的外周血结果，入选标准如下：细胞比例低于30%、多系发育异常和/或克隆性细胞遗传学异常，并与10例经典AA患者进行比较。检查外周血的自动参数、白细胞分类计数、红细胞、白细胞、血小板的形态变化，以及循环原始细胞、巨核细胞碎片和微小巨核细胞的存在情况。
AA患者往往血小板和单核细胞计数较低，淋巴细胞百分比较高。以下形态学表现仅见于PHRA，而不见于AA：低色素红细胞、核左移、循环原始细胞、带有长丝的核分叶过多、颗粒减少、环形和类Pelger核中性粒细胞、杜勒小体、循环微小巨核细胞和巨核细胞碎片。
我们得出结论，仔细检查外周血可能提供足够的信息，以便在疾病早期区分PHRA和AA。同样，经典AA患者在常规随访期间如果随后出现异常血液检查结果，应怀疑发生了克隆性演变，这需要通过骨髓检查和细胞遗传学分析来确认。
