Kraus M D, Bartlett N L, Fleming M D, Dorfman D M
Department of Pathology, Washington University School of Medicine, St. Louis, Missouri 63110, USA.
Am J Surg Pathol. 1998 Oct;22(10):1255-66. doi: 10.1097/00000478-199810000-00011.
Splenomegaly is uncommon in myelodysplasia (MDS) and, although cytopenias may be severe, therapeutic splenectomy is rarely performed. We report the histologic, histochemical, and immunophenotypic findings of nine cases of surgical splenectomy and four postmortem spleens from MDS patients. Four histologic patterns were identified: one dominated by erythrophagocytosis, one characterized by red pulp plasmacytosis, one with extramedullary hematopoiesis as the only salient finding, and one with marked red pulp expansion caused by a monocytic proliferation. Wright-Giemsa and histochemical stains were performed on touch preparations in three cases and played a critical role in the precise subclassification of one MDS patient's hematologic disorder, which ultimately proved to be chronic myelomonocytic leukemia. Splenectomy led to sustained improvement of cytopenias in three cases, but did not eliminate transfusion dependence for the remaining patients. Three splenectomy cases exhibited clustered Leder-negative mononuclear elements: two of these patients experienced disease progression to refractory anemia with excess blasts in transformation or acute myelogenous leukemia during post-splenectomy follow-up, whereas none of the three splenectomy patients without clustered mononuclear elements did. We conclude that splenomegaly in MDS usually reflects the sequelae of dyspoiesis rather than evidence of a proliferative phase, that clustering of Leder-negative large cells may correlate with either a substantial monocytic component or, possibly, increased risk of disease progression, and that the spleen can provide diagnostic as well as prognostic information in MDS patients with splenomegaly.
脾肿大在骨髓增生异常综合征（MDS）中并不常见，尽管血细胞减少可能很严重，但很少进行治疗性脾切除术。我们报告了9例MDS患者手术脾切除及4例死后脾脏的组织学、组织化学和免疫表型结果。识别出四种组织学模式：一种以红细胞吞噬为主，一种以红髓浆细胞增多为特征，一种以外髓造血为唯一显著发现，另一种以单核细胞增殖导致红髓明显扩张为特征。对3例患者的触片进行了瑞氏-吉姆萨染色和组织化学染色，这对一名MDS患者血液系统疾病的精确亚分类起到了关键作用，该患者最终被证实为慢性粒单核细胞白血病。脾切除术后3例患者的血细胞减少持续改善，但其余患者仍未能消除输血依赖。3例脾切除患者出现Leder阴性单核细胞聚集：其中2例患者在脾切除术后随访期间疾病进展为难治性贫血伴原始细胞增多的转化型或急性髓系白血病，而3例无单核细胞聚集的脾切除患者均未出现这种情况。我们得出结论，MDS中的脾肿大通常反映了造血异常的后遗症而非增殖期的证据，Leder阴性大细胞的聚集可能与大量单核细胞成分相关，或者可能与疾病进展风险增加相关，并且脾脏可以为有脾肿大的MDS患者提供诊断及预后信息。
