恶性血液病继发骨髓纤维化71例病理及临床研究

 【摘要】 目的 探讨继发性骨髓纤维化(secondary myelofibrosis，SMF)患者的临床及骨髓病理学特征。方法 对71例继发性骨髓纤维化患者的临床表现、外周血涂片、骨髓涂片及骨髓活检情况进行了回顾性研究，同时对不同疾病骨髓纤维化程度与巨核细胞数目作了相关分析。结果 分析71例SMF患者，其原发病情况为慢性粒细胞白血病(CML)20例(28.2%)，急性淋巴细胞白血病（ALL）6例（8.5%），急性髓系细胞白血病(AML)、骨髓增生异常综合症（MDS）、淋巴瘤各10例（14.1%），多发性骨髓瘤（MM）、淋巴增殖性疾病（LPD）各4例（5.6%），骨髓增殖性肿瘤（MPN）3例，慢性淋巴细胞白血病（CLL）2例，骨髓转移癌、传染性单核细胞增多症各1例。骨髓纤维化程度与巨核细胞数具有明显相关性（P＜0.05）。结论 继发性骨髓纤维化患者骨髓切片巨核细胞增生明显，且与纤维化程度有相关性。这可能与巨核细胞克隆性增殖释放的多种细胞因子积极参与骨纤的发生发展密切相关。

Pathological and clinical study of 71 Cases of Myelofibrosis Secondary to Malignant hematonosis

Abstract] Objective To investigate the characteristic of the clinical features and the bone marrow pathology of secondary myelofibrosis(SMF). Methods 71 patients with secondary myelofibrosis were analyzed retrospectively.And the relationship between myelofibrosis degree and megakaryocyte count were analyzed .Results Analysis of 71cases of patients with SMF, the original incidence is chronic myeloid leukemia (CML) 20 patients (28.2%), acute myeloid leukemia (AML),myelodysplastic syndrome (MDS), and lymphoma 10 patients (14.1%) ,respectively.Acute lymphoblastic leukemia (ALL) 6 (8.5%), multiple myeloma (MM), lymphoproliferative disorders (LPD) 4 cases (5.6%), respectively. Myeloproliferative neoplasms (MPN) 3 cases, chronic lymphocytic leukemia (CLL) 2 cases , infectious mononucleosis 1 case. There was a significant difference of the megakaryocyte’ count and association of myelofibrosis was found. Conclusion Megakaryocyte proliferated obviously in patients with SMF, and there was a close correlation between megakaryocyte’ count and myelofibrosis degree. Several kinds of cytokine which released by megakaryocyte may play an important role in the pathogenesis and progression of myelofibrosis.