非霍奇金淋巴瘤继发骨髓纤维化的骨髓病理学特征及其与疾病预后的关系

Pathological Characteristics of Bone Marrow in Non-Hodgkin's Lymphoma Patients with Secondary Myelofibrosis and Their Relationship with Prognosis

目的:观察非霍奇金淋巴瘤(NHL)继发骨髓纤维化患者的骨髓病理学特征及其与疾病预后的关系。方法:回顾性分析2012年1月至2013年12月期间西安交通大学医学院第一附属医院血液内科收治的14例NHL继发骨髓纤维化患者的临床资料,观察继发骨髓纤维化患者的骨髓病理学特征,同时比较同期收住的未合并骨髓纤维化的30例NHL患者与继发骨髓纤维化患者间总生存率及无疾病进展生存率的差异。结果:继发骨髓纤维化的14例NHL患者均为Ⅳ期,其中9例为淋巴瘤细胞白血病,继发骨髓纤维化患者骨髓纤维组织不同程度增生,Gomori染色+至+++,经治疗后骨髓淋巴瘤细胞减少或成为增生性骨髓象时,Gomori染色转为阴性,疾病复发时再次出现骨髓纤维化。未继发骨髓纤维化的30例NHLⅣ期患者的1、3、5年总存率及无疾病进展生存率分别为100%、93.1%、57.6%和100%、92.6%、52.6%。继发骨髓纤维化的14例患者的1、3、5年总存率及无疾病进展生存率分别为92.9%、81.3%、48.8%和71.8%、62.3%、47.9%。结论:NHL继发骨髓纤维化患者多处于Ⅳ期,尤其是淋巴瘤细胞白血病患者中继发骨髓纤维化者多见。继发骨髓纤维化患者骨髓活检表现为纤维组织不同程度增生,疾病缓解时骨髓纤维化程度减轻或消失。继发骨髓纤维化的NHL患者总生存率及无疾病进展生存率较未继发骨髓纤维化患者降低,继发骨髓纤维化是疾病预后不良的指标之一。

Objective： To investigate the pathological characteristics of bone marrow in non-Hodgkin＇s lymphoma （NHL） patients with secondary myelofibrosis and their relationship with disease prognosis. Methods： The pathological characteristics of bone marrow in 14 NHL patients with secondary myelofibrosis and 30 NHL patients without secondary myelofibrosis received from January 2012 to December 2013 in Department of Hematology, the First Affiliated Hospital Xi＇an Jiaotong University Medical School were analysed, and overall survival and progress-free survival rates of NHL patients with and without secondary myelofibrosis were analyzed and compared. Results： 14 cases of NHL with secondary myelofibrosis including 9 cases of lymphoma cell leukemia were all at stage IV and had hyperplasia of bone marrow fibrous tissue, the Gomori staining were all positive. When the lymphoma cells in bone marrow decreased or negative, their Gomori staining were negative. If the disease relapsed, the Gomori staining became positive again. There were 30 cases of NHL at stage IV wihtout secondary myelofibrosis. The overall survival rates and progression-free survival rates at 1,3,5 years in these patients were 100%, 93.1%, 57.6% and 100%, 92.6% ,52.6% respectively. The overall survival rates and progression-free survival rates at 1,3,5 years in 14 NHL patients with secondary myelofibrosis were 92.9%, 81.3%, 48. 8% and was 71. 8%, 62. 3%, 47.9%, respectively. Conclusion： NHL patients with secondary myelofibrosis are almost at stage IV, especially in the patients with lymphoma cell leukemia. They had different degree of hyperplasia of bone marrow fibrous tissue, and the myelofibrosis would be reduced or disappeared when the disease in remission. The overall and progression -free survival rates decrease in NHL patients with secondary myelofibrosis, compared with patients without secondary myelofibrosis. Secondary myelofibrosis is one of the indicators of poor prognosis.