Tanizawa Roberta Sandra da Silva, Zerbini Maria Claudia Nogueira, Rosenfeld Ricardo, Kumeda Cristina Aiko, Azevedo Raymundo Soares, Siqueira Sheila Aparecida Coelho, Velloso Elvira Deolinda Rodrigues Pereira
Universidade de São Paulo, Faculdade de Medicina, Hospital das Clínicas, São Paulo, SP, Brazil.
Universidade de São Paulo, Faculdade de Medicina, São Paulo, SP, Brazil.
Rev Bras Hematol Hemoter. 2017 Jan-Mar;39(1):4-12. doi: 10.1016/j.bjhh.2016.09.015. Epub 2016 Dec 22.
Secondary myeloid neoplasms comprise a group of diseases arising after chemotherapy, radiation, immunosuppressive therapy or from aplastic anemia. Few studies have addressed prognostic factors in these neoplasms.
Forty-two patients diagnosed from 1987 to 2008 with secondary myeloid neoplasms were retrospectively evaluated concerning clinical, biochemical, peripheral blood, bone marrow aspirate, biopsy, and immunohistochemistry and cytogenetic features at diagnosis as prognostic factors. The International Prognostic Scoring System was applied. Statistical analysis employed the Kaplan-Meier method, log-rank and Fisher's exact test.
Twenty-three patients (54.8%) were male and the median age was 53.5 years (range: 4-88 years) at diagnosis of secondary myeloid neoplasms. Previous diseases included hematologic malignancies, solid tumors, aplastic anemia, autoimmune diseases and conditions requiring solid organ transplantations. One third of patients (33%) were submitted to chemotherapy alone, 2% to radiotherapy, 26% to both modalities and 28% to immunosuppressive agents. Five patients (11.9%) had undergone autologous hematopoietic stem cell transplantation. The median latency between the primary disease and secondary myeloid neoplasms was 85 months (range: 23-221 months). Eight patients were submitted to allogeneic hematopoietic stem cell transplantation to treat secondary myeloid neoplasms. Important changes in bone marrow were detected mainly by biopsy, immunohistochemistry and cytogenetics. The presence of clusters of CD117 cells and p53 cells were associated with low survival. p53 was associated to a higher risk according to the International Prognostic Scoring System. High prevalence of clonal abnormalities (84.3%) and thrombocytopenia (78.6%) were independent factors for poor survival.
This study demonstrated that cytogenetics, bone marrow biopsy and immunohistochemistry are very important prognostic tools in secondary myeloid neoplasms.
继发性髓系肿瘤是一组在化疗、放疗、免疫抑制治疗后或由再生障碍性贫血引发的疾病。针对这些肿瘤的预后因素，相关研究较少。
回顾性评估了1987年至2008年间诊断为继发性髓系肿瘤的42例患者，将诊断时的临床、生化、外周血、骨髓穿刺、活检、免疫组化及细胞遗传学特征作为预后因素进行分析。应用国际预后评分系统。采用Kaplan-Meier法、对数秩检验和Fisher精确检验进行统计分析。
23例（54.8%）患者为男性，诊断继发性髓系肿瘤时的中位年龄为53.5岁（范围：4 - 88岁）。既往疾病包括血液系统恶性肿瘤、实体瘤、再生障碍性贫血、自身免疫性疾病以及需要实体器官移植的疾病。三分之一的患者（33%）仅接受化疗，2%接受放疗，26%接受两种治疗方式，28%接受免疫抑制剂治疗。5例患者（11.9%）接受了自体造血干细胞移植。原发性疾病与继发性髓系肿瘤之间的中位间隔时间为85个月（范围：23 - 221个月）。8例患者接受了异基因造血干细胞移植以治疗继发性髓系肿瘤。骨髓的重要变化主要通过活检、免疫组化和细胞遗传学检测发现。CD117细胞簇和p53细胞的存在与低生存率相关。根据国际预后评分系统，p53与较高风险相关。克隆异常的高发生率（84.3%）和血小板减少症（78.6%）是生存不良的独立因素。
本研究表明，细胞遗传学、骨髓活检和免疫组化是继发性髓系肿瘤非常重要的预后评估工具。
