继发性骨髓纤维化（Secondary Myelofibrosis, SMF）是一种由其他疾病或因素引起的骨髓纤维化，常见于骨髓增生异常综合征（MDS）、慢性髓性白血病（CML）、实体瘤转移、感染或自身免疫性疾病等。以下是诊疗指南的关键要点：

1. 诊断

• 临床表现：包括疲劳、贫血、脾肿大、发热、盗汗等非特异性症状。

• 实验室检查：血常规显示贫血、白细胞减少或增多、血小板减少；骨髓活检是金标准，显示纤维组织增生和异常造血。

• 影像学检查：如超声或CT评估脾脏大小和结构。

• 鉴别诊断：需排除原发性骨髓纤维化（PMF）和其他继发原因，通过病史、基因检测（如JAK2、CALR、MPL突变）和原发病评估。

2. 治疗

• 治疗原发病：针对 underlying cause，如化疗 for MDS or CML, 抗感染治疗, or immunosuppression for autoimmune disorders.

• 支持治疗：输血 for anemia, 生长因子（如EPO）to stimulate red blood cell production, and management of symptoms like pain or infections.

• 靶向治疗：JAK inhibitors (e.g., ruxolitinib) may be used off-label for symptom control in some cases, but efficacy varies based on the underlying cause.

• 干细胞移植：考虑 for eligible patients with high-risk disease or when other treatments fail, but it carries significant risks.

• 监测和随访：Regular blood tests and imaging to track disease progression and adjust treatment as needed.

3. 预后

预后取决于原发病的控制情况、患者年龄、 comorbidities, and response to therapy. Early diagnosis and multidisciplinary care are crucial for improving outcomes.