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首页> 外文期刊>International Journal of General Medicine >Myelofibrosis-associated complications: pathogenesis, clinical manifestations, and effects on outcomes
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Myelofibrosis-associated complications: pathogenesis, clinical manifestations, and effects on outcomes

机译：骨髓纤维化相关并发症：发病机制，临床表现及其对预后的影响

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Abstract: Myelofibrosis (MF) is a rare chronic BCR-ABL1 (breakpoint cluster region-Abelson murine leukemia viral oncogene homologue 1)-negative myeloproliferative neoplasm characterized by progressive bone marrow fibrosis, inefficient hematopoiesis, and shortened survival. The clinical manifestations of MF include splenomegaly, consequent to extramedullary hematopoiesis, cytopenias, and an array of potentially debilitating abdominal and constitutional symptoms. Dysregulated Janus kinase (JAK)-signal transducer and activator of transcription signaling underlies secondary disease-associated effects in MF, such as myeloproliferation, bone marrow fibrosis, constitutional symptoms, and cachexia. Common fatal complications of MF include transformation to acute leukemia, thrombohemorrhagic events, organ failure, and infections. Potential complications from hepatosplenomegaly include portal hypertension and variceal bleeding, whereas extramedullary hematopoiesis outside the spleen and liver – depending on the affected organ – may result in intracranial hypertension, spinal cord compression, pulmonary hypertension, pleural effusions, lymphadenopathy, skin lesions, and/or exacerbation of abdominal symptoms. Although allogeneic stem cell transplantation is the only potentially curative therapy, it is suitable for few patients. The JAK1/JAK2 inhibitor ruxolitinib is effective in improving splenomegaly, MF-related symptoms, and quality-of-life measures. Emerging evidence that ruxolitinib may be associated with a survival benefit in intermediate- or high-risk MF suggests the possibility of a disease-modifying effect. Consequently, ruxolitinib could provide a treatment backbone to which other (conventional and novel) therapies may be added for the prevention and effective management of specific MF-associated complications.
机译：摘要：骨髓纤维化（MF）是一种罕见的慢性BCR-ABL1（断点簇区域-Abelson鼠白血病病毒病毒癌基因同源物1）-阴性的骨髓增生性肿瘤，其特征在于进行性骨髓纤维化，低效率的造血作用和缩短的生存期。 MF的临床表现包括脾肿大，髓外造血，血细胞减少和一系列可能使人虚弱的腹部和体质症状。失调的Janus激酶（JAK）信号转导子和转录信号激活因子是MF中继发于疾病的继发性疾病相关作用的基础，例如骨髓增殖，骨髓纤维化，体质症状和恶病质。 MF的常见致命并发症包括转化为急性白血病，血栓出血事件，器官衰竭和感染。肝脾肿大的潜在并发症包括门静脉高压症和静脉曲张破裂出血，而脾脏和肝脏外部的髓外造血（取决于受影响的器官）可能导致颅内高压，脊髓压迫，肺动脉高压，胸腔积液，淋巴结肿大，皮肤病变和/或腹部症状加重。尽管同种异体干细胞移植是唯一可能的治疗方法，但它仅适用于少数患者。 JAK1 / JAK2抑制剂鲁索替尼可有效改善脾肿大，MF相关症状和生活质量。鲁索替尼可能与中危或高危MF患者的生存获益相关的新兴证据表明，有可能改善疾病。因此，ruxolitinib可以提供治疗主干，可以在其中添加其他（传统和新颖）疗法来预防和有效管理与MF相关的特定并发症。

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