骨髓增生性肿瘤的肾脏损害:一种未被报道的并发症。

IF 0.6 4区 医学 Q4 HEMATOLOGY
Rintu Sharma, Arihant Jain, Charanpreet Singh, Aditya Jandial, Deepesh Lad, Alka Khadwal, Gaurav Prakash, Ritambhra Nada, Raja Ramachandra, Neelam Varma, Pankaj Malhotra
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引用次数: 0

摘要

骨髓增生性疾病(mpn)的肾脏受累是罕见的,在诊断和治疗方面提出了独特的挑战。mpn患者肾功能障碍的确切发病机制尚不清楚。这些患者大多是老年人,并伴有导致肾功能障碍的合并症。虽然有症状的患者通常表现为蛋白尿、高血压、无血和血尿,但许多患者在偶然发现肾脏损害时仍无症状。采用肾活检进行早期诊断,并及时开始使用皮质类固醇、细胞减少药物和靶向药物治疗,对于获得良好的结果至关重要。我们提出了一系列的病例描述我们的方法和管理策略的MPN患者谱(原发性骨髓纤维化,原发性血小板增多,慢性髓性白血病和纤维化前骨髓纤维化)活检证实肾功能障碍的病因。虽然髓外造血是我们所有病例的共同发现,但我们讨论了MPN可以靶向肾脏的各种其他机制。尽管广泛使用细胞减少和靶向治疗,但这种情况需要个性化的方法以及多学科的努力。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Renal Impairment in Myeloproliferative Neoplasms: An Under-reported Complication.

Renal involvement in myeloproliferative disorders (MPNs) is rare and presents unique challenges in terms of diagnosis and treatment. The exact pathogenesis of renal dysfunction in MPNs remains unclear. Most of these patients are elderly and have comorbid conditions that contribute to their renal dysfunction. Although symptomatic patients typically present with proteinuria, hypertension, anasarca, and hematuria, many patients remain asymptomatic with incidental detection of renal impairment. Early diagnosis using renal biopsy and prompt initiation of treatment with corticosteroids, cytoreductive drugs, and targeted agents are crucial for achieving a favorable outcome. We present a series of case vignettes describing our approach and management strategies in a spectrum of MPN patients (primary myelofibrosis, essential thrombocytosis, chronic myeloid leukemia, and pre-fibrotic myelofibrosis) with biopsy proven etiologies of renal dysfunction. Although extramedullary hematopoiesis was a common finding in all our cases, we discuss the various other mechanisms by which MPN can target the kidneys. Despite the widespread use of cytoreductive and targeted therapies, such situations require an individualized approach along with multidisciplinary effort.

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来源期刊
CiteScore
1.70
自引率
0.00%
发文量
82
审稿时长
>12 weeks
期刊介绍: Indian Journal of Hematology and Blood Transfusion is a medium for propagating and exchanging ideas within the medical community. It publishes peer-reviewed articles on a variety of aspects of clinical hematology, laboratory hematology and hemato-oncology. The journal exists to encourage scientific investigation in the study of blood in health and in disease; to promote and foster the exchange and diffusion of knowledge relating to blood and blood-forming tissues; and to provide a forum for discussion of hematological subjects on a national scale. The Journal is the official publication of The Indian Society of Hematology & Blood Transfusion.
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