继发于系统性红斑狼疮的自身免疫性骨髓纤维化:在资源有限的情况下的诊断挑战。

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL
Oxford Medical Case Reports Pub Date : 2026-07-27 eCollection Date: 2026-07-01 DOI:10.1093/omcr/omag139
Abbas Godian, Noorein Omar, Zainab Malingumu, Basil Tumaini, Elizabeth Msangi
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引用次数: 0

摘要

自身免疫性骨髓纤维化(AIMF)是一种罕见的、潜在可逆的骨髓衰竭原因,通常与系统性自身免疫性疾病有关。我们报告了一位来自撒哈拉以南非洲的26岁女性的诊断挑战病例,她表现为输血依赖性全血细胞减少症和最初的干抽骨髓,由于经济限制,进一步的检测受到限制。7个月后,不断发展的临床和免疫学特征(包括发烧、白细胞减少、血小板减少、疟疾疹、ANA阳性和无菌渗出性胸腔积液)符合2019年系统性红斑狼疮(SLE)的EULAR/ACR标准。继发于SLE的AIMF随后被诊断出来,她对糖皮质激素和羟氯喹表现出快速的血液学反应,保持输血独立超过5个月。该病例强调了在资源有限的情况下诊断AIMF的复杂性,并强调了在评估不明原因全血细胞减少症时持续临床怀疑和适应性诊断策略的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Autoimmune myelofibrosis secondary to systemic lupus erythematosus: a diagnostic challenge in a resource-limited setting.

Autoimmune myelofibrosis (AIMF) is a rare, potentially reversible cause of bone marrow failure often linked to systemic autoimmune disease. We report a diagnostically challenging case of a 26-year-old woman from sub-Saharan Africa who presented with transfusion-dependent pancytopenia and an initial dry-tap marrow, with further testing limited by financial constraints. Seven months later, evolving clinical and immunologic features-including fever, leukopenia, thrombocytopenia, malar rash, positive ANA, and a sterile exudative pleural effusion-fulfilled the 2019 EULAR/ACR criteria for systemic lupus erythematosus (SLE). AIMF secondary to SLE was subsequently diagnosed, and she demonstrated a rapid hematologic response to glucocorticoids and hydroxychloroquine, remaining transfusion-independent for over five months. This case underscores the diagnostic complexity of AIMF in resource-limited settings and highlights the importance of sustained clinical suspicion and adaptable diagnostic strategies when evaluating unexplained pancytopenia.

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来源期刊
Oxford Medical Case Reports
Oxford Medical Case Reports MEDICINE, GENERAL & INTERNAL-
CiteScore
0.90
自引率
0.00%
发文量
125
审稿时长
19 weeks
期刊介绍: Oxford Medical Case Reports (OMCR) is an open access, peer-reviewed online journal publishing original and educationally valuable case reports that expand the field of medicine. The journal covers all medical specialities including cardiology, rheumatology, nephrology, oncology, neurology, and reproduction, comprising a comprehensive resource for physicians in all fields and at all stages of training. Oxford Medical Case Reports deposits all articles in PubMed Central (PMC). Physicians and researchers can find your work through PubMed , helping you reach the widest possible audience. The journal is also indexed in the Web of Science Core Collection . Oxford Medical Case Reports publishes case reports under the following categories: Allergy Audiovestibular medicine Cardiology and cardiovascular systems Critical care medicine Dermatology Emergency medicine Endocrinology and metabolism Gastroenterology and hepatology Geriatrics and gerontology Haematology Immunology Infectious diseases and tropical medicine Medical disorders in pregnancy Medical ophthalmology Nephrology Neurology Oncology Paediatrics Pain Palliative medicine Pharmacology and pharmacy Psychiatry Radiology, nuclear medicine, and medical imaging Respiratory disorders Rheumatology Sexual and reproductive health Sports Medicine Substance abuse.
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