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解读阵发性夜间血红蛋白尿：溶血性贫血的一种特殊模式

Deciphering Paroxysmal Nocturnal Hemoglobinuria: An Unusual Paradigm of Hemolytic Anemia.

作者信息

Athota Soumya, Gopalan Sowmya, Arthur Preetam, Jayaram Ananthvikas, Satish Anjali

机构信息

Internal Medicine, Sri Ramachandra Institute of Higher Education and Research, Chennai, IND.

Hematopathology, Anand Diagnostic Laboratory, Bengaluru, IND.

出版信息

Cureus. 2024 Aug 19;16(8):e67194. doi: 10.7759/cureus.67194. eCollection 2024 Aug.

DOI:10.7759/cureus.67194
PMID:39295707
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11409906/
Abstract

Paroxysmal nocturnal hemoglobinuria (PNH), a clonal hematopoietic stem cell disorder, arises from the increased sensitivity of red blood cells (RBC) to complement due to an acquired deficiency of certain glycosylphosphatidylinositol (GPI)-linked proteins, resulting in chronic intravascular hemolysis, arterial and venous thrombotic phenomena, multi-organ damage, and failure. We present an intriguing case of hemolytic anemia, initially suspected to be drug-induced, and later found to be associated with PNH, despite being a subclinical clone. A clinician should not hesitate to repeat fluorescent-labeled aerolysin (FLAER) cytometry if the clinical picture strongly favors a diagnosis of PNH. This case marks the importance of testing for PNH clones in autoimmune hemolytic anemia (AIHA) since their prevalence is not negligible and may correspond to a prominent hemolytic pattern, a higher thrombotic risk, and a higher therapeutic indication, such as eculizumab. This underscores the significance of conducting a thorough evaluation for occult causes of treatment-unresponsive hemolytic anemia, paving options for an early and alternative therapeutic approach.

摘要

阵发性睡眠性血红蛋白尿症（PNH）是一种克隆性造血干细胞疾病，由于某些糖基磷脂酰肌醇（GPI）连接蛋白的后天缺乏，导致红细胞（RBC）对补体的敏感性增加，从而引起慢性血管内溶血、动脉和静脉血栓形成现象、多器官损伤及功能衰竭。我们报告了一例有趣的溶血性贫血病例，最初怀疑是药物性的，后来发现与PNH有关，尽管是亚临床克隆。如果临床表现强烈支持PNH诊断，临床医生应毫不犹豫地重复进行荧光标记嗜水气单胞菌溶素（FLAER）细胞计数。该病例凸显了在自身免疫性溶血性贫血（AIHA）中检测PNH克隆的重要性，因为其患病率不可忽视，可能对应显著的溶血模式、更高的血栓形成风险以及更高的治疗指征，如依库珠单抗。这强调了对治疗无反应的溶血性贫血的隐匿病因进行全面评估的重要性，为早期和替代治疗方法提供了选择。

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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4ae2/11409906/93adb641ca5e/cureus-0016-00000067194-i10.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4ae2/11409906/fe881c91afee/cureus-0016-00000067194-i03.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4ae2/11409906/93adb641ca5e/cureus-0016-00000067194-i10.jpg

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本文引用的文献

1
Severe hemolysis with negative direct antiglobulin test: A case report.直接抗球蛋白试验阴性的严重溶血：一例报告
Ann Med Surg (Lond). 2022 Aug 18;81:104444. doi: 10.1016/j.amsu.2022.104444. eCollection 2022 Sep.
2
CD157 Can Replace CD24 and CD14 in a Single-Tube Flow-Cytometric Assay to Detect Paroxysmal Nocturnal Hemoglobinuria (PNH) Clones on Both Neutrophils and Monocytes: A Prospective Study From North India.在单管流式细胞术检测中，CD157可替代CD24和CD14用于检测中性粒细胞和单核细胞上的阵发性睡眠性血红蛋白尿（PNH）克隆：一项来自印度北部的前瞻性研究。
Cureus. 2022 Apr 8;14(4):e23965. doi: 10.7759/cureus.23965. eCollection 2022 Apr.
3
When does a PNH clone have clinical significance?
阵发性睡眠性血红蛋白尿症（PNH）克隆何时具有临床意义？
Hematology Am Soc Hematol Educ Program. 2021 Dec 10;2021(1):143-152. doi: 10.1182/hematology.2021000245.
4
Clinical and prognostic significance of small paroxysmal nocturnal hemoglobinuria clones in myelodysplastic syndrome and aplastic anemia.骨髓增生异常综合征和再生障碍性贫血中小阵发性睡眠性血红蛋白尿克隆的临床和预后意义。
Leukemia. 2021 Nov;35(11):3223-3231. doi: 10.1038/s41375-021-01190-9. Epub 2021 Mar 4.
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Consensus statement for diagnosis and treatment of paroxysmal nocturnal haemoglobinuria.阵发性睡眠性血红蛋白尿诊断与治疗的共识声明
Hematol Transfus Cell Ther. 2021 Jul-Sep;43(3):341-348. doi: 10.1016/j.htct.2020.06.006. Epub 2020 Jul 6.
6
Small Paroxysmal Nocturnal Hemoglobinuria Clones in Autoimmune Hemolytic Anemia: Clinical Implications and Different Cytokine Patterns in Positive and Negative Patients.自身免疫性溶血性贫血中小血管阵发性睡眠性血红蛋白尿克隆：阳性和阴性患者的不同细胞因子模式及临床意义。
Front Immunol. 2020 Jun 4;11:1006. doi: 10.3389/fimmu.2020.01006. eCollection 2020.
7
Coombs-positive Paroxysmal Nocturnal Haemoglobinuria.库姆斯阳性阵发性夜间血红蛋白尿症
Oxf Med Case Reports. 2020 Mar 30;2020(3):omz125. doi: 10.1093/omcr/omz125. eCollection 2020 Mar.
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