Some patients with pancytopenia do not conform to any diagnostic criteria of known haematological or non‐haematological diseases; however, they respond well to corticosteroid, high‐dose intravenous immunoglobulin and rituximab treatment. This abnormality is termed immunorelated pancytopenia (IRP). Later studies indicated that IRP might be a kind of autoimmune disease in which T helper (Th) type 2 cell function is enhanced, resulting in the hyperfunction of B lymphocytes, which then produce excess autoantibodies that attack the bone marrow (BM) and cause cytopenia. Hypofunction of regulatory T (Treg) cells and enhanced Th17 cell function, an elevated percentage of plasmacytoid dendritic cells (pDCs) and a decreased percentage of natural killer (NK) cells help to promote the process. Moreover, increased expression of a synergistic stimulator of B lymphocytes, CD70 and the reactive overexpression of the BCR inhibitory coreceptor CD22 also support this claim. Candidate autoantigens targeted by autoantibodies on haematopoietic cell membranes have also been reported in IRP. This review is focused on studies that demonstrate the role of immune responses in the pathogenesis of IRP. Current diagnostic criteria and treatments for IRP are also referenced to provide a thorough understanding. Distinguishing IRP from idiopathic cytopenias of undetermined significance (ICUS) and other haematological disorders, for example myelodysplastic syndrome (MDS), aplastic anaemia (AA), paroxysmal nocturnal hemoglobinuria (PNH) and Evans syndrome, may help patients with pancytopenia benefit from proper treatment. Further studies are required to achieve new insight into the pathophysiology of IRP with regard to the immune system, which will be instrumental for the development of novel therapies for inhibiting disease initiation and/or progression.

中文翻译：

---

免疫应答在免疫相关全血细胞减少症发病机制中的作用。

一些全血细胞减少症患者不符合任何已知血液学或非血液学疾病的诊断标准；但是，它们对皮质类固醇，大剂量静脉内免疫球蛋白和利妥昔单抗治疗反应良好。这种异常称为免疫相关性全血细胞减少症（IRP）。后来的研究表明，IRP可能是一种自身免疫疾病，其中T型辅助（Th）2型细胞功能增强，导致B淋巴细胞功能亢进，然后产生过多的自身抗体攻击骨髓（BM）并引起血细胞减少。调节性T（Treg）细胞功能低下和Th17细胞功能增强，浆状树突状细胞（pDC）百分比升高以及自然杀伤（NK）细胞百分比降低有助于促进这一过程。此外，B淋巴细胞，CD70协同刺激物的表达增加以及BCR抑制性共受体CD22的反应性过表达也支持这一主张。在IRP中也已经报道了由自身抗体靶向造血细胞膜的候选自身抗原。这篇综述的重点是证明免疫应答在IRP发病机理中的作用的研究。还参考了IRP的当前诊断标准和治疗方法，以提供全面的了解。将IRP与意义不明的特发性血细胞减少症（ICUS）和其他血液系统疾病（例如骨髓增生异常综合症（MDS），再生障碍性贫血（AA），阵发性夜间血红蛋白尿（PNH）和Evans综合征）区分开来，可以帮助患有全血细胞减少症的患者从适当治疗中受益。