Identification of TRAPPC4 as a Key Autoantigen in Immune-Related Pancytopenia: Epitope Characterization and Immune Activation Mechanisms.

Objective: Immune-related pancytopenia (IRP) is characterized by autoantibody-mediated destruction or suppression of bone marrow cells, leading to pancytopenia. This study aimed to explore the role of TRAPPC4 (trafficking protein particle complex subunit 4) as a key autoantigen in IRP, including epitope identification and immune activation mechanisms.

Methods: A total of 90 participants were included in the study, divided into four groups: 30 newly diagnosed IRP patients, 25 IRP remission patients, 20 patients with control hematologic conditions (severe aplastic anemia [SAA] and myelodysplastic syndrome [MDS]), and 15 healthy controls.

Ferritin Light Chain: A Candidate Autoantigen in Immuno-Related Pancytopenia.

The characteristic feature of immune-related pancytopenia (IRP) is autoantibody-mediated bone marrow (BM) damage and peripheral blood cytopenia. We found that the potential antigen of IRP was Ferritin light chain (FTL) by SEREX (serological analysis of recombinant cDNA expression libraries) in the previous study. In this study, we tried to explore the antigenic epitopes of FTL and verify its antigenicity in IRP.

Antibodies specific to ferritin light chain polypeptide are frequently detected in patients with immune‑related pancytopenia.

Immuno-related pancytopenia (IRP) is characterized by pancytopenia resulting from bone marrow suppression or destruction mediated by auto‑antibodies. In our previous study, a K562 cDNA library was established, which was used to screen for seven possible auto‑antigens produced by hematopoietic cells in patients with IRP, including ferritin light chain (FTL). In the present study, FTL was expressed and purified, and the levels of the auto‑antibodies specific to FTL were measured.

Roles of immune responses in the pathogenesis of immunorelated pancytopenia.

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.

Abnormal numbers of CD4+ T lymphocytes and abnormal expression of CD4+ T lymphocyte‑secreted cytokines in patients with immune‑related haemocytopenia.

In the past decade, a group of cases with persisting haemocytopenia were separated from those with idiopathic cytopenia of undetermined significance due to the optimal response of these patients to immunosuppression therapy and due to the detection of autoantibodies in the bone marrow of haemopoietic cells. This condition was termed immune‑related haemocytopenia (IRH). However, the quantity of T lymphocytes remained unknown.

Lower level of IL‑35 and its reduced inhibition in Th17 cells in patients with bone marrow mononuclear cells Coombs test‑positive hemocytopenia.

Interleukin (IL)-35 is the latest member of IL-12 family, which plays an important role in other autoimmune diseases. Bone marrow mononuclear cells Coombs test‑positive hemocytopenia, also termed immunorelated hemocytopenia (IRH) is a type of autoimmune-associated diseases. The present study investigated the relationship of IL‑35 in patients with IRH.

Screening novel autoantigens targeted by serum IgG autoantibodies in immunorelated pancytopenia by SEREX.

Immunorelated pancytopenia (IRP) is characterized by pancytopenia caused by autoantibody-mediated destruction or suppression of bone marrow. However, the autoantigens targeted by autoantibodies in IRP remain unclear. In the present study, we screened novel autoantigens in IRP by serological analysis of recombinant cDNA expression libraries and compared anti-UQCR10 antibody levels between IRP and normal controls detected by immunoblotting.

HOTAIR is upregulated in acute myeloid leukemia and that indicates a poor prognosis.

Hox transcript antisense intergenic RNA (HOTAIR) is a long non-coding RNA, its overexpression has been documented in various human solid tumor and it can be considered as a potential cancer biomarker. However, little is known about the role of HOTAIR in acute myeloid leukemia (AML). In this study, We evaluated HOTAIR expression in bone marrow of de novo AML patients, AML-CR patients and normal controls by real-time quantitative reverse transcription-PCR (qRT-PCR), then we inhibited hotair expression of two cell lines by siRNA and evaluated their proliferation by CCK, finally we analyzed its relationship with the clinicopathological parameters of AML.