Evidence map›Paper›PMID 42536510›Full record

ArticleMedicine2026

Allogeneic haematopoietic stem cell transplantation for refractory perforating intestinal Behçet disease in a patient with aplastic anaemia: A case report.

Xiuyun Rao, Xu Luo, Li Dai, Qian Wang

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In one paragraph

Article in Medicine, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

4 authors.

Xiuyun RaoGuizhou Provincial Key Laboratory for Digestive System Diseases, Guizhou Medical University and Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China.ORCID 0009-0006-2804-5333
Xu LuoGuizhou Provincial Key Laboratory for Digestive System Diseases, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China.
Li DaiGuizhou Provincial Key Laboratory for Digestive System Diseases, Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China.
Qian WangGuizhou Provincial Key Laboratory for Digestive System Diseases, Guizhou Medical University and Affiliated Hospital of Guizhou Medical University, Guiyang, Guizhou, China.ORCID 0009-0008-0539-4818

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

rationaleAplastic anemia (AA) rarely coexists with intestinal Behçet disease (iBD), and the post-partum period may further complicate its expression. Managing refractory iBD with recurrent perforation on a background of long-standing AA is challenging, and evidence on optimal treatment is limited. PATIENT CONCERNS: A 42-year-old woman with non-severe aplastic anemia (NSAA) diagnosed in 2005 (AA duration ≈ 20 years) developed recurrent fever, oral ulcers, and penetrating ulcers of the ileocaecum and terminal ileum shortly after cesarean delivery in June 2019, with one episode of vulvar ulceration. DIAGNOSES: Histology showed transmural vasculitis with focal obliterative endarteritis, confirming iBD; HLA-B51 was negative. Pre-transplant marrow was markedly hypocellular without dysplasia, and karyotype (46,XX), flow cytometry, myelodysplastic syndrome/AML and myeloproliferative neoplasm gene panels, and paroxysmal nocturnal hemoglobinuria screening were all negative, supporting acquired AA rather than hypoplastic myelodysplastic syndrome.

interventionsDespite corticosteroids, tacrolimus, tofacitinib, and tocilizumab, she sustained 2 intestinal perforations (May and September, 2023). As iBD was refractory and AA had progressed to very severe AA, she underwent human leukocyte antigen (HLA)-matched sibling peripheral-blood allogeneic hematopoietic stem cell transplantation (allo-HSCT) in June 2024 with fludarabine/busulfan/cyclophosphamide/ anti-thymocyte globulin conditioning. OUTCOMES: Neutrophil and platelet engraftment occurred around D+11 and D+15, with 100% donor chimerism at D+16 and no acute or chronic graft-versus-host disease; only transient, self-limiting cytomegalovirus (CMV) and Epstein-Barr virus DNAemia occurred, without CMV disease or post-transplant lymphoproliferative disorder. Blood counts recovered progressively, with hemoglobin 127 grams/L and platelets 118 × 109/L at the last follow-up (≈12 months), only mild residual cytopenia, transfusion independence, and sustained remission of iBD on maintenance immunosuppression. She then returned to work and was lost to follow-up. LESSONS: This case suggests that allo-HSCT may be an effective option for selected patients with AA and refractory iBD, achieving sustained remission of both the marrow failure and the intestinal disease. As this is a single case with limited follow-up (≈12 months), longer follow-up and additional cases are needed.

Indexed as

Anemia, AplasticBehcet SyndromeHematopoietic Stem Cell TransplantationIntestinal PerforationAdultFemaleHumansTransplantation, Homologousallogeneic hematopoietic stem cell transplantationaplastic anemiabone marrow failureintestinal Behçet diseaseintestinal perforation

Identifiers

PMID42536510
PMCPMC13433067

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