Guidelines for the diagnosis and management of adult aplastic anaemia: A British Society for Haematology Guideline

Guideline Hematology Eltrombopag Bone marrow failure Aplastic anemia
DOI: 10.1111/bjh.19236 Publication Date: 2024-01-22T05:24:37Z
ABSTRACT
Summary Pancytopenia with hypocellular bone marrow is the hallmark of aplastic anaemia (AA) and diagnosis confirmed after careful evaluation, following exclusion alternate including hypoplastic myelodysplastic syndromes. Emerging use molecular cyto‐genomics helpful in delineating immune mediated AA from inherited failures (IBMF). Camitta criteria used to assess disease severity, which along age availability human leucocyte antigen compatible donor are determinants for therapeutic decisions. Supportive care blood platelet transfusion support, anti‐microbial prophylaxis prompt management opportunistic infections remain key throughout course. The standard first‐line treatment newly diagnosed acquired severe/very severe patients horse anti‐thymocyte globulin ciclosporin‐based immunosuppressive therapy (IST) eltrombopag or allogeneic haemopoietic stem cell transplant (HSCT) a matched sibling donor. Unrelated HSCT adults should be considered lack response IST, up front young readily available unrelated Management IBMF, pregnancy elderly require special attention. In view rarity complexity management, appropriate discussion multidisciplinary meetings involvement expert centres strongly recommended improve patient outcomes.
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