title: Hemophagocytic bone marrow aplasia with plasma cells in a RAG2-deficient SCID case after a nonconditioned transplantation from a fully matched sibling.
abstract: We report a RAG2-deficient patient with severe combined immunodeficiency and hemophagocytic bone marrow aplasia with plasma cells after a nonconditioned transplantation from a fully matched sibling. After engraftment, disseminated BCGosis appeared because of graft versus host disease prophylaxis. On the 55th day, eosinophilia, neutropenia, and thrombocytopenia developed. Aplasia, hemophagocytic histiocytes, and plasma cells were found on his bone marrow with very high level of serum immunoglobulin E. We could not discriminate exactly whether BCGosis or alloimmune response is the cause of hemophagocytic aplasia with plasma cells. Despite the second hematopoietic stem cell transplantation with a reduced intensity conditioning regime, his marrow aplasia did not recover and he died. This case suggests that BCGosis might be associated with hemophagocytic marrow aplasia with plasma cells in an alloimmune reaction.
authors: Departments of *Pediatric Immunology-Allergy †Pediatric Hematology, School of Medicine, Ondokuz Mayis University, Samsun, Turkey.
authors: Alisan
authors: A
authors: Yildiran
authors: Tunc
authors: T
authors: Fişgin
authors: Sukru N
authors: SN
authors: Güner
authors: Mehtap
authors: M
authors: Kiliç
authors: Recep
authors: R
authors: Sancak
authors: Emel
authors: E
authors: Özyürek
authors: Davut
authors: D
authors: Albayrak
authors: Feride
authors: F
authors: Duru
mesh_terms: D001500:BCG Vaccine / Q000009:adverse effects* / Q000276:immunology; D001855:Bone Marrow Diseases / Q000209:etiology* / Q000473:pathology; D004268:DNA-Binding Proteins / Q000235:genetics*; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology* / Q000473:pathology; D008297:Male; D009687:Nuclear Proteins / Q000235:genetics*; D010950:Plasma Cells / Q000473:pathology; D016511:Severe Combined Immunodeficiency / Q000235:genetics / Q000601:surgery*; D019172:Transplantation Conditioning; D014184:Transplantation, Homologous / Q000009:adverse effects
chemical_list: D001500:BCG Vaccine; D004268:DNA-Binding Proteins; D009687:Nuclear Proteins; C499311:RAG2 protein, human