title: Autoimmune haemolytic anaemia as the presenting manifestation of agnogenic myeloid metaplasia.
abstract: We report an unusual case of a 40-year-old female patient with a severe case of direct Coombs positive haemolytic anaemia, moderate hepatomegaly and marked splenomegaly. Her initial response to steroids was transient and was rapidly followed by a relapse. Therefore, she underwent splenectomy both as a therapeutic measure and to rule out an underlying lymphoproliferative disorder. Histopathological examination of the excised spleen, as well a liver biopsy, revealed extensive extramedullary haematopoiesis, while significant marrow fibrosis was noted in a trephine biopsy sample. These findings confirmed the concomitant diagnosis of agnogenic myeloid metaplasia with myelofibrosis.
authors.affiliation: Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Unmesh
authors.initials: U
authors.lastname: Mohite
authors.forename: Anil
authors.initials: A
authors.lastname: Pathare
authors.forename: Salam
authors.initials: S
authors.lastname: al Kindi
authors.forename: Devendra
authors.initials: D
authors.lastname: Hiwase
authors.forename: Nirmala
authors.initials: N
authors.lastname: Vadakkepat
authors.forename: Huxley
authors.initials: H
authors.lastname: Knox-Macaulay
mesh_terms: D000328:Adult; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006410:Hematopoiesis; D006801:Humans; D055728:Primary Myelofibrosis / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D013154:Spleen / Q000473:pathology; D013156:Splenectomy