Autoimmune haemolytic anaemia as the presenting manifestation of agnogenic myeloid metaplasia.
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.
Mohite
Unmesh
U
Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman.
Pathare
Anil
A
al Kindi
Salam
S
Hiwase
Devendra
D
Vadakkepat
Nirmala
N
Knox-Macaulay
Huxley
H
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