Favourable response to splenectomy in familial myelodysplastic syndrome.
Familial myelodysplastic syndrome occurring at a young age is a very rare childhood haematological malignancy. Two siblings, aged three and 18 years, from a consanguineous marriage, presented with pancytopenia and was subsequently diagnosed to have myelodysplastic syndrome. Both remained clinically stable throughout the illness. Splenectomy appeared to have fully corrected the cytopenia in one of them.
Abdul-Wahab
J
J
Department of Paediatrics, Faculty of Medicine, International Islamic University of Malaysia, PO Box 141, Kuantan 27510, Malaysia. nasyrah_wahab@yahoo.com
Naznin
M
M
Suhaimi
A
A
Amir-Hamzah
A R
AR
D000293:Adolescent; D000328:Adult; D002675:Child, Preschool; D003241:Consanguinity; D005260:Female; D006801:Humans; D006971:Hypersplenism / Q000209:etiology / Q000601:surgery; D019190:Iron Overload / Q000209:etiology; D008297:Male; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000150:complications / Q000235:genetics / Q000628:therapy*; D010198:Pancytopenia / Q000209:etiology / Q000628:therapy*; D013156:Splenectomy; D013163:Splenomegaly / Q000209:etiology / Q000601:surgery*; D065227:Transfusion Reaction