A myelokathexis-like variant of myelodysplasia.
A 34-year-old woman presented with pancytopenia, profound neutropenia and repeated infections. A bone marrow specimen revealed hypercellularity with trilineage dysplasia and bizarre granulocytic morphology identical to that of the rarely reported myelokathexis. Investigations revealed defects in platelet and neutrophil functions and markedly reduced colony-to-cluster ratio in bone marrow culture. She required treatment with antibiotics, surgical intervention for a gangrenous abdominal wall, and eventually underwent a successful allogeneic bone marrow transplant from her sister. This case suggests that myelokathexis is an unusual form of myelodysplasia with a relatively benign course and presenting at a young age.
Rassam
S M
SM
Department of Haematology, Royal Free Hospital, London, U.K.
Roderick
P
P
al-Hakim
I
I
Hoffbrand
A V
AV
D000328:Adult; D001853:Bone Marrow / Q000473:pathology; D005260:Female; D006412:Hematopoietic Stem Cells / Q000473:pathology; D006801:Humans; D009190:Myelodysplastic Syndromes / Q000145:classification / Q000175:diagnosis* / Q000473:pathology