Myelodysplasia following aplastic anaemia-paroxysmal nocturnal haemoglobinuria syndrome after treatment with immunosuppression and G-CSF: evidence for the emergence of a separate clone.
Myelodysplasia (MDS) and aplastic anaemia-paroxysmal nocturnal haemoglobinuria (AA/PNH) syndrome developed in a severe aplastic anaemia (AA) patient after treatment with immunosuppressive (IS) therapy. Glycosylphosphatidyl inositol (GPI)-linked proteins were determined, and during the AA/PNH phase, a high proportion of neutrophils were found to be negative, without clinical evidence of haemolysis. However, MDS developed with cytogenetic abnormalities of monosomy 7,9q- and a rearranged chromosome 6; the GPI-linked protein negative cells were completely replaced by positively expressing cells. This represents the emergence of a GPI-linked protein positive myelodysplasia clone arising separately from an AA/PNH clone.
Jin
J Y
JY
Department of Cellular and Molecular Sciences, St George's Hospital Medical School, London.
Tooze
J A
JA
Marsh
J C
JC
Matthey
F
F
Gordon-Smith
E C
EC
D000368:Aged; D000741:Anemia, Aplastic / Q000150:complications / Q000628:therapy*; D017261:Glycosylphosphatidylinositols / Q000378:metabolism; D016179:Granulocyte Colony-Stimulating Factor / Q000009:adverse effects*; D006457:Hemoglobinuria, Paroxysmal / Q000209:etiology*; D006801:Humans; D007165:Immunosuppression Therapy / Q000009:adverse effects*; D008297:Male; D009000:Monocytes / Q000378:metabolism; D009190:Myelodysplastic Syndromes / Q000209:etiology*; D009504:Neutrophils / Q000378:metabolism; D013577:Syndrome
D017261:Glycosylphosphatidylinositols; D016179:Granulocyte Colony-Stimulating Factor