Response of aplastic anaemia and scleroderma to cyclosporin.
Scleroderma and aplastic anaemia (AA) occurred simultaneously in a patient. Treatment with antilymphocyte globulin (ALG) resulted in some improvement of the scleroderma and a partial, temporary response of the AA. Both the scleroderma and AA then responded dramatically to cyclosporin (CSA) therapy. Subsequently, a positive Ham's test, together with a reduction in the phosphatidyl-inositolglycan (PIG) anchored membrane proteins decay accelerating factor (DAF, CD55) and membrane inhibitor of reactive lysis (MIRL, CD59), confirmed a diagnosis of paroxysmal nocturnal haemoglobinuria (PNH) affecting erythroid, myeloid and lymphoid cell lineages. We hypothesize that the pathogenesis of the bone marrow failure in this patient was a stem cell defect with a secondary immune response involving T-lymphocytes that may have simultaneously triggered the pancytopenia and scleroderma.
Tooze
J A
JA
Department of Haematology, St George's Hospital, London.
Marsh
J C
JC
Wickham
N
N
Duke
O L
OL
Behrens
J
J
Gordon-Smith
E C
EC
D000741:Anemia, Aplastic / Q000188:drug therapy*; D003524:Cyclosporins / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010976:Platelet Count; D017713:Platelet Transfusion; D012594:Scleroderma, Localized / Q000188:drug therapy*
D003524:Cyclosporins