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Response of aplastic anaemia and scleroderma to cyclosporin
Author(s) -
Tooze J. A.,
Marsh J. C. W.,
Wickham N.,
Duke O. L.,
Behrens J.,
GordonSmith E. C.
Publication year - 1993
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.1993.tb03236.x
Subject(s) - medicine , scleroderma (fungus) , aplastic anemia , dermatology , immunology , bone marrow , inoculation
Summary 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, CD5 5) and membrane inhibitor of reactive lysis (MIRL. CD59). confirmed a diagnosis of paroxysmal nocturnal haenioglobinuria (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.