TY - JOUR
T1 - Thrombotic thrombocytopenic purpura in an asplenic patient with hereditary spherocytosis
T2 - Failure of plasmapheresis, antiplatelet therapy, and corticosteroids
AU - Becton, David L.
AU - Kietzei, Morris
AU - Arnold, Watson C.
AU - Berry, D. H.
PY - 1988
Y1 - 1988
N2 - Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder characterized by microangiopathic hemolysis, central nervous system and renal dysfunction, and a very poor prognosis. Recently, however, plasma exchange or infusion therapy has proven effective in the majority of patients with TTP. Wc report a patient who developed TTP several years after splenectomy for hereditary spherocytosis. Despite aggressive therapy with plasmapheresis (PP), plasma infusion, antiplatelet drugs, and corticosteroids, the patient had progression of TTP that eventually resulted in his death. The occurrence of TTP in an asplenic patient with an intrinsic red cell disorder, a previously unreported association, may predict a poor prognosis.
AB - Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem disorder characterized by microangiopathic hemolysis, central nervous system and renal dysfunction, and a very poor prognosis. Recently, however, plasma exchange or infusion therapy has proven effective in the majority of patients with TTP. Wc report a patient who developed TTP several years after splenectomy for hereditary spherocytosis. Despite aggressive therapy with plasmapheresis (PP), plasma infusion, antiplatelet drugs, and corticosteroids, the patient had progression of TTP that eventually resulted in his death. The occurrence of TTP in an asplenic patient with an intrinsic red cell disorder, a previously unreported association, may predict a poor prognosis.
KW - Antiplatelet therapy
KW - Plasmapheresis
KW - Spherocytosis
KW - Thrombotic thrombocytopenic purpura
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U2 - 10.1097/00043426-198821000-00003
DO - 10.1097/00043426-198821000-00003
M3 - Article
C2 - 3189717
AN - SCOPUS:0023835426
SN - 1077-4114
VL - 10
SP - 5
EP - 8
JO - Journal of pediatric hematology/oncology
JF - Journal of pediatric hematology/oncology
IS - 1
ER -