A Case Report of Rituximab Therapy for Recurrent Thrombotic Thrombocytopenia Purpura
Korean Journal of Hematology
; : 193-197, 2009.
Article
em Ko
| WPRIM
| ID: wpr-720085
Biblioteca responsável:
WPRO
ABSTRACT
Thrombotic thrombocytopenic purpura (TTP) is a rare medical condition characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurologic manifestations, and infrequently, renal involvement. In many cases, TTP is associated with deficiency of the von Willebrand factor-cleaving protease, ADAMTS13, and treatment with plasma exchange is effective in the majority of patients. We report a patient with acute TTP in whom remission was not achieved by initial treatment consisting of plasma exchange and corticosteroids. In view of the severe autoantibody-mediated ADAMTS-13 deficiency, treatment was initiated with rituximab, a chimeric monoclonal antibody directed against the CD 20 antigen present on B lymphocytes. The patient received 4 weekly infusions of rituximab (375 mg/m2). Four weeks after the last infusion of rituximab, a complete clinical and laboratory remission was documented. We conclude that rituximab should be considered in patients with TTP with acquired ADAMTS-13 deficiency, who fail to respond to standard treatment with plasma exchange and corticosteroids. Rituximab may result in a lowered requirement for plasmapheresis and avoid the complications of salvage immunosuppressive therapy.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Troca Plasmática
/
Púrpura
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Púrpura Trombocitopênica Trombótica
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Trombocitopenia
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Nucleotídeos de Timina
/
Linfócitos B
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Plasmaferese
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Corticosteroides
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Anticorpos Monoclonais Murinos
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Rituximab
Limite:
Humans
Idioma:
Ko
Revista:
Korean Journal of Hematology
Ano de publicação:
2009
Tipo de documento:
Article