Rituximab for refractory Evans syndrome and other immune‐mediated hematologic diseases

The authors describe a 21‐year‐old man with long‐lasting Evans syndrome refractory to corticosteroids and immunosuppressive agents; the patient responded to four weekly infusions of rituximab. The patient relapsed with thrombocytopenia 7 months post‐therapy and was successfully re‐treated with two w...

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Bibliographic Details
Published in:American journal of hematology 2004-11, Vol.77 (3), p.303-310
Main Authors: Mantadakis, Elpis, Danilatou, Vassiliki, Stiakaki, Eftichia, Kalmanti, Maria
Format: Article
Language:English
Subjects:
Adult
Anemia, Hemolytic, Autoimmune - blood
Anemia, Hemolytic, Autoimmune - drug therapy
Anemia, Hemolytic, Autoimmune - immunology
Anemias. Hemoglobinopathies
Antibodies, Monoclonal - adverse effects
Antibodies, Monoclonal - therapeutic use
Antibodies, Monoclonal, Murine-Derived
autoimmune hemolytic anemia
Biological and medical sciences
chronic ITP
Diseases of red blood cells
Evans syndrome
Follow-Up Studies
Hematologic and hematopoietic diseases
Humans
Male
Medical sciences
Platelet Count
Platelet diseases and coagulopathies
Purpura, Thrombocytopenic, Idiopathic - blood
Purpura, Thrombocytopenic, Idiopathic - drug therapy
Purpura, Thrombocytopenic, Idiopathic - immunology
Rituximab
Time Factors
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Description
Summary:The authors describe a 21‐year‐old man with long‐lasting Evans syndrome refractory to corticosteroids and immunosuppressive agents; the patient responded to four weekly infusions of rituximab. The patient relapsed with thrombocytopenia 7 months post‐therapy and was successfully re‐treated with two weekly doses of the same monoclonal antibody. He remains in remission for 7‐plus months after the second treatment. Therapy was well tolerated, and no infectious complications occurred, despite avoiding administration of prophylactic gammaglobulin. Rituximab appears safe and modestly effective in a variety of immune‐mediated hematologic diseases, including autoimmune hemolytic anemia, chronic immune thrombocytopenia, Evans syndrome, pure red cell aplasia, mixed type II cryoglobulinemia, cold agglutinin disease, and Waldenström's macroglobulinemia. However, as most of the published literature consists of case reports and small case series, international collaboration is essential in order to better define the efficacy and safety of this agent in children and adults with hematologic diseases. Am. J. Hematol. 77:303–310, 2004. © 2004 Wiley‐Liss, Inc.
ISSN:0361-8609
1096-8652
DOI:10.1002/ajh.20180