Abstract
Evans syndrome is an autoimmunopathy characterized by thrombocytopenia and autoimmune hemolytic anemia with poor response to immunosuppression. A 2-yr-old boy with Evans syndrome showed only short-lasting responses to immunosuppressive treatment including double autologous peripheral stem cell transplantation (SCT). Intracranial bleeding required emergency splenectomy and external ventricular drainage. Unrelated umbilical cord blood was given following conditioning with busulfan, thiotepa, etoposide and antithymocyte globulin. One year after SCT the patient shows stable blood counts without immunosuppression. This is the first child reported with Evans syndrome successfully treated by means of unrelated cord blood transplantation.
MeSH terms
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Adrenal Cortex Hormones / adverse effects
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Adrenal Cortex Hormones / therapeutic use
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Anemia, Hemolytic, Autoimmune / complications
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Anemia, Hemolytic, Autoimmune / drug therapy
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Anemia, Hemolytic, Autoimmune / surgery*
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Cerebral Hemorrhage / etiology
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Cerebral Hemorrhage / pathology
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Child, Preschool
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Cord Blood Stem Cell Transplantation*
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Danazol / therapeutic use
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Drug Resistance
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Female
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Humans
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Immunoglobulins, Intravenous / therapeutic use
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Immunosuppressive Agents / therapeutic use
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Magnetic Resonance Imaging
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Male
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Peripheral Blood Stem Cell Transplantation
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Purpura, Thrombocytopenic, Idiopathic / complications
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Purpura, Thrombocytopenic, Idiopathic / drug therapy
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Purpura, Thrombocytopenic, Idiopathic / surgery*
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Remission Induction
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Splenectomy
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Transplantation Conditioning
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Transplantation, Autologous
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Transplantation, Homologous
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Vincristine / therapeutic use
Substances
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Adrenal Cortex Hormones
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Immunoglobulins, Intravenous
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Immunosuppressive Agents
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Vincristine
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Danazol