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[Evans syndrome in a child].

H Shalev1, A Gedalia, S Alkarnawi

  • 1Dept. of Pediatrics, Soroka Medical Center, Beer Sheba.

Harefuah
|May 1, 1992
PubMed
Summary
This summary is machine-generated.

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This case study details a young girl diagnosed with Evans' syndrome, a rare autoimmune disorder causing red blood cell and platelet destruction. Treatment with corticosteroids and intravenous gamma-globulin proved effective in managing her condition.

Area of Science:

  • Pediatrics
  • Immunology
  • Hematology

Background:

  • Evans' syndrome is a rare autoimmune disorder characterized by concurrent hemolytic anemia and immune thrombocytopenia.
  • Childhood-onset Evans' syndrome presents diagnostic and therapeutic challenges, particularly in cases refractory to standard treatments.

Observation:

  • A 9-month-old Bedouin girl presented with sequential Coombs-positive hemolytic anemia and immune thrombocytopenia.
  • Initial laboratory workup excluded other underlying diseases, leading to a diagnosis of Evans' syndrome.

Findings:

  • Corticosteroid therapy initially achieved normal hemoglobin and thrombocyte counts within one month.
  • Relapses occurred upon corticosteroid dose tapering, indicating steroid dependency.

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Implications:

  • High-dose intravenous gamma-globulin was effective in managing relapses, suggesting its utility in refractory childhood Evans' syndrome.
  • This case highlights the complex management of pediatric Evans' syndrome and the potential role of alternative therapies.