Refractory immune hemolytic anemia with a high thermal amplitude, low affinity igg anti-pra cold autoantibody

R. R. Ramos, B. R. Curtis, J. E. Sadler, C. S. Eby, H. Chaplin

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

A 54 y.o. woman presented with acute Coombs-negative hemolytic anemia at an outside hospital where she received 25 RBC transfusions and did not respond to prednisone or splenectomy. On transfer to our hospital, routine DAT and IAT were weakly positive, occasionally negative. When a modified "cold" antiglobulin test was employed, the result was strongly positive for IgG, weakly positive for C3d. Cold agglutinin titer was 32, and the Donath-Landsteiner test was negative. The autoantibody exhibited Pra specificity. The patient failed IV-IgG, high dose IV pulse steroids and cyclophosphamide, and continued to require daily transfusions. She responded 21 days after receiving daily plasma exchange (x3), with pulse cyclophosphamide on the third day, followed by escalating daily oral cyclophosphamide.

Original languageEnglish
Pages (from-to)149-154
Number of pages6
JournalAutoimmunity
Volume12
Issue number2
DOIs
StatePublished - 1992

Keywords

  • Low affinity autoantibody
  • Refractory autoimmune hemolytic anemia
  • Wide thermal amplitude autoantibody

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