Faculty Collaboration Database

Refractory immune hemolytic anemia with a high thermal amplitude, low affinity IgG anti-Pra cold autoantibody. Autoimmunity 1992;12(2):149-54

Date

01/01/1992

Pubmed ID

1617112

DOI

10.3109/08916939209150322

Scopus ID

2-s2.0-0026645493 (requires institutional sign-in at Scopus site)   11 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.

Author List

Ramos RR, Curtis BR, Sadler JE, Eby CS, Chaplin H

Author

Brian Curtis PhD Director in the Platelet & Neutrophil Immunology Laboratory department at BloodCenter of Wisconsin

MESH terms used to index this publication - Major topics in bold

Agglutinins
Anemia, Hemolytic, Autoimmune
Antibody Specificity
Autoantibodies
Cold Temperature
Cryoglobulins
Humans
Immunoglobulin G
Male
Middle Aged
Rosette Formation