Refractory Immune Hemolytic Anemia With A High Thermal Amplitude, Low Affinity IgG Anti-PraCold Autoantibody
作者:
RamosR. R.,
CurtisB. R.,
SadlerJ. E.,
EbyC. S.,
ChaplinH.,
期刊:
Autoimmunity
(Taylor Available online 1992)
卷期:
Volume 12,
issue 2
页码: 149-154
ISSN:0891-6934
年代: 1992
DOI:10.3109/08916939209150322
出版商: Taylor&Francis
关键词: Refractory autoimmune hemolytic anemia;low affinity autoantibody;wide thermal amplitude autoantibody;Praautoantibody specificity
数据来源: Taylor
摘要:
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 Praspecificity.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.
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