Fatal outcome in a patient with autoimmune hemolytic anemia associated with an IgM bithermic anti-ITP. Transfusion 1994 May;34(5):427-31
Date
05/01/1994Pubmed ID
8191568DOI
10.1046/j.1537-2995.1994.34594249056.xScopus ID
2-s2.0-0028236059 (requires institutional sign-in at Scopus site) 10 CitationsAbstract
BACKGROUND: Several cold autoantibodies (usually IgG) with IT specificity have been reported previously, as have autoantibodies with joint I and P blood group specificities (IP1, ITP1, iP1, IP). A fatal outcome associated with an IgM cold autoantibody of ITP specificity is reported.
CASE REPORT: A 54-year-old man suffered from progressively severe cold autoimmune hemolytic anemia for 9 months. Hemoglobin concentration ranged from 6 to 7 g per dL (60-70 g/L) and reticulocytes from 3 to 5 percent (0.030-0.050). The direct antiglobulin test was weakly positive for IgM and strongly positive for C3d. The serum contained a cold agglutinin that reacted strongest with cord i red cells (RBCs) > adult I RBCs > adult i RBCs, which is consistent with IT specificity. The Donath-Landsteiner test was positive; the reaction was neutralized by globoside. The serum reacted weakly or was negative with RBCs from five group p blood donors, which suggests anti-ITP specificity. Dithiothreitol treatment of the serum abolished the cold agglutinin reactivity, which suggests that the anti-IT was IgM. The patient received > 40 RBC transfusions and failed to respond to oral steroids, oral cytoxan, high-dose pulse intravenous steroids, and plasma exchange at room temperature and at 35 degrees C. He died of sepsis following an unsuccessful trial of chlorambucil. Autopsy revealed unsuspected disseminated non-Hodgkin's lymphoma.
CONCLUSION: Serologic studies are consistent with our patient's having a single IgM cold autoantibody with IT and P specificities (anti-ITP) and requiring both specificities on the same RBC to permit maximal antibody expression.
Author List
Ramos RR, Curtis BR, Eby CS, Ratkin GA, Chaplin HAuthor
Brian Curtis PhD Director in the Platelet & Neutrophil Immunology Laboratory department at BloodCenter of WisconsinMESH terms used to index this publication - Major topics in bold
AdsorptionAgglutinins
Anemia, Hemolytic, Autoimmune
Antibody Specificity
Autoantibodies
Blood Protein Electrophoresis
Cold Temperature
Cryoglobulins
Fatal Outcome
Humans
I Blood-Group System
Immunoglobulin M
Immunoglobulin kappa-Chains
Male
Middle Aged
Neutralization Tests
P Blood-Group System
Paraproteins
Phenotype
