Life‐threatening, antiglobulin test‐negative, acute autoimmune hemolytic anemia due to a non‐complement‐activating IgG1 kappa cold antibody with Pra specificity
- 12 November 1990
- journal article
- case report
- Published by Wiley in Transfusion
- Vol. 30 (9), 838-843
- https://doi.org/10.1046/j.1537-2995.1990.30991048792.x
Abstract
A 21-year-old man with fulminant cold autoantibody hemolytic anemia (CAHA) was hospitalized with hemoglobinemia, hemoglobinuria, hemoglobin concentration of 3.3 gm per dL, a negative direct antiglobulin test (DAT) with polyspecific and anti-C3d reagents, a negative Donath-Landsteiner test, and a cold agglutinin titer of 80. He failed to respond to corticosteroids, multiple plasma exchanges, and cyclophosphamide; he required 54 transfusions in 10 days to maintain a hemoglobin concentration of 6.0 to 10.0 g per dL. He improved dramatically after a splenectomy was performed. The wide-thermal-amplitude cold agglutinin proved to be an IgG1 kappa antibody with Pra specificity. The patient's serum exhibited normal complement activation. When the DAT was carried out at 0 to 4 degrees C, the result was strongly positive for IgG; the indirect antiglobulin test at 0 to 4 degrees C was positive with the patient's serum diluted 1 in 640. Within 6 months, he was in complete remission and receiving no therapy. As compared with eight patients with CAHA that was exclusively IgG-mediated, this patient is remarkable for his requirement for many transfusions and for DATs that were consistently negative for C3d.Keywords
This publication has 26 references indexed in Scilit:
- Cold AgglutinationTransfusion Medicine Reviews, 1989
- Mitogen-induced human IgG subclass expression. II. IgG1 and IgG3 subclasses are preferentially stimulated by a combination of Staphylococcus aureus Cowan I and pokeweed mitogenHuman Immunology, 1989
- High-Dose Methylprednisolone in Acute Immune Cold HemolysisNew England Journal of Medicine, 1985
- Characterization of red blood cells strongly coated in vitro by C3 via the alternative pathwayTransfusion, 1980
- Biologic Activity of Cold-Reacting AutoantibodiesNew England Journal of Medicine, 1977
- Biologic Activity of Cold-Reacting AutoantibodiesNew England Journal of Medicine, 1977
- Low-Titer Cold-Hemagglutinin DiseaseNew England Journal of Medicine, 1977
- Preparation of Red Cells Coated with C4 and C3 Subcomponents and Production of Anti-C4d and Anti-C3dVox Sanguinis, 1976
- An Enhancing Effect of Albumin on the Determination of Cold HemagglutininsVox Sanguinis, 1971
- An Incomplete Cold‐Reactive γG Antibody with i Specificity in Infectious Mononucleosis1Vox Sanguinis, 1969