Resolution of severe Donath-Landsteiner autoimmune hemolytic anemia temporally associated with institution of plasmapheresis.
OBJECTIVE
To report a case of severe postinfectious autoimmune hemolytic anemia (AIHA) owing to the Donath-Landsteiner (DL) antibody resolving with plasmapheresis, and to review the pathophysiology of this underrecognized cause of pediatric AIHA and its potential susceptibility to plasmapheresis therapy.


METHODS
Descriptive case report.


METHODS
A pediatric intensive care unit in a university children's hospital.


METHODS
A 5-yr-old Hispanic female had gastroenteritis followed by progressive intravascular hemolysis, initially attributed to acute postinfectious cold hemagglutinin (immunoglobulin M) disease.


METHODS
With no slowing in the rate of hemolysis, a continued need for frequent transfusions, and a lack of response to corticosteroid and intravenous immunoglobulin therapy, a 3-day course of plasmapheresis was administered.


RESULTS
The patient presented to an emergency department with an initial hematocrit of 22%, which fell to 12% by hospital admission. She received nine transfusions over 7 days, with her hematocrit reaching a nadir of 11% on the 5th day of hospitalization. Once plasmapheresis was initiated, she required no further transfusion. Analysis of serum from initial presentation demonstrated biphasic hemolysis, confirming the presence of the DL antibody.


CONCLUSIONS
In AIHA, in which the direct antiglobulin test detects primarily C3 rather than immunoglobulin G, especially in children, the DL antibody must be considered. Confirming the diagnosis rapidly may be critical, especially in cases of severe hemolysis, because this may help direct therapy. A low titer of DL antibody can mediate severe intravascular hemolysis given its propensity to sensitize, detach, and rebind erythrocytes with changes in temperature in the microcirculation. However, given the transient and relatively brief production of the DL antibody in postviral illness, early clearance of the offending antibody may be possible with plasmapheresis, without the expectation for significant rebound antibody production, potentially decreasing the length of hospital stay and the need for transfusions.
Roy-Burman
Arup
A
Division of Critical Care Medicine, the Department of Pediatrics, University of California, San Francisco, USA. arup@itsa.ucsf.edu
Glader
Bertil E
BE
D000744:Anemia, Hemolytic, Autoimmune / Q000175:diagnosis / Q000628:therapy*; D001323:Autoantibodies / Q000097:blood*; D001803:Blood Transfusion; D002675:Child, Preschool; D005260:Female; D006801:Humans; D010956:Plasmapheresis
D001323:Autoantibodies; C067477:Donath-Landsteiner antibody