CLINICAL VIGNETTE: THE DONATH-LANDSTEINER ANTIBODY AND THE RED BLOOD CELL: A LOVE-HATE RELATIONSHIP.

Abstract

Objective To remind pediatricians of the potential severity and appropriate treatment of paroxysmal cold hemoglobinuria (PCH). Case A 14-month-old boy presented with fever, lethargy, and pallor after 7 days of URI symptoms treated with acetaminophen and ibuprofen. No history of bleeding or bruising. PMH: negative. PE: T 37C, BP 90/61, P167, O2 sat. 89% on RA. Lethargic pale boy with clear lungs, 2/6 systolic murmur, hepatomegaly without splenomegaly, no edema, purpura or ecchymoses. Lab: Hgb 2.8, retic/corrected 14.4/3.7, WBC 44k with normal differential, platelets 344k, BUN/Cr 27/0.5, AST 170, ALT 39, bili 2.7, direct Coombs neg, anti-C3b/C3d 3+ positive, cold-agglutinin neg, IgG neg, Donath-Landsteiner (DL) antibody positive and dark urine with few casts. The patient was treated with blankets, steroids, several 3 cc/kg PRBC transfusions (warmed), and furosemide while in the PICU and did not develop any further signs of cardiac failure or renal dysfunction. The patient was discharged home 5 days later with a Hgb of 7.6 and clear urine on tapering doses of steroids. Discussion This case illustrates one of the rare forms of hemolytic anemia, PCH, which is caused by the DL antibody. The disease occurs 1 to 3 weeks after a viral illness, predominantly in young children. The DL antibody binds to the P antigen on RBCs in temperatures