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 < 37°C, but after dissociating from the RBC, it activates the complement pathway, resulting in intravenous hemolysis. This patient's profound anemia contributed to his lethargy, tachycardia, and impending high-output failure and required careful fluid and blood product management to prevent acute renal failure, which can occur in the setting of hemoglobinuria superimposed on a prerenal state. High-dose steroids are often used in severe cases, whereas milder cases resolve spontaneously. Transfusions are given to patients with Hgb < 5 or signs of heart failure. The patient should be kept warm and IV fluids should be warmed as well. Rarely, chronic PCH can develop if low levels of the DL antibody continue to be produced even years after resolution of the acute episode.
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