Jason Woods MD, Children’s National Health System
with Kathy Brown MD, Children’s National Health System

The Case
3 yo previously healthy male presenting with fatigue, brown urine, and anemia.  The challenge of this case was to determine the etiology while providing timely/safe interventions.

Here’s How You Answered Our Questions

Make sure to get a type and screen (at CNHS a Coombs is included). Most providers felt that a transfusion is warranted, but consider the timing (ED vs floor/PICU) and potential need for IVIG/steroids. Providers felt that Heme should be involved early in this case. Repeat a UA to determine if nitrites truly present before antibiotics.

Denouement
Patient was hemodynamically stable, tired appearing, but non-toxic. Full CBC confirmed Hgb 5.6, platelets of 279. PT/INR and PTT were normal. Urine dip showed 3+ blood but no RBC on micro. Nitrites, leuk esterase and WBC on micro were negative. Coombs, ASO, Anti-DNAse B were negative. C3, C4, and hemoglobin electrophoresis were normal.

Testing for G6PD was positive. Patient was started on slow PRBC transfusion in the ED and admitted to the ICU for continued management. Discharged with diagnosis of G6PD, confirmed on hematology visit (after blood from transfusions had cleared from body). Of note, the newborn screens in Virginia and Maryland do not screen for GDPD, but Washington DC does.

Teaching Points
Dewesh made some great comments about acute hemolytic anemia.

Transfusions (Info from UpToDate):
If the anemia is causing CV compromise (typically when the Hgb <5 g/dL), strong consideration must be given to transfusing the patient (even in AIHA). Although transfusions can lead to additional hemolysis, it must be emphasized that, despite the risks, transfusion therapy should not be withheld from a patient and life-threatening anemia.

Of note, if the autoantibody is reactive with all donor units (ie, the antibody is “pan-reactive”), no units of blood will be deemed “fully compatible” based on the crossmatch, although certain units may be designated “least incompatible” with the patient’s serum. Available adsorption techniques to remove autoantibodies may allow the identification of clinically important alloantibodies that also may be present.  When the actual erythrocyte transfusion is given, acute symptomatic transfusion reactions are not common, even when transfusing “least incompatible” units of blood. On the rare occasion when a transfusion results in more severe hemolysis, with substantial hemoglobinemia and hemoglobinuria, vigorous hydration will help prevent renal dysfunction.

If the autoantibodies fix complement and cause intravascular hemolysis, a prudent procedure is to begin the transfusion at a slow rate, checking both plasma and urine samples periodically for the presence of free hemoglobin. Warming the blood during infusion is useful for patients with cold-reactive autoantibodies.