PMC9054456_01
Urine sample on admission demonstrating large blood but only minimal red blood cells (25-50) per high-power field with dark brown color. This is consistent with hemoglobinuria, one of the classic presentations of childhood PCH.
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Urinalysis showed positive nitrites and leukocyte esterase but no bacteria as well as large blood with only 25-50 red blood cells (RBCs) per high-power field, and urine was dark brown in color (Figure 1).
Daily minimum hemoglobin throughout hospitalization. Presenting Hgb was 10.9 g/dL, which dropped to 8.2 g/dL prior to the conclusion of hospital day 1. On hospital day 6, multiple attempts were made to obtain a hemoglobin value, but each sample was rendered invalid due to hemolysis. A spun hematocrit was measured at 13.8%.
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By hospital day 2, hemoglobin dropped to 6.7 g/dL and by hospital day 4 to a nadir of 5.3 g/dL (Figure 2).
Peak creatinine trend in mg/dL throughout hospitalization. CRRT was initiated on hospital day 4, prior to the creatinine plotted for that day.
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The patient developed acute kidney injury (AKI) with worsening oliguria, a BUN of 60 mg/dL, and creatinine of 1.58 mg/dL (Figure 3). Her renal function significantly improved from a creatinine peak that exceeded 5 mg/dL to a level of 2 mg/dL at discharge, with no ongoing requirements for dialysis (Figure 3).
Lactate dehydrogenase (LD) trend in IU/L throughout hospitalization. The plasma exchange was initiated on hospital day 9, and eculizumab was given on hospital day 15.
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The hemolytic process significantly improved, as demonstrated by the rapid decline in lactate dehydrogenase levels (Figure 4), following initiation of plasmapheresis. She was transferred to the pediatric floor with improving urine output and significant reduction in hemolysis (last LD 545 IU/L) with no further PRBC transfusion requirements (Figure 4).