Conservative Management of Alloimmune Hemolysis and Cholestasis With Extreme Laboratory Abnormalities

• Published in: Pediatrics (Evanston) Vol. 147; no. 2; p. e20193367
• Main Authors: Kotch, Chelsea, Friedman, David F, Wilkins, Benjamin J, Samelson-Jones, Benjamin J
• Format: Journal Article
• Language: English
• Published: United States American Academy of Pediatrics 01-02-2021
• Subjects: Alanine; Alanine transaminase; Biomarkers - blood; Blood diseases; Care and treatment; Case reports; Chelation; Cholestasis; Cholestasis - blood; Cholestasis - diagnosis; Cholestasis - etiology; Cholestasis - therapy; Combined Modality Therapy; Conservative Treatment - methods; Diagnosis; Diseases; Erythroblastosis, Fetal - blood; Erythroblastosis, Fetal - diagnosis; Erythroblastosis, Fetal - physiopathology; Erythroblastosis, Fetal - therapy; Female; Ferritin; Fetuses; Gallbladder diseases; Hematologic diseases; Hemolytic disease; Humans; Infant, Newborn; Infants; Infants (Newborn); Iron; Isoimmunization; Jaundice, Obstructive; Laboratories; Male; Neonatal diseases; Neonates; Pediatrics; Risk factors; Severity of Illness Index; United States
• ISSN: 0031-4005; 1098-4275
• DOI: 10.1542/peds.2019-3367

Alloimmune hemolytic disease of the fetus or newborn (HDFN) is a rare cause of neonatal cholestasis. HDFN-associated cholestasis has most often been reported secondary to anti-D alloimmunization. In utero transfusions are also an identified risk factor. A variety of diagnostic and therapeutic strategies have been described, mostly in case reports. Here, we report 2 cases of HDFN-associated cholestasis that were notable for extreme laboratory abnormalities including a peak ferritin of 24 700 ng/mL and a peak alanine aminotransferase of 1406 U/L (33.5-fold upper limit of normal). One case was due to alloimmunization other than anti-D. These cases help define the range of laboratory derangements that are consistent with HDFN-associated cholestasis, including extreme hyperferritinemia. Although in a number of cases, researchers have reported the use of iron chelation in these infants, herein, we describe successful management without iron chelation.