Sara D Prince1, Lena E Winestone2, Sandra J Nance3, David F Friedman2. 1. Nemours Alfred I. DuPont Hospital for Children, Wilmington, Delaware. 2. Division of Pediatric Hematology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania. 3. Immunohematology Reference Laboratories, American Red Cross, Philadelphia, Pennsylvania.
Abstract
BACKGROUND: Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune hemolytic anemia caused by the Donath-Landsteiner antibody (D-L antibody). In children, this is typically a transient immune-mediated hemolysis that follows a viral illness and does not recur. Recurrent acute or chronic PCH due to D-L antibody is very rare. CASE REPORT: We have reported a unique case of recurrent PCH in a 5-year-old boy with two acute episodes of hemolysis separated by 21 months of hematologic remission. Each episode was severe requiring red blood cell transfusions, intravenous methylprednisolone, and intravenous immunoglobulin during the second episode. Testing identified recurrence of the D-L antibody with the classic anti-P biphasic hemolysis. CONCLUSION: This demonstrates that PCH can be a recurrent disease in the pediatric population (in the absence of syphilis) with the classical D-L antibody.
BACKGROUND:Paroxysmal cold hemoglobinuria (PCH) is a form of autoimmune hemolytic anemia caused by the Donath-Landsteiner antibody (D-L antibody). In children, this is typically a transient immune-mediated hemolysis that follows a viral illness and does not recur. Recurrent acute or chronic PCH due to D-L antibody is very rare. CASE REPORT: We have reported a unique case of recurrent PCH in a 5-year-old boy with two acute episodes of hemolysis separated by 21 months of hematologic remission. Each episode was severe requiring red blood cell transfusions, intravenous methylprednisolone, and intravenous immunoglobulin during the second episode. Testing identified recurrence of the D-L antibody with the classic anti-P biphasic hemolysis. CONCLUSION: This demonstrates that PCH can be a recurrent disease in the pediatric population (in the absence of syphilis) with the classical D-L antibody.
Authors: Jennifer Delun Williams; Ram K Jayaprakash; Heena Kithany; Mark Peter Tighe Journal: Arch Dis Child Educ Pract Ed Date: 2021-08-25 Impact factor: 1.167