Korean J Pediatr Hematol Oncol.  1998 Apr;5(1):171-176.

A Case of Paroxysmal Cold Hemoglobinuria Following Epstein-Barr Virus Infection

• Park SM
• Park SH
• Oh SH
• Lyu CJ
• Kim HO
• Yang CH
• Kim KY

Abstract

Paroxysmal cold hemoglobinuria(PCH), a rare type of autoimmune hemolytic process caused by a complement-binding IgG anti-P biphasic hemolysin, is associated with a positive Donath-Landsteiner(D-L) test. Historically, PCH has been associated with syphilis, but is now most often seen following viral infections especially in children. Epstein-Barr virus(EBV), a member of Herpesviridae, can cause various hematologic complications such as neutropenia, thrombocytopenia, aplastic anemia, virus associated hemophagocytic syndrome and hemolytic anemia. We report a case of PCH following EBV infection confirmed by EBV panel test and D-L test. The patient, a 4-year-old male was seen at Yonsei Medical Center in April, 1997, complained high fever, jaundice and hematuria for 2 days. Direct antiglobulin test using anti-C3d monovalent antisera was strong positive, but was negative against anti-IgG monovalent antisera. D-L test showed a characteristic biphasic hemolysin pattern. EBV panel test showed primary infection with a result of EBV EA(early antigen) IgM(+), EBV EA IgG(-), EBV NA(nuclear antigen) IgG(-). He was improved with transfusion of packed red cells and discharged on 13 th hospital day. He was completely recovered and then no relapse occurred during follow up. PCH is thought to be a rare form of autoimmune hemolytic anemia, but recent studies suggest that PCH may account for a large percentage of cases of autoimmune hemolytic anemia as acute transient form, especially in children. Therefore, in unexplained hemolytic anemia, more careful serological examination and attention will result in high detection rate of PCH and cause of PCH.

Keyword

Paroxysmal cold hemoglobinuria(PCH); Donath-Landsteiner(D-L) test; Epstein- Barr virus(EBV)

MeSH Terms

Anemia, Aplastic
Anemia, Hemolytic
Anemia, Hemolytic, Autoimmune
Child
Child, Preschool
Coombs Test
Epstein-Barr Virus Infections
Fever
Follow-Up Studies
Hematuria
Hemoglobinuria, Paroxysmal*
Herpesviridae
Herpesvirus 4, Human*
Humans
Immune Sera
Immunoglobulin G
Jaundice
Lymphohistiocytosis, Hemophagocytic
Male
Neutropenia
Recurrence
Syphilis
Thrombocytopenia
Immune Sera
Immunoglobulin G