[A boy with summer onset paroxysmal cold hemoglobinuria induced by Donath-Landsteiner antibody with anti-I specificity].

Item request has been placed! ×
Item request cannot be made. ×
loading   Processing Request
Read More Add to Saved list
  • Additional Information
    • Source:
      Publisher: Japan Society Of Clinical Hematology Country of Publication: Japan NLM ID: 2984782R Publication Model: Print Cited Medium: Print ISSN: 0485-1439 (Print) Linking ISSN: 04851439 NLM ISO Abbreviation: Rinsho Ketsueki Subsets: MEDLINE
    • Publication Information:
      Publication: Tokyo : Japan Society Of Clinical Hematology
      Original Publication: Tokyo, Rinsho Ketsueki Kondankai.
    • Subject Terms:
      Antibody Specificity*; Autoantibodies/*blood ; Hemoglobinuria, Paroxysmal/*etiology; Air Conditioning/adverse effects ; Body Temperature Regulation/physiology ; Child, Preschool ; Erythrocytes/immunology ; Hemoglobinuria, Paroxysmal/diagnosis ; Hemoglobinuria, Paroxysmal/drug therapy ; Humans ; Immunoglobulin M/blood ; Male ; Prednisolone/therapeutic use ; Seasons ; Treatment Outcome
    • Abstract:
      We encountered 4-year-old boy who developed paroxysmal cold hemoglobinuria (PCH) in the middle of August. He was admitted due to progressive jaundice and pallor following fever and nausea. Laboratory data revealed severe anemia, increased serum indirect bilirubin and LDH, and decreased serum haptoglobin. Direct/indirect Coombs tests were positive. These findings yielded a diagnosis of autoimmune hemolytic anemia. Serological test for syphilis was negative. The patient's symptoms and signs promptly subsided after treatment with prednisolone, which was started on the 2nd hospital day. The patient has been in a disease-free state for 16 months without any medication. After discharge, he was finally given a diagnosis of PCH because a Donath-Landsteiner test was positive. The antibody belonged to an IgM subclass and showed anti-I specificity. Considering that development of PCH is common in winter, this case was unique because it developed in August. We speculated that exposure to a cool air-conditioned atmosphere prior to hospitalization and the cooling mechanism of the body after admission were involved in the summer onset of PCH and its prolonged clinical course.
    • Accession Number:
      0 (Autoantibodies)
      0 (Immunoglobulin M)
      9PHQ9Y1OLM (Prednisolone)
    • Publication Date:
      Date Created: 20000301 Date Completed: 20000405 Latest Revision: 20131121
    • Publication Date:
      20240829
    • Accession Number:
      10695397