Skip to main content
Log in

Severe low-titer cold-hemagglutinin disease responsive to steroid pulse therapy

  • Case Report
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

We report the case of a 12-year-old boy suffering from severe low-titer cold-hemagglutinin disease with excruciating colicky abdominal pain, jaundice, and acute hemolytic anemia requiring transfusion. Cold hemagglutinins of the IgM type and a positive direct antiglobulin test, predominantly against C3d, were found. Steroid pulse therapy with 20 mg/kg body wt. methylprednisolone for 3 consecutive days was given. Abdominal pain disappeared within 12 h of the first steroid infusion and hemolysis was halted. We conclude that a therapeutic trial with steroid pulse therapy in severe low-titer cold-hemagglutinin disease is warranted.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Atkinson JP, Schreiber AD, Frank MM (1973) Effects of corticosteroids and splenectomy on the immune clearence and destruction of erythrocytes. J Clin Invest 52:1509–1517

    PubMed  Google Scholar 

  2. Cohen J (1993) Cardiovascular collapse after high-dose methylprednisolone. Pediatr Hematol Oncol 10:5–8

    Google Scholar 

  3. Fries L, Brickman C, Frank M (1983) Monocyte receptors for the Fc portion of IgG increase in number in autoimmune hemolytic anemia and other hemolytic states and are decreased by glucocorticoid therapy. J Immunol 131:1240–1245

    PubMed  Google Scholar 

  4. Habibi B, Homber JC, Schaison G (1974) Autoimumune hemolytic anemia in children. Am J Med 56:61–69

    PubMed  Google Scholar 

  5. Lahav M, Rosenberg I, Wysenbeek AJ (1989) Steroid-responsive idiopathic cold agglutinin disease: a case report. Acta Haematol 81:166–168

    PubMed  Google Scholar 

  6. Mendoza SA, Reznik VM, Griswold WR (1990) Treatment of steroid-resistant focal segmental glomerulosclerosis with pulse methylprednisolone and alkylating agents. Pediatr Nephrol 4:303–307

    PubMed  Google Scholar 

  7. Mollison PL, Engelfiet CP, Contreras M (1993) Blood red cell antibodies against self antigens, bound antigens and induced antigens. In: Transfusion in clinical medicine, 9th edn. Black-well Scientific, Oxford, pp 283–320

    Google Scholar 

  8. Nydegger U, Kazatchkine M, Miescher P (1991) Immunopathologic and clinical features of hemolytic anemia due to cold agglutinins. Semin Hematol 28:66–77

    PubMed  Google Scholar 

  9. Roelcke D, Uhlenbruck G (1970) Letter to the editor. Vox Sang 18:478

    PubMed  Google Scholar 

  10. Schreiber AD, Herskovitz B, Goldwein M (1977) Low-titer cold-hemagglutinin disease. N Engl J Med 296:1490–1494

    PubMed  Google Scholar 

  11. Schreiber A, Parsons J, McDermott P (1975) Effect of corticosteroids on the human monocyte IgG and complement receptors. J Clin Invest 56:1189–1197

    PubMed  Google Scholar 

  12. Seldon M, Isbister J, Raik E, Biggs J (1980) A fatal case of cold autoimmune hemolytic anemia. Am J Clin Pathol 73:716–717

    PubMed  Google Scholar 

  13. Shaikow AV, Maximov AA, Speransky AI (1992) Repetitive use of pulse therapy with methylprednisolone and cyclophosphamide in addition to oral methotrexate in children with systemic juvenile rheumatoid arthritis: preliminary results of long-term study. J Rheumatol 19:612–616

    PubMed  Google Scholar 

  14. Zupanska W, Lawkowicz B, Forska J (1976) Autoimmune haemolytic anemia in children. Br J Haematol 34:511–520

    PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Nanan, R., Scheurlen, W., Gerlich, M. et al. Severe low-titer cold-hemagglutinin disease responsive to steroid pulse therapy. Ann Hematol 71, 101–102 (1995). https://doi.org/10.1007/BF01699254

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01699254

Key words

Navigation