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International Journal of Hematology

, Volume 78, Issue 5, pp 450–452 | Cite as

Hemophagocytic Syndrome: An Unusual Manifestation of Acute Human Immunodeficiency Virus Infection

Case Report

Abstract

An 18 year-old heterosexual man was hospitalized because of fever, chills, a sore throat, and a dry cough for 8 days. He had had sexual intercourse with a new partner within the 4 months prior to admission. At admission, the patient presented a clinical picture compatible with hemophagocytic syndrome (HPS). The presence of hemophagocytosis was subsequently determined pathologically from bone marrow and lymph node specimens. An exhaustive diagnostic work-up failed to reveal any causative etiology, the symptoms improving after 2 doses of intravenous immunoglobulin (IVIG) infusion, given at a dose of 0.5 mg/kg each, the 2 doses being administered 1 week apart. Three months subsequent to the patient’s initial presentation, acute human immunodeficiency virus (HIV) infection was diagnosed, and the patient received highly active antiretrovirus therapy (HAART) from the time of diagnosis. The patient remained well for the following 2 years. HPS in the advanced stages of HIV infection has previously been described, but HPS during seroconversion of an acute form of the infection is rare. We most definitely suggest, however, that acute HIV infection be included in the list of potential causes of HPS. IVIG therapy appears to be an appropriate therapeutic modality, and HAART also is effective, for prevention of recurrence of HPS in a patient with acute HIV infection.

Key words

Hemophagocytic syndrome Acute human immunodeficiency virus infection 

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References

  1. 1.
    Tsuda H. Hemophagocytic syndrome (HPS) in children and adults.Int J Hematol. 1997;65:215–226.CrossRefGoogle Scholar
  2. 2.
    Grateau G, Bachmeyer C, Blanche P, et al. Haemophagocytic syndrome in patients infected with the human immunodeficiency virus: nine cases and a review.J Infect. 1997;34:219–225.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Tiab M, Lacroix FM, Hamidou M, Gaillard F, Raffi F. Reactive haemophagocytic syndrome in AIDS.AIDS. 1996;10:108–111.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Risdall RJ, Makenna RW, Nesbit ME, et al. Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis.Cancer. 1979;44:993–1002.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Niedt GW, Schinella RA. Acquired immunodeficiency syndrome: clinicopathologic study of 56 autopsies.Arch Pathol Lab Med. 1985;109:727–734.PubMedPubMedCentralGoogle Scholar
  6. 6.
    Koduri PR, Carandang G, DeMarais P, Patel AR. Hyperferritinemia in reactive hemophagocytic syndrome: report of four adult cases.Am J Hematol. 1995;49:247–249.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Blanche P, Robert F, Dupouy-Camet J, Sicard D. Toxoplasmosis- associated hemophagocytic syndrome in a patient with AIDS: diagnosis by the polymerase chain reaction.Clin Infect Dis. 1994;19: 989–990.CrossRefPubMedGoogle Scholar
  8. 8.
    Bourquelot P, Oksenhendler E, Wolff M, et al. Hemophagocytic syndrome in HIV infection [in French]. Presse Med. 1993;22:1217–1220.PubMedGoogle Scholar
  9. 9.
    Reiner AP, Spivak JL. Hematophagic histiocytosis: a report of 23 new patients and a review of the literature.Medicine (Baltimore). 1988;67:369–388.CrossRefGoogle Scholar
  10. 10.
    Chokephaibulkit K, Veerakul G, Vanprapar N, et al. Penicilliosis- associated hemophagocytic syndrome in a human immunodeficiency virus-infected child: the first case report in children.J Med Assoc Thai. 2001;84:426–429.PubMedGoogle Scholar
  11. 11.
    Albrecht H, Schafer H, Stellbrink HJ, Greten H. Epstein-Barr virus-associated hemophagocytic syndrome: a cause of fever of unknown origin in human immunodeficiency virus infection.Arch Pathol Lab Med. 1997;121:853–858.PubMedGoogle Scholar
  12. 12.
    Chiu SS, Chan GC, Loong F. Epstein-Barr virus (EBV) induced hemophagocytic syndrome followed by EBV associated T/NK lymphoma in a child with perinatal human immunodeficiency virus (HIV) infection.Med Pediatr Oncol. 2001;36:326–328.CrossRefPubMedGoogle Scholar
  13. 13.
    Low P, Neipel F, Rascu A, et al. Suppression of HHV-8 viremia by foscarnet in an HIV-infected patient with Kaposi’s sarcoma and HHV-8 associated hemophagocytic syndrome.Eur J Med Res. 1998;3:461–464.PubMedGoogle Scholar
  14. 14.
    Pellegrin JL, Merlio JP, Lacoste D, et al. Syndrome of macrophagic activation with hemophagocytosis in human immunodeficiency virus infection [in French].Rev Med Interne. 1992;13:438–440.CrossRefPubMedGoogle Scholar
  15. 15.
    Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis.Int J Hematol. 2000;72:1–11.PubMedGoogle Scholar
  16. 16.
    Freeman B, Rathore MH, Salman E, Joyce MJ, Pitel P. Intravenously administered immune globulin for the treatment of infection- associated hemophagocytic syndrome.J Pediatr. 1993;123:479–481.CrossRefPubMedGoogle Scholar
  17. 17.
    Gotoh M, Matsuda J, Gohchi K, Sanaka T, Kawasugi K. Successful recovery from human immunodeficiency virus (HIV)-associated haemophagocytic syndrome treated with highly active anti-retroviral therapy in a patient with HIV infection.Br J Haematol. 2001;112: 1090.CrossRefPubMedGoogle Scholar

Copyright information

© The Japanese Society of Hematology 2003

Authors and Affiliations

  1. 1.Division of Infectious Diseases, Department of Internal MedicineTaipei Veterans General Hospital and National Yang-Ming UniversityTaiwanRepublic of China
  2. 2.Division of Medical Oncology, Department of Internal MedicineTaipei Veterans General Hospital and National Yang-Ming UniversityTaiwanRepublic of China

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