Yonsei Med J.  2008 Apr;49(2):325-328. 10.3349/ymj.2008.49.2.325.

Acute Human Immunodeficiency Virus Syndrome Presenting with Hemophagocytic Lymphohistiocytosis

• Park KH
• Yu HS
• Jung SI
• Shin DH
• Shin JH

• KMID: 1084508
• DOI: http://doi.org/10.3349/ymj.2008.49.2.325

Abstract

Hemophagocytic lymphohistiocytosis (HLH) has been described in patients with advanced stages of human immunodeficiency virus (HIV) infection, but rarely occurs during the seroconversion stage of acute HIV infection. We report a case of acute HIV syndrome that presented with virus-associated HLH. The patient recovered spontaneously without any immunomodulating therapy. This case suggests that acute HIV infection should be included in the differential diagnosis of HLH and indicates that HLH associated with acute HIV infection can have a favorable outcome.

Keyword

Acute human immunodeficiency virus syndrome; hemophagocytic lymphohistiocytosis

MeSH Terms

Acquired Immunodeficiency Syndrome/complications/*diagnosis
Adult
Diagnosis, Differential
HIV Infections/complications/*diagnosis
Humans
Korea
Lymphohistiocytosis, Hemophagocytic/etiology/*pathology
Male

Figure

• 

  Fig. 1 Microscopic finding of the bone marrow aspiration smear from our patient revealing a marked hemophagocytosis by histiocytes. (A) The large arrow indicates the phagocytosis of neutrophils and platelets by benign histiocytes (Wright's stain, × 1,000). (B) Arrowheads indicate hemophagocytes (Wright's stain, × 200).

Reference

1. Tsuda H. Hemophagocytic syndrome (HPS) in children and adults. Int J Hematol. 1997. 65:215–226.

2. Grateau G, Bachmeyer C, Blanche P, Jouanne M, Tulliez M, Galland C, et al. Haemophagocytic syndrome in patients infected with the human immunodeficiency virus: nine cases and a review. J Infect. 1997. 34:219–225.

3. Tiab M, Mechinaud Lacroix F, Hamidou M, Gaillard F, Raffi F. Reactive haemophagocytic syndrome in AIDS. AIDS. 1996. 10:108–111.

4. Pantaleo G, Graziosi C, Fauci AS. New concepts in the immunopathogenesis of human immunodeficiency virus infection. N Engl J Med. 1993. 328:327–335.

5. Henter JI, Tondini C, Pritchard J. Histiocyte disorders. Crit Rev Oncol Hematol. 2004. 50:157–174.

6. Costello C. Haematological abnormalities in human immunodeficiency virus (HIV) disease. J Clin Pathol. 1988. 41:711–715.

7. Niedt GW, Schinella RA. Acquired immunodeficiency syndrome. Clinicopathologic study of 56 autopsies. Arch Pathol Lab Med. 1985. 109:727–734.

8. Sun HY, Chen MY, Fang CT, Hsieh SM, Hung CC, Chang SC. Hemophagocytic lymphohistiocytosis: an unusual initial presentation of acute HIV infection. J Acquir Immune Defic Syndr. 2004. 37:1539–1540.

9. Bhatia S, Bauer F, Bilgrami SA. Candidiasis-associated hemophagocytic lymphohistiocytosis in a patient infected with human immunodeficiency virus. Clin Infect Dis. 2003. 37:e161–e166.

10. Castilletti C, Preziosi R, Bernardini G, Caterini A, Gomes V, Calcaterra S, et al. Hemophagocytic syndrome in a patient with acute human immunodeficiency virus infection. Clin Infect Dis. 2004. 38:1792–1793.

11. Verbsky JW, Grossman WJ. Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives. Ann Med. 2006. 38:20–31.

12. Aricò M, Danesino C, Pende D, Moretta L. Pathogenesis of haemophagocytic lymphohistiocytosis. Br J Haematol. 2001. 114:761–769.

13. Norris PJ, Pappalardo BL, Custer B, Spotts G, Hecht FM, Busch MP. Elevations in IL-10, TNF-alpha, and IFN-gamma from the earliest point of HIV Type 1 infection. AIDS Res Hum Retroviruses. 2006. 22:757–762.

14. Sproat LO, Pantanowitz L, Lu CM, Dezube BJ. Human immunodeficiency virus-associated hemophagocytosis with iron-deficiency anemia and massive splenomegaly. Clin Infect Dis. 2003. 37:e170–e173.

15. Shin DH, Kim US, Park SW, Kim HB, Kim JW, Oh MD, et al. A case of acute retroviral syndrome presenting as a fever of unknown origin. Korean J Infect Dis. 1998. 30:406–409.

16. Jung SJ, Jung ES, Kim JY, Jeong HE, Park DW, Sohn JW, Kim MJ. Three cases of primary human immunodeficiency virus infection and clinical implication of its early recognition [in Korean]. Korean J Med. 2005. 69:S988–S993.

17. Lim YH, Chang SJ, Won YW, Hong SM, Cheon SM, Shin JH, et al. Two cases of acute human immunodeficiency virus syndrome presenting as an acute febrile disease. Korean J Med. 2005. 69:451–456.