Skip to main content

Advertisement

SpringerLink
Log in

Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

Hemophagocytic syndrome (HPS) is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe two cases of untreated systemic lupus erythematosus (SLE) with HPS that presented as one of the manifestations of SLE. The onset of HPS was after parturition for one patient, and after abortion for the other. Bone marrow examination revealed severe hemophagocytosis in both patients. One patient responded to pulsed methylprednisone alone, and the other responded to pulsed methylprednisone plus intravenous immunoglobulin (IVIG). We believe accurate diagnosis, intensive therapy, and sufficient supportive cares are essential in improving patients’ prognosis.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Abbreviations

HPS:

hemophagocytic syndrome

SLE:

systemic lupus erythematosus

ESR:

erythrocyte sedimentation

AST:

aspartate aminotransferase

LDH:

lactate dehydrogenase

ALT:

alanine aminotransferase

γ-GT:

γ-glutamyl transpeptidase

AKP:

alkaline phosphatase

TG:

triglyceride

CH:

cholesterol

ANA:

antinuclear antibody

PSL:

prednisolone

MP:

methylprednisolone

TNF-α:

tumor necrosis factor-α

IVIG:

intravenous immunoglobulin

DIC:

disseminated intravascular coagulation

References

  1. Wong KF, Hui PK, Chan JK, Chan YW, Ha SY (1991) The acute lupus hemophagocytic syndrome. Ann Intern Med 114:387–390

    PubMed  CAS  Google Scholar 

  2. Kumakura S, Ishikura H, Umegae N, Yamagata S, Kobayashi S (1997) Autoimmune-associated hemophagocytic syndrome. Am J Med 102:113–115

    Article  PubMed  CAS  Google Scholar 

  3. Reiner AP, Spivak JL (1988) Hemophagocytic histiocytosis: a report of 23 new patients and review of the literature. Medicine (Baltimore) 67:369–388

    CAS  Google Scholar 

  4. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40:1725

    Article  PubMed  CAS  Google Scholar 

  5. Risdall RJ, McKenna RW, Nesbit ME et al (1979) Virus-associated hemophagocytic syndrome. A benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 44:993–1002

    Article  PubMed  CAS  Google Scholar 

  6. Gill DS, Spencer A, Cobcroft RG (1994) High-dose gamma-globulin therapy in the reactive hemophagocytic syndrome. Br J Haematol 88:204–206

    PubMed  CAS  Google Scholar 

  7. Papo T, Andre MH, Amoura Z et al (1999) The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus. J Rheumatol 26:927–930

    PubMed  CAS  Google Scholar 

  8. Isome M, Suzuki J, Takahashi A et al (2005) Epstein–Barr virus-associated hemophagocytic syndrome in a patient with lupus nephritis. Pediatr Nephrol 20:226–228

    Article  PubMed  Google Scholar 

  9. Kaneko K, Matsuda M, Sekijima Y et al (2005) Acute respiratory distress syndrome due to systemic lupus erythematosus with hemophagocytic syndrome: an autopsy report. Clin Rheumatol 24:158–161

    Article  PubMed  Google Scholar 

  10. Mootsikapun P, Sirijerachai C, Chansung K, Nanagara R (2004) Acute lupus hemophagocytic syndrome: report of a case and review of the literature. J Med Assoc Thai 87:333–339

    PubMed  Google Scholar 

  11. Dhote R, Simon J, Papo T et al (2003) Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Arthritis Rheum 49:633–639

    Article  PubMed  Google Scholar 

  12. Funauchi M, Ohno M, Yamagata T et al (2003) Effects of liposteroid on the hemophagocytic syndrome in systemic lupus erythematosus. Lupus 12:483–485

    Article  PubMed  CAS  Google Scholar 

  13. Yang WK, Fu LS, Lan JL et al (2003) Mycobacterium avium complex-associated hemophagocytic syndrome in systemic lupus erythematosus patient: report of one case. Lupus 12:312–316

    Article  PubMed  CAS  Google Scholar 

  14. Lloyd MS, Bethel KJ, Pischel KD (2002) Clinical image: a hungry histiocyte: lupus-related hemophagocytic syndrome. Arthritis Rheum 46:2537

    Article  PubMed  Google Scholar 

  15. Sakamoto O, Ando M, Yoshimatsu S et al (2002) Systemic lupus erythematosus complicated by cytomegalovirus-induced hemophagocytic syndrome and colitis. Intern Med 41:151–155

    PubMed  Google Scholar 

  16. Takahashi N, Chubachi A, Kume M et al (2001) A clinical analysis of 52 adult patients with hemophagocytic syndrome: the prognostic significance of the underlying diseases. Int J Hematol 74:209–213

    Article  PubMed  CAS  Google Scholar 

  17. Huang YC, Lin YT, Yang YH et al (2001) Acute lupus pneumonitis mimicking pulmonary tuberculosis: a case report. J Microbiol Immunol Infect 34:143–146

    PubMed  CAS  Google Scholar 

  18. Matsumoto Y, Naniwa D, Banno S, Sugiura Y (1998) The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports. Ther Apher 2:300–304

    PubMed  CAS  Google Scholar 

  19. Muiesan P, Dhawan A, Wendon J et al (1998) Hemophagocytosis: a potential complication in small bowel transplantation. Transplantation 66:794–796

    Article  PubMed  CAS  Google Scholar 

  20. Finkielman JD, Grinberg AR, Paz LA et al (1996) Case report: reactive hemophagocytic syndrome associated with disseminated strongyloidiasis. Am J Med Sci 312:37–39

    Article  PubMed  CAS  Google Scholar 

  21. Balduini CL, Noris P, Loni C, Aiosa C (1997) Hemophagocytic syndrome responding to high dose gammaglobulin as presenting feature of sarcoidosis. Am J Hematol 54:88–89

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgements

This work was supported by a research grant from NSFC 30371332 and Shanghai Leading Academic Discipline project, Project number T0203.

Author information

Authors and Affiliations

  1. Cheng-De Yang, Department of Rheumatology, Renji Hospital, Medical School of Shanghai Jiao Tong University, #145 Shan Dong Zhong Road, Shanghai, 200001, People’s Republic of China

    Jie Qian & Cheng-De Yang

Authors
  1. Jie Qian
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Cheng-De Yang
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Cheng-De Yang.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Qian, J., Yang, CD. Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review. Clin Rheumatol 26, 807–810 (2007). https://doi.org/10.1007/s10067-006-0245-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10067-006-0245-y

Keywords

Search

Navigation