1887

Abstract

Haemophagocytic lymphohistiocytosis is a rare but potentially fatal disease resulting from dysregulated activation and proliferation of lymphocytes. We present a case of haemophagocytic syndrome occurring in a 5-year-old Italian boy as a complication of Mediterranean spotted fever. The characteristics of this case have been analysed and contextualized among those of another 15 cases of haemophagocytic syndrome associated with rickettsial diseases found through a systematic review of the international literature.

Loading

Article metrics loading...

/content/journal/jmm/10.1099/jmm.0.025833-0
2011-04-01
2024-04-18
Loading full text...

Full text loading...

/deliver/fulltext/jmm/60/4/537.html?itemId=/content/journal/jmm/10.1099/jmm.0.025833-0&mimeType=html&fmt=ahah

References

  1. Abbott K. C., Vukelja S. J., Smith C. E., McAllister C. K., Konkol K. A., O'Rourke T. J., Holland C. J., Ristic M. 1991; Hemophagocytic syndrome: a cause of pancytopenia in human ehrlichiosis. Am J Hematol 38:230–234 [CrossRef]
    [Google Scholar]
  2. Berner Y., Keysary A., Berrebi A. 1989; Transient histiocytic hemophagocytosis and pancytopenia in Mediterranean spotted fever. Isr J Med Sci 25:660–661
    [Google Scholar]
  3. Burns S., Saylors R., Mian A. 2010; Hemophagocytic lymphohistiocytosis secondary to Ehrlichia chaffeensis infection: a case report. J Pediatr Hematol Oncol 32:e142–e143 [CrossRef]
    [Google Scholar]
  4. Cascio A., Dones P., Romano A., Titone L. 1998; Clinical and laboratory findings of boutonneuse fever in Sicilian children. Eur J Pediatr 157:482–486 [CrossRef]
    [Google Scholar]
  5. Chen Y.-C., Chao T.-Y., Lin J.-C. 2000; Scrub typhus-associated hemophagocytic syndrome. Infection 28:178–179 [CrossRef]
    [Google Scholar]
  6. Dierberg K. L., Dumler J. S. 2006; Lymph node hemophagocytosis in rickettsial diseases: a pathogenetic role for CD8 T lymphocytes in human monocytic ehrlichiosis (HME)?. BMC Infect Dis 6:121 [CrossRef]
    [Google Scholar]
  7. Doran T. I., Parmley R. T., Logas P. C., Chamblin S. 1989; Infection with Ehrlichia canis in a child. J Pediatr 114:809–812 [CrossRef]
    [Google Scholar]
  8. Dumler J. S., Barbet A. F., Bekker C. P., Dasch G. A., Palmer G. H., Ray S. C., Rikihisa Y., Rurangirwa F. R. 2001; Reorganization of genera in the families Rickettsiaceae and Anaplasmataceae in the order Rickettsiales : unification of some species of Ehrlichia with Anaplasma , Cowdria with Ehrlichia and Ehrlichia with Neorickettsia , descriptions of six new species combinations and designation of Ehrlichia equi and ‘HGE agent’ as subjective synonyms of Ehrlichia phagocytophila . Int J Syst Evol Microbiol 51:2145–2165 [CrossRef]
    [Google Scholar]
  9. Dumler J. S., Madigan J. E., Pusterla N., Bakken J. S. 2007; Ehrlichioses in humans: epidemiology, clinical presentation, diagnosis, and treatment. Clin Infect Dis 45:S45–S51 [CrossRef]
    [Google Scholar]
  10. Emminger W., Zlabinger G. J., Fritsch G., Urbanek R. 2001; CD14dim/CD16bright monocytes in hemophagocytic lymphohistiocytosis. Eur J Immunol 31:1716–1719 [CrossRef]
    [Google Scholar]
  11. Feng H. M., Walker D. H. 2000; Mechanisms of intracellular killing of Rickettsia conorii in infected human endothelial cells, hepatocytes, and macrophages. Infect Immun 68:6729–6736 [CrossRef]
    [Google Scholar]
  12. Fournier P. E., Raoult D. 2009; Current knowledge on phylogeny and taxonomy of Rickettsia spp. Ann N Y Acad Sci 11661–11 [CrossRef]
    [Google Scholar]
  13. Freeman H. R., Ramanan A. V. 2011; Review of haemophagocytic lymphohistiocytosis. Arch Dis Child 96 (in press doi: 10.1136/adc.2009.176610
    [Google Scholar]
  14. Gouriet F., Rolain J. M., Raoult D. 2006; Rickettsia slovaca infection, France. Emerg Infect Dis 12:521–523 [CrossRef]
    [Google Scholar]
  15. Gupta S., Weitzman S. 2010; Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy. Expert Rev Clin Immunol 6:137–154 [CrossRef]
    [Google Scholar]
  16. Henter J. I., Horne A., Aricó M., Egeler R. M., Filipovich A. H., Imashuku S., Ladisch S., McClain K., Webb D. other authors 2007; HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48:124–131 [CrossRef]
    [Google Scholar]
  17. Iwasaki H., Hashimoto K., Takada N., Nakayama T., Ueda T., Nakamura T. 1994; Fulminant Rickettsia tsutsugamushi infection associated with haemophagocytic syndrome. Lancet 343:1236 [CrossRef]
    [Google Scholar]
  18. Iwasaki H., Mahara F., Takada N., Fujita H., Ueda T. 2001; Fulminant Japanese spotted fever associated with hypercytokinemia. J Clin Microbiol 39:2341–2343 [CrossRef]
    [Google Scholar]
  19. Jackson M. D., Kirkman C., Bradford W. D., Walker D. H. 1986; Rocky Mountain spotted fever: hepatic lesions in childhood cases. Pediatr Pathol 5:379–388 [CrossRef]
    [Google Scholar]
  20. Keréveur A., McIlroy D., Samri A., Oksenhendler E., Clauvel J. P., Autran B. 1999; Up-regulation of adhesion and MHC molecules on splenic monocyte/macrophages in adult haemophagocytic syndrome. Br J Haematol 104:871–877 [CrossRef]
    [Google Scholar]
  21. Kobayashi T., Takizawa H., Hiroshima K., Uruma T., Enokihara H., Okuyama A. 1992; A case of new type scrub typhus (tsutsugamushi disease) presenting with acute respiratory failure and hemophagocytic syndrome. Nihon Kyobu Shikkan Gakkai Zasshi 30:447–452
    [Google Scholar]
  22. Lee E. H., Rikihisa Y. 1998; Protein kinase A-mediated inhibition of gamma interferon-induced tyrosine phosphorylation of Janus kinases and latent cytoplasmic transcription factors in human monocytes by Ehrlichia chaffeensis . Infect Immun 66:2514–2520
    [Google Scholar]
  23. Marty A. M., Dumler J. S., Imes G., Brusman H. P., Smrkovski L. L., Frisman D. M. 1995; Ehrlichiosis mimicking thrombotic thrombocytopenic purpura. Case report and pathological correlation. Hum Pathol 26:920–925 [CrossRef]
    [Google Scholar]
  24. Miyakawa K., Ohsugi K., Kuriyama C., Sugahara S., Kikuchi A., Ohta M. 2006; [Tsutsugamushi disease with hemophagocytosis complicated by parvovirus B19 infection.]. Nippon Naika Gakkai Zasshi 95:2544–2546 (in Japanese [CrossRef]
    [Google Scholar]
  25. Mouffok N., Parola P., Lepidi H., Raoult D. 2009; Mediterranean spotted fever in Algeria – new trends. Int J Infect Dis 13:227–235 [CrossRef]
    [Google Scholar]
  26. Nagafuji K., Nonami A., Kumano T., Kikushige Y., Yoshimoto G., Takenaka K., Shimoda K., Ohga S., Yasukawa M. other authors 2007; Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis. Haematologica 92:978–981 [CrossRef]
    [Google Scholar]
  27. Ozkan A., Ozkalemkas F., Ali R., Karadogan S., Ozkocaman V., Ozcelik T., Tunali A. 2006; Mediterranean spotted fever: presentation with pancytopenia. Am J Hematol 81:646–647
    [Google Scholar]
  28. Pérez-de Pedro I., Macías-Vega N., Miranda-Candón I., Camps-García M. T. 2008; Severe Rickettsia conorii infection associated with hemophagocytic syndrome. Enferm Infecc Microbiol Clin 26:597–598 [CrossRef]
    [Google Scholar]
  29. Premaratna R., Williams H. S., Chandrasena T. G., Rajapakse R. P., Kularatna S. A., de Silva H. J. 2009; Unusual pancytopenia secondary to haemophagocytosis syndrome in rickettsioses. Trans R Soc Trop Med Hyg 103:961–963 [CrossRef]
    [Google Scholar]
  30. Raoult D. 2009; Rickettsioses, Ehrlichioses, and Anaplasmosis. In Mandell, Douglas and Bennett's Principles and Practice of Infectious Diseases pp 2495–2538 Edited by Mandell G. L., Bennett J. E., Dolin R. Philadelphia, PA: Churchill Livingstone;
    [Google Scholar]
  31. Rouphael N. G., Talati N. J., Vaughan C., Cunningham K., Moreira R., Gould C. 2007; Infections associated with haemophagocytic syndrome. Lancet Infect Dis 7:814–822 [CrossRef]
    [Google Scholar]
  32. Rovery C., Brouqui P., Raoult D. 2008; Questions on Mediterranean spotted fever a century after its discovery. Emerg Infect Dis 14:1360–1367 [CrossRef]
    [Google Scholar]
  33. Sahni S. K., Rydkina E. 2009; Host-cell interactions with pathogenic Rickettsia species. Future Microbiol 4:323–339 [CrossRef]
    [Google Scholar]
  34. Silva-Herzog E., Detweiler C. S. 2008; Intracellular microbes and haemophagocytosis. Cell Microbiol 10:2151–2158 [CrossRef]
    [Google Scholar]
  35. Sotomayor E. A., Popov V. L., Feng H. M., Walker D. H., Olano J. P. 2001; Animal model of fatal human monocytotropic ehrlichiosis. Am J Pathol 158:757–769 [CrossRef]
    [Google Scholar]
  36. Sotto A., Corne P., Bessis D., Lamaury I., Taïb J., Janbon F. 1994; Hemophagocytosis syndrome in Mediterranean boutonneuse fever. Presse Med 23:1582
    [Google Scholar]
  37. Takami A., Yamauchi H., Asakura H., Ishiyama K., Nakao S. 2002; Tsutsugamushi disease (scrub typhus)-associated hemophagocytic syndrome. Int J Hematol 75:337–338 [CrossRef]
    [Google Scholar]
  38. Verbsky J. W., Grossman W. J. 2006; Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives. Ann Med 38:20–31 [CrossRef]
    [Google Scholar]
  39. Wada M., Yamane Y., Tsumori M., Kato Y. 2001; Tsutsugamushi disease-associated hemophagocytic syndrome. Rinsho Kenkyu 78:1173–1176
    [Google Scholar]
  40. Woodard B. H., Farnham R., Bradford W. D. 1981; Fatal Rocky Mountain spotted fever. Hematologic and lymphoreticular observations. Arch Pathol Lab Med 105:452–453
    [Google Scholar]
http://instance.metastore.ingenta.com/content/journal/jmm/10.1099/jmm.0.025833-0
Loading
/content/journal/jmm/10.1099/jmm.0.025833-0
Loading

Data & Media loading...

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error