Haemophagocytic syndrome and rickettsial diseases.

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.

[1]  A. Ramanan,et al.  Review of haemophagocytic lymphohistiocytosis , 2010, Archives of Disease in Childhood.

[2]  A. Mian,et al.  Hemophagocytic Lymphohistiocytosis Secondary to Ehrlichia chaffeensis Infection: A Case Report , 2010, Journal of pediatric hematology/oncology.

[3]  S. Gupta,et al.  Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy , 2010, Expert review of clinical immunology.

[4]  T. Chandrasena,et al.  Unusual pancytopenia secondary to haemophagocytosis syndrome in rickettsioses. , 2009, Transactions of the Royal Society of Tropical Medicine and Hygiene.

[5]  D. Raoult,et al.  Current Knowledge on Phylogeny and Taxonomy of Rickettsia spp. , 2009, Annals of the New York Academy of Sciences.

[6]  S. Sahni,et al.  Host-cell interactions with pathogenic Rickettsia species. , 2009, Future microbiology.

[7]  D. Raoult,et al.  Mediterranean spotted fever in Algeria--new trends. , 2009, International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases.

[8]  E. Silva-Herzog,et al.  Intracellular microbes and haemophagocytosis , 2008, Cellular microbiology.

[9]  I. Pedro,et al.  Infección grave por Rickettsia conorii asociada a síndrome hemofagocítico , 2008 .

[10]  D. Raoult,et al.  Questions on Mediterranean Spotted Fever a Century after Its Discovery , 2008, Emerging infectious diseases.

[11]  N. Rouphael,et al.  Infections associated with haemophagocytic syndrome , 2007, The Lancet Infectious Diseases.

[12]  J. Bakken,et al.  Ehrlichioses in humans: epidemiology, clinical presentation, diagnosis, and treatment. , 2007, Clinical infectious diseases : an official publication of the Infectious Diseases Society of America.

[13]  K. Takenaka,et al.  Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis. , 2007, Haematologica.

[14]  M. Aricò,et al.  HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis , 2007, Pediatric blood & cancer.

[15]  M. Ohta,et al.  [Tsutsugamushi disease with hemophagocytosis complicated by Parvovirus B19 infection]. , 2006, Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine.

[16]  V. Ozkocaman,et al.  Mediterranean spotted fever: Presentation with pancytopenia , 2006, American journal of hematology.

[17]  J. Dumler,et al.  Lymph node hemophagocytosis in rickettsial diseases: a pathogenetic role for CD8 T lymphocytes in human monocytic ehrlichiosis (HME)? , 2006, BMC infectious diseases.

[18]  D. Raoult,et al.  Rickettsia slovaca Infection, France , 2006, Emerging infectious diseases.

[19]  W. Grossman,et al.  Hemophagocytic lymphohistiocytosis: Diagnosis, pathophysiology, treatment, and future perspectives , 2006, Annals of medicine.

[20]  K. Ishiyama,et al.  Tsutsugamushi Disease (Scrub Typhus)—Associated Hemophagocytic Syndrome , 2002, International journal of hematology.

[21]  S. Ray,et al.  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 , 2001, International journal of systematic and evolutionary microbiology.

[22]  G. Zlabinger,et al.  CD14dim/CD16bright monocytes in hemophagocytic lymphohistiocytosis , 2001, European journal of immunology.

[23]  H. Iwasaki,et al.  Fulminant Japanese Spotted Fever Associated with Hypercytokinemia , 2001, Journal of Clinical Microbiology.

[24]  V. Popov,et al.  Animal model of fatal human monocytotropic ehrlichiosis. , 2001, The American journal of pathology.

[25]  D. Walker,et al.  Mechanisms of Intracellular Killing of Rickettsia conorii in Infected Human Endothelial Cells, Hepatocytes, and Macrophages , 2000, Infection and Immunity.

[26]  T. Chao,et al.  Scrub Typhus-Associated Hemophagocytic Syndrome , 2000, Infection.

[27]  É. Oksenhendler,et al.  Up‐regulation of adhesion and MHC molecules on splenic monocyte/macrophages in adult haemophagocytic syndrome , 1999, British journal of haematology.

[28]  Y. Rikihisa,et al.  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 , 1998, Infection and Immunity.

[29]  A. Cascio,et al.  Clinical and laboratory findings of boutonneuse fever in Sicilian children , 1998, European Journal of Pediatrics.

[30]  J. Dumler,et al.  Ehrlichiosis mimicking thrombotic thrombocytopenic purpura. Case report and pathological correlation. , 1995, Human pathology.

[31]  A. Sotto,et al.  [Hemophagocytosis syndrome in Mediterranean boutonneuse fever]. , 1994, Presse medicale.

[32]  H. Iwasaki,et al.  Fulminant Rickettsia tsutsugamushi infection associated with haemophagocytic syndrome , 1994, The Lancet.

[33]  K. Hiroshima,et al.  [A case of new type scrub typhus (tsutsugamushi disease) presenting with acute respiratory failure and hemophagocytic syndrome]. , 1992, Nihon Kyobu Shikkan Gakkai zasshi.

[34]  K. Abbott,et al.  Hemophagocytic syndrome: A cause of pancytopenia in human ehrlichiosis , 1991, American journal of hematology.

[35]  A. Berrebi,et al.  Transient histiocytic hemophagocytosis and pancytopenia in Mediterranean spotted fever. , 1989, Israel journal of medical sciences.

[36]  R. Parmley,et al.  Infection with Ehrlichia canis in a child. , 1989, The Journal of pediatrics.

[37]  W. Bradford,et al.  Fatal Rocky Mountain spotted fever. Hematologic and lymphoreticular observations. , 1981, Archives of pathology & laboratory medicine.

[38]  R. Fontaine,et al.  Fatal Rocky Mountain spotted fever. , 1978, JAMA.

[39]  M. D. Jackson,et al.  Rocky mountain spotted fever: hepatic lesions in childhood cases. , 1986, Pediatric pathology.