hemophagocytic lymphohistiocytosis


Summary: A group of related disorders characterized by LYMPHOCYTOSIS; HISTIOCYTOSIS; and hemophagocytosis. The two major forms are familial and reactive.

Top Publications

  1. Thompson P, Allen C, Horton T, Jones J, Vinks A, McClain K. Severe neurologic side effects in patients being treated for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2009;52:621-5 pubmed publisher
    b>Hemophagocytic Lymphohistiocytosis (HLH) is characterized by uncontrolled inflammation that is generally fatal without immune modulating chemotherapy...
  2. Gupta S, Weitzman S. Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy. Expert Rev Clin Immunol. 2010;6:137-54 pubmed
    b>Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory syndrome with prolonged high fever, hepatosplenomegaly and characteristic laboratory findings...
  3. Karapinar B, Yilmaz D, Balkan C, Akin M, Ay Y, Kvakli K. An unusual cause of multiple organ dysfunction syndrome in the pediatric intensive care unit: hemophagocytic lymphohistiocytosis. Pediatr Crit Care Med. 2009;10:285-90 pubmed publisher
    To report our experience in children with primary or secondary hemophagocytic lymphohistiocytosis (HLH) presented with multiple organ dysfunction syndrome (MODS) in pediatric intensive care unit (PICU)...
  4. Janka G. Hemophagocytic lymphohistiocytosis: when the immune system runs amok. Klin Padiatr. 2009;221:278-85 pubmed publisher
    b>Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an exaggerated but ineffective immune response leads to severe hyperinflammation...
  5. Chang Y, Lu P, Lu M, Wang J, Tung C, Shaw C. Sequential transplants for respective relapse of Hodgkin disease and hemophagocytic lymphohistiocytosis: a treatment dilemma. J Pediatr Hematol Oncol. 2009;31:778-81 pubmed publisher
    The association of hemophagocytic lymphohistiocytosis (HLH) with Hodgkin disease (HD) seems to be uncommon. A 9-year-old boy, diagnosed with HD-HLH, received chemotherapy and remained in complete remission for 21 months...
  6. Chen J, Fleming M, Pinkus G, Pinkus J, Nichols K, Mo J, et al. Pathology of the liver in familial hemophagocytic lymphohistiocytosis. Am J Surg Pathol. 2010;34:852-67 pubmed publisher
    Familial hemophagocytic lymphohistiocytosis is a rare, rapidly progressive disorder characterized by an activation of the immune system resulting in a systemic proliferation of lymphocytes and histiocytes...
  7. Beutel K, Gross Wieltsch U, Wiesel T, Stadt U, Janka G, Wagner H. Infection of T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-Asian origin. Pediatr Blood Cancer. 2009;53:184-90 pubmed publisher
    Epstein-Barr virus (EBV) is one of the most frequent triggers of hemophagocytic lymphohistiocytosis (HLH). EBV-associated HLH (EBV-HLH) and ectopic infection of T cells has been particularly described in patients from Far East Asia...
  8. Decaminada N, Cappellini M, Mortilla M, Del Giudice E, Sieni E, Caselli D, et al. Familial hemophagocytic lymphohistiocytosis: clinical and neuroradiological findings and review of the literature. Childs Nerv Syst. 2010;26:121-7 pubmed publisher
    Familial hemophagocytic lymphohistiocytosis (FHL) is a rare multisystem congenital disorder characterized by uncontrolled proliferation and infiltration of activated lymphocytes and histiocytes, secreting high amounts of inflammatory ..
  9. Yoon H, Kim H, Yoo K, Sung K, Koo H, Kang H, et al. UNC13D is the predominant causative gene with recurrent splicing mutations in Korean patients with familial hemophagocytic lymphohistiocytosis. Haematologica. 2010;95:622-6 pubmed publisher
    Familial hemophagocytic lymphohistiocytosis is a fatal disease characterized by immune dysregulation from defective function of cytotoxic lymphocytes...

More Information


  1. Marsh R, Satake N, Biroschak J, Jacobs T, Johnson J, Jordan M, et al. STX11 mutations and clinical phenotypes of familial hemophagocytic lymphohistiocytosis in North America. Pediatr Blood Cancer. 2010;55:134-40 pubmed publisher
    Mutations in STX11 are responsible for Familial Hemophagocytic Lymphohistiocytosis (FHLH) type 4, a rare primary immunodeficiency which has previously been observed only in patients of Kurdish, Turkish, and Lebanese ethnic background...
  2. Marsh R, Madden L, Kitchen B, Mody R, McClimon B, Jordan M, et al. XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease. Blood. 2010;116:1079-82 pubmed publisher
    ..SH2D1A mutation), XIAP deficiency was originally observed to be associated with a high incidence of hemophagocytic lymphohistiocytosis (HLH) and a lack of lymphoma, suggesting that classification of XIAP deficiency as a cause of XLP ..
  3. Aronson I, Worobec S. Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: an overview. Dermatol Ther. 2010;23:389-402 pubmed publisher
    ..The cytophagocytic disorder in these patients is a hemophagocytic lymphohistiocytosis (HLH), similar to the infection-activated reaction associated with perforin mutations found in ..
  4. Lin T, Ferlic Stark L, Allen C, Kozinetz C, McClain K. Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality. Pediatr Blood Cancer. 2011;56:154-5 pubmed publisher
    b>Hemophagocytic lymphohistiocytosis (HLH) is difficult to diagnose and treat. Highly elevated ferritin is strongly associated with HLH and levels may provide a prognostic marker...
  5. Castillo L, Carcillo J. Secondary hemophagocytic lymphohistiocytosis and severe sepsis/ systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation. Pediatr Crit Care Med. 2009;10:387-92 pubmed publisher
    In an effort to attain earlier diagnoses in children with hemophagocytic lymphohistiocytosis (HLH), the International Histiocyte Society has now broadened their diagnostic criteria to no longer differentiate primary (HLH) and secondary ..
  6. Strout M, Seropian S, Berliner N. Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis. Nat Rev Clin Oncol. 2010;7:415-20 pubmed publisher
    ..Idiopathic, atypical hemophagocytic lymphohistiocytosis. Initial treatment with antibiotics was followed by immunosuppressive therapy (including intravenous ..
  7. Brown D, McCoy M, Pilonieta M, Nix R, Detweiler C. Chronic murine typhoid fever is a natural model of secondary hemophagocytic lymphohistiocytosis. PLoS ONE. 2010;5:e9441 pubmed publisher
    b>Hemophagocytic lymphohistiocytosis (HLH) is a hyper-inflammatory clinical syndrome associated with neoplastic disorders especially lymphoma, autoimmune conditions, and infectious agents including bacteria, viruses, protozoa and fungi...
  8. Zur Stadt U, Rohr J, Seifert W, Koch F, Grieve S, Pagel J, et al. Familial hemophagocytic lymphohistiocytosis type 5 (FHL-5) is caused by mutations in Munc18-2 and impaired binding to syntaxin 11. Am J Hum Genet. 2009;85:482-92 pubmed publisher
    ..Disturbance of this highly regulated process underlies familial hemophagocytic lymphohistiocytosis (FHL), a genetically heterogeneous autosomal-recessive disorder characterized by a severe ..
  9. Danielian S, Basile N, Rocco C, Prieto E, Rossi J, Barsotti D, et al. Novel syntaxin 11 gene (STX11) mutation in three Argentinean patients with hemophagocytic lymphohistiocytosis. J Clin Immunol. 2010;30:330-7 pubmed publisher
    b>Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with major diagnostic and therapeutic difficulties, basically comprising two different conditions: primary and secondary forms...
  10. Côte M, Ménager M, Burgess A, Mahlaoui N, Picard C, Schaffner C, et al. Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells. J Clin Invest. 2009;119:3765-73 pubmed publisher
    Familial hemophagocytic lymphohistiocytosis (FHL) is a genetically heterogeneous autosomal recessive immune disorder characterized by the occurrence of uncontrolled activation of lymphocytes and macrophages infiltrating multiple organs...
  11. Fox C, Shannon Lowe C, Gothard P, Kishore B, Neilson J, O CONNOR N, et al. Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in adults characterized by high viral genome load within circulating natural killer cells. Clin Infect Dis. 2010;51:66-9 pubmed publisher
    Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) is a rare and aggressive disease usually encountered in the context of primary EBV infection. In most analyzed cases, EBV has been found predominantly in T cells...
  12. Niller H. Myelodysplastic syndrome (MDS) as a late stage of subclinical hemophagocytic lymphohistiocytosis (HLH): a putative role for Leptospira infection. A hypothesis. Acta Microbiol Immunol Hung. 2010;57:181-9 pubmed publisher
    It is proposed that hemophagocytic lymphohistiocytosis (HLH) and myelodysplastic syndromes (MDS) may be temporally distinct phases of pathophysiologically related disease processes...
  13. Ohga S, Kudo K, Ishii E, Honjo S, Morimoto A, Osugi Y, et al. Hematopoietic stem cell transplantation for familial hemophagocytic lymphohistiocytosis and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan. Pediatr Blood Cancer. 2010;54:299-306 pubmed publisher
    Post-transplant outcomes of hemophagocytic lymphohistiocytosis (HLH) patients were analyzed in Japan where Epstein-Barr virus (EBV)-associated severe forms are problematic...
  14. Meeths M, Entesarian M, Al Herz W, Chiang S, Wood S, Al Ateeqi W, et al. Spectrum of clinical presentations in familial hemophagocytic lymphohistiocytosis type 5 patients with mutations in STXBP2. Blood. 2010;116:2635-43 pubmed publisher
    b>Hemophagocytic lymphohistiocytosis (HLH) is an often-fatal hyperinflammatory syndrome characterized by fever, hepatosplenomegaly, cytopenia, and in some cases hemophagocytosis...
  15. Toga A, Wada T, Sakakibara Y, Mase S, Araki R, Tone Y, et al. Clinical significance of cloned expansion and CD5 down-regulation in Epstein-Barr Virus (EBV)-infected CD8+ T lymphocytes in EBV-associated hemophagocytic lymphohistiocytosis. J Infect Dis. 2010;201:1923-32 pubmed publisher
    Epstein-Barr virus (EBV) is the pathogen that most commonly triggers infection-associated hemophagocytic lymphohistiocytosis (HLH) and ectopically infects CD8(+) T cells in EBV-associated HLH (EBV-HLH)...
  16. Cetica V, Santoro A, Gilmour K, Sieni E, Beutel K, Pende D, et al. STXBP2 mutations in children with familial haemophagocytic lymphohistiocytosis type 5. J Med Genet. 2010;47:595-600 pubmed publisher
    ..Additional polymorphisms are also described. These data expand current knowledge on the genetic heterogeneity of FHL and suggest that patients with FHL5 may have different results in degranulation assays under different conditions. ..
  17. Koh M, Sadarangani S, Chan Y, Chan M, Tan A, Tan S, et al. Aggressive subcutaneous panniculitis-like T-cell lymphoma with hemophagocytosis in two children (subcutaneous panniculitis-like T-cell lymphoma). J Am Acad Dermatol. 2009;61:875-81 pubmed publisher
    ..We present two pediatric patients with aggressive forms of subcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome, and discuss the current literature. ..
  18. Wood S, Meeths M, Chiang S, Bechensteen A, Boelens J, Heilmann C, et al. Different NK cell-activating receptors preferentially recruit Rab27a or Munc13-4 to perforin-containing granules for cytotoxicity. Blood. 2009;114:4117-27 pubmed publisher
    The autosomal recessive immunodeficiencies Griscelli syndrome type 2 (GS2) and familial hemophagocytic lymphohistiocytosis type 3 (FHL3) are associated with loss-of-function mutations in RAB27A (encoding Rab27a) and UNC13D (encoding ..
  19. Avasthi R, Mohanty D, Chaudhary S, Mishra K. Disseminated tuberculosis: interesting hematological observations. J Assoc Physicians India. 2010;58:243-4 pubmed
    ..The report highlights the significance of hematological picture in final confirmation of tuberculosis which may otherwise be passed off as nutritional or other unrelated causes. ..
  20. van der Werff ten Bosch J, Kollen W, Ball L, Brinkman D, Vossen A, Lankester A, et al. Atypical varicella zoster infection associated with hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2009;53:226-8 pubmed publisher
    Two adolescents, on immunosuppressive therapy for graft-versus-host disease, developed hemophagocytic lymphohistiocytosis (HLH) after varicella zoster virus (VZV) reactivation...
  21. Pachlopnik Schmid J, Moshous D, Boddaert N, Neven B, Dal Cortivo L, Tardieu M, et al. Hematopoietic stem cell transplantation in Griscelli syndrome type 2: a single-center report on 10 patients. Blood. 2009;114:211-8 pubmed publisher
    ..only curative treatment for Griscelli syndrome type 2, an inherited immune disorder causing fatal hemophagocytic lymphohistiocytosis (HLH). Optimal therapeutic modalities are not yet well known...
  22. Abdelkefi A, Jamil W, Torjman L, Ladeb S, Ksouri H, Lakhal A, et al. Hemophagocytic syndrome after hematopoietic stem cell transplantation: a prospective observational study. Int J Hematol. 2009;89:368-373 pubmed publisher
    ..The rapid diagnosis of HPS and the early initiation of an appropriate treatment are essential for patient management. ..
  23. Kerzel S, Zemlin M, Kömhoff M, Klaus G, Maier R. Hyperferritinemia as the diagnostic clue in life-threatening hemophagocytic lymphohistiocytosis. Klin Padiatr. 2009;221:318-21 pubmed publisher
    ..elevations of serum ferritin have been almost exclusively reported for hemophagocytic syndromes, as hemophagocytic lymphohistiocytosis (HLH)...
  24. Escoto H, Ringewald J, Kalpatthi R. Etoposide-related cardiotoxicity in a child with haemophagocytic lymphohistiocytosis. Cardiol Young. 2010;20:105-7 pubmed publisher
    ..He recovered well with supportive measures and after discontinuation of etoposide. We discuss the possible mechanisms and differential diagnoses of myocardial infarction in our patient. ..
  25. Vermeulen M, de Haas V, Mulder M, Flohil C, Fetter W, van de Kamp J. Hydrops fetalis and early neonatal multiple organ failure in familial hemophagocytic lymphohistiocytosis. Eur J Med Genet. 2009;52:417-20 pubmed publisher
    Familial hemophagocytic lymphohistiocytosis (FHLH) is a genetic heterogeneous autosomal recessive disorder. We report two siblings with FHLH caused by a PRF1 mutation...
  26. Chung H, Park C, Lim J, Jang S, Chi H, Im H, et al. Establishment of a reference interval for natural killer cell activity through flow cytometry and its clinical application in the diagnosis of hemophagocytic lymphohistiocytosis. Int J Lab Hematol. 2010;32:239-47 pubmed publisher
    Recently, the Histiocyte Society revised the diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH) to include low or absent natural killer (NK) cell activity, according to local laboratory reference...
  27. Shinoda H, Yoshida A, Teruya Feldstein J. Malignant histiocytoses/disseminated histiocytic sarcoma with hemophagocytic syndrome in a patient with mediastinal germ cell tumor. Appl Immunohistochem Mol Morphol. 2009;17:338-44 pubmed publisher
    ..Atypical histiocytes stained positive for CD68, CD163, CD4, CD45 (LCA), and S-100. Cytogenetics studies were negative for i12p. The patient was refractory to therapy and ultimately died 5 months after diagnosis. ..
  28. Mancao M, Imran H, Chandra S, Estrada B, Figarola M, Sosnowski J, et al. Eastern equine encephalitis virus infection and hemophagocytic lymphohistiocytosis in a 5-month-old infant. Pediatr Infect Dis J. 2009;28:543-5 pubmed publisher
    ..b>Hemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening disease associated with the inability of an overactive immune ..
  29. Brennan A, Chia J, Trapani J, Voskoboinik I. Perforin deficiency and susceptibility to cancer. Cell Death Differ. 2010;17:607-15 pubmed publisher
    ..A complete loss of CL function results in an aggressive immunoregulatory disorder, familial hemophagocytic lymphohistiocytosis, whereas a partial loss of function seems to be a factor strongly predisposing to hematological ..
  30. Balta G, Okur H, Unal S, Yarali N, Gunes A, Unal S, et al. Assessment of clinical and laboratory presentations of familial hemophagocytic lymphohistiocytosis patients with homozygous W374X mutation. Leuk Res. 2010;34:1012-7 pubmed publisher
    ..Those with very high ferritin (>20,000ng/ml) had extremely low fibrinogen levels. Two-thirds of patients receiving HLH protocol died within 20 days of therapy. ..
  31. Premaratna R, Williams H, Chandrasena T, Rajapakse R, Kularatna S, de Silva H. Unusual pancytopenia secondary to haemophagocytosis syndrome in rickettsioses. Trans R Soc Trop Med Hyg. 2009;103:961-3 pubmed publisher
    ..Empirical use of anti-rickettsial antibiotics in such situations could be beneficial, when facilities to diagnose rickettsial diseases are not readily available. ..
  32. Martin A, Marques L, Soler Palacin P, Caragol I, Hernandez M, Figueras C, et al. Visceral leishmaniasis associated hemophagocytic syndrome in patients with chronic granulomatous disease. Pediatr Infect Dis J. 2009;28:753-4 pubmed publisher
    ..We report visceral leishmaniasis-associated infection-associated hemophagocytic syndrome in 3 patients with chronic granulomatous disease. ..
  33. Meeths M, Bryceson Y, Rudd E, Zheng C, Wood S, Ramme K, et al. Clinical presentation of Griscelli syndrome type 2 and spectrum of RAB27A mutations. Pediatr Blood Cancer. 2010;54:563-72 pubmed publisher
    ..Since five of six patients studied herein initially were diagnosed as having FHL, we conclude that the diagnosis of GS2 may be overlooked, particularly in fair-haired patients with haemophagocytic syndromes. ..
  34. Wang Z, Wang Y, Feng C, Tian L, Chen X. [The early diagnosis and clinical analysis of 57 cases of acquired hemophagocytic lymphohistiocytosis]. Zhonghua Nei Ke Za Zhi. 2009;48:312-5 pubmed
    ..interleukin-2 receptors (sCD(25)) and glycosylated ferritin in the early diagnostic of acquired hemophagocytic lymphohistiocytosis (HLH)...
  35. Tai C, Chang L, Lin D, Lin K, Jou S, Yang Y. A case of natural killer cell lymphoma presenting with bilateral pleural effusions and hemophagocytic lymphohistocytosis. Pediatr Blood Cancer. 2009;52:666-9 pubmed publisher
    ..Morphologic and immunohistochemical tests revealed an association of NK cell lymphoma with Epstein-Barr virus. A literature review helped identify other cases of patients with NK cell lymphoma, their treatment and outcomes. ..
  36. Darteyre S, Ludwig C, Jeziorski E, Schved J, Rodiere M. [Hemophagocytic lymphohistiocytosis and Epstein-Barr virus infection in children]. Med Mal Infect. 2010;40:18-26 pubmed publisher
    The authors had for aim to review pediatric cases of EBV-associated hemophagocytic lymphohistiocytosis observed, between January 1999 and December 2007, to compare their characteristics to literature data, and to suggest a pragmatic ..
  37. Born A, Muller K, Marquart H, Heilmann C, Schejbel L, Vissing J. Myositis in Griscelli syndrome type 2 treated with hematopoietic cell transplantation. Neuromuscul Disord. 2010;20:136-8 pubmed publisher
    ..Our results suggest that muscle involvement should be considered in patients with hemophagocytic syndrome to ensure proper treatment. ..
  38. Hinze C, Fall N, Thornton S, Mo J, Aronow B, Layh Schmitt G, et al. Immature cell populations and an erythropoiesis gene-expression signature in systemic juvenile idiopathic arthritis: implications for pathogenesis. Arthritis Res Ther. 2010;12:R123 pubmed publisher
    ..The aim of this study was to determine the association of this signature with peripheral blood mononuclear cell (PBMC) subpopulations and its specificity for sJIA as compared with related conditions...
  39. Nodomi S, Kato I, Daifu T, Saida S, Morishima T, Matsubara H, et al. [Early relapse of hemophagocytic syndrome after reduced-intensity cord blood transplantation for relapsed acute lymphoblastic leukemia]. Rinsho Ketsueki. 2010;51:201-6 pubmed
    ..The origin of macrophages was 2(nd) donor derived. After administration of steroids, intravenous immunoglobulin and VP-16, the patient exhibited complete chimerism and remained in complete remission for over one year. ..
  40. RamaChandran S, Ariffin H. Secondary acute myeloid leukemia after etoposide therapy for haemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2009;53:488-90 pubmed publisher
  41. Taki H, Shinoda K, Hounoki H, Ogawa R, Hayashi R, Sugiyama E, et al. Presenting manifestations of hemophagocytic syndrome in a male patient with systemic lupus erythematosus. Rheumatol Int. 2010;30:387-8 pubmed publisher
    ..The patient was treated with high-dose prednisolone, resulting in an excellent outcome. Early diagnosis of HPS by bone marrow biopsy is important for the successful treatment. ..
  42. Botelho C, Ferrer F, Francisco L, Maia P, Mendes T, Carreira A. Acute lupus hemophagocytic syndrome: report of a case. Nefrologia. 2010;30:247-51 pubmed publisher
    ..There are only few cases reported in the literature. However, much is still needed in order to better understand its causes, all the immunopathogenic mechanisms, as well as its clinical and therapeutic aspects. ..
  43. García Astudillo L, Fontalba A, Mazorra F, Marin M, Castellanos A, Fernandez S, et al. Severe course of community-acquired pneumonia in an adult patient who is heterozygous for Q481P in the perforin gene: are carriers of the mutation free of risk?. J Investig Allergol Clin Immunol. 2009;19:311-6 pubmed
    Most cases of autosomal recessive hemophagocytic lymphohistiocytosis (HLH) are associated with over 50 mutations in the perforin gene. Some of these mutations have no clear functional association...
  44. Belyea B, Hinson A, Moran C, Hwang E, Heath J, Barfield R. Spontaneous resolution of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2010;55:754-6 pubmed publisher
    Secondary hemophagocytic lymphohistiocytosis (sHLH) is a reactive, proliferative disorder of the immune system resulting in lymphohistiocytic proliferation, hemophagocytosis, and cytokine dysregulation...
  45. Kamiya K, Kurasawa K, Arai S, Maezawa R, Hanaoka R, Kumano K, et al. Rituximab was effective on refractory thrombotic thrombocytopenic purpura but induced a flare of hemophagocytic syndrome in a patient with systemic lupus erythematosus. Mod Rheumatol. 2010;20:81-5 pubmed publisher
    ..This case showed that TTP with normal ADAMTS-13 activity is B-cell dependent and indicated that B-cell depletion might exacerbate some autoimmune conditions in SLE. ..
  46. Niece J, Rogers Z, Ahmad N, Langevin A, McClain K. Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race. Pediatr Blood Cancer. 2010;54:424-8 pubmed publisher
    Early recognition and aggressive treatment of hemophagocytic lymphohistiocytosis (HLH) has changed a uniformly fatal disease to one 55% survive...
  47. Erduran E, Makuloglu M, Mutlu M. A rare hematological manifestation of brucellosis: reactive hemophagocytic syndrome. J Microbiol Immunol Infect. 2010;43:159-62 pubmed publisher
    ..The Brucella agglutination test was positive. The patient improved after treatment with Rifampin (15 mg/kg/day) and trimethoprim-sulfamethoxazole (10 mg/kg/day). ..
  48. Maakaroun N, Moanna A, Jacob J, Albrecht H. Viral infections associated with haemophagocytic syndrome. Rev Med Virol. 2010;20:93-105 pubmed publisher
    ..HPS triggered by viral infections generally does not respond to specific antiviral therapy but may be treated with immunosuppressive/immunomodulatory agents and, in refractory cases, with bone marrow transplantation. ..
  49. Sovinz P, Schwinger W, Lackner H, Benesch M, Moser A, Raicht A, et al. Severe epstein-barr virus encephalitis with hemophagocytic syndrome: rapid clearance of virus following allogeneic hematopoietic stem cell transplantation from a seropositive donor. Pediatr Infect Dis J. 2010;29:553-6 pubmed publisher
    Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis with encephalitis is a life-threatening condition that requires treatment with immunochemotherapy; refractory patients are eligible for allogeneic hematopoietic stem cell ..
  50. Celkan T, Berrak S, Kazanci E, Ozyurek E, Unal S, Ucar C, et al. Malignancy-associated hemophagocytic lymphohistiocytosis in pediatric cases: a multicenter study from Turkey. Turk J Pediatr. 2009;51:207-13 pubmed
    This study evaluates the clinical and laboratory data of children with secondary hemophagocytic lymphohistiocytosis (sHLH) related to malignancy...
  51. Pande A, Bhattacharyya M, Pain S, Ghosh A, Samanta A. Diagnostic yield of bone marrow examination in HIV associated FUO in ART naïve patients. J Infect Public Health. 2010;3:124-9 pubmed publisher
    ..7%. It was found to be positive in 33.3% of the patients, who had the final diagnosis of an infective etiology and 100% of the patients, who had a final diagnosis of an underlying hematological etiology. ..
  52. Harms P, Schmidt L, Smith L, Newton D, Pletneva M, Walters L, et al. Autopsy findings in eight patients with fatal H1N1 influenza. Am J Clin Pathol. 2010;134:27-35 pubmed publisher
    ..The possibility of hemophagocytic syndrome should be investigated in severely ill patients with H1N1 infection. ..
  53. Suzuki N, Morimoto A, Ohga S, Kudo K, Ishida Y, Ishii E. Characteristics of hemophagocytic lymphohistiocytosis in neonates: a nationwide survey in Japan. J Pediatr. 2009;155:235-8.e1 pubmed publisher
    To assess the etiology, prognosis, and appropriate treatment of hemophagocytic lymphohistiocytosis (HLH) in neonates...
  54. Lee W, Lee D, Kim C, Won C, Chang S, Lee M, et al. Dermatopathic lymphadenitis with generalized erythroderma in a patient with epstein-barr virus-associated hemophagocytic lymphohistiocytosis. Am J Dermatopathol. 2010;32:357-61 pubmed publisher
    Here, we describe a patient with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) who simultaneously presented with generalized erythroderma and dermatopathic lymphadenitis (DL)...
  55. Puliyel M, Rose W, Kumar S, Moses P, Gibikote S. Prolonged neurologic course of familial hemophagocytic lymphohistiocytosis. Pediatr Neurol. 2009;41:207-10 pubmed publisher
    Familial hemophagocytic lymphohistiocytosis is a very unusual cause for demyelination and the clinician would do well to be aware of the condition, especially when children present with atypical findings on magnetic resonance imaging ..
  56. Karras A. What nephrologists need to know about hemophagocytic syndrome. Nat Rev Nephrol. 2009;5:329-36 pubmed publisher
    ..This review summarizes the presentation, causes, pathophysiology and renal features of HPs for the benefit of the practicing nephrologist. ..
  57. Chen Y, Shang S, Zhang C, Liu T, Yang Z, Tang Y. Hemophagocytic lymphohistiocytosis at initiation of kawasaki disease and their differential diagnosis. Pediatr Hematol Oncol. 2010;27:244-9 pubmed publisher
    The authors report a case of hemophagocytic lymphohistiocytosis that became apparent and was confirmed by a specific Th1/Th2 cytokine pattern at the initiation of Kawasaki disease in an 18-month-old child...
  58. Zheng Y, Yang Y, Zhao W, Wang H. Novel swine-origin influenza A (H1N1) virus-associated hemophagocytic syndrome--a first case report. Am J Trop Med Hyg. 2010;82:743-5 pubmed publisher
    ..Three weeks later, the patient was discharged without any complaints, indicating the etiological role of H1N1infection in HPS. ..
  59. Kim B, Jeon Y, Kim C. Kaposi sarcoma herpes virus-associated hemophagocytic syndrome complicated by multicentric castleman disease and kaposi sarcoma in a HIV-negative immunocompetent patient: an autopsy case. J Korean Med Sci. 2009;24:970-4 pubmed publisher
    ..This is the first case of multiple KSHV associated diseases including MCD and KS with KSHV-associated hemophagocytic syndrome in an HIV-negative, non-transplant, immunocompetent patient. ..
  60. Jin Y, Xie Z, Yang S, Lu G, Shen K. Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: a retrospective study of 78 pediatric cases in mainland of China. Chin Med J (Engl). 2010;123:1426-30 pubmed
    The clinical characteristics of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) are largely unreported in the pediatric patients in mainland of China...
  61. My L, Lien L, Hsieh W, Imamura T, Anh T, Anh P, et al. Comprehensive analyses and characterization of haemophagocytic lymphohistiocytosis in Vietnamese children. Br J Haematol. 2010;148:301-10 pubmed publisher
    ..Familial HLH is rare. The frequent reactivation and high mortality demands a more appropriate therapeutic regimen in tropical areas like Vietnam. ..
  62. Chung H, Chi H, Jang S, Park C. Epstein-Barr virus infection associated with bone marrow fibrin-ring granuloma. Am J Clin Pathol. 2010;133:300-4 pubmed publisher
    ..Patients with EBV-associated hemophagocytic lymphohistiocytosis (5/17 patients) and chronic active EBV infection (4/17) constituted 53% of patients with EBV-..
  63. Suresh D, Athanassaki I, Jeha G, Heptulla R. Total parenteral nutrition associated with severe insulin resistance following hematopoietic stem cell transplantation in patients with hemophagocytic syndrome: report on two cases. Pediatr Diabetes. 2010;11:70-3 pubmed publisher
    ..These cases aim to increase awareness and stimulate research to unravel the associated underling mechanisms. ..
  64. Halfon P, Retornaz F, Mathieu D, Helbert T, Philibert P, Pegliasco H. Virus-Associated Hemophagocytic Syndrome related to acute CMV and HBV sexual co-infection: a case report. J Clin Virol. 2009;46:189-91 pubmed publisher
    ..CMV and hepatitis B virus (HBV) can be sexually transmitted. However, co-infection with these viruses has never been reported and the clinical follow-up after acute HBV-CMV infection is not known...
  65. Dierick M, Lacquet F, Verhelst C, Vonck A, Van Garsse L. [Systemic lupus erythematosus over hemophagocytic lymphohistiocytosis]. Acta Clin Belg. 2009;64:150-9 pubmed
    ..In some cases there is need for a specific treatment with corticosteroids, intravenous immunoglobulin, immunosuppressive therapy or etoposide. ..
  66. Hsieh W, Lan B, Chen Y, Chang Y, Chuang H, Su I. Efficacy of peroxisome proliferator activated receptor agonist in the treatment of virus-associated haemophagocytic syndrome in a rabbit model. Antivir Ther. 2010;15:71-81 pubmed publisher
    ..Importantly, rosiglitazone could also inhibit viral replication in vitro and in vivo. PPAR agonists could represent a potentially new agent for the therapy of VAHS. ..
  67. Pachlopnik Schmid J, Ho C, Chretien F, Lefebvre J, Pivert G, Kosco Vilbois M, et al. Neutralization of IFNgamma defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice. EMBO Mol Med. 2009;1:112-24 pubmed publisher
    ..These data strongly suggest that neutralization of IFNgamma could be used in humans to safely alleviate the clinical manifestations of haemophagocytosis. ..
  68. Chia J, Yeo K, Whisstock J, Dunstone M, Trapani J, Voskoboinik I. Temperature sensitivity of human perforin mutants unmasks subtotal loss of cytotoxicity, delayed FHL, and a predisposition to cancer. Proc Natl Acad Sci U S A. 2009;106:9809-14 pubmed publisher
    ..preventing a catastrophic collapse of immune homeostasis, manifested in infancy as Type 2 familial hemophagocytic lymphohistiocytosis (FHL)...
  69. Fardet L, Galicier L, Vignon Pennamen M, Regnier S, Noguera M, De Labarthe A, et al. Frequency, clinical features and prognosis of cutaneous manifestations in adult patients with reactive haemophagocytic syndrome. Br J Dermatol. 2010;162:547-53 pubmed publisher
    ..A generalized, nonpruriginous, transient, maculopapular rash is frequently observed in patients with RHS. Although nonspecific, awareness of this cutaneous involvement may assist physicians in the initial diagnosis of RHS. ..
  70. Ohno T, Ueda Y, Kishimoto W, Arimoto Miyamoto K, Takeoka T, Tsuji M. Epstein-Barr virus-induced infectious mononucleosis after two separate episodes of virus-associated hemophagocytic syndrome. Intern Med. 2009;48:1169-73 pubmed
    ..No reactivation of EBV infection was noted over the following 6 years. The patient may have attained immune competency in adulthood, which was somehow impaired during his adolescence. ..
  71. Ide K, Setoguchi Mukai A, Nakagawa T, Uetsuka K, Nakayama H, Fujino Y, et al. Disseminated histiocytic sarcoma with excessive hemophagocytosis in a cat. J Vet Med Sci. 2009;71:817-20 pubmed
    ..An autopsy revealed the presence of neoplastic histiocytic cells in the bone marrow, liver, pancreatic lymph node and glomeruli. This is the first case of histiocytic sarcoma in a cat to be reported in Japan. ..
  72. Neta M, Wen X, Moore P, Bienzle D. Structure and sequence variation of the canine perforin gene. Vet Immunol Immunopathol. 2010;133:314-20 pubmed publisher
    ..Investigation of more dogs is required to confirm a specific genotype. Future studies should focus on the potential contribution of reduced perforin expression and/or function to HHS in dogs. ..
  73. Okamoto M, Yamaguchi H, Isobe Y, Yokose N, Mizuki T, Tajika K, et al. Analysis of triglyceride value in the diagnosis and treatment response of secondary hemophagocytic syndrome. Intern Med. 2009;48:775-81 pubmed
    Secondary hemophagocytic syndrome (hemophagocytic lymphohistiocytosis, HLH) follows viral infection, malignant disorders, and autoimmune disease. Criteria for HLH diagnosis, which were proposed in 2004, include hypertriglyceridemia...
  74. Yeh H, Liu M, Chang K, Liu S, Chen C. Adult-onset Still's disease complicated with hemophagocytic syndrome. J Formos Med Assoc. 2010;109:85-8 pubmed
    ..During the disease course, extensive studies could not identify any viral infection or other known underlying etiology for the reactive hemophagocytosis. Currently, the patient is in remission on low-dose prednisolone and azathioprine. ..
  75. De Maria A, Zolezzi A, Passalacqua G, Leva M, Tarchino F, Spaggiari P, et al. Melkersson-Rosenthal syndrome associated with parvovirus B19 viraemia and haemophagocytic lymphohistiocytosis. Clin Exp Dermatol. 2009;34:e623-5 pubmed publisher
    ..This observation suggests a possible association between PB19 and HL, and opens the possibility of its association also with acute-onset MRS. Further investigations for the presence of PB19 in cases of MRS are warranted. ..
  76. Uneda S, Murata S, Sonoki T, Matsuoka H, Nakakuma H. Successful treatment with liposomal doxorubicin for widespread Kaposi's sarcoma and human herpesvirus-8 related severe hemophagocytic syndrome in a patient with acquired immunodeficiency syndrome. Int J Hematol. 2009;89:195-200 pubmed publisher
    ..HHV-8 clearance with liposomal doxorubicin has recently been reported. Liposomal doxorubicin suppressed not only the widespread KS tumors, but also HHV-8 viremia resulting in decreased HPS in this patient. ..