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Hemophagocytic Lymphohistiocytosis (Hemophagocytic Syndrome)

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

Also Known As:

Hemophagocytic Syndrome; Lymphohistiocytosis, Hemophagocytic; Primary Hemophagocytic Lymphohistiocytosis; Familial Hemophagocytic Lymphocytoses; Familial Hemophagocytic Lymphohistiocytoses; Familial Hemophagocytic Lymphohistiocytosis; Hemophagocytic Lymphocytoses, Familial; Hemophagocytic Lymphocytosis, Familial; Hemophagocytic Lymphohistiocytoses; Hemophagocytic Lymphohistiocytoses, Familial; Hemophagocytic Lymphohistiocytoses, Primary; Hemophagocytic Lymphohistiocytosis, Primary; Hemophagocytic Syndrome, Infection Associated; Infection-Associated Hemophagocytic Syndrome; Lymphocytoses, Familial Hemophagocytic; Lymphocytosis, Familial Hemophagocytic; Lymphohistiocytoses, Familial Hemophagocytic; Lymphohistiocytoses, Hemophagocytic; Lymphohistiocytoses, Primary Hemophagocytic; Lymphohistiocytosis, Familial Hemophagocytic; Lymphohistiocytosis, Primary Hemophagocytic; Primary Hemophagocytic Lymphohistiocytoses; Familial Hemophagocytic Lymphocytosis; Hemophagocytic Lymphohistiocytosis, Familial; Hemophagocytic Syndrome, Infection-Associated; Hemophagocytic Syndrome, Reactive; Hemophagocytic Syndromes; Reactive Hemophagocytic Syndrome

Networked: 866 relevant articles (39 outcomes, 43 trials/studies)

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases
- Lymphatic Diseases: 2658
  - Histiocytosis: 321
    - Non-Langerhans-Cell Histiocytosis: 35
      - Hemophagocytic Lymphohistiocytosis: 866
        Familial erythrophagocytic lymphohistiocytosis: 23
        3 familial Hemophagocytic lymphohistiocytosis: 1
        2 familial Hemophagocytic lymphohistiocytosis
        4 familial Hemophagocytic lymphohistiocytosis

Related Diseases

1.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
2.	Juvenile Rheumatoid Arthritis (Juvenile Idiopathic Arthritis)
3.	Arthritis (Polyarthritis)
4.	Adult-Onset Still's Disease
5.	Thrombocytopenia (Thrombopenia)

Experts

1.	Imashuku, Shinsaku: 15 articles (09/2013 - 02/2002)
2.	Jordan, Michael B: 13 articles (07/2015 - 08/2004)
3.	Filipovich, Alexandra H: 13 articles (12/2014 - 01/2002)
4.	Ishii, Eiichi: 13 articles (08/2013 - 06/2002)
5.	Henter, Jan-Inge: 12 articles (12/2015 - 01/2002)
6.	Ohga, Shouichi: 11 articles (10/2015 - 09/2004)
7.	Janka, Gritta: 11 articles (06/2015 - 01/2002)
8.	Aricò, Maurizio: 11 articles (05/2015 - 10/2002)
9.	Wang, Zhao: 9 articles (11/2015 - 12/2008)
10.	de Saint Basile, Geneviève: 9 articles (05/2010 - 07/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hemophagocytic Lymphohistiocytosis:

1.	Cyclosporine (Ciclosporin)FDA LinkGeneric 08/01/2015 - "Several aspects of this report of primary hemophagocytic lymphohistiocytosis are remarkable and include the association with post-partum, the severe radiologic and histologic macrovesicular steatosis, and the dramatic efficacy of cyclosporine." 01/01/2013 - "Although treatment with high-dose glucocorticoid (GC) and cyclosporine (CsA) was started, her condition did not improve because of complication with severe hemophagocytic syndrome (HPS). " 03/01/2005 - "Key words; Cyclosporin neurotoxicity; Epstein-Barr virus associated-Hemophagocytic syndrome; Magnetic Resonance Image (MRI)." 03/01/2005 - "Cyclosporin neurotoxicity with Epstein-Barr virus-associated hemophagocytic syndrome." 02/01/2004 - "Both patients were treated with oral cyclosporine in an effort to suppress the cytokine release from T-cells that has been thought to induce the hemophagocytic syndrome. "
2.	Etoposide (VP 16)FDA LinkGeneric 10/01/2002 - "This is the first case in the literature which suggests the efficacy of etoposide against refractory autoimmune-associated hemophagocytic syndrome." 02/01/2014 - "Etoposide-containing conditioning regimen reduces the occurrence of hemophagocytic lymphohistiocytosis after SCT." 01/01/2014 - "Etoposide selectively ablates activated T cells to control the immunoregulatory disorder hemophagocytic lymphohistiocytosis." 03/01/2003 - "[Successful use of etoposide in an elderly patient with chronic recurrent hemophagocytic syndrome]." 02/01/2002 - "We studied the impact of etoposide on the prognosis of 81 patients (77 of whom were children <15 years old) with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH). "
3.	rituximab (Mabthera)FDA Link 09/01/2013 - "Are regimens containing rituximab effective in the initial treatment of Epstein-Barr virus-positive natural killer cell lymphoproliferative disease-associated hemophagocytic lymphohistiocytosis?" 08/01/2007 - "Use of rituximab in conjunction with immunosuppressive chemotherapy as a novel therapy for Epstein Barr virus-associated hemophagocytic lymphohistiocytosis." 02/01/2007 - "Rituximab alone was effective for the treatment of a diffuse large B-cell lymphoma associated with hemophagocytic syndrome." 05/01/2015 - "Two patients who received 375 mg/m(2) rituximab died of Pneumocystis carinii pneumonia and hemophagocytic syndrome. " 08/01/2007 - "On the basis of the observations that the anti-CD-20 monoclonal antibody rituximab reduces disease burden in individuals with EBV-associated lymphoproliferative disorders, we treated a patient with severe EBV-hemophagocytic lymphohistiocytosis using a combination of rituximab and chemotherapy. "
4.	Dexamethasone (Maxidex)FDA LinkGeneric 12/01/2014 - "To study the profile of children with Hemophagocytic lymphohistiocytosis (HLH) and compare the outcome of treatment with intravenous immunoglobulin therapy and Dexamethasone vs. HLH-2004 protocol. " 04/01/2012 - "Dexamethasone palmitate successfully attenuates hemophagocytic syndrome after allogeneic stem cell transplantation: macrophage-targeted steroid therapy." 07/01/2008 - "Hemophagocytic lymphohistiocytosis in a pancreas-kidney transplant recipient: response to dexamethasone and cyclosporine." 07/01/2010 - "Here we report a case of reactive hemophagocytic lymphohistiocytosis [macrophage activation syndrome] complicating systemic onset juvenile idiopathic arthritis, which was treated successfully with dexamethasone and cyclosporine. " 05/01/2004 - "The patient improved after treatment, according to the hemophagocytic lymphohistiocytosis protocol (HLH-94) of the Histiocyte Society, with dexamethasone, etoposide, and cyclosporine and unrelated cord blood stem cell transplantation. "
5.	DNA (Deoxyribonucleic Acid)IBA 05/01/2008 - "In the present study, DNA sequencing of the genes SRGN, ARF6, AP3B1, and SH2D1A was performed in a well defined cohort of 18 families with familial hemophagocytic lymphohistiocytosis (FHL). " 07/01/2000 - "In this study we have analyzed LMP1 DNA isolated from 10 Japanese patients with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH). " 06/01/2014 - "We performed DNA sequencing of UNC13D and targeted analysis of these three mutations in 1,709 North American patients with a suspected clinical diagnosis of hemophagocytic lymphohistiocytosis (HLH). " 03/01/2005 - "Epstein-Barr virus (EBV) DNA was positive in her peripheral blood, and we diagnosed the case as EBV-associated hemophagocytic syndrome (EB-VAHS) after excluding other malignancies. " 12/01/2004 - "The patient had hemophagocytic syndrome with excessive circulating levels of EBV DNA that was refractory to immunochemotherapy. "
6.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 02/01/2010 - "We report the case of a patient with systemic lupus erythematosus (SLE) who first revealed hemophagocytic syndrome (HPS), which was treated successfully with glucocorticoid and intravenous cyclophosphamide. " 02/01/2014 - "Refractory hemophagocytic syndrome in systemic lupus erythematosus successfully treated with intermittent intravenous cyclophosphamide: three case reports and literature review." 01/01/1999 - "Anaphylactoid reaction to cyclophosphamide in a patient with T cell lymphoma and hemophagocytic syndrome." 10/01/2004 - "[A case of mixed connective tissue disease successfully treated for hemophagocytic syndrome with intermittent intravenous injection of cyclophosphamide]." 11/01/2008 - "Autopsies were performed in 9 patients and disclosed disseminated herpetic infection in 6 patients (caused by herpes simplex in 4 cases, varicella in 1, and CMV in 1) and hemophagocytic syndrome in 3. A higher frequency of renal failure (54% vs. 19%, p = 0.024), antiphospholipid syndrome (33% vs. 6%, p = 0.023), treatment with cyclophosphamide (82% vs. 37%, p = 0.008), and multisystemic involvement at presentation (58% vs. 8%, p < 0.001); and a lower frequency of antiviral therapy (18% vs. 76%, p < 0.001) were found in patients who died, compared with survivors. "
7.	AntibodiesIBA 07/01/2013 - "We encountered a neonatal patient with hemophagocytic lymphohistiocytosis (HLH) whose mother was positive for anti-Ro/SSA and anti-La/SSB antibodies. " 07/01/2013 - "Hemophagocytic lymphohistiocytosis in a newborn infant born to a mother with Sjögren syndrome antibodies." 05/01/2007 - "Emergence of anti-red blood cell antibodies triggers red cell phagocytosis by activated macrophages in a rabbit model of Epstein-Barr virus-associated hemophagocytic syndrome." 04/01/2015 - "Recipient derived, donor directed, antigranulocyte antibodies led to life-threatening and prolonged neutropenia and residual recipient lymphocytes reestablished hemophagocytic lymphohistiocytosis after withdrawal of immune suppression despite high levels of whole blood chimerism. " 02/15/2004 - "The patient had high titers of antibodies to EBV, EBV RNA in lymph nodes, T-cell lymphoproliferative disease, and hemophagocytic lymphohistiocytosis. "
8.	InterferonsIBA 09/01/2014 - "To establish a hemophagocytic lymphohistiocytosis (HLH)-like mouse model induced by CpG oligodeoxynucleotide (CpG-ODN1826) and interferon (IFN)-γ for further study on therapy. " 01/01/2014 - "[Secondary hemophagocytic syndrome due to Peg-interferon administration]." 01/01/2013 - "Hemophagocytic lymphohistiocytosis induced by direct stimulation of macrophages by interferon (IFN) was suspected. " 10/07/2010 - "The presence of CD8(+) T cells and interferon-γ (IFN-γ) production is required to induce disease in mouse models of hemophagocytic lymphohistiocytosis. " 06/01/2004 - "Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy."
9.	Proteins (Proteins, Gene)IBA 01/01/2014 - "Understanding the molecular mechanisms during this process and the role of individual proteins was greatly improved by the study of familial hemophagocytic lymphohistiocytosis (FHL). " 01/01/2014 - "In this review, we describe how detailed characterization of patients with genetic hemophagocytic lymphohistiocytosis has resulted in improvement in knowledge regarding contribution of individual proteins to the functional machinery of cytotoxic T- and NK-cells. " 01/01/2013 - "Patients with hemophagocytic lymphohistiocytosis (HLH) exhibit immune hyper-activation as a consequence of genetic defects in secretory granule proteins of cytotoxic T lymphocytes (CTL) and natural killer (NK) cells. " 01/01/2012 - "In some inherited human disorders, a defect in selected proteins involved in the cellular cytotoxicity mechanism results in specific clinical syndromes, grouped under the name of familial hemophagocytic lymphohistiocytosis. " 12/01/2014 - "Macrophage activation syndrome (MAS), a life-threatening complication of systemic juvenile idiopathic arthritis (JIA), resembles familial hemophagocytic lymphohistiocytosis (HLH), a constellation of autosomal-recessive immune disorders resulting from deficiency in cytolytic pathway proteins. "
10.	gamma-Globulins (gamma-Globulin)IBA 11/01/2000 - "High-dose gamma-globulins appear to be more effective in infection-associated hemophagocytic syndrome, with a mean dose of 1.6gm/kg for one or two cycles. " 11/01/2000 - "A multicentric randomized study is required to evaluate high-dose gamma-globulin in the treatment of reactive hemophagocytic syndrome." 11/01/2000 - "We report 17 patients, of which 9 had infection-associated hemophagocytic syndrome, all treated with high-dose gamma-globulin. " 11/01/2000 - "Treatment should be directed to the etiology, but successful treatment with high-dose gamma-globulin has been reported, especially in viral-associated hemophagocytic syndrome. " 05/10/1996 - "[Aged patient with virus-associated hemophagocytic syndrome successfully treated with large dose gamma-globulin therapy]."

Therapies and Procedures

1.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 03/01/2011 - "One patient remains in durable remission, the second patient subsequently developed severe hemophagocytic lymphohistiocytosis with multi-organ failure and died, and the third patient underwent a successful allogeneic bone marrow transplantation. " 01/01/2011 - "Unrelated bone marrow transplantation induced long-term remission in a patient with life-threatening Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis." 08/01/1999 - "A case of hemophagocytic lymphohistiocytosis with prolonged remission after syngeneic bone marrow transplantation." 07/01/1998 - "Familial hemophagocytic lymphohistiocytosis: improved neurodevelopmental outcome after bone marrow transplantation." 10/01/2002 - "Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation."
2.	Drug Therapy (Chemotherapy) 12/01/2010 - "This study was aimed to investigate the therapeutic efficacy of HLH-2004 chemotherapy in patients with secondary hemophagocytic lymphohistiocytosis (sHLH). " 02/01/2010 - "Because chemotherapy for hemophagocytic syndrome was transiently effective for underlying LCH as well, the diagnosis of LCH was delayed until its recurrence. " 05/01/2001 - "In the SCTCL group 4 developed hemophagocytic syndrome, 4 died within 2 years of diagnosis, and 3 went into remission following chemotherapy. " 09/01/2015 - "[Acquired hemophagocytic syndrome treated with HLH 94-04 chemotherapy protocol: Report of four cases]." 11/01/2013 - "This report describes a 6-year-old girl with symptoms misdiagnosed as hemophagocytic lymphohistiocytosis and treated with chemotherapy before the recognition that her symptoms and laboratory values were consistent with a somatic FAS mutation leading to ALPS. "
3.	Transplantation (Transplant Recipients) 11/01/1996 - "One 3-year-old girl with hemophagocytic lymphohistiocytosis is in remission at +304 days post-transplantation. " 05/01/2015 - "A five-month-old male infant with familial hemophagocytic lymphohistiocytosis underwent cord blood transplantation using reduced-intensity conditioning. " 01/01/2015 - "Virus-associated hemophagocytic syndrome (HPS) is a potentially fatal complication of immunosuppression for transplantation. " 01/01/2015 - "Virus-associated hemophagocytic syndrome in renal transplant recipients: report of 2 cases from a single center." 05/01/2014 - "BK virus-associated hemophagocytic syndrome in a renal transplant recipient."
4.	Splenectomy 03/01/2012 - "Zhang et al. have reported a case of an adult man with hemophagocytic lymphohistiocytosis (HLH) with an unknown etiology who has achieved a remission of HLH following comprehensive treatment based on splenectomy. " 05/01/2015 - "Our aim was to evaluate the clinical value of splenectomy as a treatment for relapsed hemophagocytic lymphohistiocytosis (HLH) of unknown cause in adults. " 05/01/2015 - "Splenectomy as a treatment for adults with relapsed hemophagocytic lymphohistiocytosis of unknown cause." 03/01/2012 - "Splenectomy as a therapeutic approach in refractory hemophagocytic lymphohistiocytosis." 09/01/2011 - "Splenectomy for an adult patient with refractory secondary hemophagocytic lymphohistiocytosis."
5.	Aftercare (After-Treatment) 01/01/2009 - "Severe hemophagocytic syndrome developing after treatment initiation for disseminated Mycobacterium tuberculosis: case report and literature review." 05/01/2001 - "After treatment with steroid and antibiotics under a diagnosis of bacteria-associated hemophagocytic syndrome, the patient recovered rapidly. " 02/01/2007 - "Severe autoimmune hemolytic anemia after unrelated umbilical cord blood transplant for familial hemophagocytic lymphohistiocytosis: significant improvement after treatment with rituximab." 05/01/2004 - "The patient improved after treatment, according to the hemophagocytic lymphohistiocytosis protocol (HLH-94) of the Histiocyte Society, with dexamethasone, etoposide, and cyclosporine and unrelated cord blood stem cell transplantation. " 01/01/2012 - "A case of hemophagocytic lymphohistiocytosis in a patient with chronic lymphocytic leukemia after treatment with fludarabine, cyclophosphamide, and rituximab chemotherapy, with autopsy findings."