3,539 results match your criteria Lymphohistiocytosis


Neurological Abnormality Could be the First and Only Symptom of Familial Hemophagocytic Lymphohistiocytosis: Report of Two Families.

Chin Med J (Engl) 2018 Dec;131(24):3004-3006

Beijing Key Laboratory of Pediatric Hematology Oncology; National Key Discipline of Pediatrics (Capital Medical University); Key Laboratory of Major Diseases in Children, Ministry of Education; Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing 100045, China.

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December 2018
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Hemophagocytic Lymphohistiocytosis during the Postpartum Stage of Pregnancy: A Report of Eight Cases.

Acta Haematol 2018 Dec 7;141(1):55-60. Epub 2018 Dec 7.

Haemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome that can be classified as either primary or secondary. Secondary HLH can be triggered by a variety of diseases. Pregnancy-related HLH has already been observed clinically. Read More

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December 2018

Outcomes in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan.

Int J Hematol 2018 Dec 7. Epub 2018 Dec 7.

Department of Pediatrics, Ehime University Graduate School of Medicine, Toon, Japan.

Recent advances in intensive chemo- and immunotherapy have contributed to the outcome of hemophagocytic lymphohistiocytosis (HLH); however, the prognosis of HLH in children differs by HLH subtype. In Japan, secondary HLH, particularly Epstein-Barr virus-associated HLH (EBV-HLH), is the most common HLH subtype. The prognosis of HLH has improved in recent years. Read More

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December 2018

Macrophage activation syndrome in a patient with axial spondyloarthritis on adalimumab.

Clin Rheumatol 2018 Dec 7. Epub 2018 Dec 7.

Division of Rheumatology, Department of Medicine, University of Washington School of Medicine, Seattle, WA, USA.

Macrophage activation syndrome (MAS) is a rare and potentially fatal condition characterized by excessive activation and uncontrolled proliferation of T lymphocytes and macrophages, leading to overwhelming systemic inflammation and cytokine release. MAS has been reported with viral infections, autoimmune disorders, malignancies, and medications. We describe a case of a patient with axial spondyloarthritis (axSpA) treated with adalimumab, who presented with MAS. Read More

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December 2018

Severe Fever with Thrombocytopenia Syndrome Patients with Hemophagocytic Lymphohistiocytosis Retrospectively Identified in Korea, 2008-2013.

J Korean Med Sci 2018 Dec 16;33(50):e319. Epub 2018 Nov 16.

Biomedical Research Institute, Pusan National University Hospital, Busan, Korea.

The incidence of severe fever with thrombocytopenia syndrome (SFTS) has increased in Korea since a first report in 2013. We investigated whether SFTS existed before 2013 using real-time reverse transcription polymerase chain reaction and stored blood samples from febrile patients with thrombocytopenia. Four cases of SFTS were identified, with the earliest occurring in 2008. Read More

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December 2018

Recent advances in CAR T-cell toxicity: Mechanisms, manifestations and management.

Blood Rev 2018 Nov 14. Epub 2018 Nov 14.

Experimental Transplantation and Immunology Branch, National Cancer Institute, Building 10, Suite 3-3330, Bethesda, MD 20892, United States. Electronic address:

Chimeric antigen receptor (CAR) T-cell therapy is an effective new treatment for hematologic malignancies. Two CAR T-cell products are now approved for clinical use by the U.S. Read More

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November 2018

The first case of Klebsiella pneumoniae liver abscess with hemophagocytic lymphohistiocytosis.

J Microbiol Immunol Infect 2018 Nov 20. Epub 2018 Nov 20.

Division of Infectious Diseases, Department of Medicine, Taipei Veterans General Hospital, Taipei 112, Taiwan; Institute of Emergency and Critical Care Medicine, National Yang-Ming University, Taipei, Taiwan. Electronic address:

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November 2018

A multicenter study of patients with multisystem Langerhans cell histiocytosis who develop secondary hemophagocytic lymphohistiocytosis.

Cancer 2018 Dec 6. Epub 2018 Dec 6.

Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Background: Langerhans cell histiocytosis (LCH) is a rare myeloid neoplasm characterized by the presence of abnormal CD1a-positive (CD1a )/CD207 histiocytes. Hemophagocytic lymphohistiocytosis (HLH) represents a spectrum of hyperinflammatory syndromes typified by the dysregulated activation of the innate and adaptive immune systems. Patients with LCH, particularly those with multisystem (MS) involvement, can develop severe hyperinflammation mimicking that observed in HLH. Read More

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December 2018
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Inflammatory and Infectious Syndromes Associated with Cancer Immunotherapies.

Clin Infect Dis 2018 Dec 6. Epub 2018 Dec 6.

Department of Medicine, Harvard Medical School, Boston, MA, USA.

Immunotherapy using antibodies to immune checkpoint molecules or targeted chimeric antigen receptor-modified T (CAR-T) cells represent dramatic advances in cancer treatment. These therapies mediate immune-related adverse events (irAEs) which may mimic or amplify infectious presentations. Checkpoint inhibitor therapy may be associated with diverse irAEs including mild skin, endocrine and autoimmune manifestations or severe inflammatory processes including colitis, pneumonitis, myocarditis, and shock. Read More

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December 2018
2 Reads

Outcomes Analysis of Children Diagnosed With Hemophagocytic Lymphohistiocytosis in the PICU.

Pediatr Crit Care Med 2018 Dec 4. Epub 2018 Dec 4.

Department of Critical Care, Children's National Health System, Washington, DC.

Objectives: Describe a single center experience of hemophagocytic lymphohistiocytosis in a PICU over a 10-year period, to identify clinical features that may be associated with worse outcomes, including mortality, hospital and ICU length of stay, and functional and cognitive impairments on discharge.

Design: Retrospective electronic medical record review, 2007-2017.

Setting: PICU located in a large urban academic quaternary care children's hospital. Read More

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December 2018
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Long-term safety and activity of axicabtagene ciloleucel in refractory large B-cell lymphoma (ZUMA-1): a single-arm, multicentre, phase 1-2 trial.

Lancet Oncol 2018 Nov 30. Epub 2018 Nov 30.

University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Background: Axicabtagene ciloleucel is an autologous anti-CD19 chimeric antigen receptor (CAR) T-cell therapy. In the previous analysis of the ZUMA-1 registrational study, with a median follow-up of 15·4 months (IQR 13·7-17·3), 89 (82%) of 108 assessable patients with refractory large B-cell lymphoma treated with axicabtagene ciloleucel achieved an objective response, and complete responses were noted in 63 (58%) patients. Here we report long-term activity and safety outcomes of the ZUMA-1 study. Read More

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November 2018
24.690 Impact Factor

Fludarabine/melphalan 100 mg/m Conditioning Therapy Followed by Allogeneic Hematopoietic Cell Transplantation for Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis.

Biol Blood Marrow Transplant 2018 Nov 30. Epub 2018 Nov 30.

Department of Hematology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Our previous research indicated that a reduced-intensity conditioning regimen (fludarabine and melphalan at 100 mg/m) was useful in allogeneic hematopoietic cell transplantation (HCT) for patients with lymphoma. This retrospective study evaluated the reduced-intensity conditioning regimen in allogeneic HCT for adult patients with hemophagocytic lymphohistiocytosis (HLH). Sixteen patients with HLH were evaluated, including 6 patients who were enrolled in a prospective clinical trial (NCT00772811) and 10 patients who received the same conditioning regimen (fludarabine at 30 mg/m/day on days -6 to -2 and melphalan at 100 mg/m on day -2). Read More

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November 2018
3 Reads

Epidemiology and Outcomes of Dengue in Kidney Transplant Recipients: A 20-year Retrospective Analysis and Comparative Literature Review.

Clin Transplant 2018 Dec 2:e13458. Epub 2018 Dec 2.

Division of Infectious Diseases, Department of Medicine, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Background: Kidney transplant (KT) recipients in dengue-endemic areas are at risk of exposure. We investigated the epidemiology and outcomes from dengue in KT recipients at our transplant center and conducted a literature review.

Materials And Methods: We conducted a 20-year retrospective study of KT recipients who were diagnosed with laboratory-confirmed dengue from January 1997 to September 2017 according to the 2009 WHO classification. Read More

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December 2018
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Cytologic Analysis of Epstein-Barr Virus-Associated T/Natural Killer-Cell Lymphoproliferative Diseases.

Authors:
Akihiro Yachie

Front Pediatr 2018 16;6:327. Epub 2018 Nov 16.

Department of Pediatrics, School of Medicine, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan.

Rapid, precise diagnosis of Epstein-Barr virus-associated T lymphocyte or natural killer cell lymphoproliferative diseases is clinically important to prevent disease progression and avoid fatal outcomes for patients. In addition to detecting increased copy numbers of Epstein-Barr virus, identification of the lymphocyte subpopulation targeted by the virus infection is crucial to reaching the final diagnosis. However, these procedures are laborious and require large amounts of sample. Read More

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November 2018
2 Reads

The rheumatology/hematology interface: CAPS and MAS diagnosis and management.

Hematology Am Soc Hematol Educ Program 2018 Nov;2018(1):313-317

Department of Medicine, Division of Hematology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.

Catastrophic antiphospholipid antibody syndrome (CAPS) and macrophage activation syndrome (MAS) are both life-threatening hematologic disorders that infrequently afflict patients with rheumatologic disease. CAPS is characterized by fulminant multiorgan damage related to small vessel thrombosis in the setting of persistent antiphospholipid antibodies. It can occur in patients with rheumatologic diseases such as systemic lupus erythematosus but can also affect patients who do not have rheumatologic disease. Read More

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November 2018
1 Read

Disruption of IL-33 Signaling Limits Early CD8+ T Cell Effector Function Leading to Exhaustion in Murine Hemophagocytic Lymphohistiocytosis.

Front Immunol 2018 20;9:2642. Epub 2018 Nov 20.

Division of Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, United States.

Danger signals mediated through ST2, the interleukin-33 (IL-33) receptor, amplify CD8 T cell-mediated inflammation in the murine model of familial hemophagocytic lymphohistiocytosis type 2 (FHL2), and blockade of ST2 provides a potential therapeutic strategy in this disease. However, the long-term effects of disrupting IL-33/ST2 signaling on the CD8 T cell compartment are unknown. Here, we examined the evolution of the T cell response in murine FHL type 2 in the absence of ST2 signaling and found that CD8 T cells gradually undergo exhaustion, similar to a related nonfatal FHL model. Read More

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November 2018
2 Reads

Nodular Panniculitis with Hemophagocytic Lymphohistiocytosis.

Chin Med J (Engl) 2018 Dec;131(23):2860-2861

Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China.

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December 2018

Retrospective study of haemophagocytic syndrome hospitalisations in children in the USA.

BMJ Paediatr Open 2018 9;2(1):e000337. Epub 2018 Nov 9.

Division of Pediatric Critical Care, Duke Children's Hospital, Duke University, Durham, North Carolina, USA.

Introduction: The haemophagocytic syndrome (HS) is a rare condition that presents with uncontrolled inflammation leading to multiorgan failure and is associated with significant morbidity and mortality. Current national estimates of children hospitalised due to HS are unknown. Characterising and understanding the burden of HS-related hospitalisations at a national level is the initial step in optimising the overall care. Read More

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November 2018
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[Hemophagocytic lymphohistiocytosis following autologous hematopoietic stem cell transplantation for advanced extranodal NK/T-cell lymphoma: a case report and literatures review].

Zhonghua Xue Ye Xue Za Zhi 2018 Nov;39(11):956-959

Department of Hematology, the First Affiliated Hospital of Wannan Medical College, Yijishan Hospital, Wuhu 241001, China.

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November 2018

[The 466th case:myasthenia,fever,blurred vision and pancytopenia].

Zhonghua Nei Ke Za Zhi 2018 Dec;57(12):942-944

Department of Rheumatology and Immunology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

A 45-year-old woman was admitted to the Department of Rheumatology and Immunology, Peking Union Medical College Hospital, due to weakness of the upper limbs, fever, and blurred vision. She was clinically diagnosed as systemic lupus erythematosus overlapped primary biliary cirrhosis, with renal, retinal, hematological and musculoskeletal involvement, combined with severe pulmonary infection and respiratory failure. Treated with glucocorticoids, ursodeoxycholic acid, antibiotics and respiratory support, the patient got better. Read More

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December 2018
2 Reads

Complex Investigation of a Pediatric Haematological Case: Haemophagocytic Syndrome Associated with Visceral Leishmaniasis and Epstein⁻Barr (EBV) Co-Infection.

Int J Environ Res Public Health 2018 Nov 27;15(12). Epub 2018 Nov 27.

Department of Surgical and Biomedical Sciences, Paediatric Clinic, Università degli Studi di Perugia, 06132 Perugia, Italy.

Background: Visceral leishmaniasis (VL) is an anthropozoonosis caused by an intracellular parasite belonging to the genus . In the Mediterranean region, and are responsible for VL and dogs are the main reservoir. Haemophagocytic lymphohistiocytosis (HLH) represents a complication of VL and consists of unrestrained activation and proliferation of lymphocytes and macrophages, leading to uncontrolled immune activation. Read More

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November 2018

Hemophagocytic lymphohistiocytosis secondary to Babesia in an immunocompetent adult.

Am J Hematol 2018 Nov 27. Epub 2018 Nov 27.

Department of Internal Medicine, Division of Hematology/Oncology, NYU Winthrop Hospital, Mineola, New York.

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November 2018
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Risk factors affecting outcome of unrelated cord blood transplantation for children with familial haemophagocytic lymphohistiocytosis.

Br J Haematol 2018 Nov 21. Epub 2018 Nov 21.

Eurocord, Hôpital Saint Louis, Paris, France.

Allogeneic haematopoietic stem cell transplantation is still the only available curative option for Familial Haemophagocytic Lymphohistiocytosis (FHLH). Most studies report outcomes after bone marrow or peripheral blood stem cell transplantation. We analysed the outcomes of 118 children with FHLH undergoing single-unit umbilical cord blood transplantation performed from 1996 to 2014. Read More

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November 2018
3 Reads

Hematopoietic Stem Cell Transplantation for the Treatment of Epstein-Barr Virus-Associated T- or NK-Cell Lymphoproliferative Diseases and Associated Disorders.

Front Pediatr 2018 6;6:334. Epub 2018 Nov 6.

Department of Hematology/Oncology, Osaka Women's and Children's Hospital, Izumi, Japan.

Chronic active Epstein-Barr virus infection (CAEBV) is a prototype of EBV-associated T- and/or NK-cell (EBV T/NK-cell) lymphoproliferative disorders. Most subtypes of these are lethal. We established a unified treatment strategy composed of step 1 (immunochemotherapy: steroids, cyclosporine A, and etoposide), step 2 (multi-drug block chemotherapy), and step 3 (allogeneic hematopoietic stem cell transplantation; HSCT) for CAEBV and its related diseases. Read More

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November 2018
6 Reads

Hematopoietic cell transplantation for asymptomatic X-linked lymphoproliferative syndrome type 1.

Allergy Asthma Clin Immunol 2018 14;14:82. Epub 2018 Nov 14.

1Department of Hematology and Oncology, Children's Cancer Center, Kobe Children's Hospital, Minatojima-Minamimachi 1-6-7, Chuo-ku, Kobe, 650-0047 Japan.

Background: X-linked lymphoproliferative disease type 1 (XLP1) is a rare primary immune deficiency, which is caused by gene mutations. XLP1 is commonly associated with Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis, hypogammaglobulinemia, and/or lymphoma. The only curative treatment for XLP1 is allogeneic hematopoietic cell transplantation. Read More

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November 2018
3 Reads

Hemophagocytic Lymphohistiocytosis in Dermatomyositis

Balkan Med J 2018 11 20. Epub 2018 Nov 20.

Department of Internal Medicine, Division of Allergy, Immunology and Rheumatology, Taipei Veterans General Hospital, Taipei, Taiwan

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November 2018

Bilateral pulmonary nodules and intravascular pulmonary histiocytosis: A rare presentation of hemophagocytic lymphohistiocytosis secondary to Epstein-Barr Virus infection.

Respir Med Case Rep 2019 4;26:11-13. Epub 2018 Nov 4.

Internal Medicine Department, Hurley Medical Center/ Michigan State University, Flint, MI, USA.

A 61-year-old male presented with worsening dyspnoea and constitutional symptoms for few weeks followed by bloody diarrhoea and loss and fever. Physical exam revealed tachycardia, respiratory distress, and splenomegaly without lymphadenopathy. Work up showed pancytopenia, hypofibrinogenemia, acute kidney injury requiring haemodialysis, high ferritin level, positive IgG and IgM for EBV and positive soluble CD25. Read More

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November 2018
3 Reads

Comparison of Different Diagnostic Guidelines for the Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: Single Centre Experience.

Acta Clin Croat 2018 Jun;57(2):307-311

Department of Pediatrics, Zagreb University Hospital Centre, University of Zagreb School of Medicine, Zagreb, Croatia.

Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA), caused by exaggerated but ineffective immune response. The aim of the study was to compare the capacity of the HLH-2004 guidelines with the capacity of the MAS guidelines from 2005, and with the new set of classification criteria from 2016 in diagnosing MAS complicating sJIA. The study included 35 children aged 1-18 diagnosed with sJIA according to ILAR criteria and treated at the Department of Pediatrics, Division of Immunology and Rheu-matology, Zagreb University Hospital Centre, in the period from 2009 to 2015. Read More

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June 2018
2 Reads

Successful resolution of Hemophagocytic lymphohistiocytosis associated to brucellosis in the adult.

Tunis Med 2018 Jul;96(7):458-461

Hemophagocyticlymphohistocytosis (HLH) is a proliferation of histiocytes with importanthemophagocytosisoccurring in different organs such as the spleen and the bone marrow. HLH is now increasingly diagnosed in the context of infections, malignancies and connective tissue diseases. Although brucellosis is an endemic infection in Tunisia, its association with HLH is a very rare condition which should be considered in patients with splenomegaly and cytopenia. Read More

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July 2018
5 Reads

Successful rescue of acute liver failure and hemophagocytic lymphohistiocytosis following varicella infection: A case report and review of literature.

World J Clin Cases 2018 Nov;6(13):659-665

Department of Emergency, Peking University People's Hospital, Beijing 100044, China.

Herein we report a case of acute liver failure (ALF) and hemophagocytic lymphohistiocytosis (HLH) induced by varicella infection, successfully rescued by a combination therapy of acyclovir, supportive care, and immunosuppression with dexamethasone and etoposide. A previously healthy 16-year-old boy presented with generalized rash, fever, severe abdominal pain, and abnormal liver function within 4 d. Chickenpox was suspected, and acyclovir and intravenous immunoglobulin were started on admission. Read More

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November 2018
3 Reads

Hemophagocytic lymphohistiocytosis is a sign of poor outcome in pediatric Epstein-Barr virus-associated post-transplant lymphoproliferative disease after allogeneic hematopoietic stem cell transplantation.

Pediatr Transplant 2018 Nov 11:e13319. Epub 2018 Nov 11.

Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.

EBV-related PTLD developing after HSCT is a potentially life-threatening disease. HLH is uncommon after allogeneic HSCT. Data on outcome of patients with PTLD and concomitant HLH after allogeneic HSCT are limited. Read More

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November 2018
7 Reads

Liver-associated immune abnormalities.

Autoimmun Rev 2018 Nov 5. Epub 2018 Nov 5.

University of Toronto, Toronto, Ontario, Canada; Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, Hospital for Sick Children, Toronto, Canada.

In recent years, the cross talk between the liver and the immune system is being uncovered, in part by studying liver involvement in primary immune deficiencies (PID) and in part by investigating the alterations of the immune system following orthotopic liver transplantation (OLT). Here we review some of the reciprocal interactions between the liver and the immune system. Patients with PID, particularly those involving inherited defects in T and B cells or innate immunity are prone to infections and inflammatory responses that often involve the liver. Read More

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November 2018
6 Reads

Visceral Leishmaniasis Associated with Macrophage Activation Syndrome and Diffuse Alveolar Hemorrhage in a Lupus Patient.

Acta Med Port 2018 Oct 31;31(10):593-596. Epub 2018 Oct 31.

Serviço de Medicina Interna. Centro Hospitalar de Trás-os-Montes e Alto Douro. Vila Real. Portugal.

Systemic lupus erythematosus is a heterogeneous and unpredictable autoimmune disease which can be complicated to approach and treat. Hemophagocytic lymphohistiocytosis and diffuse alveolar hemorrhage are rare disease complications. The authors describe a clinical case of a 32-year-old woman with lupus and fever of unknown origin. Read More

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October 2018
1 Read

Management of Hemophagocytic Lympho-Histiocytosis in Critically Ill Patients.

J Intensive Care Med 2018 Nov 1:885066618810403. Epub 2018 Nov 1.

1 Medical ICU, AP_HP Saint Louis hospital, Paris, France.

Hemophagocytic syndrome remains a rare but life-threatening complication and is associated with intensive care unit (ICU) admission. The pathophysiology is based on a defect of cytotoxicity in T cells that results in a state of hyperinflammation in the presence of a trigger. As a consequence, patients may develop multiorgan failure. Read More

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November 2018
1 Read

Thrombotic thrombocytopenic purpura and hemophagocytic lymphohistiocytosis in an elderly man: A case report.

Medicine (Baltimore) 2018 Nov;97(44):e13025

Hematology Unit, Medical Specialty Center, Bangkok Hospital Hat Yai, Bangkok Dusit Medical Services, Hat Yai, Songkhla, Thailand.

Rationale: Thrombotic thrombocytopenic purpura (TTP) and hemophagocytic lymphohistiocytosis (HLH) are rare hematologic conditions and have high mortality. Both TTP and HLH result from deregulation of the immune system. There are no published reports of coexisting TTP and HLH in elderly patients. Read More

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November 2018
1 Read
5.720 Impact Factor

Langerhans cell histiocytosis complicated with hemophagocytic lymphohistiocytosis in a boy with a novel XIAP mutation: A case report.

Medicine (Baltimore) 2018 Nov;97(44):e13019

Department of Pediatrics and Key Laboratory of Birth Defect and Related Disorders of Women and Children (Sichuan University), Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu, Sichuan, China.

Rationale: X-linked lymphoproliferative syndromes (XLPs) are rare, yet often fatal primary immunodeficiency diseases, which rarely manifest as Langerhans cell histiocytosis (LCH) complicated with hemophagocytic lymphohistiocytosis (HLH). Clinical data of a case of XLP-2 manifesting as LCH complicated with HLH was retrospectively analyzed to determine the etiology and causal gene.

Patient Concerns And Diagnosis: The diagnosis of multisystem LCH was confirmed by skin biopsy and other examinations in a 13-month boy with recurrent ear discharge, fever and hemorrhagic papules for 3 months. Read More

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November 2018
1 Read

Acute myeloid leukemia with new complex t(8;21;22) induced hemophagocytic lymphohistiocytosis: A case report.

Medicine (Baltimore) 2018 Nov;97(44):e12762

Department of Hematology, Hematology Research Laboratory, West China Hospital of Sichuan University, Chengdu, Sichuan, People's Republic of China.

Rationale: The balanced translocation t(8;21;22)(q22;q22;q11.2) is not reported previously, although t(8;21)(q22;q22) is seen in approximately 7% of adults and most frequent abnormality in children with newly diagnosed acute myeloid leukemia (AML). AML-associated hemophagocytic lymphohistiocytosis (HLH) is a rare event, reported only of limited numbers. Read More

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November 2018
3 Reads

Adult onset haemophagocytic lymphohistiocytosis prognosis is affected by underlying disease: analysis of a single-institution series of 174 patients.

Swiss Med Wkly 2018 09 23;148:w14641. Epub 2018 Sep 23.

Senior Department of Haematology, The First Affiliated Hospital, Medical School of Zhejiang University, Hangzhou, Zhejiang Province, China.

Background: Haemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterised by activation of the mononuclear phagocytic system, and often leads to progressive multiple organ failure. The diagnosis of HLH is made late by most physicians.

Methods: To confirm the diagnosis of acquired HLH made in a single-institution series of adult patients with HLH-04 criteria, we applied the HScore and evaluated prognostic factors associated with clinical outcome. Read More

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September 2018
3 Reads

Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome.

Nat Genet 2018 Dec 29;50(12):1650-1657. Epub 2018 Oct 29.

Department of Human Genetics, McGill University, Montreal, Quebec, Canada.

Subcutaneous panniculitis-like T cell lymphoma (SPTCL), a non-Hodgkin lymphoma, can be associated with hemophagocytic lymphohistiocytosis (HLH), a life-threatening immune activation that adversely affects survival. T cell immunoglobulin mucin 3 (TIM-3) is a modulator of immune responses expressed on subgroups of T and innate immune cells. We identify in ~60% of SPTCL cases germline, loss-of-function, missense variants altering highly conserved residues of TIM-3, c. Read More

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December 2018
1 Read

Haplo-identical Hematopoietic Stem Cell Transplant is a Possible Cure in a Patient with Relapsed Hemophagocytic Lymphohistiocytosis Syndrome.

Indian J Hematol Blood Transfus 2018 Oct 2;34(4):760-761. Epub 2018 May 2.

Department of Hemato-Oncology, Rajiv Gandhi Cancer Institute and Research Centre, Sector-5, Rohini, New Delhi, Delhi 110085 India.

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October 2018
0.234 Impact Factor

Dynamic Formation of Microvillus Inclusions During Enterocyte Differentiation in -Deficient Intestinal Organoids.

Cell Mol Gastroenterol Hepatol 2018 14;6(4):477-493.e1. Epub 2018 Aug 14.

German Cancer Consortium (Deutsches Konsortium für Translationale Krebsforschung), Heidelberg, Germany.

Background & Aims: Microvillus inclusion disease (MVID) is a congenital intestinal malabsorption disorder caused by defective apical vesicular transport. Existing cellular models do not fully recapitulate this heterogeneous pathology. The aim of this study was to characterize 3-dimensional intestinal organoids that continuously generate polarized absorptive cells as an accessible and relevant model to investigate MVID. Read More

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August 2018
6 Reads

Hemophagocytic lymphohistiocytosis with neurological manifestations in an infant with scrub typhus: a rare fatal occurrence.

Trop Doct 2018 Oct 26:49475518804696. Epub 2018 Oct 26.

4 Director Professor and Head, Department of Pediatrics, Maulana Azad Medical College, New Delhi, India.

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of unchecked activation of the immune system leading to phagocytosis of blood cells and proliferation of histiocytes in solid organs. HLH can be primary or secondary to infective, autoimmune and malignant conditions. Scrub typhus is an infective illness caused by Orientia tsutsugamushi, transmitted by mite. Read More

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October 2018
7 Reads

A fatal case of enterovirus infection with secondary hemophagocytosis-case report with review of literature.

APMIS 2018 Nov;126(11):877-882

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Enterovirus is a common viral infection, which can affect multiple organ systems with an array of clinical presentation such as meningitis, encephalitis, myocarditis, and disseminated infections. The illness is usually asymptomatic and self-limited but few cases can be severe and life-threatening especially when associated with hemophagocytosis. We discuss a fatal case of disseminated enterovirus infection and the histomorphological features of the infection. Read More

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November 2018
17 Reads
1.920 Impact Factor

Epstein-Barr Virus-Positive T/NK-Cell Lymphoproliferative Diseases in Chinese Mainland.

Front Pediatr 2018 9;6:289. Epub 2018 Oct 9.

Beijing Key Laboratory of Pediatric Respiratory Infectious Diseases, Key Laboratory of Major Diseases in Children, Ministry of Education, National Clinical Research Center for Respiratory Diseases, National Key Discipline of Pediatrics (Capital Medical University), Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.

Epstein-Barr virus-positive T/NK-cell lymphoproliferative disorders (EBV T/NK LPD) encompass a heterogeneous group of disorders, including chronic active Epstein-Barr virus infection (CAEBV), Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), systemic EBV T-cell lymphoma of childhood and hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) and so on, predominantly affecting children and young adults with high mortality. Patients with EBV T/NK LPD have overlapping clinical symptoms as well as histologic and immunophenotypic features. In this review, we summarized the clinical features of EBV T/NK LPD in Chinese patients from the published articles. Read More

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October 2018
5 Reads

Fatal Septic Shock in a Patient with Hemophagocytic Lymphohistiocytosis Associated with an Infectious Mononucleosis.

Case Rep Crit Care 2018 25;2018:9756050. Epub 2018 Sep 25.

Caserta Hospital, Department of Anesthesia and Intensive Care, Italy.

The authors describe the case of a young woman who developed a clinical pictures resembling a septic shock-related multiple organ dysfunction syndrome a couple of months after having been diagnosed suffering from a hemophagocytic lymphohistiocytosis associated with an infectious mononucleosis. Despite the aggressive treatment, which included antibiotics, vasopressors, IV immunoglobulins, and the use of an extracorporeal device aimed to remove mediators released both during sepsis and the cytokine storm determined by the hemophagocytic lymphohistiocytosis, the patient died. At the autopsy, an extremely uncommon aggressive lymphoma of Epstein-Barr virus-positive T-lymphocytes with systemic involvement was discovered. Read More

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September 2018
1 Read

Hypercytokinemia with hemophagocytic syndrome due to human metapneumovirus.

Pediatr Int 2018 Oct;60(10):974-976

Department of Pediatrics, Jichi Medical University, Shimotsuke, Tochigi, Japan.

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October 2018
1 Read

HLH - Unusual Trigger and Positive Outcome.

J Assoc Physicians India 2018 Mar;66(3):85-86

Professor and Head of Department, Sri Ramachandra University and Research Institute, Chennai, Tamil Nadu.

Hemophagocytic Lymphohistiocytosis (HLH), is an uncommon, aggressive and life threatening syndrome of excessive immune activation. We report an unusual case of HLH, in a 34 year old male, who was admitted with Subarachnoid hemorrhage and cerebellar contusion in a Neurosurgical Intensive care unit, whose trigger is not clear. Read More

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March 2018
6 Reads

Usefulness of the skin biopsy as a tool in the diagnosis of silvery hair syndrome.

Pediatr Dermatol 2018 Nov 18;35(6):780-783. Epub 2018 Oct 18.

Department of Dermatology, National Institute of Pediatrics, Mexico City, Mexico.

Background/objectives: Silvery hair syndrome is a rare, autosomal-recessive entity characterized by silvery gray hair, eyebrows, and eyelashes and may be associated or not with immunologic or neurologic alterations. Two main types have been recognized: Chediak-Higashi syndrome and Griscelli syndrome. Hair shaft examination under light microscopy has been a useful tool to differentiate Chediak-Higashi syndrome from Griscelli syndrome, although distribution of melanin varies according to hair color related to ethnicity. Read More

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November 2018
1 Read