3,617 results match your criteria Lymphohistiocytosis


Hemophagocytic Lymphohistiocytosis (HLH) Triggered by Wasp Venom Immunotherapy Is Rare but Noteworthy.

J Investig Allergol Clin Immunol 2019 Feb;29(1):49-51

Department of Internal Medicine and Allergology, Wroclaw Medical University, Wroclaw, Poland.

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http://dx.doi.org/10.18176/jiaci.0322DOI Listing
February 2019

Novel mutations of STXBP2 and LYST associated with adult haemophagocytic lymphohistiocytosis with Epstein-Barr virus infection: a case report.

BMC Med Genet 2019 Feb 19;20(1):34. Epub 2019 Feb 19.

Department of Hematology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, Hubei, China.

Background: Haemophagocytic lymphohistiocytosis is a life-threatening disease resulting from primary or secondary hyper-inflammatory disorders. The typical symptoms include persistent fever, splenomegaly, cytopenia and significant elevation of serum ferritin.

Case Presentation: We report a 30-year-old Chinese female patient who was diagnosed with chronic active Epstein-Barr virus infection more than 9 months prior and has since been presenting with cutaneous lymphoproliferative disorders mimicking hydroa vacciniforme and subsequent haemophagocytic lymphohistiocytosis. Read More

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http://dx.doi.org/10.1186/s12881-019-0765-3DOI Listing
February 2019

Haemophagocytic lymphohistiocytosis secondary to presumed congenital tuberculosis in a neonate.

J Paediatr Child Health 2019 Feb 18. Epub 2019 Feb 18.

Department of Allergy and Immunology, Children's Hospital at Westmead, Sydney, New South Wales, Australia.

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http://dx.doi.org/10.1111/jpc.14393DOI Listing
February 2019

Haploidentical Stem Cell Transplantation with Post-Transplant Cyclophosphamide for Primary Immune Deficiency Disorders in Children: Challenges and Outcome from a Tertiary Care Center in South India.

J Clin Immunol 2019 Feb 18. Epub 2019 Feb 18.

Department of Pediatric Hematology, Oncology, Blood and Marrow Transplantation, Apollo Hospitals, 320, Padma complex, Anna salai, Teynampet, Chennai, Tamil Nadu, 600035, India.

Haploidentical stem cell transplantation (haplo SCT) has emerged as an acceptable alternative to matched family donor transplantation for children diagnosed to have primary immune deficiency disorders (PIDs). We present data over 4 years on the challenges and efficacy of unmanipulated T cell replete haplo SCTs with post-transplant cyclophosphamide (PTCy) in children diagnosed to have PIDs. We performed a retrospective study in the pediatric blood and marrow transplantation unit where all children less than 18 years of age diagnosed to have PIDs and who underwent haplo SCT with PTCy from January 2014 to February 2018 were included in the study. Read More

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http://dx.doi.org/10.1007/s10875-019-00600-zDOI Listing
February 2019

Liver Transplant in a Patient With Hemophagocytic Lymphohistiocytosis.

Exp Clin Transplant 2019 Jan;17(Suppl 1):226-229

From the Department of Transplantation, Baskent University, Ankara, Turkey.

Hemophagocytic lymphohistiocytosis is a rare and life-threatening systemic disease that can cause hepatic infiltration and present as acute liver failure. Here, we report a case of a 3-year-old pediatric patient who presented with acute liver failure and hepatic encephalopathy secondary to hemophagocytic lymphohistiocytosis. She had left lateral segment liver transplant from her father. Read More

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http://dx.doi.org/10.6002/ect.MESOT2018.P80DOI Listing
January 2019

Successful Management of Late-Onset Cytomegalovirus-Induced Hemophagocytic Lymphohistiocytosis in Kidney Transplant Recipient After Coronary Artery Bypass Graft Surgery.

Exp Clin Transplant 2019 Jan;17(Suppl 1):207-211

From the Nephrology Department, Hamd Al-Essa Organ Transplant Center of Kuwait, Kuwait.

Hemophagocytic syndrome combines febrile hepatosplenomegaly, pancytopenia, hypofibrinemia, and hepatic dysfunction. It is characterized by bone marrow and organ infiltration of activated, nonmalignant macrophages that phagocytize blood cells. It is rare among renal transplant recipients. Read More

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http://dx.doi.org/10.6002/ect.MESOT2018.P67DOI Listing
January 2019

Acute Respiratory Distress Syndrome Secondary to Histoplasmosis-induced Hemophagocytic Lymphohistiocytosis.

Arch Bronconeumol 2019 Feb 15. Epub 2019 Feb 15.

Division of Pulmonary and Critical Care Medicine, Medstar Georgetown University Hospital, Washington, DC, United States.

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http://dx.doi.org/10.1016/j.arbres.2018.12.011DOI Listing
February 2019

Requirement for etoposide in the treatment of pregnancy related hemophagocytic lymphohistiocytosis: a multicenter retrospective study.

Orphanet J Rare Dis 2019 Feb 18;14(1):50. Epub 2019 Feb 18.

Department of Obstetrics and Gynecology, China-Japan Friendship Hospital, Beijing, China.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare severe clinical syndrome. HLH manifesting during pregnancy has been paid much attention in recent years. Despite the specificity of pregnancy-related HLH, there has not been any consensus regarding its treatment. Read More

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http://dx.doi.org/10.1186/s13023-019-1033-5DOI Listing
February 2019

The Immunology of Macrophage Activation Syndrome.

Front Immunol 2019 1;10:119. Epub 2019 Feb 1.

Pediatric Rheumatology, University of Alabama Birmingham, Birmingham, AL, United States.

Synonymous with secondary hemophagocytic lymphohistiocytosis, macrophage activation syndrome (MAS) is a term used by rheumatologists to describe a potentially life-threatening complication of systemic inflammatory disorders, most commonly systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus (SLE). Clinical and laboratory features of MAS include sustained fever, hyperferritinemia, pancytopenia, fibrinolytic coagulopathy, and liver dysfunction. Soluble interleukin-2 receptor alpha chain (sCD25) and sCD163 may be elevated, and histopathology often reveals characteristic increased hemophagocytic activity in the bone marrow (and other tissues), with positive CD163 (histiocyte) staining. Read More

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http://dx.doi.org/10.3389/fimmu.2019.00119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367262PMC
February 2019

Clinical characteristics of extranodal NK/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis.

Cancer Manag Res 2019 23;11:997-1002. Epub 2019 Jan 23.

Department of Medical Oncology, Fudan University Shanghai Cancer Center, Shanghai 200032, China,

Background: Extranodal natural killer (NK) / T cell lymphoma is a subtype of non-Hodgkin's lymphoma (NHL) that usually has an aggressive clinical course. It is the predominant trigger of lymphoma-associated hemophagocytic syndrome (LAHS), which is highly lethal and with extremely poor prognosis. This study is aiming to characterize the associated clinical features and prognostic factors of the disease. Read More

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https://www.dovepress.com/clinical-characteristics-of-extran
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http://dx.doi.org/10.2147/CMAR.S183784DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6350644PMC
January 2019
4 Reads

Prevalence of deaths in a cohort of girls and women with cryopreserved ovarian tissue.

Acta Obstet Gynecol Scand 2019 Feb 15. Epub 2019 Feb 15.

The Fertility Clinic, section 4071, University Hospital of Copenhagen Rigshospitalet, Copenhagen, Denmark.

Introduction: Ovarian tissue cryopreservation (OTC) is increasingly offered to women in need of fertility preservation. However, little is known about the risk of these women dying before they use the preserved material.

Material And Methods: From 1999 to 2016, 927 girls and women underwent OTC in our center, prior to receiving gonadotoxic treatment. Read More

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http://dx.doi.org/10.1111/aogs.13576DOI Listing
February 2019
1 Read

Visceral leishmaniasis with haemophagocytic lymphohistiocytosis.

BMJ Case Rep 2019 Feb 13;12(2). Epub 2019 Feb 13.

Department of General Medicine, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

A 27-year-old man presented with high-grade intermittent fever for 4 months, generalised fatigue for 2 months, intermittent gum bleeds for 1 month and loss of weight of 15 kg. He appeared cachectic with generalised wasting, had pallor and features of reticuloendothelial system proliferation. His liver span was 17 cm. Read More

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http://dx.doi.org/10.1136/bcr-2018-226361DOI Listing
February 2019
1 Read

Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome.

Pediatr Rheumatol Online J 2019 Feb 14;17(1). Epub 2019 Feb 14.

Division of Immunolgy, Boston Children's Hospital, Boston, MA, USA.

Background: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-019-0309-6DOI Listing
February 2019
5 Reads

Haploinsufficiency of UNC13D increases the risk of lymphoma.

Cancer 2019 Feb 13. Epub 2019 Feb 13.

Childhood Cancer Research Unit, Department of Women's and Children's Health, Karolinska Institutet, Karolinska University Hospital Solna, Stockholm, Sweden.

Background: Experimental models have demonstrated that immune surveillance by cytotoxic lymphocytes can protect from spontaneous neoplasms and cancer. In humans, defective lymphocyte cytotoxicity is associated with the development of hemophagocytic lymphohistiocytosis, a hyperinflammatory syndrome. However, to the best of the authors' knowledge, the degree to which human lymphocyte cytotoxicity protects from cancer remains unclear. Read More

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http://dx.doi.org/10.1002/cncr.32011DOI Listing
February 2019

Haemophagocytic Lymphohistiocytosis in a 16-Year-Old Boy.

Mymensingh Med J 2019 Jan;28(1):241-244

Dr Md Atikur Rahman, Phase-B Resident, Department of Internal Medicine, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh; E-mail:

Haemophagocytosis encompasses phagocytosis of erythrocytes, leukocytes, platelets and their precursors by macrophages in bone marrow and other tissues. Haemophagocytic lymphohistiocytosis (HLH) usually presents with high fever, pancytopenia, splenomegaly, lymphadenopathy, haemophagocytosis in bone marrow, liver, lymphnodes or CSF. We report coagulase negative Staphylococcus induced HLH in a 16 year old boy presenting with high grade fever, lymphadenopathy, hepatosplenomegaly, pancytopenia with neutropenic sepsis in the department of Medicine, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh on 28th November 2016. Read More

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January 2019
1 Read

Disseminated CMV infection and HLH in a patient with well-controlled HIV and ulcerative colitis.

BMJ Case Rep 2019 Feb 11;12(2). Epub 2019 Feb 11.

Department of Genitourinary Medicine and HIV, Imperial College Healthcare NHS Trust, London, UK.

We present a case of haemophagocytic lymphohistiocytosis (HLH) in the context of disseminated cytomegalovirus (CMV) viraemia in a 50-year-old man with well-controlled HIV infection and ulcerative colitis (UC), for which he was receiving azathioprine. Peak CMV viral load was 371 000 copies/ml with evidence of end-organ CMV in the lungs and colon. A bone marrow biopsy showed evidence of haemophagocytosis of platelets, neutrophils and erythrocytes. Read More

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http://dx.doi.org/10.1136/bcr-2018-227916DOI Listing
February 2019
1 Read

[Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis: Rarity and Severity in an Immunocompetent Adult].

Acta Med Port 2019 Feb 1;32(1):78-80. Epub 2019 Feb 1.

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

Hemophagocytic lymphohistiocytosis is a rare, aggressive and life-threatening syndrome, characterized by an excessive immune activation. It is triggered by multiple stimuli, with infections having an important role. The most common infectious trigger is viral infection, particularly by Epstein-Barr virus. Read More

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http://dx.doi.org/10.20344/amp.9474DOI Listing
February 2019
1 Read

Risk-factors Associated with Poor Outcomes in VEO-IBD Secondary to XIAP Deficiency: A Case Report and Literature Review.

J Pediatr Gastroenterol Nutr 2019 Feb 7. Epub 2019 Feb 7.

Division of Gastroenterology, Hepatology and Nutrition, Boston Children's Hospital, Boston, MA.

Very early onset inflammatory bowel disease (VEO-IBD) represents a diagnostic and treatment challenge. Here we present a case of VEO-IBD secondary to a mutation in BIRC4 gene, which encodes X-linked inhibitor of apoptosis protein (XIAP), in a 17 month-old male with severe failure to thrive, intractable diarrhea and hepatosplenomegaly. Endoscopy and histology identified only mild duodenitis and ileitis, but severe pancolitis with crypt abscesses and epithelium apoptosis. Read More

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http://Insights.ovid.com/crossref?an=00005176-900000000-9653
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http://dx.doi.org/10.1097/MPG.0000000000002297DOI Listing
February 2019
6 Reads

Clinical and endoscopic complications of Epstein-Barr virus in inflammatory bowel disease: an illustrative case series.

Int J Colorectal Dis 2019 Feb 9. Epub 2019 Feb 9.

Department of Gastroenterology and Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.

Background And Aim: Epstein-Barr virus (EBV) is a proposed trigger in the etiopathogenesis of inflammatory bowel disease (IBD) and is associated with lymphoproliferative diseases. Nevertheless, testing for EBV DNA in the intestinal mucosa and screening for EBV infection before initiation of a drug therapy are not routinely performed. The aim of this article is to increase awareness of the relevance of EBV infection in specific clinical situations. Read More

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http://dx.doi.org/10.1007/s00384-019-03257-7DOI Listing
February 2019

Association of hemophagocytic lymphohistiocytosis with kidney lesions in acute African swine fever virus infection

Ann Parasitol 2018 ;64(4):343-350

Department of Medical Biology and Parasitology, Armenia Yerevan State Medical University, 2 Koryun St., Yerevan 0025, Armenia

Glomerulonephritis due to African swine fever (ASF) is well documented. However, there is absence of good understanding of mechanisms involved in the development of pathology development. This study examines glomerulonephritis in association with acute infection induced by II genotype (Georgia 2007) of ASF virus. Read More

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January 2018
1 Read

Fifteen-minute consultation: Recognising primary immune deficiencies in children.

Arch Dis Child Educ Pract Ed 2019 Feb 7. Epub 2019 Feb 7.

Department of Paediatrics, Institute of Clinical Sciences, University of Gothenburg, Gothenburg, Sweden.

Children with primary immunodeficiency syndromes present with broad variation of clinical features and the consequences are often severe if not promptly recognised. Here, support is provided for the general paediatrician to recognise primary immunodeficiencies among the many children they meet in their clinical practice. Read More

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http://dx.doi.org/10.1136/archdischild-2018-315484DOI Listing
February 2019
1 Read

Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma With Hemophagocytosis Showing Complete Resolution With the BFM90 Protocol: Case Report and Review of Literature.

J Pediatr Hematol Oncol 2019 Feb 5. Epub 2019 Feb 5.

Clinical Haematology and Bone Marrow Transplantation, Christian Medical College, Ludhiana, Punjab.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct subtype of peripheral T-cell lymphoma associated with aggressive clinical behavior. Since its original description, it has continued to be a rare disease, and <200 cases have been reported in literature. We report an 11-year-old boy who presented with SPTCL and hemophagocytic lymphohistiocytosis (HLH) and responded to high-dose multiagent chemotherapy. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001434DOI Listing
February 2019
4 Reads

A Review of Neuropathological Features of Familial and Adult Hemophagocytic Lymphohistiocytosis.

J Neuropathol Exp Neurol 2019 Jan 14. Epub 2019 Jan 14.

Department of Pathology, University of Colorado School of Medicine, Aurora, Colorado.

Hemophagocytic lymphohistiocytosis (HLH) is a hematological disorder that can be due to genetic (primary HLH) causes or excessive activation of the immune system in association with infection, malignancy, rheumatologic disorders, or immune suppression (secondary HLH). Hemophagocytic lymphohistiocytosis remains an under-recognized condition among neuropathologists, especially the secondary forms, where it may be diagnosed only at brain biopsy or autopsy due to confounding comorbidities. The CNS is frequently affected, but neuropathological features are underappreciated. Read More

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https://academic.oup.com/jnen/advance-article/doi/10.1093/jn
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http://dx.doi.org/10.1093/jnen/nlz001DOI Listing
January 2019
3 Reads

A Unique Case of Severe Anemia Secondary to Copper Deficiency in an Adult Patient.

Cureus 2018 Nov 26;10(11):e3636. Epub 2018 Nov 26.

Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, USA.

Anemia is a frequently encountered problem in the healthcare system. Common causes of anemia include blood loss, followed by impaired red blood cell production and red blood cell destruction. This case demonstrates the need for cognizance of the less frequent causes of anemia. Read More

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http://dx.doi.org/10.7759/cureus.3636DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6351007PMC
November 2018

Identification of a novel CCDC22 mutation in a patient with severe Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and aggressive natural killer cell leukemia.

Int J Hematol 2019 Jan 31. Epub 2019 Jan 31.

Department of Hematology/Oncology, Wakayama Medical University, Wakayama, Japan.

Aggressive natural killer cell leukemia (ANKL) is a rare neoplasm characterized by the systemic infiltration of Epstein-Barr virus (EBV)-associated NK cells, and rapidly progressive clinical course. We report the case of a 45-year-old man with intellectual disability who developed ANKL, and describe the identification of a novel genetic mutation of coiled-coil domain-containing 22 (CCDC22). He presented with persistent fever, severe pancytopenia, and hepatosplenomegary. Read More

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http://dx.doi.org/10.1007/s12185-019-02595-0DOI Listing
January 2019
3 Reads

Recognition of hemophagocytic lymphohistiocytosis in sickle cell vasoocclusive crises is a potentially lifesaving diagnosis.

Haematologica 2019 Jan 31. Epub 2019 Jan 31.

Department of Medicine, Boston University School of Medicine, Boston MA, USA;

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http://dx.doi.org/10.3324/haematol.2018.206458DOI Listing
January 2019
1 Read

[Refractory/relapsed hemophagocytic lymphohistiocytosis treated with ruxolitinib: three cases report and literatures review].

Zhonghua Xue Ye Xue Za Zhi 2019 Jan;40(1):73-75

Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.

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http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2019.01.014DOI Listing
January 2019
3 Reads

Hemophagocytic lymphohistiocytosis followed by an episode of peritoneal dialysis associated peritonitis: a case report.

BMC Nephrol 2019 Jan 29;20(1):27. Epub 2019 Jan 29.

Renal Division, Department of Medicine, Peking University First Hospital; Peking University Institute of Nephrology, Key Laboratory of Renal Disease, Ministry of Health of China; and Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, 8 Xishiku Street; Xicheng District, Beijing, China.

Background: Hemophagocytic lymphohistiocytosis (HLH) is characterized by excessive activation of the immune system due to infection, autoimmune diseases, or malignancy. As an aggressive and life-threatening clinical syndrome, HLH secondary to peritoneal dialysis associated peritonitis (PDAP) has never been reported.

Case Presentation: A 34-year-old female peritoneal dialysis (PD) patient was hospitalized for fever, progressively multi-organ damage (including cytopenias, abnormalities of coagulation and liver enzyme) after an episode of organism-specific peritonitis. Read More

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http://dx.doi.org/10.1186/s12882-019-1217-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352365PMC
January 2019

Therapeutic plasma exchange for pediatric nonrenal disease indications and outcomes: A single-center experience.

Asian J Transfus Sci 2018 Jul-Dec;12(2):127-135

Department of Paediatrics, Apollo Children's Hospitals, Chennai, Tamil Nadu, India.

Introduction: Outcome data in pediatric plasma exchange, especially in nonrenal indications are scarce. We aimed to evaluate its role and outcome in our patients.

Subjects And Methods: A retrospective study of children admitted in the year 2016 to the Pediatric Intensive Care Unit requiring plasma exchange for nonrenal indications was undertaken. Read More

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http://dx.doi.org/10.4103/ajts.AJTS_123_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327757PMC
January 2019
3 Reads

[Isolated digestive localization of Hodgkin lymphoma in an HIV-infected patient, detected after hemophagocytic lymphohistiocytosis].

Med Mal Infect 2019 Jan 25. Epub 2019 Jan 25.

Service de médecine interne, maladies infectieuses et immunologie clinique, hôpital Robert-Debré, CHU Reims, avenue Général-Koenig, 51100 Reims, France.

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http://dx.doi.org/10.1016/j.medmal.2019.01.005DOI Listing
January 2019

[Hemophagocytic syndrome in a patient followed for IgG4-related disease in intensive care unit].

Tuberk Toraks 2018 Dec;66(4):353-358

Division of Intensive Care, Department of Anesthesiology and Reanimation, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey.

IgG4-related disease (IgG4-RD) is a set of diseases that can affect multiple organs, produce an immune-mediated fibroinflammatory response, and lead to tissue destruction and organ failure. Hemophagocytic syndrome is a life-threatening hyperinflammatory fatal disease caused by defect and excessive macrophage activity in natural killer cells. The disease can often be confused with other immune-mediated diseases such as cancer, infection, interstitial lung disease, sjogren's syndrome, wegener's vasculitis, or temporal arteritis. Read More

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http://dx.doi.org/10.5578/tt.67630DOI Listing
December 2018
4 Reads

Innovative analysis of predictors for overall survival from systemic non-Hodgkin T cell lymphoma using quantile regression analysis.

Chin Med J (Engl) 2019 Feb;132(3):294-301

Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.

Background: Non-Hodgkin T/NK cell lymphoma is a rare and widely variable type of lymphoma with the most dismal prognosis. This study aimed to investigate varied impact of the clinical indicators to the overall survival (OS).

Methods: We conducted a retrospective study to identify the non-invasive clinical features of T cell lymphoma that can predict prognosis with an innovative analysis method using quantile regression. Read More

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http://dx.doi.org/10.1097/CM9.0000000000000088DOI Listing
February 2019
1 Read

An 18-Year-Old Male With X-linked Lymphoproliferative Syndrome Type 1 Who Developed Primary Central Nervous System Lymphoma 6 Months After Primary Epstein-Barr Virus Infection.

J Pediatr Hematol Oncol 2019 Jan 22. Epub 2019 Jan 22.

Departments of Pediatrics.

X-linked lymphoproliferative syndrome type 1 (XLP1) is a rare congenital immunodeficiency disease. We report the case of an 18-year-old male who developed hemophagocytic lymphohistiocytosis (HLH) with neurological complications after primary Epstein-Barr virus (EBV) infection and subsequently developed EBV-related central nervous system lymphoma (CNSL). Given the vulnerability to EBV, he was finally diagnosed with XLP1 and treated with whole-brain irradiation along with chemotherapy and subsequent allogeneic hematopoietic stem cell transplantation from a SH2D1A wild-type sibling donor. Read More

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http://dx.doi.org/10.1097/MPH.0000000000001424DOI Listing
January 2019
5 Reads

A Case of Parvovirus-Related Haemophagocytic Lymphohistiocytosis in a Patient with HbH Disease.

Case Rep Med 2018 24;2018:8057045. Epub 2018 Dec 24.

Department of Haematology, Tan Tock Seng Hospital, Singapore.

Hemophagocytic lymphohistiocytosis (HLH) is rare and life-threatening medical emergency. Parvovirus infection is rarely associated with HLH. We report a case of parvovirus-related HLH in a patient with alpha thalassaemia (HbH disease). Read More

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https://www.hindawi.com/journals/crim/2018/8057045/
Publisher Site
http://dx.doi.org/10.1155/2018/8057045DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323427PMC
December 2018
2 Reads

Specific sequences of infectious challenge lead to secondary hemophagocytic lymphohistiocytosis-like disease in mice.

Proc Natl Acad Sci U S A 2019 Feb 23;116(6):2200-2209. Epub 2019 Jan 23.

Department of Immunobiology, Yale University School of Medicine, New Haven, CT 06520;

Secondary hemophagocytic lymphohistiocytosis (sHLH) is a highly mortal complication associated with sepsis. In adults, it is often seen in the setting of infections, especially viral infections, but the mechanisms that underlie pathogenesis are unknown. sHLH is characterized by a hyperinflammatory state and the presence hemophagocytosis. Read More

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http://dx.doi.org/10.1073/pnas.1820704116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6369774PMC
February 2019
2 Reads

Soluble ST2 and CD163 as Potential Biomarkers to Differentiate Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome.

Mediterr J Hematol Infect Dis 2019 1;11(1):e2019008. Epub 2019 Jan 1.

Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, China.

The differentiation of primary hemophagocytic lymphohistiocytosis (pHLH) and macrophage activation syndrome (MAS) poses a challenge to hematologists. The aim of this study was (1) to compare the levels of soluble ST2 (sST2), sCD163, IFN-γ, IL-10, IL-18, TNF-α and Serum soluble interleukin-2 receptor (sCD25) in patients with pHLH and MAS and (2) to investigate whether they can help differentiate the two diseases. A total of 52 participants were recruited in this study, including 12 pHLH patients, 20 MAS patients, and 20 healthy subjects. Read More

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https://www.mjhid.org/index.php/mjhid/article/view/2019.008
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http://dx.doi.org/10.4084/MJHID.2019.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328040PMC
January 2019
5 Reads

Macrophage Activation Marker Soluble CD163 Associated with Fatal and Severe Ebola Virus Disease in Humans.

Emerg Infect Dis 2019 Feb;25(2):290-298

Ebola virus disease (EVD) is associated with elevated cytokine levels, and hypercytokinemia is more pronounced in fatal cases. This type of hyperinflammatory state is reminiscent of 2 rheumatologic disorders known as macrophage activation syndrome and hemophagocytic lymphohistiocytosis, which are characterized by macrophage and T-cell activation. An evaluation of 2 cohorts of patients with EVD revealed that a marker of macrophage activation (sCD163) but not T-cell activation (sCD25) was associated with severe and fatal EVD. Read More

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http://dx.doi.org/10.3201/eid2502.181326DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6346465PMC
February 2019
3 Reads

Lysosomal Acid Lipase: From Cellular Lipid Handler to Immunometabolic Target.

Trends Pharmacol Sci 2019 02 18;40(2):104-115. Epub 2019 Jan 18.

Department of Excellence of Pharmacological and Biomolecular Sciences (DisFeB), Università Degli Studi di Milano, Milan 20133, Italy; SISA Centre, Bassini Hospital, Cinisello Balsamo, 20092, Italy. Electronic address:

Lysosomal acid lipase (LAL) hydrolyzes cholesteryl esters (CEs) and triglycerides (TGs) to free cholesterol (FC) and free fatty acids (FFAs), which are then used for metabolic purposes in the cell. The process also occurs in immune cells that adapt their metabolic machinery to cope with the different energetic requirements associated with cell activation, proliferation, and polarization. LAL deficiency (LALD) causes severe lipid accumulation and affects the immunometabolic signature in animal models. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01656147183023
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http://dx.doi.org/10.1016/j.tips.2018.12.006DOI Listing
February 2019
2 Reads

Overview of EBV-Associated T/NK-Cell Lymphoproliferative Diseases.

Front Pediatr 2018 4;6:417. Epub 2019 Jan 4.

Department of Allergy and Clinical Immunology, National Research Institute for Child Health and Development, Tokyo, Japan.

Epstein-Barr virus-associated T/natural killer-cell lymphoproliferative diseases (EBV-T/NK-LPDs) are a group of rare diseases resulting from ectopic infection of T or natural killer (NK) lymphocytes with Epstein-Barr virus (EBV). EBV-T/NK-LPDs include chronic active EBV infection, EBV-associated hemophagocytic lymphohistiocytosis, hydroa vacciniforme-like lymphoproliferative disease, and severe mosquito bite allergy. Extra-nodal NK/T-cell lymphoma-nasal type and aggressive NK-cell leukemia can also be included in this broad spectrum. Read More

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https://www.frontiersin.org/article/10.3389/fped.2018.00417/
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http://dx.doi.org/10.3389/fped.2018.00417DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6328478PMC
January 2019
4 Reads

Deficiency of perforin and hCNT1, a novel inborn error of pyrimidine metabolism, associated with a rapidly developing lethal phenotype due to multi-organ failure.

Biochim Biophys Acta Mol Basis Dis 2019 Jan 15. Epub 2019 Jan 15.

Departments of Clinical Chemistry, Pediatrics and Clinical Genetics, Amsterdam UMC, University of Amsterdam, Amsterdam Gastroenterology & Metabolism, 1105 AZ Amsterdam, the Netherlands. Electronic address:

Pyrimidine nucleotides are essential for a vast number of cellular processes and dysregulation of pyrimidine metabolism has been associated with a variety of clinical abnormalities. Inborn errors of pyrimidine metabolism affecting enzymes in the pyrimidine de novo and degradation pathway have been identified but no patients have been described with a deficiency in proteins affecting the cellular import of ribonucleosides. In this manuscript, we report the elucidation of the genetic basis of the observed uridine-cytidineuria in a patient presenting with fever, hepatosplenomegaly, persistent lactate acidosis, severely disturbed liver enzymes and ultimately multi-organ failure. Read More

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http://dx.doi.org/10.1016/j.bbadis.2019.01.013DOI Listing
January 2019
2 Reads

A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis.

Clin Case Rep 2019 Jan 28;7(1):164-169. Epub 2018 Nov 28.

Department of Hematology and Oncology, D'Amour Center for Cancer Care, Baystate Medical Center University of Massachusetts Springfield Massachusetts.

Hepatosplenic gamma-delta T-cell lymphoma with concurrent hemophogocytic lymphohistiocytosis is a rare but well-recognized clinical scenario, associated with a grim prognosis. Clinicians must be aware of this aggressive type of lymphoma so that a prompt diagnosis can be made with timely initiation of systemic therapy and referral for bone marrow transplant. Read More

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http://dx.doi.org/10.1002/ccr3.1924DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333078PMC
January 2019
1 Read

Combined hemophagocytic syndrome and thrombotic microangiopathy due to mixed infection with influenza virus and pneumococcal pneumonia.

Clin Case Rep 2019 Jan 22;7(1):131-134. Epub 2018 Nov 22.

Department of Respiratory Medicine Saitama Cardiovascular and Respiratory Center Saitama Japan.

Development of hemophagocytic syndrome and thrombotic microangiopathy due to community-acquired pneumonia is rare, but delayed management of these complications can lead to a poor prognosis. Infection by both and influenza virus can cause these complications; thus, physicians should pay attention to them when treating influenza-associated pneumococcal pneumonia. Read More

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http://doi.wiley.com/10.1002/ccr3.1842
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http://dx.doi.org/10.1002/ccr3.1842DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6333073PMC
January 2019
5 Reads

Unusual manifestation of disseminated herpes simplex virus type 2 infection associated with pharyngotonsilitis, esophagitis, and hemophagocytic lymphohisitocytosis without genital involvement.

BMC Infect Dis 2019 Jan 17;19(1):65. Epub 2019 Jan 17.

Division of Rheumatology, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, 3-18-22 Hon-komagome, Tokyo, 1138677, Bunkyo-ku, Japan.

Background: Herpes simplex virus (HSV) has various presentations, depending on the patient's immune status, age, and the route of transmission. In adults, HSV type 1 is found predominantly in the oral area, and HSV type 2 (HSV-2) is commonly found in the genital area. HSV-2 infection without genital lesions is uncommon. Read More

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https://bmcinfectdis.biomedcentral.com/articles/10.1186/s128
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http://dx.doi.org/10.1186/s12879-019-3721-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6337778PMC
January 2019
6 Reads

Case Report: Hemophagocytic Lymphohistiocytosis Caused by Disseminated Histoplasmosis in a Venezuelan Patient with HIV and Epstein-Barr Virus Reactivation Who Traveled to Japan.

Am J Trop Med Hyg 2019 Feb;100(2):365-367

AIDS Clinical Center, National Center for Global Health and Medicine, Tokyo, Japan.

We describe a Venezuelan visitor to Japan who was diagnosed with hemophagocytic lymphohistiocytosis (HLH). The patient was also diagnosed with human immunodeficiency virus (HIV) and Epstein-Barr virus infection by the Western blot and polymerase chain reaction (PCR) tests, respectively. The cause of HLH was considered to be these two infections at first; however, the patient did not recover with antiretroviral/anti-herpes virus therapy. Read More

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http://dx.doi.org/10.4269/ajtmh.18-0478DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367636PMC
February 2019
2 Reads

Haemophagocytic lymphohistiocytosis with collapsing lupus podocytopathy as an unusual manifestation of systemic lupus erythematosus with APOL1 double-risk alleles.

BMJ Case Rep 2019 Jan 14;12(1). Epub 2019 Jan 14.

Division of Nephrology, Department of Medicine, Jacobi Medical Center at Albert Einstein College of Medicine, Bronx, New York, USA.

Haemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by excessive immune activation. Secondary HLH has been described in autoimmune diseases. We detail the case of a 28-year-old African American woman who developed HLH in the setting of systemic lupus erythematosus with collapsing lupus podocytopathy superimposed on mesangial proliferative lupus nephritis class II. Read More

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http://dx.doi.org/10.1136/bcr-2018-227860DOI Listing
January 2019
2 Reads

Chronic active EBV infection associated with NK cell lymphoma and hemophagocytic lymphohistiocytosis in a 27-year-old woman: A case report.

Medicine (Baltimore) 2019 Jan;98(2):e14032

Department of Infectious Diseases, The Third Affiliated Hospital of Hebei Medical University, Shijiazhuang.

Rationale: Chronic active Epstein-Barr virus infection (CAEBV) is a common infectious disease that often affects multiple organs or systems. However, it is liable to be neglected and misdiagnosed owing to its insidious onset, lack of specific findings in the early phase, and a general lack of awareness among clinicians. PATIENT CONCERNS:: a 27-year-old woman case has been described who was initially misdiagnosed as drug-induced liver injury due to onset presentation of mild splenomegaly, recurrent liver dysfunction, and disputable pathological evidence of liver biopsy. Read More

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http://dx.doi.org/10.1097/MD.0000000000014032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6336602PMC
January 2019
5 Reads
5.723 Impact Factor

Emapalumab: First Global Approval.

Drugs 2019 Jan;79(1):99-103

Springer, Private Bag 65901, Mairangi Bay, Auckland, 0754, New Zealand.

Emapalumab-Izsg (hereafter referred to as emapalumab) [Gamifant] is a monoclonal antibody directed against interferon gamma that is available as an intravenous infusion. Emapalumab is being developed by Novimmune and Swedish Orphan Biovitrum for the treatment of haemophagocytic lymphohistiocytosis (HLH). In November 2018, emapalumab received its first global approval in the USA, for the treatment of paediatric (newborn and older) and adult patients with primary HLH, who have refractory, recurrent or progressive disease or intolerance to conventional HLH therapy. Read More

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http://dx.doi.org/10.1007/s40265-018-1046-8DOI Listing
January 2019