4,168 results match your criteria Lymphohistiocytosis


A comprehensive analysis of adult patients with secondary hemophagocytic lymphohistiocytosis: a prospective cohort study.

Ann Hematol 2020 May 21. Epub 2020 May 21.

Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro Gangnam-gu, Seoul, 06351, South Korea.

Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal condition with various underlying disorders in adult patients and is diagnosed based on the HLH-2004 criteria, which were established based on experience in pediatric patients. However, few studies have prospectively evaluated the treatment outcomes and diagnostic performance of HLH criteria in adult patients with secondary HLH. Thus, we performed a single-center, prospective cohort study of adult patients with suspected HLH, and we analyzed treatment outcomes of patients enrolled between 2017 and 2019 as an interim analysis (ClinicalTrials. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00277-020-04083-6DOI Listing

IL-1 blockade with anakinra in acute leukaemia patients with severe COVID-19 pneumonia appears safe and may result in clinical improvement.

Br J Haematol 2020 May 21. Epub 2020 May 21.

Department of Haematology, University College London Hospitals NHS Foundation Trust, London, United Kingdom.

As of 17th May, 2020 the number of patients infected by coronavirus disease 2019 (COVID-19) worldwide has exceeded 4.5 million (WHO 2020). A subgroup of patients with COVID-19 pneumonia develop a hyperinflammatory syndrome which has a similar cytokine release profile to secondary haemophagocytic lymphohistiocytosis (HLH) (Huang, et al 2020). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/bjh.16873DOI Listing

[Treatment experience of hemophagocytic lymphohistiocytosis with severe central nervous system involvement in a child].

Zhongguo Dang Dai Er Ke Za Zhi 2020 May;22(5):519-522

Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China.

View Article

Download full-text PDF

Source

Early Use of Blood Purification in Severe Epstein-Barr Virus-Associated Hemophagocytic Syndrome.

Pediatrics 2020 May 19. Epub 2020 May 19.

Second Department of Pediatrics, Affiliated Hospital of Zunyi Medical University, Zunyi, China; and

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) is a common type of hemophagocytic lymphohistiocytosis (HLH) that exhibits high rates of morbidity and fatalities. Multiorgan failure caused by Epstein-Barr virus (EBV)-induced hypercytokinemia is one of the main reasons for early deaths. Blood purification techniques have been successfully applied in previously treated hypercytokinemia. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1542/peds.2019-3197DOI Listing

A Case of Pediatric Visceral Leishmaniasis-related Hemophagocytic Lymphohistiocytosis diagnosed by mNGS.

Int J Infect Dis 2020 May 18. Epub 2020 May 18.

Department of Pediatric Intensive Care Unit, Hebei Children's Hospital, Shijiazhuang, Hebei, China.

Background: Visceral leishmaniasis-related hemophagocytic lymphohistiocytosis (VL-HLH) is a secondary hemophagocytic syndrome, which can be life-threatening, caused by and transmitted by infected sandflies. Rapid and accurate identification of is crucial for clinical strategies.

Case Report: Here, we report an infantile infection in a non-epidemic area of China. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijid.2020.05.056DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7233218PMC

Neurologic complications of COVID-19.

Am J Emerg Med 2020 May 16. Epub 2020 May 16.

Department of Emergency Medicine, Rush University Medical Center, Chicago, IL, United States of America.

Background: Much of the focus regarding the global pandemic of coronavirus disease of 2019 (COVID-19) has been on the cardiovascular, pulmonary, and hematologic complications. However, neurologic complications have arisen as an increasingly recognized area of morbidity and mortality.

Objective: This brief report summarizes the neurologic complications associated with COVID-19 with an emphasis on the emergency medicine clinician. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ajem.2020.05.024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229718PMC

Weathering the COVID-19 storm: Lessons from hematologic cytokine syndromes.

Blood Rev 2020 May 15:100707. Epub 2020 May 15.

Division of Hematology, University of British Columbia, Canada.

A subset of patients with severe COVID-19 develop profound inflammation and multi-organ dysfunction consistent with a "Cytokine Storm Syndrome" (CSS). In this review we compare the clinical features, diagnosis, and pathogenesis of COVID-CSS with other hematological CSS, namely secondary hemophagocytic lymphohistiocytosis (sHLH), idiopathic multicentric Castleman disease (iMCD), and CAR-T cell therapy associated Cytokine Release Syndrome (CRS). Novel therapeutics targeting cytokines or inhibiting cell signaling pathways have now become the mainstay of treatment in these CSS. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.blre.2020.100707DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7227559PMC

Inherited chromosomally integrated human herpesvirus-6 in a patient with XIAP deficiency.

Transpl Infect Dis 2020 May 18:e13331. Epub 2020 May 18.

Deprtment of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Tokyo, Japan.

Human herpesvirus-6 (HHV-6) is a common pathogen affecting the human population. Primary HHV-6 infection generally occurs during infancy and causes exanthema subitum. Moreover, HHV-6 may exhibit inherited chromosomally integrated HHV-6 (iciHHV-6) in certain individuals. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/tid.13331DOI Listing

Storm, typhoon, cyclone or hurricane in patients with COVID-19? Beware of the same storm that has a different origin.

RMD Open 2020 05;6(1)

University of Oviedo, Instituto de Investigación Sanitaria del Principado de Asturias, Oviedo, Spain.

Some of the articles being published during the severe acute respiratory syndrome-coronavirus (SARS-CoV)-2 pandemic highlight a link between severe forms of coronavirus disease 2019 (COVID-19) and the so-called cytokine storm, also with increased ferritin levels. However, this scenario is more complex than initially thought due to the heterogeneity of hyperinflammation. Some patients with coronavirus 2019 disease (COVID-19) develop a fully blown secondary haemophagocytic lymphohistiocytosis (sHLH), whereas others, despite a consistent release of pro-inflammatory cytokines, do not fulfil sHLH criteria but still show some features resembling the phenotype of the hyperferritinemic syndrome. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/rmdopen-2020-001295DOI Listing

Children with lymphoma presenting with hemophagocytic lymphohistiocytosis.

Turk J Pediatr 2020 ;62(2):284-288

Department of Pediatric Hematology and Oncology, University of Health Sciences, Dr. Abdurrahman Yurtaslan Ankara Oncology Training and Research Hospital, Ankara, Turkey.

Background: < 7b > Hemophagocytic lymphohistiocytosis (HLH) may precede malignancy, in particular lymphomas and leukemias. However, the causative factors, appropriate treatment and the prognosis of this association is not established.

Case: Herein, we present two patients, one with nodular sclerosing Hodgkin lymphoma (HL) and concomitant Epstein-Barr virus (EBV) infection, and the other with anaplastic large cell lymphoma (ALCL), presented as malignancy associated HLH. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.24953/turkjped.2020.02.016DOI Listing
January 2020

Trigger-dependent differences determine therapeutic outcome in murine primary hemophagocytic lymphohistiocytosis.

Eur J Immunol 2020 May 17. Epub 2020 May 17.

Institute for Immunodeficiency, Center for Chronic Immunodeficiency (CCI), Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.

Familial hemophagocytic lymphohistiocytosis (FHL) is a hyperinflammatory syndrome affecting patients with genetic cytotoxicity defects. Perforin-deficient (PKO) mice recapitulate the full clinical picture of FHL after infection with lymphocytic choriomeningitis virus (LCMV). Hyperactivated CD8 T cells and IFNγ have been identified as the key drivers of FHL and represent targets for therapeutic interventions. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/eji.201948123DOI Listing

Macrophage activation syndrome in children with Kawasaki disease: diagnostic and therapeutic approaches.

World J Pediatr 2020 May 16. Epub 2020 May 16.

Department of Pediatrics, Bucheon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, 327 Sosa-ro, Wonmi-gu, Bucheon, 14647, Republic of Korea.

Background: Macrophage activation syndrome (MAS) is a rare, life-threatening complication of Kawasaki disease (KD). Early recognition and treatment of MAS are very important, but sometimes it is difficult to distinguish MAS from a severe form of KD.

Data Sources: A PubMed search was performed in Clinical Queries using the key terms "macrophage activation syndrome or secondary hemophagocytic lymphohistiocytosis (HLH)" and "Kawasaki disease". Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12519-020-00360-6DOI Listing

Etoposide-based therapy for severe forms of COVID-19.

Med Hypotheses 2020 May 8;142:109826. Epub 2020 May 8.

Laboratory of Acquired and Constitutional Genetic Diseases (MAGECA), Faculty of Medicine, Batna 2 University, Batna, Algeria. Electronic address:

The new coronavirus infection COVID-19 has quickly become a global health emergency. Mortality is principally due to severe Acute Respiratory Distress Syndrome (ARDS) which relays only on supportive treatment. Numerous pathological, clinical and laboratory findings rise the similarity between moderate to severe COVID-19 and haemophagocytic lymphohistiocytosis (HLH). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.mehy.2020.109826DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7207128PMC

FAVORABLE ANAKINRA RESPONSES IN SEVERE COVID-19 PATIENTS WITH SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS.

Cell Host Microbe 2020 May 14. Epub 2020 May 14.

4 Department of Internal Medicine, National and Kapodistrian University of Athens, Medical School, 124 62 Athens, Greece.

Dysregulation of inflammation is hypothesized to play a crucial role in the severe complications of COVID-19, with IL-1/IL-6 pathway being central. Here, we report on the treatment of eight severe COVID-19 pneumonia patients-- seven hospitalized in intensive care units (ICUs) in Greece and one non-ICU patient in the Netherlands-- with the interleukin-1 receptor antagonist Anakinra. All patients scored positive for the hemophagocytosis score (HScore) and were diagnosed with secondary hemophagocytic lymphohistocytosis (sHLH) characterized by pancytopenia, hyper-coagulation, acute kidney injury and hepatobiliary dysfunction. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.chom.2020.05.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7221383PMC

Possible role of low-dose etoposide therapy for hemophagocytic lymphohistiocytosis by COVID-19.

Authors:
Akiyoshi Takami

Int J Hematol 2020 May 12. Epub 2020 May 12.

Division of Hematology, Department of Internal Medicine, Aichi Medical University School of Medicine, 1-1 Yazakokarimata, Nagakute, 480-1195, Japan.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12185-020-02888-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7216850PMC

Hemophagocytic Lymphohistiocytosis in Renal Transplant Recipients: A 2-Case Report.

Transplant Proc 2020 May 7. Epub 2020 May 7.

Renal Transplant Unit, Azienda Ospedaliera Brotzu, Cagliari, Italy.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by an excessive immune activation. HLH can be triggered by a variety of events that disrupt immune homeostasis, such as infections and immunosuppression. HLH presents with heterogeneous clinical symptoms and laboratory findings such as pancytopenia, elevated liver enzymes, impaired renal function, and hyperferritinemia. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.transproceed.2020.02.047DOI Listing

Retrospective single-centre analysis of diagnostic approach to adult-onset haemophagocytic lymphohistiocytosis.

Intern Med J 2020 May 10. Epub 2020 May 10.

Department of Immunology, Canberra Hospital, Woden, ACT, Australia.

Haemophagocytic lymphohistiocytosis (HLH) is rare disorder characterised by immune activation leading to cytokine storm and end-organ failure. It is associated with poor prognosis. HLH is predominantly a disease of early childhood, where it often results from genetic defects that impair cytotoxic function of natural killer cells and T cells. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/imj.14891DOI Listing

Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy.

Best Pract Res Clin Rheumatol 2020 May 7:101515. Epub 2020 May 7.

Division of Rheumatology, University of Washington, Seattle, WA, USA.

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening state of immune hyperactivation that arises in the setting of genetic mutations and infectious, inflammatory, or neoplastic triggers. Sustained, aberrant activation of cytotoxic CD8 T cells and resultant inflammatory cytokine release are core pathogenic mechanisms. Key clinical features include high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.berh.2020.101515DOI Listing

Targeted busulfan-based reduced-intensity conditioning and HLA-matched HSCT cure hemophagocytic lymphohistiocytosis.

Blood Adv 2020 May;4(9):1998-2010

Department of Immunology. Hematology, Oncology and SCT, University Children's Hospital, Zurich, Switzerland.

Reduced-intensity/reduced-toxicity conditioning and allogeneic T-cell replete hematopoietic stem cell transplantation are curative in patients with hemophagocytic lymphohistiocytosis (HLH). Unstable donor chimerism (DC) and relapses are clinical challenges . We examined the effect of a reduced-intensity conditioning regimen based on targeted busulfan to enhance myeloid DC in HLH. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/bloodadvances.2020001748DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7218427PMC

Epstein-Barr Virus Infection-associated Hemophagocytic Lymphohistiocytosis.

Cureus 2020 Apr 6;12(4):e7563. Epub 2020 Apr 6.

Hematology, Dartmouth-Hitchcock Norris Cotton Cancer Center, Lebanon, USA.

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome characterized by uncontrolled immune activation. There is an aberrant activation of lym-phocytes and macrophages that results in hypercytokinemia. We aim to describe a case of secondary HLH due to primary Epstein-Barr virus (EBV) infection. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.7759/cureus.7563DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7202582PMC

Familial Hemophagocytic Lymphohistiocytosis Type 3.

Indian J Pediatr 2020 May 6. Epub 2020 May 6.

Department of Pediatrics, Newborn Medicine, Children's Hospital, Capital Institute of Pediatrics, 2# Yabao Road, Beijing, China.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s12098-020-03295-yDOI Listing
May 2020
0.919 Impact Factor

Chemotherapy-sparing treatment of haemophagocytic lymphohistiocytosis with intravenous immunoglobulins and corticosteroids.

BMJ Case Rep 2020 May 5;13(5). Epub 2020 May 5.

Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Haemophagocytic lymphohistiocytosis (HLH) can be a rapidly fatal disease. Current treatment in adults is extrapolated from the HLH-2004 protocol that specifies a regimen of etoposide, dexamethasone and cyclosporine. However, HLH presents as a spectrum of disease severity. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2020-234490DOI Listing

Genotype characteristics and immunological indicator evaluation of 311 hemophagocytic lymphohistiocytosis cases in China.

Orphanet J Rare Dis 2020 May 6;15(1):112. Epub 2020 May 6.

Department of Hematology, Beijing Friendship Hospital, Capital Medical University, 95 Yong An Road, Xicheng District, Beijing, 10050, China.

Background: Primary hemophagocytic lymphohistiocytosis (pHLH) is a genetic disorder that is classically diagnosed by genetic testing. Secondary HLH (sHLH) is usually caused by infections, malignancies, or autoimmune disorders, but may display some mutations or polymorphisms. Rapid immunological assays examining natural killer (NK) cell activity, degranulation function (CD107a), and protein expression related to genetic deficiencies have been recommended for early pHLH identification. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13023-020-01390-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7201972PMC

An interferon-gamma release assay as a novel biomarker in systemic lupus erythematosus.

Rheumatology (Oxford) 2020 May 6. Epub 2020 May 6.

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

Objective: The mycobacterium tuberculosis (TB) IFN-γ release assay (TB-IGRA) assesses peripheral blood cell release of IFN-γ upon ex vivo exposure to mitogen (IGRA-MT), TB antigen or a negative/nil control (IGRA-NL); IGRA-NL is a measure of spontaneous IFN-γ release (SIR). Here, we investigate the diagnostic associations of elevated SIR and the potential use of IGRA-NL as a novel biomarker in SLE.

Methods: We analysed diagnostic code frequencies among 11 823 individuals undergoing TB-IGRA testing between 2010 and 2015 in a large urban US health-care system. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/keaa161DOI Listing

Emapalumab in Children with Primary Hemophagocytic Lymphohistiocytosis.

N Engl J Med 2020 05;382(19):1811-1822

From the Department of Pediatrics, Sapienza, University of Rome (F.L.), and the Department of Pediatric Hematology-Oncology (F.L.) and Division of Rheumatology (F.D.B.), IRCCS Bambino Gesù Children's Hospital, Rome, Pediatric Hematology-Oncology, Woman and Child Hospital, Azienda Ospedaliera Universitaria Integrata, Verona (S.C.), the Pediatric Hematology-Oncology Unit, Department of Pediatrics, University of Milano-Bicocca, Monza Brianza per il Bambino e la sua Mamma Foundation, Monza (C.R.), the Clinic of Pediatric Hematology-Oncology, University Hospital of Padova, Padua (M.-C.P.), and the Division of Pediatric Onco-Hematology, Regina Margherita Hospital, Turin (F.F.) - all in Italy; the Divisions of Immunobiology and Bone Marrow Transplantation and Immune Deficiency, Department of Pediatrics (M.B.J.), and the Division of Rheumatology (A.G.), Cincinnati Children's Hospital Medical Center, and the University of Cincinnati College of Medicine (M.B.J.) - all in Cincinnati; the Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston (C.A.); the Department of Hematology, Great Ormond Street Hospital for Children, London (A.R.); the Department of Pediatric Hematology-Oncology, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Boston (B.D.); the Center for Cancer and Blood Disorders, Children's Hospital Colorado, Aurora (T.P.G.); the Departments of Pediatric Hematology-Oncology and Hematology and Oncology, Fundación para la Investigación Biomédica Hospital Infantil Universitario Niño Jesús, Centro de Investigación Biomédica en Red de Enfermedades Raras, Madrid (J.S.), and the Department of Pediatric Hematology and Oncology, Hospital Universitari Vall d'Hebron, Barcelona (J.-L.D.D.); the Department of Pediatric Hematology and Oncology, University Children's Hospital, Muenster, Germany (M.A.); the Center for Cancer and Blood Disorders, Phoenix Children's Hospital, Phoenix, AZ (M.H.); NovImmune, Plan-les-Ouates, Switzerland (G.L., M.B., C.M.); and MnS Modelling and Simulation, Dinant, Belgium (P.J.).

Background: Primary hemophagocytic lymphohistiocytosis is a rare syndrome characterized by immune dysregulation and hyperinflammation. It typically manifests in infancy and is associated with high mortality.

Methods: We investigated the efficacy and safety of emapalumab (a human anti-interferon-γ antibody), administered with dexamethasone, in an open-label, single-group, phase 2-3 study involving patients who had received conventional therapy before enrollment (previously treated patients) and previously untreated patients who were 18 years of age or younger and had primary hemophagocytic lymphohistiocytosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1056/NEJMoa1911326DOI Listing

A case of complete atrioventricular block in secondary hemophagocytic syndrome/hemophagocytic lymphohistiocytosis recovered by plasma exchange and cytokine absorbing therapy with AN69ST continuous hemodiafiltration.

Immunol Med 2020 May 6:1-8. Epub 2020 May 6.

Department of Rheumatology, Yokohama Rosai Hospital, Yokohama, Japan.

We report a case of incipient systemic lupus erythematosus (SLE) that rapidly progressed to complete atrioventricular block (cAVB). A 20-year-old man was admitted with facial erythema, painless oral aphtha, polyarthritis, and myalgia of each extremity. On admission, he developed first-degree atrioventricular block, pericarditis, pleuritis, renal failure, hemophagocytic lymphohistiocytosis, neurophychiatric SLE (left cerebellar infarction), and bacteremia. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/25785826.2020.1761145DOI Listing

Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome.

Lancet Rheumatol 2020 May 4. Epub 2020 May 4.

Department of Rheumatology, Sheffield Teaching Hospitals NHS Foundation Trust and Sheffield Children's Hospital NHS Foundation trust, Sheffield, UK.

The term cytokine storm syndromes describes conditions characterised by a life-threatening, fulminant hypercytokinaemia with high mortality. Cytokine storm syndromes can be genetic or a secondary complication of autoimmune or autoinflammatory disorders, infections, and haematological malignancies. These syndromes represent a key area of interface between rheumatology and general medicine. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/S2665-9913(20)30096-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7198216PMC

Successful treatment of mycobacterial infection associated hemophagocytic lymphohistiocytosis with etoposide and anti-tuberculous therapy: a case report.

BMC Infect Dis 2020 May 5;20(1):321. Epub 2020 May 5.

Department of Medical Intensive Care Unit, the Third Affiliated Hospital of Sun Yat-Sen University, No. 600 Tian He Road, Guangzhou, 510630, Guangdong Province, China.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially life-threatening disorder characterized by an exacerbated but ineffective inflammatory response, which can be classified as primary and secondary HLH. HLH associated with Mycobacterium tuberculosis is uncommon. This case report accounted an immunocompetent patient who was confirmed to be Mycobacterium infection, or rather, highly suspected tuberculosis (TB) associated HLH, with a favorable outcome. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12879-020-05016-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7201620PMC

Reactivation of Epstein-Barr Virus Hepatitis in T/Natural Killer (NK) Cells Mimicking Liver T/NK-Cell Lymphoma.

Gastroenterology Res 2020 Apr 22;13(2):81-84. Epub 2020 Apr 22.

Department of Pathology and Laboratory Medicine, Kaiser Permanente Sacramento Medical Center, Sacramento, CA, USA.

Diagnosis of Epstein-Barr virus (EBV)-associated hepatitis, chronic active EBV infection, and EBV-associated lymphoproliferative diseases, is always challenging due to the overlapping symptoms and lack of diagnostic criteria. We report such a case of a 40-year-old man with unremarkable past medical history. He presented with fever of unknown origin for 1 month with jaundice for 2 days. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.14740/gr1283DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7188360PMC

The chimeric antigen receptor-intensive care unit (CAR-ICU) initiative: Surveying intensive care unit practices in the management of CAR T-cell associated toxicities.

J Crit Care 2020 Apr 15;58:58-64. Epub 2020 Apr 15.

Critical Care Medicine, Department of Anesthesiology and Critical Care Medicine, Memorial Sloan Kettering Cancer Center, Weill Cornell Medical College, New York, NY, United States of America.

Purpose: A task force of experts from 11 United States (US) centers, sought to describe practices for managing chimeric antigen receptor (CAR) T-cell toxicity in the intensive care unit (ICU).

Materials And Methods: Between June-July 2019, a survey was electronically distributed to 11 centers. The survey addressed: CAR products, toxicities, targeted treatments, management practices and interventions in the ICU. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jcrc.2020.04.008DOI Listing

FUNCTIONAL AND GENETIC TESTING IN ADULTS WITH HLH DO NOT REVEAL A CYTOTOXICITY DEFECT BUT RATHER AN INFLAMMATORY PROFILE.

Blood 2020 Apr 30. Epub 2020 Apr 30.

Hopital de la Conception, Marseille, France.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory condition. Primary HLH occurs early in life due to monogenic biallelic mutations affecting lymphocyte cytotoxicity. Secondary HLH occurs mostly in adults secondary to infections, lymphoma or rheumatic diseases. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood.2019003664DOI Listing
April 2020
10.452 Impact Factor

Clinical and imaging features in adult patients with bone marrow haemophagocytosis with and without haemophagocytic lymphohistiocytosis: a single-institution experience.

Clin Radiol 2020 Apr 27. Epub 2020 Apr 27.

Department of Radiology, University Hospitals Cleveland Medical Center, 11100 Euclid Ave, Cleveland, OH 44106, USA.

Aim: To evaluate clinical, laboratory, imaging findings, and outcomes of adult patients with bone marrow haemophagocytosis (BMH) who meet the diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH) with those who do not meet the criteria.

Materials And Methods: A pathology database search was performed from 2009 to 2019 to identify adult patients with BMH. Electronic medical records of 41 patients were reviewed to distinguish those who fulfil the HLH-2004 diagnostic guidelines, which identified 22 patients (11 men; mean age, 53. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.crad.2020.03.037DOI Listing

Soluble Interleukin-2 Receptor (sIL-2r) Level is a Limited Test for the Diagnosis of Adult Secondary Hemophagocytic Lymphohistiocytosis (HLH).

Eur J Haematol 2020 Apr 30. Epub 2020 Apr 30.

Tisch Cancer Institute, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Background: Soluble interleukin-2 receptor (sIL-2r) level is used as a diagnostic tool in hemophagocytic lymphohistiocytosis (HLH). However, evidence supporting its use among adults is inadequate.

Objective And Methods: We conducted a retrospective study to assess the performance characteristics of sIL-2r for the diagnosis of adult HLH. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/ejh.13433DOI Listing

HLH Associated with Disseminated Tuberculosis.

N Engl J Med 2020 04;382(18):1749

University of Wisconsin School of Medicine, Madison, WI

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1056/NEJMicm1910558DOI Listing

Use of Tocilizumab for COVID-19-Induced Cytokine Release Syndrome: A Cautionary Case Report.

Chest 2020 Apr 25. Epub 2020 Apr 25.

Division of Infectious Disease, Department of Medicine, Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ.

Novel coronavirus disease 2019 (COVID-19) emerged in late December 2019 in Wuhan, China. Since then, COVID-19 has become a pandemic affecting more than 1.5 million people worldwide. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.chest.2020.04.024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7195070PMC

The Difficulty of Diagnosing Kikuchi-Fujimoto Disease in Infants and Children Under Six Years Old: Case Report and Literature Review.

Authors:
Yasuji Inamo

Cureus 2020 Mar 24;12(3):e7383. Epub 2020 Mar 24.

Pediatrics, Nihon University School of Medicine, Tokyo, JPN.

We came across a 20-month-old boy with Kikuchi-Fujimoto disease (KFD) who showed atypical symptoms that were difficult to diagnose. His symptoms were different from those experienced in common clinical KFD cases. Hence, we report his case presentation and review the literature on the difference in KFD symptoms between infants and young children (under six years of age), and school-age children (6 to 20 years). Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.7759/cureus.7383DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7179985PMC

Case series of three adult patients with exceptional clinical presentations of haemophagocytic lymphohistiocytosis.

Neth J Med 2020 Apr;78(3):136-141

Department of Rheumatology and Clinical immunology, University Medical Center Utrecht, Utrecht, the Netherlands.

Macrophage activation syndrome (MAS) is a secondary form of haemophagocytic lymphohistiocytosis (HLH). MAS-HLH is an underrecognised and life-threatening condition associated with a heterogeneous group of diseases including connective tissue disease and inflammatory disorders. Here, we report three cases of adult patients with MAS-HLH triggered by different entities, including systemic lupus erythematosus, Griscelli syndrome type 2, and Adult onset Still's disease. Read More

View Article

Download full-text PDF

Source

UNC13D mutation presenting as fulminant familial hemophagocytic lymphohistiocytosis.

J Microbiol Immunol Infect 2020 Apr 11. Epub 2020 Apr 11.

Department of Pediatrics, National Taiwan University Children's Hospital, Taipei, Taiwan. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jmii.2020.04.001DOI Listing

Hemophagocytic Lymphohistiocytosis in Patients With Primary Immunodeficiency.

J Pediatr Hematol Oncol 2020 Apr 21. Epub 2020 Apr 21.

Department of Pediatrics, Division of Pediatric Immunology.

Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled and excessive immune responses with high mortality. We aimed to define mortality-related parameters in HLH secondary to primary immunodeficiency (PID). A total of 28 patients with HLH between the years 2013 and 2017 were enrolled in the study. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MPH.0000000000001803DOI Listing

IL6-R blocking with tocilizumab in critically ill patients with hemophagocytic syndrome.

Crit Care 2020 04 22;24(1):166. Epub 2020 Apr 22.

Département de Néphrologie et Transplantation d'organes, Centre Hospitalier Universitaire de Toulouse, F-31000, Toulouse, France.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13054-020-02878-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7175448PMC

A man in his forties with increasing shortness of breath.

Tidsskr Nor Laegeforen 2020 Apr 23;140(6). Epub 2020 Mar 23.

Background: Diffuse large B-cell lymphoma is an aggressive non-Hodgkin lymphoma. The patients are often critically ill with a variety of symptoms, but the disease is potentially curable.

Case Presentation: A previously healthy man in his forties was admitted to the local hospital feeling unwell, with dyspnoea, cough, fever and weight loss. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4045/tidsskr.19.0692DOI Listing

[Relationship between Single Nucleotide Polymorphisms of IL2RA, IL-10 Gene and Epstein-Barr Virus Associated Hemophagocytic Lymphohistiocytosisin in children].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2020 Apr;28(2):646-651

Department of Pediatrics, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou 510000, Guangdong Province, China,E-mail: com.

Objective: To investigate the correlation of single nucleotide polymorphisms (SNPs) of IL2RA and IL-10 gene with the pathogenesis of Epstein Barr Virus associated hemophagocytic lymphohistiocytosis (EBV-HLH) in children and the effect of correlated SNPs on the prognosis of children with EBV-HLH.

Methods: For EBV-HLH group (51 cases), EBV-associated infectious mononucleosis (EBV-IM) group (48 cases) and EBV-positive healthy children group (52 cases), the genotypes at rs2104286, rs12722489, rs11594656 of IL2RA gene and rs1800896, rs1800871 and rs1800872 of IL-10 gene were detected with the SNaPshot technique. The distribution differences of genotype frequency and allele frequency of each SNP in each group were analyzed, and the correlated SNPs were taken as the research object for survival analysis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.19746/j.cnki.issn.1009-2137.2020.02.048DOI Listing

Association of soluble interleukin-2 receptor alpha with laboratory parameters and clinical findings of hemophagocytic lymphohistiocytosis patients: The first report from South of Iran.

J Educ Health Promot 2020 28;9:48. Epub 2020 Feb 28.

Department of Pathology, Shiraz University of Medical Sciences, Shiraz, Iran.

Introduction: Hemophagocytic lymphohistiocytosis (HLH) is caused by overactivation of immune system. Gene mutations, infections, malignant, and autoimmune trigger the development of the disease.

Materials And Methods: Clinical data and peripheral blood samples of 21 patients suspected of HLH were collected in Shiraz Medical Centers 2017-2018. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4103/jehp.jehp_579_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7161657PMC
February 2020

A Rare and Fatal Case of Hemophagocytic Lymphohistiocytosis Associated with Sarcoidosis.

Am J Case Rep 2020 Apr 21;21:e921306. Epub 2020 Apr 21.

Department of Rheumatology, Henry Ford Hospital, Detroit, MI, USA.

BACKGROUND Sarcoidosis is a systemic inflammatory disorder characterized by a classic pathologic feature of non-caseating granulomas involving any organ system. Hemophagocytic lymphohistiocytosis (HLH) is a catastrophic cytokine surge characterized by dysregulation of the macrophage response, which can be rapidly fatal. Recognition of HLH has been increasing over the past decade. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.12659/AJCR.921306DOI Listing

Enterovirus-associated hemophagocytic lymphohistiocytosis with multiorgan failure.

Proc (Bayl Univ Med Cent) 2020 Apr 6;33(2):248-250. Epub 2020 Mar 6.

Division of Infectious Diseases, Department of Internal Medicine, Baylor University Medical CenterDallasTexas.

Hemophagocytic lymphohistiocytosis is a highly fatal hyperinflammatory syndrome that is increasingly being recognized in adults. It can be primary or secondary in the setting of malignancy, autoimmune disorders, infections, or acquired immune deficiencies. We present a case of a 50-year-old man with enterovirus-associated multiorgan system dysfunction and hemophagocytic lymphohistiocytosis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/08998280.2020.1731051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7155957PMC

[Clinical analysis of 81 adult patients with hemophagocytic lymphohistiocytosis].

Zhonghua Xue Ye Xue Za Zhi 2020 Mar;41(3):248-250

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3760/cma.j.issn.0253-2727.2020.03.011DOI Listing

Outcome analysis of pediatric hemophagocytic lymphohistiocytosis.

J Formos Med Assoc 2020 Apr 16. Epub 2020 Apr 16.

Division of Critical Care, Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan, ROC; Division of Cardiology, Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Kaohsiung, Taiwan, ROC. Electronic address:

Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease associated with rapid clinical deterioration and the need for intensive care; therefore, it is essential to identify clinical parameters related to mortality and establish prognostic factors correlated with unfavorable outcome in high risk patients whose treatment may fail.

Methods: Between January 2004 and December 2018, a total of 51 pediatric patients (less than 18 years old) who fulfilled the diagnostic criteria of HLH-2004 with documented results of bone marrow investigations at Kaohsiung Chang Gung Memorial Hospital were enrolled. The treatment protocol was based on hemophagocytic lymphohistiocytosis-94 (HLH-94) and HLH-2004. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jfma.2020.03.025DOI Listing

Severe cerebral involvement in adult-onset hemophagocytic lymphohistiocytosis.

J Clin Neurosci 2020 Apr 16. Epub 2020 Apr 16.

Department of Internal Medicine, Division of Neurology, Lahey Hospital and Medical Center, Burlington, MA 01805, United States; Departments of Neurology and Neurosurgery, Tufts University School of Medicine, Boston, MA 02111, United States. Electronic address:

The diagnosis of hemophagocytic lymphohistiocytosis (HLH) with cerebral involvement is challenging given the rarity of HLH and its resemblance to the much more common severe sepsis. Timely diagnosis and treatment may be lifesaving. We report two cases demonstrating different and rare forms of severe brain involvement in adult patients with HLH: acute necrotizing encephalopathy, and diffuse hemorrhagic disease due to disseminated intravascular coagulation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jocn.2020.04.054DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7162634PMC