4,857 results match your criteria Lymphohistiocytosis


Quantitative analysis of the natural history of prolidase deficiency: description of 17 families and systematic review of published cases.

Genet Med 2021 May 26. Epub 2021 May 26.

Department of Dermatology, University Hospital Münster, Münster, Germany.

Purpose: Prolidase deficiency is a rare inborn error of metabolism causing ulcers and other skin disorders, splenomegaly, developmental delay, and recurrent infections. Most of the literature is constituted of isolated case reports. We aim to provide a quantitative description of the natural history of the condition by describing 19 affected individuals and reviewing the literature. Read More

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Postpartum disseminated HSV-1 infection with hemophagocytic lymphohistiocytosis and fulminant neonatal herpes infection.

J Infect Dis 2021 May 26. Epub 2021 May 26.

Department of Infectious Diseases, Aarhus University Hospital, Aarhus, Denmark.

The present study describes a 19-year-old woman with systemic herpes simplex virus (HSV)-1 infection postpartum, and a fatal course of neonatal herpesvirus infection. The mother experienced an unusual disease course with hemophagocytic lymphohistiocytosis (HLH) and persistence of HSV-1 DNA for 15 weeks. Functional investigation of cells from the mother demonstrated significantly impaired induction of antiviral interferons and cytokines in response to viruses and various ligands in the context of normal activation of the transcription factors NF-κB and IRF3. Read More

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Multiple-Organ Involvement in Familial Hemophagocytic Lymphohistiocytosis Type 2 Shown on FDG PET/CT.

Clin Nucl Med 2021 May 26. Epub 2021 May 26.

From the Department of Nuclear Medicine, Beijing Friendship Hospital of Capital Medical University, Xi Cheng District, Beijing, China.

Abstract: FDG PET/CT in an 18-year-old man with known familial hemophagocytic lymphohistiocytosis (HLH) type 2 demonstrated abnormally in the brain, liver, and lymph nodes. Pathology showed no sign of lymphoproliferative disease. The patient underwent the chemotherapy of HLH. Read More

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Therapy-related acute myeloid leukemia with KMT2A-SNX9 gene fusion associated with a hyperdiploid karyotype after hemophagocytic lymphohistiocytosis.

Cancer Genet 2021 May 7;256-257:86-90. Epub 2021 May 7.

Pediatric Hematology-Oncology Program, Research Center, Instituto Nacional de Câncer, Rio de Janeiro, Brazil. Electronic address:

Therapy-related acute myeloid leukemia (t-AML) following treatment with topoisomerase-II inhibitors has been increasingly reported. These compounds (e.g. Read More

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Rubella vaccine-induced granulomas are a novel phenotype with incomplete penetrance of genetic defects in cytotoxicity.

J Allergy Clin Immunol 2021 May 22. Epub 2021 May 22.

Division of Pediatric Hematology and Oncology, Department of Pediatrics, University Hospital Erlangen, Erlangen, Germany.

Background: Rubella virus-induced granulomas have been described in patients with various inborn errors of immunity. Most defects impair T-cell immunity, suggesting a critical role of T cells in rubella elimination. However, the molecular mechanism of virus control remains elusive. Read More

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Polymorphisms and haplotypes of IL2RA, IL10, IFNG, IRF5, and CCR2 are associated with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children.

Pediatr Blood Cancer 2021 May 24:e29097. Epub 2021 May 24.

Division of Hematology/Oncology, Department of Pediatrics, The Seventh Affiliated Hospital, Sun Yat-Sen University, Shenzhen, China.

Objective: Cytokine storms are central to the development of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH). Previous studies have shown that single-nucleotide polymorphisms (SNPs) of cytokine genes may be associated with the development of EBV-HLH in children. As such, we investigated the association between susceptibility to EBV-HLH in children and SNPs and haplotypes of genes encoding interleukin-2 receptor subunit alpha (IL2RA), interleukin-10 (IL10), interferon gamma (IFNG), interferon regulatory factor 5 (IRF5), and C-C chemokine receptor 2 (CCR2). Read More

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Case Report: Visceral Leishmaniasis and Hemophagocytic Lymphohistiocytosis: Three Clinical Cases, Three Different Pattern.

Am J Trop Med Hyg 2021 May 24. Epub 2021 May 24.

1Hematology Service and Transfusional Unit, University Hospital-Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil.

Visceral leishmaniasis (VL) is a neglected tropical disease with more than 30,000 cases annually reported worldwide. In Brazil, about 3,700 cases are annually reported. The VL clinical presentation is variable, from asymptomatic to severe cases with a high risk of death. Read More

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Juvenile myelomonocytic leukemia in CBL syndrome associated with germline splice-site mutations: Two case reports and a literature review.

Clin Case Rep 2021 May 15;9(5):e04260. Epub 2021 May 15.

Translational Research in Pediatric Oncology, Hematopoietic Transplantation & Cell Therapy Hospital La Paz Institute for Health Research (INGEMM-IdiPAZ) Madrid Spain.

The clinical and laboratory criteria for hemophagocytic lymphohistiocytosis should be taken into account during the juvenile myelomonocytic leukemia diagnosis, specifically in CBL syndrome, to reveal the presence of primary rather than secondary associated hemophagocytosis. Read More

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A case report of idiopathic Hemophagocytic lymphohistiocytosis in an immunocompetent adult.

Clin Case Rep 2021 May 28;9(5):e04006. Epub 2021 Mar 28.

Hematology Department Centro Hospitalar do Baixo Vouga Aveiro Portugal.

Hemophagocytic lymphohistiocytosis poses a diagnostic dilemma due to the absence of specific clinical and laboratory findings, especially in adults. Despite greater recognition of the disease, secondary idiopathic forms are still reported. Read More

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Enzyme replacement therapy and hematopoietic stem cell transplant: a new paradigm of treatment in Wolman disease.

Orphanet J Rare Dis 2021 May 21;16(1):235. Epub 2021 May 21.

Department of Blood and Marrow Transplantation, Royal Manchester Children's Hospital, Oxford Road, Manchester, UK.

Background: Wolman disease is a rare, lysosomal storage disorder in which biallelic variants in the LIPA gene result in reduced or complete lack of lysosomal acid lipase. The accumulation of the substrates; cholesterol esters and triglycerides, significantly impacts cellular function. Untreated patients die within the first 12 months of life. Read More

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Macrophage Activation Syndrome in a Case of Myasthenia Gravis with Concurrent Cytomegalovirus Infection.

Acta Neurol Taiwan 2020 Dec;29(4):114-118

Department of Neurology, Shin Kong Wu Ho- Su Memorial Hospital.

Objective: Macrophage activation syndrome (MAS) or reactive hemophagocytic lymphohistiocytosis (HLH) refers to a set of clinical symptoms caused by the excessive activation and proliferation of macrophages. It was linked with autoimmune disease such as systemic-onset juvenile rheumatoid arthritis, systemic lupus erythematosus, rheumatoid arthritis, and dermatomyositis, etc. Herein we report a case of myasthenia gravis (MG) with concurrent cytomegalovirus (CMV) infection developed MAS. Read More

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December 2020

Analysis of cytokine risk factors in the early death of patients with secondary phagocytic lymphocytic histiocytosis.

Am J Transl Res 2021 15;13(4):2388-2398. Epub 2021 Apr 15.

Department of Hematology, The First People's Hospital of Yunnan Province Kunming, China.

Secondary hemophagocytic lymphohistiocytosis (sHLH) is an excessive inflammatory response syndrome caused by immune abnormalities. Up to date, the risk factors for cytokines causing early death in sHLH patients have not been elucidated. Our study reviewed the cytokine expression levels in peripheral blood of 50 sHLH patients. Read More

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Pediatric Hemophagocytic Lymphohistiocytosis - A Single Center Study of 118 Patients.

Indian Pediatr 2021 May 20. Epub 2021 May 20.

Department of Pediatric Hematology Oncology and BMT unit, Institute of Child Health, Sir Ganga Ram Hospital, New Delhi, India. Correspondence to: Dr Anupam Sachdeva, Director Pediatric Hematology Oncology and BMT, Institute of Child Health, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi 110 060, India.

Objective: To describe the epidemiological features, outcomes and prognostic factors in diagnosis of pediatric hemophagocytic lymphohistiocytosis (HLH).

Methods: Retrospective observational study. 118 children fulfilled the inclusion criteria for HLH between January, 2010 and December, 2019. Read More

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A recently explored aspect of the iceberg named COVID-19: multisystem inflammatory syndrome in children (MIS-C).

Turk Arch Pediatr 2021 Jan 1;56(1):3-9. Epub 2021 Jan 1.

Department of Pediatric Rheumatology, İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, İstanbul, Turkey.

Humanity has recently gained a novel foe named coronavirus disease 2019. Although data so far mostly suggest that children are more likely to have a favorable disease course, new concerns have been raised because of recently reported pediatric cases with hyperinflammatory conditions resembling Kawasaki disease, toxic shock syndrome, and macrophage activation syndrome/hemophagocytic lymphohistiocytosis. Because the increasing evidence suggests that this recent hyperinflammatory condition emerged in the coronavirus disease 2019 era is a distinct clinical picture, the Centers for Disease Control and Prevention named this novel disease multisystem inflammatory syndrome in children. Read More

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January 2021

Gastric cancer complicated with hemophagocytic lymphohistiocytosis: case report and a brief review.

J Gastrointest Oncol 2021 Apr;12(2):892-899

Department of Gastrointestinal Surgery, Peking University People's Hospital, Beijing, China.

Hemophagocytic lymphohistiocytosis (HLH) comprises a group of severe immune function disorders that can lead to immune-mediated organ damage. There are two subtypes of HLH: primary and secondary. Secondary HLH is associated with infectious, oncologic, chemotherapeutic, and other underlying causes, and studies on HLH triggered by tumors have mainly focused on hematological malignancies. Read More

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Complicated case of COVID-19 disease with overlapping features of thrombotic thrombocytopenic purpura and haemophagocytic lymphohistiocytosis.

BMJ Case Rep 2021 May 19;14(5). Epub 2021 May 19.

Clinical Hematology, King George's Medical University, Lucknow, Uttar Pradesh, India

Haemophagocytic lymphohistiocytosis has been reported as an uncommon complication of severe COVID-19 disease while thrombotic thrombocytopenic purpura has been rarely reported. Here, we are reporting a 21-year-old man who developed a combination of these complications during the hospital stay in the post-COVID-19 recovery period. He presented with fever and bilateral COVID-19-related pneumonia requiring invasive ventilation. Read More

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[Successful treatment of hemophagocytic syndrome caused by auricle infection: one case report].

Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2021 May;56(5):493-495

Department of Otorhinolaryngology Head and Neck Surgery, the Affiliated Yantai Yuhuangding Hospital of Qingdao University, Yantai 264000, China.

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Analysis of the occurrence of hemophagocytic lymphohistiocytosis (HLH) features in patients with sepsis: a prospective study.

Sci Rep 2021 May 18;11(1):10529. Epub 2021 May 18.

Department of Adults' Infectious Diseases, Medical University of Warsaw, 61 Żwirki i Wigury St., 02-091, Warsaw, Poland.

HLH syndrome may mimic sepsis but requires entirely different treatment. The aim of the study was to assess the occurrence of HLH features in patients with sepsis and the influence these exert on the patients' prognosis. The prospective study included 108 patients with suspected sepsis who were routinely evaluated according to HLH criteria. Read More

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Reactions Related to CAR-T Cell Therapy.

Front Immunol 2021 28;12:663201. Epub 2021 Apr 28.

Department of General Surgery, Second Hospital of Lanzhou University, Lanzhou, China.

The application of chimeric antigen receptor (CAR) T-cell therapy as a tumor immunotherapy has received great interest in recent years. This therapeutic approach has been used to treat hematological malignancies solid tumors. However, it is associated with adverse reactions such as, cytokine release syndrome (CRS), immune effector cell-associated neurotoxicity syndrome (ICANS), off-target effects, anaphylaxis, infections associated with CAR-T-cell infusion (CTI), tumor lysis syndrome (TLS), B-cell dysplasia, hemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) and coagulation disorders. Read More

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Hemophagocytic lymphohistiocytosis associated with Epstein-Barr virus-positive diffuse large B-cell lymphoma, NOS of bone marrow-liver-spleen type: an autopsy case report.

J Clin Exp Hematop 2021 Jun 14;61(2):102-108. Epub 2021 May 14.

Department of Hematology, Juntendo University Urayasu Hospital, Chiba, Japan.

Lymphoma-associated hemophagocytic lymphohistiocytosis (HLH) has a significantly poor prognosis among secondary HLH. We describe the rare case of a 74-year-old female with secondary HLH presenting with a rapidly fatal course. Post-mortem examination revealed Epstein-Barr virus (EBV) -positive diffuse large B-cell lymphoma (DLBCL). Read More

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Limited utility of the HScore in detecting secondary haemophagocytic lymphohistiocytosis in COVID-19: response.

Br J Haematol 2021 May 16. Epub 2021 May 16.

Department of Haematological Medicine, King's College Hospital-NHS Foundation Trust, London, UK.

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Prostate Cancer and Reactive Haemophagocytic Lymphohistiocytosis.

Eur J Case Rep Intern Med 2021 1;8(4):002425. Epub 2021 Apr 1.

Medical Oncology Department, Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium.

We report the case of a 76-year-old man presenting with reactive haemophagocytic lymphohistiocytosis (rHLH) in the setting of disseminated prostate cancer. This often fatal syndrome must be diagnosed early in order to maximize survival. Treatment should be initiated whenever the clinical diagnosis is suspected, even if the HLH-2004 criteria are not met. Read More

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Lenalidomide-Associated Hemophagocytic Lymphohistiocytosis With Plasma Cell Phagocytosis.

Cureus 2021 Apr 10;13(4):e14409. Epub 2021 Apr 10.

Hematology and Oncology, Brooke Army Medical Center, San Antonio, USA.

Hemophagocytic lymphohistiocytosis (HLH) is a severe systemic inflammatory syndrome that is often fatal. In the adult population, it is believed to develop secondary to immune dysregulation due to rheumatologic, infectious, malignant, and recently, immunomodulatory drugs. It's co-occurrence with phagocytosis by non-macrophage cells has not been previously well defined. Read More

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Macrophage Activation in COVID-19 Patients in Intensive Care Unit.

J Med Cases 2020 Jul 29;11(7):211-214. Epub 2020 Jun 29.

Service de Reanimation Medicale, Groupe Hospitalier de la Region Mulhouse Sud Alsace, Mulhouse, France.

We report six cases of patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, admitted to intensive care unit (ICU), for whom bone marrow aspirate revealed hemophagocytosis. We compared their clinical presentation and laboratory findings to those that can be encountered during a hemophagocytic lymphohistiocytosis. These observations might evoke a macrophage activation mechanism different from the one encountered in the hemophagocytic lymphohistiocytosis (HLH). Read More

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Macrophage activation syndrome in systemic juvenile idiopathic arthritis.

Authors:
Masaki Shimizu

Immunol Med 2021 May 13:1-9. Epub 2021 May 13.

Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

Macrophage activation syndrome (MAS) is a severe, potentially life-threatening complication of systemic juvenile idiopathic arthritis (s-JIA). An immunological feature is the excessive activation and proliferation of T lymphocytes and macrophages. Massive hypercytokinemia is strongly associated with its pathogenesis, particularly the overproduction of interleukin (IL)-1, IL-6 and IL-18; interferon (IFN)-γ; and tumor necrosis factor (TNF)-α. Read More

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Ruxolitinib, a JAK1/2 Inhibitor, Ameliorates Cytokine Storm in Experimental Models of Hyperinflammation Syndrome.

Front Pharmacol 2021 22;12:650295. Epub 2021 Apr 22.

Incyte Research Institute, Wilmington, DE, United States.

Hyperinflammatory syndromes comprise a heterogeneous group of disorders characterized by severe inflammation, multiple organ dysfunction, and potentially death. In response to antigenic stimulus (e.g. Read More

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[Clinical features and treatment outcome of patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis].

Zhonghua Xue Ye Xue Za Zhi 2021 Apr;42(4):324-331

Department of Hematology, Huadong Hospital, Fudan University, Shanghai 200040, China.

To investigate the clinical features and effect of prognostic factors in patients with different pathological types of non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis. We collected and analyzed the clinical data of 89 patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis who were treated at Huadong Hospital from March 2013 to May 2020. The data were analyzed via log-rank and Cox multivariate analyses. Read More

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Case 14-2021: A 64-Year-Old Woman with Fever and Pancytopenia.

N Engl J Med 2021 05;384(19):1849-1857

From the Department of Medicine, Newton-Wellesley Hospital, Newton (M.D.G.), and the Departments of Radiology (D.P.M.), Medicine (A.W., M.B.B.), and Pathology (J.A.V.), Massachusetts General Hospital, and the Departments of Radiology (D.P.M.), Medicine (A.W., M.B.B.), and Pathology (J.A.V.), Harvard Medical School, Boston - all in Massachusetts.

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Anaplasmosis with associated haemophagocytic lymphohistiocytosis.

Br J Haematol 2021 May 11. Epub 2021 May 11.

Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.

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