5,421 results match your criteria Lymphohistiocytosis

EBV protection- and susceptibility-related HLA alleles and EBV status in the Chinese population: A single-center study.

Immun Inflamm Dis 2022 Jul;10(7):e666

Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing, People's Republic of China.

Background: Although most adults are infected by Epstein-Barr virus (EBV), some patients develop highly lethal diseases associated with EBV infection, including EBV-hemophagocytic lymphohistiocytosis (EBV-HLH), chronic active EBV infections (CAEBV), and lymphoma, the pathogeneses of which remain to be investigated. The human leukocyte antigen (HLA) complex may be associated with the viral infection pathway, and, therefore, HLA alleles may be associated with EBV-related diseases and subpopulations of infected cells, studies related to EBV-associated diseases, and subpopulations of infected cells that were conducted in China are scarce.

Methods: In this study, we analyzed the high-resolution HLA genotypes of 269 patients with EBV-associated diseases and 213 EBV-seronegative hematopoietic stem cell donors using PCR-SBT assay and elucidated the associations of HLA-A, -B, -C, -DRB1, and -DQB1 alleles with EBV-associated diseases in the Chinese population, Benjamini-Hochberg correction to adjust for multiple testing. Read More

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Brain Magnetic Resonance Imaging Findings of Pediatric Hemophagocytic Lymphohistiocytosis Could Be Diagnostic and Life-Saving.

Pediatr Neurol 2022 May 31;133:40-47. Epub 2022 May 31.

Department of Pediatric Neurology, Selçuk University Faculty of Medicine, Konya, Turkey.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare and fatal disease and may also present with central nervous system findings at the beginning without specific diagnostic criteria. Brain magnetic resonance imaging (MRI) findings are diverse and can also be diagnostic. We aimed to emphasize the importance of brain MRI findings in the early diagnosis of this fatal disease. Read More

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Recurrent parvovirus B19 viremia resulting in two episodes of hemophagocytic lymphohistiocytosis.

Virol J 2022 Jun 25;19(1):107. Epub 2022 Jun 25.

Department of Gastroenterology, Hepatology and Infectious Diseases, Medical Faculty and University Hospital Düsseldorf, Heinrich-Heine-University, Düsseldorf, Germany.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory condition with uncontrolled activation of lymphocytes and macrophages. Besides a primary (genetic) form, HLH can also be triggered by malignant, autoimmune and infectious diseases. HLH recurrences are rarely described, usually only in primary HLH. Read More

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COVID-19, haemophagocytic lymphohistiocytosis, and infection-induced cytokine storm syndromes.

Lancet Infect Dis 2022 Jul;22(7):937-938

Department of Medicine, University of British Columbia, Vancouver, BC V5Z1M9, Canada; Centre for Health Education Scholarship, University of British Columbia, Vancouver, BC V5Z1M9, Canada. Electronic address:

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Bone Marrow Histology in Hemophagocytic Lymphohistiocytosis.

Arch Pathol Lab Med 2022 Jun 23. Epub 2022 Jun 23.

The Graduate Institute of Microbiology, College of Medicine, National Taiwan University (Teng), Taipei, Taiwan.

Context.—: Bone marrow (BM) samples are obtained through aspiration and trephine biopsy. Hemophagocytic lymphohistiocytosis (HLH) has been largely studied in BM aspirate smears. Read More

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Peripheral platelet phagocytosis in an extremely low birth weight infant:a case report.

Fukushima J Med Sci 2022 Jun 23. Epub 2022 Jun 23.

Department of Pediatrics, Tokyo University School of Medicine.

A 768 g female neonate, born at 25 weeks' gestation, developed sepsis due to methicillin-resistant Staphylococcus epidermidis on day 14. Severe thrombocytopenia was observed, and hemophagocytic macrophages were identified in her peripheral blood smear. Cytokine profiles at the time of onset suggested that an inflammatory cytokine storm had activated lymphocytes and macrophages, leading to platelet phagocytosis. Read More

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Hemophagocytic Lymphohistiocytosis Associated With Hemolytic Uremic Syndrome in a Child: A Case Report and Systematic Literature Review.

J Pediatr Hematol Oncol 2022 Jul 13;44(5):e905-e910. Epub 2021 Aug 13.

Hematology, University of Health Sciences Ankara Training and Research Hospital, Ankara, Turkey.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder resulting from excessive activation and nonmalignant proliferation of T-lymphocytes and macrophages. Hemolytic uremic syndrome (HUS) is a disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Reports pertaining to the association between HLH and HUS are rarely published; however, we report on a 4-year-old boy who was diagnosed with both conditions and treated successfully with high-dose steroid and intravenous immunoglobulin. Read More

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Use of rituximab in SARS-CoV-2-positive renal transplant recipient with EBV reactivation and probable haemophagocytic lymphohistiocytosis.

CEN Case Rep 2022 Jun 22. Epub 2022 Jun 22.

Department of Clinical Nephrology and Transplantation, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.

We present a case of a rapid clinical recovery in a critically ill kidney transplant recipient with SARS-CoV-2 positivity, Epstein-Barr virus (EBV) reactivation and probable secondary hemophagocytic lymphohistiocytosis (HLH) treated with etoposide-free regimen, based on dexamethasone and a single dose of rituximab. Although rituximab is often a part of EBV-HLH treatment strategy, its use in simultaneous Coronavirus 2019 disease (COVID-19) and solid-organ transplantation has not been reported yet. We review the current evidence for the potential of SARS-CoV-2 to trigger EBV reactivation, leading to a severe clinical illness. Read More

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[Interpretation of Chinese expert consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis associated with lymphoma (2022)].

N Wei Z Wang

Zhonghua Yi Xue Za Zhi 2022 Jun;102(24):1779-1781

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

Chinese expert consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis (HLH) associated with lymphoma was revised on the basis of evidence-based medical evidence in combination with domestic and international research progress. It added the classification of "according to the difference in the causes of HLH". For the first time, the revised version included the classification of lymphoma-associated HLH induced by immunotherapy. Read More

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Dramatic recovery after etoposide phosphate infusion for hemophagocytic lymphohistiocytosis/macrophage activation syndrome following treatment with tisagenlecleucel in a young patient with relapsed acute lymphoblastic leukemia: a case report.

Acta Haematol 2022 Jun 20. Epub 2022 Jun 20.

The occurrence of a secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome (HLH/MAS) after CAR-T cells infusion is very rare and mostly fatal. Treatment recommendations for such a complication are not yet established. Here we report the dramatic recovery of a HLH/MAS following tisagenlecleucel infusion in a young patient with relapsed acute lymphoblastic leukemia using etoposide phosphate (EP). Read More

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Drugs for paediatric hyperinflammatory syndromes.

Drugs Context 2022 27;11. Epub 2022 May 27.

Department of Paediatrics and Adolescent Medicine, University of Hong Kong, Hong Kong.

Background: Many syndromes are associated with exaggerated inflammation. Children with hyperinflammatory syndromes often present with vague and non-specific symptoms that pose diagnostic and management challenges. The recent literature seems biased towards referring these syndromes only to the multisystem inflammatory syndrome in children (MIS-C) that is associated with COVID-19. Read More

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Case Report: Chimeric Antigen Receptor T Cells Induced Late Severe Cytokine Release Syndrome.

Front Oncol 2022 1;12:893928. Epub 2022 Jun 1.

Department of Hematology, Nanfang Hospital, Southern Medical University, Guangzhou, China.

Background: Severe cytokine release syndrome (sCRS) has emerged as an adverse complication in the early period of chimeric antigen receptor T cell (CART) therapy, while whether sCRS occurs in the late period remains unknown. Here, we reported two patients with late sCRS.

Case Presentation: Case 1 was a 34-year-old female with refractory Philadelphia chromosome-positive B cell acute lymphoblastic leukemia. Read More

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Clinical Profile, Treatment, and Outcome of Patients with Secondary Hemophagocytic Lymphohistiocytosis in Critically Ill Patients: A Prospective Observational Study.

Indian J Crit Care Med 2022 May;26(5):564-567

Department of Medicine, AIIMS, New Delhi, India.

Introduction: The objective of the study was to evaluate the clinical profile and outcome of patients with secondary hemophagocytic lymphohistiocytosis (HLH) in critically ill patients.

Materials And Methods: A prospective observational study was conducted where critically ill adult patients presenting with fever and bicytopenia were evaluated according to the HLH-2004 diagnostic criteria for the presence of secondary HLH. The underlying trigger, clinical profile, treatment, and outcome of patients with HLH were analyzed. Read More

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Secondary Hemophagocytic Lymphohistiocytosis: Think of the Devil Lurking!

Indian J Crit Care Med 2022 May;26(5):545-546

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

Pannu AK, Bhalla A. Secondary Hemophagocytic Lymphohistiocytosis: Think of the Devil Lurking! Indian J Crit Care Med 2022;26(5):545-546. Read More

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Baseline clinical features predicting macrophage activation syndrome in patients with systemic adult-onset Still's disease receiving interleukin-6 inhibitor treatment.

Int J Rheum Dis 2022 Jun 19. Epub 2022 Jun 19.

Department of Respiratory Medicine, Allergy and Clinical Immunology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan.

Aim: Macrophage activation syndrome (MAS), a severe complication of systemic adult-onset Still's disease (AOSD), has been reported to occur during interleukin-6 (IL-6) inhibitor treatment. However, predictors for MAS development are unknown. Therefore, this study investigated predictive features for MAS development after starting IL-6 inhibitor treatment in systemic AOSD patients. Read More

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Severe hemophagocytic lymphohistiocytosis in kidney transplant recipient: the etiology is on the tip of the tongue!

Intensive Care Med 2022 Jun 18. Epub 2022 Jun 18.

Medical Intensive Care Unit, Hôpital Saint-Louis, Assistance Publique Hôpitaux de Paris (APHP), Université de Paris, 1 avenue Claude Vellefaux, 75010, Paris, France.

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Pediatric hemophagocytic lymphohistiocytosis in a tropical country: Results of a multicenter study in Thailand.

Asia Pac J Clin Oncol 2022 Jun 17. Epub 2022 Jun 17.

Division of Hematology/Oncology, Department of Pediatrics, Phramongkutklao Hospital and Phramongkutklao College of Medicine, Bangkok, Thailand.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition caused by genetic mutation or various triggers disturbing the immune system.

Methods: A multicenter retrospective study of pediatric patients with HLH receiving a diagnosis between January 2005 and December 2019 from three pediatric oncology centers was conducted to explore the clinical characteristics and determine prognostic factors associated with outcomes among Thai children.

Results: In all, 78 patients with HLH with a median age at diagnosis of 3. Read More

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HLH treatment: smarter, not harder.

Blood 2022 Jun;139(24):3453-3455

Boston Children's Hospital; and.

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Comprehensive Serum Proteome Profiling of Cytokine Release Syndrome and Immune Effector Cell Associated Neurotoxicity Syndrome in B-Cell ALL Patients Receiving CART19.

Clin Cancer Res 2022 Jun 15. Epub 2022 Jun 15.

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

Purpose: Study the biology and identify markers of severe cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS) in children post chimeric antigen receptor T-cell (CART) treatment.

Experimental Design: We use comprehensive proteomic profiling to measure over 1400 serum proteins at multiple serial timepoints in a cohort of patients with B-cell acute lymphoblastic leukemia treated with the CD19 targeted CART CTL019 on two clinical trials.

Results: Identified FLT3 and MILR1 as pre-infusion predictive biomarkers of severe CRS. Read More

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Hemophagocytic lymphohistiocytosis diagnosed by bone marrow trephine biopsy in living post-COVID-19 patients: case report and mini-review.

J Mol Histol 2022 Jun 14. Epub 2022 Jun 14.

Department of Pathology, Faculty of Medicine, Medical School, School of Health Sciences, University of Thessaly, Biopolis, 41500, Larissa, Greece.

Hemophagocytic lymphohistiocytosis (HLH) constitutes a life-threatening inflammatory syndrome. Postmortem histological findings of bone marrow (BM) from COVID-19 patients showed histiocytosis and hemophagocytosis and supported the hypothesis that secondary HLH (sHLH) may be triggered by SARS-CoV-2 infection. However, there are a limited number of sHLH cases in which trephine has been performed in living post-COVID-19 patients. Read More

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Iron overload disorders.

Hepatol Commun 2022 Jun 14. Epub 2022 Jun 14.

Liver Institute Northwest and Elson S. Floyd College of Medicine, Washington State University, Washington, USA.

Iron overload disorders represent a variety of conditions that lead to increased total body iron stores and resultant end-organ damage. An elevated ferritin and transferrin-iron saturation can be commonly encountered in the evaluation of elevated liver enzymes. Confirmatory homeostatic iron regulator (HFE) genetic testing for C282Y and H63D, mutations most encountered in hereditary hemochromatosis, should be pursued in evaluation of hyperferritinemia. Read More

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Hemophagocytic lymphohistiocytosis-like presentation of malignant melanoma.

Int J Lab Hematol 2022 Jun 14. Epub 2022 Jun 14.

Department of Internal Medicine and Dermatology, CHU Réunion, Saint Denis, France.

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Murine typhus complicated by sHLH mimicking adult-onset Still's disease.

Rev Med Interne 2022 Jun 10. Epub 2022 Jun 10.

Department of Internal Medicine, University Hospital Lyon Croix-Rousse, Claude-Bernard University - Lyon 1, Lyon, France; University Claude-Bernard Lyon 1, Research on Healthcare Performance (RESHAPE), INSERM U1290, Lyon, France.

Introduction: Adult-onset Still's disease (AOSD) is a rare multisystemic disorder and a diagnostic challenge for physicians because of the wide range of differential diagnoses. Common features of AOSD and secondary hemophagocytic lymphohistiocytosis (sHLH) could favour diagnostic uncertainty, in particular in case of infection-related sHLH.

Observation: A 61-year-old man was admitted to our internal medicine department for suspected AOSD. Read More

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[Research Advance of the Mechanisms, Clinical Characteristics and Treatment Strategy of Coagulation Dysfunction in Hemophagocytic Lymphohistiocytosis --Review].

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2022 Jun;30(3):959-964

Center of Hematology, The First Affiliated Hospital of Nanchang University, Institute of Hematology, Academy of Clinical Medicine of Jiangxi Province, Institute of Lymphoma and Myeloma in Nanchang University, Nanchang 330006, Jiangxi Province,

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease caused by uncontrolled proliferation of activated macrophage, and secreting high amounts of inflammatory cytokines which lead to multi-organ dysfunction syndrome. HLH patients often show different clinical characteristics during the disease was progressed, in which coagulopathy were the most common, including thrombocytopenia and hypofibrinogenemia, those are the major cause of death in patients, and the clinicians should increase awareness of the mechanisms, clinical characteristics, prognosis and treatment. In this review, the above problems are briefly summarized, to deepen understanding of the HLH related coagulation dysfunctions, and early identification and treatment to reduce mortality, so as to provide more opportunities for HLH patients to recieve subsequent treatment. Read More

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Favorable outcomes with durable chimerism after hematopoietic cell transplantation using busulfan and fludarabine-based reduced-intensity conditioning for pediatric patients with hemophagocytic lymphohistiocytosis.

Blood Res 2022 Jun 10. Epub 2022 Jun 10.

Division of Pediatric Hematology/Oncology, Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul, Korea.

Introduction: The incorporation of a reduced-intensity conditioning (RIC) regimen in hematopoietic cell transplantation (HCT) for patients with hemophagocytic lymphohistiocytosis (HLH) has decreased early mortality but is associated with a high rate of mixed chimerism and graft failure. Here, we present a successful single-center experience using busulfan and a fludarabine-based RIC regimen for the treatment of HLH.

Methods: The medical records of pediatric patients with HLH who underwent HCT using a busulfan/fludarabine-based RIC regimen between January 2008 and December 2017 were reviewed retrospectively. Read More

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Genome Editing With TALEN, CRISPR-Cas9 and CRISPR-Cas12a in Combination With AAV6 Homology Donor Restores T Cell Function for XLP.

Front Genome Ed 2022 23;4:828489. Epub 2022 May 23.

Molecular and Cellular Immunology, UCL Great Ormond Street Institute of Child Health, London, United Kingdom.

X-linked lymphoproliferative disease is a rare inherited immune disorder, caused by mutations or deletions in the gene that encodes an intracellular adapter protein SAP (Slam-associated protein). SAP is essential for mediating several key immune processes and the immune system - T cells in particular - are dysregulated in its absence. Patients present with a spectrum of clinical manifestations, including haemophagocytic lymphohistiocytosis (HLH), dysgammaglobulinemia, lymphoma and autoimmunity. Read More

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Aggressive Natural Killer Cell Leukemia in an Adolescent Patient: A Case Report and Literature Review.

Front Pediatr 2022 23;10:829927. Epub 2022 May 23.

Key Laboratory of Birth Defects and Related Diseases of Women and Children, Ministry of Education, West China Second University Hospital, Sichuan University, Chengdu, China.

Aggressive natural killer cell leukemia (ANKL) is a rare malignant tumor, especially uncommon in children. ANKL has very aggressive clinical course and bad prognosis and is usually caused by Epstein-Barr virus infection. ANKL often has clinical manifestations of hemophagocytic lymphohistiocytosis (HLH) and can be easily treated as HLH, which might complicate this aggressive disease. Read More

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Hemophagocytosis on ascitic fluid cytology: Diagnosis of HLH.

Diagn Cytopathol 2022 Jun 8. Epub 2022 Jun 8.

Department of Pathology and Laboratory Medicine, Henry Ford Hospital, Detroit, Michigan, USA.

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of pathologic immune response characterized by excessive activation of macrophages. Hemophagocytosis is one of the diagnostic criteria for HLH, and it usually involves the bone marrow, spleen, lymph nodes, or any part of the reticuloendothelial system. Hemophagocytosis in the ascitic fluid has rarely been reported in HLH. Read More

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Familial hemophagocytic lymphohistiocytosis hepatitis is mediated by IFN-γ in a predominantly hepatic-intrinsic manner.

PLoS One 2022 7;17(6):e0269553. Epub 2022 Jun 7.

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

Interferon gamma (IFN-γ) is the main cytokine driving organ dysfunction in Familial Hemophagocytic Lymphohistiocytosis (FHL). Blockade of IFN-γ pathway ameliorates FHL hepatitis, both in animal models and in humans with FHL. Hepatocytes are known to express IFN-γ receptor (IFN-γ-R). Read More

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