☆ 4.5 Review

Cytokine Storm Syndrome

ANNUAL REVIEW OF MEDICINE (2023)

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Article Rheumatology

Severe delayed hypersensitivity reactions to IL-1 and IL-6 inhibitors link to common HLA-DRB1*15 alleles

Vivian E. Saper et al.

Summary: Drug reaction with eosinophilia and systemic symptoms (DRESS) can occur in Still's disease patients treated with IL-1/IL-6 inhibitors, with features such as eosinophilia, liver enzyme elevation, and persistent rash. HLA-DRB1*15 haplotypes are significantly enriched in Still's disease with DRESS. Pre-prescription HLA typing and vigilance for serious reactions to these drugs are recommended.

ANNALS OF THE RHEUMATIC DISEASES (2022)

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Article Hematology

An improved index for diagnosis and mortality prediction in malignancy-associated hemophagocytic lymphohistiocytosis

Adi Zoref-Lorenz et al.

Summary: This study found that the optimized HLH inflammatory (OHI) index, which combines levels of soluble CD25 and ferritin, can effectively diagnose HLH and predict mortality in patients with HMs. Additionally, the index can identify high-risk patients who may not be diagnosed with HLH according to traditional criteria.

BLOOD (2022)

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Article Rheumatology

Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 20 Moroccan adult patients

Ammouri Wafa et al.

Summary: This study aimed to investigate the clinical and laboratory manifestations, triggers, treatment, and outcome of macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE). The study found that fever, splenomegaly, lymphadenopathy, profound cytopenia, hyperferritinemia, hypertriglyceridemia, high SLEDAI, and H-Score were associated with MAS. Early diagnosis and urgent therapeutic management improved the overall prognosis.

CLINICAL RHEUMATOLOGY (2022)

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Review Obstetrics & Gynecology

Management of hemophagocytic lymphohistiocytosis in pregnancy: Case series study and literature review

Congcong Liu et al.

Summary: This study retrospectively analyzed the clinical characteristics and treatment options of 13 patients with hemophagocytic lymphohistiocytosis (HLH) during pregnancy. The results showed that early diagnosis and treatment are crucial for maternal survival, and corticosteroids are the preferred treatment for most patients.

JOURNAL OF OBSTETRICS AND GYNAECOLOGY RESEARCH (2022)

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Review Rheumatology

Janus kinase-targeting therapies in rheumatology: a mechanisms-based approach

Yoshiya Tanaka et al.

Summary: This Review discusses the therapeutic use of Janus kinase inhibitors in autoimmune rheumatic diseases, highlighting their disease-specific mechanisms of action. JAK inhibitors suppress intracellular signaling mediated by multiple cytokines, offering potential treatment options for a range of immune and inflammatory disorders.

NATURE REVIEWS RHEUMATOLOGY (2022)

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Article Allergy

Childhood multisystem inflammatory syndrome associated with COVID-19 (MIS-C): Distinct from Kawasaki disease or part of the same spectrum?

Maria Santaniello et al.

Summary: One of the most challenging and intriguing phenomena during the COVID-19 pandemic is the multisystem inflammatory syndrome in children, which shares similarities with Kawasaki disease but also presents with different symptoms.

PEDIATRIC ALLERGY AND IMMUNOLOGY (2022)

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Article Rheumatology

Who Would Have Predicted Multisystem Inflammatory Syndrome in Children?

Daniel D. Reiff et al.

Summary: This review provides an in-depth analysis of multisystem inflammatory disease in children (MIS-C) following COVID-19. It discusses the clinical findings, pathophysiology, imaging and laboratory studies, treatment algorithms, and disease outcomes. MIS-C can be challenging to diagnose due to its non-specific presentation, but accurate diagnosis can be achieved through common laboratory features, imaging findings, and historical clues, enabling appropriate treatment with therapies such as IVIG. Aggressive treatment leads to good outcomes, and longitudinal studies shed light on long-term cardiac sequelae and recovery.

CURRENT RHEUMATOLOGY REPORTS (2022)

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Article Immunology

A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China

Ziyuan Shen et al.

Summary: This study developed a prognostic model for adult HLH in China, providing a feasible and accurate stratification system for individualized treatment strategy. The model can help improve the prognosis of patients.

FRONTIERS IN IMMUNOLOGY (2022)

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Review Hematology

Malignancy-associated haemophagocytic lymphohistiocytosis

Audi Setiadi et al.

Summary: Haemophagocytic lymphohistiocytosis (HLH) is an inflammatory syndrome that can occur with cancer or immune-activating therapies for cancer. Familial HLH is characterized by uncontrolled activation of macrophages and cytotoxic T cells, while the pathophysiology of malignancy-associated HLH is not well understood. Distinguishing HLH from cancer-related abnormalities is challenging and emerging diagnostic tools are discussed. Targeted therapy, including cytokine blockade and JAK-STAT inhibition, may improve outcomes. The framework of cytokine storm syndrome can help understand malignancy-associated HLH.

LANCET HAEMATOLOGY (2022)

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Review Hematology

Haemophagocytic lymphohistiocytosis and Epstein-Barr virus: a complex relationship with diverse origins, expression and outcomes

Nader Kim El-Mallawany et al.

Summary: Epstein-Barr virus (EBV) is associated with various presentations of haemophagocytic lymphohistiocytosis (HLH), which can be life-threatening. Patients may have genetic defects or underlying infection/malignancy, with EBV playing a significant role. Increased awareness and understanding of EBV-related complications are crucial for improving survival rates.

BRITISH JOURNAL OF HAEMATOLOGY (2022)

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Article Rheumatology

Cytokine profile, ferritin and multi-visceral involvement characterize macrophage activation syndrome during adult-onset Still's disease

Piero Ruscitti et al.

Summary: The study found that in patients with adult-onset Still's disease complicated by macrophage activation syndrome, levels of cytokines (IL-1, IL-6, IFN-gamma, etc.) and inflammatory markers (ferritin) were elevated. Analysis of circulating immune cells in MAS patients showed an increase in 'classical monocytes' and a reduction in total NK cells. A total of 3477 IFN-related genes were identified in AOSD synovial tissues.

RHEUMATOLOGY (2022)

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Review Infectious Diseases

Hemophagocytic lymphohistiocytosis: An unusual presentation of disseminated tuberculosis: A case report and literature review

Poornachandra et al.

Summary: This article presents a case of acquired HLH associated with Mycobacterial tuberculosis infection, emphasizing the importance of early diagnosis and treatment for improving the clinical outcome of the patient.

JOURNAL OF CLINICAL TUBERCULOSIS AND OTHER MYCOBACTERIAL DISEASES (2022)

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Article Oncology

Diagnostic strategy for trigger identification in severe reactive hemophagocytic lymphohistiocytosis: A diagnostic accuracy study

Jean-Jacques Tudesq et al.

Summary: Management of reactive hemophagocytic lymphohistiocytosis (rHLH) requires early recognition, identification of triggers, and adequate treatment to reduce mortality. Tissue biopsies have a high diagnostic yield in identifying triggers in severe rHLH cases. While splenectomy carries a procedural risk, it can be reserved for cases where diagnostic uncertainty persists. Early initiation of appropriate therapy based on trigger identification leads to better outcomes in patients with rHLH.

HEMATOLOGICAL ONCOLOGY (2021)

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Article Rheumatology

Predicting Macrophage Activation Syndrome in Childhood-onset Systemic Lupus Erythematosus Patients at Diagnosis

Maya Gerstein et al.

Summary: The study aimed to differentiate macrophage activation syndrome (MAS) from active childhood-onset systemic lupus erythematosus (cSLE) and generated decision rules based on clinical and laboratory features. Recursive partitioning identified serum ferritin as the best discriminator between MAS and non-MAS patients. Validation in independent cohorts is needed to confirm the findings.

JOURNAL OF RHEUMATOLOGY (2021)

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Article Medicine, General & Internal

Baricitinib plus Remdesivir for Hospitalized Adults with Covid-19

A. C. Kalil et al.

Summary: Combination treatment with baricitinib plus remdesivir was superior to remdesivir alone in reducing recovery time and accelerating improvement in clinical status among patients with Covid-19, notably among those receiving high-flow oxygen or noninvasive ventilation. The combination was associated with fewer serious adverse events.

NEW ENGLAND JOURNAL OF MEDICINE (2021)

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Review Immunology

Use of the JAK Inhibitor Ruxolitinib in the Treatment of Hemophagocytic Lymphohistiocytosis

Camille Keenan et al.

Summary: Hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome driven by overactive T cells and macrophages that abundantly secrete pro-inflammatory cytokines. Etoposide-based regimens have reduced mortality associated with HLH, but the 5-year survival rate remains low. Novel cytokine-directed therapies, such as ruxolitinib, are being studied to improve outcomes in HLH.

FRONTIERS IN IMMUNOLOGY (2021)

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Article Immunology

Usefulness of Interleukin-18 as a Diagnostic Biomarker to Differentiate Adult-Onset Still's Disease With/Without Macrophage Activation Syndrome From Other Secondary Hemophagocytic Lymphohistiocytosis in Adults

Toshihiko Shiga et al.

Summary: The study suggests that IL-18 could be a useful biomarker for the differential diagnosis of AOSD and adult HLH. Serum IL-18 levels were significantly higher in the AOSD group compared to the adult HLH group, and were closely correlated with ferritin, soluble interleukin-2 receptor (sIL-2R), and other laboratory data.

FRONTIERS IN IMMUNOLOGY (2021)

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Editorial Material Medicine, Research & Experimental

A perspective on the potential detrimental role of inflammation in pig orthotopic heart xenotransplantation

Charles P. Thompson et al.

Summary: The inflammatory response following surgical procedures and xenograft exposure may have detrimental effects on pig heart graft function and recipient baboon pulmonary function, as well as impacting immune response to the graft. Strategies to reduce the inflammatory response, such as white blood cell filtration and immunosuppressive therapy, could potentially improve outcomes of genetically engineered pig heart transplantation.

XENOTRANSPLANTATION (2021)

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Review Medicine, General & Internal

Successful ruxolitinib administration for a patient with steroid-refractory idiopathic pneumonia syndrome following hematopoietic stem cell transplantation: A case report and literature review

Dan Tomomasa et al.

Summary: IPS is an acute lung complication that occurs after HSCT, and ruxolitinib may be an alternative treatment option for patients with severe IPS, as shown in a case of a myelodysplastic syndrome patient who had undergone a second HSCT.

CLINICAL CASE REPORTS (2021)

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Article Pediatrics

Kawasaki Disease and Systemic Juvenile Idiopathic Arthritis - Two Ends of the Same Spectrum

Ellen Go et al.

Summary: This study describes the disease course of a small proportion of patients with both Kawasaki disease and systemic juvenile idiopathic arthritis, characterized by refractory Kawasaki disease, high prevalence of coronary artery dilatation, and shared immunopathology potentially linking the two conditions.

FRONTIERS IN PEDIATRICS (2021)

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Article Hematology

Characterization of HLH-like manifestations as a CRS variant in CD22 CAR T cells

Daniel A. Lichtenstein et al.

Summary: In a subset of patients with cytokine release syndrome (CRS), chimeric antigen receptor (CAR) T-cell toxicities resembling hemophagocytic lymphohistiocytosis (HLH) can occur, known as carHLH. This study comprehensively characterized the manifestations and timing of carHLH, highlighting factors such as preinfusion natural killer cell lymphopenia and higher bone marrow T-cell:NK cell ratio associated with its development. Additionally, carHLH was characterized by persistent elevation of HLH-associated inflammatory cytokines following CAR T-cell expansion.

BLOOD (2021)

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Article Medicine, General & Internal

Association Between Administration of IL-6 Antagonists and Mortality Among Patients Hospitalized for COVID-19 AMeta-analysis

Manu Shankar-Hari et al.

Summary: This study identified 27 clinical trials and found that the use of IL-6 antagonists was associated with lower 28-day all-cause mortality in patients hospitalized for COVID-19 compared to usual care or placebo. Different risk effects were observed between tocilizumab and sarilumab, increasing understanding of the treatment with these drugs.

JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION (2021)

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Article Biochemistry & Molecular Biology

Early treatment of COVID-19 with anakinra guided by soluble urokinase plasminogen receptor plasma levels: a double-blind, randomized controlled phase 3 trial

Evdoxia Kyriazopoulou et al.

Summary: The SAVE-MORE phase 3 study demonstrated the efficacy of anakinra, an IL-1 alpha/beta inhibitor, in patients with COVID-19 and high serum levels of soluble plasminogen activator receptor, showing significant reduction in clinical deterioration and decreased 28-day mortality.

NATURE MEDICINE (2021)

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Editorial Material Biochemistry & Molecular Biology

Calming the cytokine storm in COVID-19

Randy Q. Cron et al.

Summary: Cytokine blockade can improve the survival rate of COVID-19 patients at risk of respiratory failure, with timing and patient selection being crucial factors.

NATURE MEDICINE (2021)

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Article Rheumatology

Performance of Cytokine Storm Syndrome Scoring Systems in Pediatric COVID-19 and Multisystem Inflammatory Syndrome in Children

Daniel D. Reiff et al.

Summary: This study evaluated the effectiveness of different scoring systems in identifying patients with cytokine storm syndrome in pediatric COVID-19 and MIS-C. Some systems were found to be more suitable for identifying CSS, but existing scoring systems might not fully capture the breadth of the disease processes in these two conditions.

ACR OPEN RHEUMATOLOGY (2021)

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Letter Hematology

Dose-adjusted EPOCH regimen as first-line treatment for non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis: a single-arm, open-label, phase II trial

Jin-Hua Liang et al.

HAEMATOLOGICA (2020)

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Letter Hematology

Ruxolitinib for refractory/relapsed hemophagocytic lymphohistiocytosis

Jingshi Wang et al.

HAEMATOLOGICA (2020)

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Article Rheumatology

Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis

Esraa M. Eloseily et al.

ARTHRITIS & RHEUMATOLOGY (2020)

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Review Pediatrics

Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management

Lauren A. Henderson et al.

PEDIATRIC DRUGS (2020)

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Article Medicine, General & Internal

Clinical features of patients infected with 2019 novel coronavirus in Wuhan, China

Chaolin Huang et al.

LANCET (2020)

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Review Genetics & Heredity

The genetics of macrophage activation syndrome

Grant S. Schulert et al.

GENES AND IMMUNITY (2020)

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Article Medicine, Research & Experimental

Clinical and immunological features of severe and moderate coronavirus disease 2019

Guang Chen et al.

JOURNAL OF CLINICAL INVESTIGATION (2020)

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Article Oncology

CD4/CD8 T-Cell Selection Affects Chimeric Antigen Receptor (CAR) T-Cell Potency and Toxicity: Updated Results From a Phase I Anti-CD22 CAR T-Cell Trial

Nirali N. Shah et al.

JOURNAL OF CLINICAL ONCOLOGY (2020)

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Article Rheumatology

On the Alert for Cytokine Storm: Immunopathology in COVID-19

Lauren A. Henderson et al.

ARTHRITIS & RHEUMATOLOGY (2020)

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Review Immunology

Diagnosis and Management of Secondary HLH/MAS Following HSCT and CAR-T Cell Therapy in Adults; A Review of the Literature and a Survey of Practice Within EBMT Centres on Behalf of the Autoimmune Diseases Working Party (ADWP) and Transplant Complications Working Party (TCWP)

Robert David Sandler et al.

FRONTIERS IN IMMUNOLOGY (2020)

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Article Medicine, General & Internal

Emapalumab in Children with Primary Hemophagocytic Lymphohistiocytosis

F. Locatelli et al.

NEW ENGLAND JOURNAL OF MEDICINE (2020)

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Article Hematology

Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis-a dangerous disease

Zhili Jin et al.

ANNALS OF HEMATOLOGY (2020)

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Article Oncology

Ruxolitinib combined with doxorubicin, etoposide, and dexamethasone for the treatment of the lymphoma-associated hemophagocytic syndrome

Lanlan Zhou et al.

JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY (2020)

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Article Hematology

Frequency and spectrum of disease-causing variants in 1892 patients with suspected genetic HLH disorders

Vanessa Gadoury-Levesque et al.

BLOOD ADVANCES (2020)

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Article Oncology

The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis

Sakshi Bami et al.

PEDIATRIC BLOOD & CANCER (2020)

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Article Rheumatology

Systemic juvenile idiopathic arthritis and recurrent macrophage activation syndrome due to a CASP1 variant causing inflammasome hyperactivation

Sofie E. Jorgensen et al.

RHEUMATOLOGY (2020)

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Article Allergy

Highways to hell: Mechanism-based management of cytokine storm syndromes

Scott W. Canna et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY (2020)

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Review Medicine, General & Internal

Cytokine Storm

David C. Fajgenbaum et al.

NEW ENGLAND JOURNAL OF MEDICINE (2020)

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Article Hematology

Recommendations for the management of hemophagocytic lymphohistiocytosis in adults

Paul La Rosee et al.

BLOOD (2019)

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Article Oncology

Haemophagocytic lymphohistiocytosis in patients treated with immune checkpoint inhibitors: analysis of WHO global database of individual case safety reports

Roberta Noseda et al.

JOURNAL FOR IMMUNOTHERAPY OF CANCER (2019)

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Article Rheumatology

Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis

Francesca Minoia et al.

ANNALS OF THE RHEUMATIC DISEASES (2019)

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Article Hematology

ASTCT Consensus Grading for Cytokine Release Syndrome and Neurologic Toxicity Associated with Immune Effector Cells

Daniel W. Lee et al.

BIOLOGY OF BLOOD AND MARROW TRANSPLANTATION (2019)

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Review Critical Care Medicine

Extracorporeal life support and systemic inflammation

Abdulrahman Al-Fares et al.

INTENSIVE CARE MEDICINE EXPERIMENTAL (2019)

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Article Medicine, General & Internal

Lamotrigine-induced hemophagocytic lymphohistiocytosis with Takotsubo cardiomyopathy: a case report

Jenny Y. Zhou et al.

JOURNAL OF MEDICAL CASE REPORTS (2019)

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Article Rheumatology

Clinical features of macrophage activation syndrome as the onset manifestation of juvenile systemic lupus erythematosus

Satoshi Sato et al.

RHEUMATOLOGY ADVANCES IN PRACTICE (2019)

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Article Hematology

Bone marrow histomorphological criteria can accurately diagnose hemophagocytic lymphohistiocytosis

Eric Gars et al.

HAEMATOLOGICA (2018)

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Article Oncology

FDA Approval Summary: Tocilizumab for Treatment of Chimeric Antigen Receptor T Cell-Induced Severe or Life-Threatening Cytokine Release Syndrome

Robert Q. Le et al.

ONCOLOGIST (2018)

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Article Hematology

Bone marrow histomorphological criteria can accurately diagnose hemophagocytic lymphohistiocytosis

Eric Gars et al.

HAEMATOLOGICA (2018)

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Article Allergy

Recommendations for the Use of Etoposide-Based Therapy and Bone Marrow Transplantation for the Treatment of HLH: Consensus Statements by the HLH Steering Committee of the Histiocyte Society

Stephan Ehl et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE (2018)

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Article Genetics & Heredity

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

Tenzin Gayden et al.

NATURE GENETICS (2018)

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Review Infectious Diseases

Hemophagocytic Lymphohistiocytosis Associated with Scrub Typhus: Systematic Review and Comparison between Pediatric and Adult Cases

Tameto Naoi et al.

TROPICAL MEDICINE AND INFECTIOUS DISEASE (2018)

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Letter Allergy

Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition

Scott W. Canna et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY (2017)

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Review Rheumatology

Cytokine Storm Syndrome: Looking Toward the Precision Medicine Era

Edward M. Behrens et al.

ARTHRITIS & RHEUMATOLOGY (2017)

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Article Immunology

Whole-Exome Sequencing Reveals Mutations in Genes Linked to Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Fatal Cases of H1N1 Influenza

Grant S. Schulert et al.

JOURNAL OF INFECTIOUS DISEASES (2016)

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Article Immunology

A Heterozygous RAB27A Mutation Associated with Delayed Cytolytic Granule Polarization and Hemophagocytic Lymphohistiocytosis

Mingce Zhang et al.

JOURNAL OF IMMUNOLOGY (2016)

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Article Rheumatology

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative

Angelo Ravelli et al.

ARTHRITIS & RHEUMATOLOGY (2016)

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Article Hematology

Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis

Yini Wang et al.

BLOOD (2015)

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Article Hematology

Hemophagocytic lymphohistiocytosis caused by dominant-negative mutations in STXBP2 that inhibit SNARE-mediated membrane fusion

Waldo A. Spessott et al.

BLOOD (2015)

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Article Hematology

Marked hyperferritinemia does not predict for HLH in the adult population

Alison M. Schram et al.

BLOOD (2015)

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Review Hematology

Ehrlichia-Induced Hemophagocytic Lymphohistiocytosis: A Case Series and Review of Literature

Zaher K. Otrock et al.

BLOOD CELLS MOLECULES AND DISEASES (2015)

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Article Immunology

Failed CTL/NK cell killing and cytokine hypersecretion are directly linked through prolonged synapse time

Misty R. Jenkins et al.

JOURNAL OF EXPERIMENTAL MEDICINE (2015)

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Editorial Material Rheumatology

Does Viral Hemorrhagic Fever Represent Reactive Hemophagocytic Syndrome?

Randy Q. Cron et al.

JOURNAL OF RHEUMATOLOGY (2015)

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Article Medicine, General & Internal

Reactive Hemophagocytic Syndrome in Adults: A Retrospective Analysis of 162 Patients

Sebastien Riviere et al.

AMERICAN JOURNAL OF MEDICINE (2014)

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Article Hematology

XMEN disease: a new primary immunodeficiency affecting Mg2+ regulation of immunity against Epstein-Barr virus

Feng-Yen Li et al.

BLOOD (2014)

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Article Biophysics

Etoposide-containing conditioning regimen reduces the occurrence of hemophagocytic lymphohistiocytosis after SCT

R. Kobayashi et al.

BONE MARROW TRANSPLANTATION (2014)

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Article Hematology

Risk factor analysis of non-Hodgkin lymphoma-associated haemophagocytic syndromes: a multicentre study

Hirozumi Sano et al.

BRITISH JOURNAL OF HAEMATOLOGY (2014)

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Article Hematology

Hemophagocytic syndrome in patients with acute myeloid leukemia undergoing intensive chemotherapy

Karen Delavigne et al.

HAEMATOLOGICA (2014)

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Article Immunology

Ebola Hemorrhagic Fever: Novel Biomarker Correlates of Clinical Outcome

Anita K. McElroy et al.

JOURNAL OF INFECTIOUS DISEASES (2014)

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Article Medicine, General & Internal

Prognostic Factors and Outcomes of Adults With Hemophagocytic Lymphohistiocytosis

Sameer A. Parikh et al.

MAYO CLINIC PROCEEDINGS (2014)

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Article Rheumatology

Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

Laurence Fardet et al.

ARTHRITIS & RHEUMATOLOGY (2014)

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Article Hematology

Cytokine release syndrome after blinatumomab treatment related to abnormal macrophage activation and ameliorated with cytokine-directed therapy

David T. Teachey et al.

BLOOD (2013)

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Article Hematology

Treatment of Epstein Barr virus-induced haemophagocytic lymphohistiocytosis with rituximab-containing chemo-immunotherapeutic regimens

DeepakBabu Chellapandian et al.

BRITISH JOURNAL OF HAEMATOLOGY (2013)

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Review Rheumatology

Reaching the Threshold: A Multilayer Pathogenesis of Macrophage Activation Syndrome

Raffaele Strippoli et al.

JOURNAL OF RHEUMATOLOGY (2013)

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Review Genetics & Heredity

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

A. Ravelli et al.

GENES AND IMMUNITY (2012)

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Article Hematology

X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the disease

Claire Booth et al.

BLOOD (2011)

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Article Oncology

Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center

Maciej Machaczka et al.

LEUKEMIA & LYMPHOMA (2011)

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Review Oncology

HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis

Jan-Inge Henter et al.

PEDIATRIC BLOOD & CANCER (2007)

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Article Hematology

A proportion of patients with lymphoma may harbor mutations of the perforin gene

R Clementi et al.

BLOOD (2005)

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Article Hematology

An animal model of hemophagocytic lymphohistiocytosis (HLH):: CD8+ T cells and interferon gamma are essential for the disorder

MB Jordan et al.

BLOOD (2004)

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