4.6 Review

Hemophagocytic lymphohistiocytosis as an etiology of bone marrow failure

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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.
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Pathology updates and diagnostic approaches to haemophagocytic lymphohistiocytosis

Alexander Kikuchi et al.

Summary: Haemophagocytic lymphohistiocytosis is a complex and often underestimated immune dysregulation syndrome that can occur in various clinical scenarios. Timely and accurate diagnosis is crucial for patient survival, requiring a high level of clinical suspicion. The presence of haemophagocytosis in certain clinical situations should prompt consideration of this syndrome and further testing.

HISTOPATHOLOGY (2022)

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)

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)

Article Hematology

RhoG deficiency abrogates cytotoxicity of human lymphocytes and causes hemophagocytic lymphohistiocytosis

Artem Kalinichenko et al.

Summary: This study identifies RhoG as a novel essential regulator in aiding the retention of cytotoxic granules near the plasma membrane, facilitating their release for the elimination of infected and malignant cells. The interaction between RhoG and Munc13-4 is crucial for the docking of cytotoxic granules to the plasma membrane and subsequent membrane fusion, shedding light on the molecular pathomechanism behind genetically determined forms of hemophagocytic lymphohistiocytosis.
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Pediatric hemophagocytic lymphohistiocytosis

Scott W. Canna et al.

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Emapalumab in Children with Primary Hemophagocytic Lymphohistiocytosis

F. Locatelli et al.

NEW ENGLAND JOURNAL OF MEDICINE (2020)

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Cytokine Storm

David C. Fajgenbaum et al.

NEW ENGLAND JOURNAL OF MEDICINE (2020)

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Hermansky-Pudlak syndrome type II and lethal hemophagocytic lymphohistiocytosis: Case description and review of the literature

Fabiola Dell'Acqua et al.

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY-IN PRACTICE (2019)

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Hemophagocytic Lymphohistiocytosis

Hanny Al-Samkari et al.

ANNUAL REVIEW OF PATHOLOGY: MECHANISMS OF DISEASE, VOL 13 (2018)

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Epstein-Barr Virus and Hemophagocytic Lymphohistiocytosis

Rebecca A. Marsh

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Cytotoxic T Cells Use Mechanical Force to Potentiate Target Cell Killing

Roshni Basu et al.

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Malignancy-associated haemophagocytic lymphohistiocytosis in children and adolescents

Kai Lehmberg et al.

BRITISH JOURNAL OF HAEMATOLOGY (2015)

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The unique aspects of presentation and diagnosis of hemophagocytic lymphohistiocytosis in adults

Sarah Nikiforow et al.

HEMATOLOGY-AMERICAN SOCIETY OF HEMATOLOGY EDUCATION PROGRAM (2015)

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Perforin and granzymes: function, dysfunction and human pathology

Ilia Voskoboinik et al.

NATURE REVIEWS IMMUNOLOGY (2015)

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Impact of interferon-γ on hematopoiesis

Alexander M. de Bruin et al.

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Mutation of NLRC4 causes a syndrome of enterocolitis and autoinflammation

Neil Romberg et al.

NATURE GENETICS (2014)

Editorial Material Pediatrics

Hemophagocytic Lymphohistiocytosis: Advances in Pathophysiology, Diagnosis, and Treatment

Shanmuganathan Chandrakasan et al.

JOURNAL OF PEDIATRICS (2013)

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A quarter century of granzymes

C. L. Ewen et al.

CELL DEATH AND DIFFERENTIATION (2012)

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Hemophagocytosis causes a consumptive anemia of inflammation

Erin E. Zoller et al.

JOURNAL OF EXPERIMENTAL MEDICINE (2011)

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Perforin deficiency and susceptibility to cancer

A. J. Brennan et al.

CELL DEATH AND DIFFERENTIATION (2010)

Article Multidisciplinary Sciences

Quiescent haematopoietic stem cells are activated by IFN-γ in response to chronic infection

Megan T. Baldridge et al.

NATURE (2010)

Article Medicine, Research & Experimental

Neutralization of IFNγ defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice

Jana Pachlopnik Schmid et al.

EMBO MOLECULAR MEDICINE (2009)

Article Hematology

Aplastic anemia

Neal S. Young et al.

CURRENT OPINION IN HEMATOLOGY (2008)

Review Immunology

Formation and function of the lytic NK-cell immunological synapse

Jordan S. Orange

NATURE REVIEWS IMMUNOLOGY (2008)

Article Hematology

Nationwide survey of hemophagocytic lymphohistiocytosis in Japan

Eiichi Ishii et al.

INTERNATIONAL JOURNAL OF HEMATOLOGY (2007)

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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis

Jan-Inge Henter et al.

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XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome

Stephaine Rigaud et al.

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Regulation of NKT cell development by SAP, the protein defective in XLP

KE Nichols et al.

NATURE MEDICINE (2005)

Review Immunology

Molecular and immunological basis of X-linked lymphoproliferative disease

S Latour et al.

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Interleukin-18 in hemophagocytic lymphohistiocytosis

H Takada et al.

LEUKEMIA & LYMPHOMA (2001)

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Rab27a is required for regulated secretion in cytotoxic T lymphocytes

JC Stinchcombe et al.

JOURNAL OF CELL BIOLOGY (2001)

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Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome

G Ménasché et al.

NATURE GENETICS (2000)