Pilot Study of Etoposide-based Therapy and Hematopoietic Cell Transplantation for Hemophagocytic Lymphohistiocytosis

NCT01547143

NAINTERVENTIONAL

Asan Medical Center

12 participants

Started 2012-03 · Completed 2016-05

Status: Terminated (as of 12-23-2025)

Conditions

Hemophagocytic Lymphohistiocytosis

Interventions

DRUG: IST and/or alloHCT

Linked Papers (from SciSciNet-V2)

Total Papers47

Background

7

Result

0

Derived

0

Unknown

40

View on ClinicalTrials.gov

Total Citations Per Year

Brief Summary

The investigators would like to propose a pilot study evaluating the efficacy of etoposide combined with immunosuppressive agents for adult secondary hemophagocytic lymphohistiocytosis (HLH), in order to prove out whether the modification of previous HLH-94 or HLH-2004 protocol for childhood patients can improve the outcome and decrease the toxicities. The results of this pilot study will be a base of a more-improved phase-2 protocol.

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Background Papers14

Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH

Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH

Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality

Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality

Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management

Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management

Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis

Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis

Genotype-phenotype study of familial haemophagocytic lymphohistiocytosis type 3

Genotype-phenotype study of familial haemophagocytic lymphohistiocytosis type 3

UNC13D is the predominant causative gene with recurrent splicing mutations in Korean patients with familial hemophagocytic lymphohistiocytosis

UNC13D is the predominant causative gene with recurrent splicing mutations in Korean patients with familial hemophagocytic lymphohistiocytosis

Treatment Outcomes with CHOP Chemotherapy in Adult Patients with Hemophagocytic Lymphohistiocytosis

Treatment Outcomes with CHOP Chemotherapy in Adult Patients with Hemophagocytic Lymphohistiocytosis

Result Papers0

No result papers linked

Derived Papers0

No derived papers linked

Unknown Papers40

HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis

4,636 citations

Munc13-4 Is Essential for Cytolytic Granules Fusion and Is Mutated in a Form of Familial Hemophagocytic Lymphohistiocytosis (FHL3)

Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation

Familial hemophagocytic lymphohistiocytosis

Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis

Nationwide Survey of Hemophagocytic Lymphohistiocytosis in Japan

Hemophagocytic lymphohistiocytosis (HLH) and related disorders

Requirement for Etoposide in the Treatment of Epstein-Barr Virus–Associated Hemophagocytic Lymphohistiocytosis

Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantation

Immunotherapy of Familial Hemophagocytic Lymphohistiocytosis With Antithymocyte Globulins: A Single-Center Retrospective Report of 38 Patients

Frequency and Severity of Central Nervous System Lesions in Hemophagocytic Lymphohistiocytosis

Haematopoietic stem cell transplantation in haemophagocytic lymphohistiocytosis

Pathogenesis of haemophagocytic lymphohistiocytosis

Stem cell transplantation with reduced-intensity conditioning for hemophagocytic lymphohistiocytosis

Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?

Treatment of Epstein‐Barr virus‐associated hemophagocytic lymphohistiocytosis (EBV‐HLH) in young adults: A report from the HLH studyl center

Genetic subtypes of familial hemophagocytic lymphohistiocytosis: correlations with clinical features and cytotoxic T lymphocyte/natural killer cell functions

Hemophagocytic lymphohistiocytosis and related disorders

Hemophagocytic Lymphohistiocytosis

The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis

Spectrum of clinical presentations in familial hemophagocytic lymphohistiocytosis type 5 patients with mutations in STXBP2

Treatment of Familial Hemophagocytic Lymphohistiocytosis With Bone Marrow Transplantation From HLA Genetically Nonidentical Donors

Hematopoietic stem cell transplantation for familial hemophagocytic lymphohistiocytosis and Epstein–Barr virus‐associated hemophagocytic lymphohistiocytosis in Japan

Treatment of Epstein-Barr Virus-related Hemophagocytic Lymphohistiocytosis (EBV-HLH); Update 2010

Causes, clinical symptoms, and outcomes of infectious diseases associated with hemophagocytic lymphohistiocytosis in Taiwanese adults

Clinical features of adult patients with secondary hemophagocytic lymphohistiocytosis from causes other than lymphoma: an analysis of treatment outcome and prognostic factors

Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step

Soluble interleukin-2 receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis [letter]

Improved outcome in haemophagocytic lymphohistiocytosis after bone marrow transplantation from related and unrelated donors: a single‐centre experience of 12 patients

Characterization of PRF1, STX11 and UNC13D genotype‐phenotype correlations in familial hemophagocytic lymphohistiocytosis

Pathology of the Liver in Familial Hemophagocytic Lymphohistiocytosis

Hematopoietic stem cell transplantation for hemophagocytic lymphohistiocytosis: a retrospective analysis of data from the Italian Association of Pediatric Hematology Oncology (AIEOP)

Experience With Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome at a Single Institution

Treatment Strategies for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis (EBV-HLH)

STX11 mutations and clinical phenotypes of familial hemophagocytic lymphohistiocytosis in North America

Establishment of a reference interval for natural killer cell activity through flow cytometry and its clinical application in the diagnosis of hemophagocytic lymphohistiocytosis

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: a retrospective study of 78 pediatric cases in mainland of China.

Spontaneous resolution of Epstein–Barr virus‐associated hemophagocytic lymphohistiocytosis

Tumor necrosis factor alpha promoter polymorphism associated with increased susceptibility to secondary hemophagocytic lymphohistiocytosis in the Korean population