Tocilizumab and Hemophagocytic Lymphohistiocytosis (HLH)
Hemophagocytic LymphohistiocytosisThis study seeks to determine the efficacy of tocilizumab (TCZ) in patients with hemophagocytic lymphohistiocytosis (HLH) and high cytokine levels (proteins involved in inflammation) in an attempt to decrease the damage caused by these proteins; and secondarily to assess its safety and impact on disease activity.
Assessing the Presence of CT-DNA in Lymphoma Associated HLH
LymphomaHaemophagocytic LymphohistiocytosisHaemophagocytic lymphohistiocytosis (HLH) is a rare life-threatening blood disease which causes severe inflammation with symptoms similar to severe sepsis. It is hard to diagnose. The most common cause of HLH in adults is lymphoma (blood cancer). Outcomes for adults with HLH and cancer are serious, and most die after days or weeks because they have been diagnosed or treated too late. It is likely that many cases where patients died of HLH with no underlying cause actually had cancer. Recently it has been found that patients with certain types of lymphoma have DNA which comes directly from their cancer (circulating tumour DNA; ctDNA). Aggressive lymphomas release a lot of ctDNA which can be detected in the blood of patients. This study will look for ctDNA in patients with HLH, and see if it is possible to use it to diagnose lymphoma earlier. Patients will provide a small additional blood sample for analysis. Diagnosing lymphoma more rapidly would mean more people could get the correct treatment for the lymphoma which has caused their HLH. They could receive the correct treatment sooner. Earlier diagnosis and treatment could improve survival for these patients.
Response Prediction in EBV-HLH Using Metabonomics Analysis
Hemophagocytic LymphohistiocytosesEBV1 moreHemophagocytic lymphohistiocytosis (HLH) is a rare, poorly recognized and underdiagnosed syndrome of excessive immune activation, which is rapidly fatal. Epstein-Barr virus (EBV) is a common trigger of HLH, particularly in Asian individuals. We aim to analyze metabolomics and cytokine profiles of patients before and after treatment to explore the metabolomic characteristics of EBV-HLH, and search for pathogenic mechanisms and therapeutic targets.
Bone Marrow Transplant With Abatacept for Non-Malignant Diseases
Hurler SyndromeFanconi Anemia13 moreThis is a single arm, phase I study to assess the tolerability of abatacept when combined with cyclosporine and mycophenolate mofetil as graft versus host disease prophylaxis in children undergoing unrelated hematopoietic stem cell transplant for serious non-malignant diseases as well as to assess the immunological effects of abatacept. Participants will be followed for 2 years.
A Study for Long-term Follow-up of Hemophagocytic Lymphohistiocytosis (HLH) Participants Who Received...
Hemophagocytic LymphohistiocytosisInternational, multicenter, long-term, follow-up study that will enrol HLH participants who have received emapalumab in previous clinical trials, in the context of the clinical development program for emapalumab or under compassionate use (CU).
Splenectomy as a Treatment for Patient With Relapsed Haemophagocytic Lymphohistiocytosis of Unknown...
Hemophagocytic LymphohistiocytosisTo evaluate the clinical value of splenectomy as a treatment for relapsed haemophagocytic lymphohistiocytosis (HLH) in patient with unknown etiology.
DEP-Ru Regimen as a Salvage Therapy for HLH
Hemophagocytic LymphohistiocytosisThis study aimed to investigate the efficacy and safety of ruxolitinib together with liposomal doxorubicin, etoposide and methylprednisolone (DEP-Ru) as a salvage therapy for refractory/relapsed hemophagocytic lymphohistiocytosis.
Pilot Study of Unrelated Donor Hematopoietic Stem Cell Transplantation in Patients With Life Threatening...
Chediak-Higashi SyndromeGraft Versus Host Disease4 moreOBJECTIVES: I. Determine the efficacy of unrelated donor hematopoietic stem cell transplantation in the treatment of patients with life threatening hemophagocytic disorders. II. Determine the rate of disease free survival, incidence of graft failure, and incidence of graft versus host disease in these patients after undergoing this treatment regimen.
Pegylated Liposomal Doxorubicin as a Induction Therapy for Lymphoma Induced Hemophagocytic Lymphohistiocytosis....
Hemophagocytic LymphohistiocytosisThis study aimed to investigate the efficacy and safety of pegylated liposomal doxorubicin together with etoposide and methylprednisolone as a induction therapy for lymphoma induced hemophagocytic lymphohistiocytosis.
L-DEP Regimen as a Salvage Therapy for Refractory Epstein Barr Virus-induced Hemophagocytic Lymphohistiocytosis...
Hemophagocytic LymphohistiocytosisThis study aimed to investigate the efficacy and safety of Pegaspargase together with liposomal doxorubicin, etoposide and high dose methylprednisolone (L-DEP) as a salvage therapy for refractory Epstein Barr virus-induced hemophagocytic lymphohistiocytosis.