A registry for hemophagocytic lymphohistiocytosis (HLH)

Recruiting

Conditions: hemophagocytic lymphohistiocytosis (HLH)
D76.1
Haemophagocytic lymphohistiocytosis

Registration Number: DRKS00010148

Lead Sponsor: niversitätsklinikum FreiburgCentrum für Chronische Immundefizienz (CCI)

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Recruiting

Sex: All

Target Recruitment: 300

Inclusion Criteria

• Patients with primary immunodeficiencies predisposing to HLH (all ages)
• Patients fulfilling >/= 4/8 diagnostic criteria for HLH as defined by the Histiocyte Society and receiving HLH directed therapy (steroids are sufficient)
• Patients presenting with a high suspicion of isolated CNS-HLH and receiving HLH directed therapy
• Patients with active HLH that is not secondary to underlying conditions such as rheumatological disease, malignancy, metabolic disease or Leishmania infection (mostly infection-only” HLH) (all ages)
• Patients (or their legal representatives) having provided informed consent

Exclusion Criteria

• Patients with malignancy
• Patients with rheumatological/autoinflammatory/autoimmune disease
• Patients with metabolic diseases
• Patients with Leishmania infection
• No written consent available

Study & Design

Study Type: observational

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Collect data to judge feasibility of a future HLH trial:<br>Number of eligible patients/year<br>Outcome associated with standard of care<br>Frequency of CNS disease<br>Time to first HLH directed drug<br>Time to Transplant<br>

Secondary Outcome Measures

Name	Time	Method
Improve the infrastructure for a future HLH study :<br>Standardization of patient registration<br>Standardization of patient documentation<br>Standardization of diagnostic evaluation<br>Implementation of national and international coordination<br>