hemophagocytic lymphohistiocytosis (HLH) D76.1
Conditions
Interventions
Group 1: Data collection:
Demographic characteristics,
Form of HLH,
HLH clinical criteria,
Diagnostic assays performed,
CNS disease,
HLH directed treatment and outcome,
HSCT and and outcome,
Chimeris
Sponsors
Universitätsklinikum FreiburgCentrum für Chronische Immundefizienz (CCI)
Eligibility
Sex/Gender
All
Inclusion criteria
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
Exclusion criteria: • Patients with malignancy • Patients with rheumatological/autoinflammatory/autoimmune disease • Patients with metabolic diseases • Patients with Leishmania infection • No written consent available
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Collect data to judge feasibility of a future HLH trial: Number of eligible patients/year Outcome associated with standard of care Frequency of CNS disease Time to first HLH directed drug Time to Transplant | — |
Secondary
| Measure | Time frame |
|---|---|
| Improve the infrastructure for a future HLH study : Standardization of patient registration Standardization of patient documentation Standardization of diagnostic evaluation Implementation of national and international coordination | — |
Countries
France, Germany, Spain, United Kingdom
Contacts
Public ContactKai Lehmberg
UKE HamburgKlinik und Poliklinik für Pädiatrische Hämatologie und Onkologie
Outcome results
None listed