An observational study about the clinical profile of Hemophagocytic Lymphohistiocytosis(a disease in which own immune cells eat up blood cells resulting in fall of blood cells) in children less than 12 years and about markers that decide good and bad prognosis of the disease.
- Conditions
- Hemophagocytic lymphohistiocytosis,
- Registration Number
- CTRI/2021/03/032323
- Brief Summary
Hemophagocytic lymphohistiocytosis is a life threatening disease associated with rapid clinical deterioration often requiring intensive care treatment.Children under 12 years fulfilling the HLH criteria are included in study.This study is to identify clinical parameters related to mortality and prognostic factors correlated with favourable/unfavorable outcome with main emphasis on S.ferritin ,which will be repeated every 5 days once until normalisation, as secondary outcome.
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- Not Yet Recruiting
- Sex
- All
- Target Recruitment
- 30
- Children less than 12yrs fulfilling criteria of Hemophagocytic lymphohistiocytosis.
- 1.A molecular diagnosis consistent with HLH (e.g., PRF mutations, SAP mutations) or 2.
- Presence of 5 of the following 8 symptoms, signs, or laboratory abnormalities: a.
- Splenomegaly c.
- Cytopenia (affecting ≥2 cell lineages; hemoglobin ≤9 g/dL [or ≤10 g/dL for infants <4 wk old], platelets <100,000/μL, neutrophils <1,000/μL) d.
- Hypertriglyceridemia (≥265 mg/dL) and/or hypofibrinogenemia (≤150 mg/dL) e.
- Hemophagocytosis in the bone marrow, spleen, or lymph nodes without evidence of malignancy f.
- Low or absent natural killer cell cytotoxicity g.
- Hyperferritinemia (≥500 ng/mL) h.
- Elevated soluble CD25 (interleukin-2Rα chain; ≥2,400 U/mL).
Macrophage Activation Syndrome (MAS) associated with Autoimmune disease 1.Systemic-onset juvenile idiopathic arthritis 2.Systemic lupus erythematosus 3.Enthesitis-related arthritis 4.Inflammatory bowel disease.
Study & Design
- Study Type
- Observational
- Study Design
- Not specified
- Primary Outcome Measures
Name Time Method Clinical, Epidemiological and Hematological profile of Hemophagocytic lymphohistiocytosis in children less than 12 years in a tertiary health center Assessment of Participants from the time of admission for the entire period of hospital stay
- Secondary Outcome Measures
Name Time Method Rate of fall of ferritin as a prognostic marker Every 5 days once S.Ferritin will be repeated until normalises
Trial Locations
- Locations (1)
M3 Unit,E Block(6th Floor),Dept-Pediatrics,Institute of Child Health and Hospital for Children,MMC
🇮🇳Chennai, TAMIL NADU, India
M3 Unit,E Block(6th Floor),Dept-Pediatrics,Institute of Child Health and Hospital for Children,MMC🇮🇳Chennai, TAMIL NADU, IndiaAishvarya RPrincipal investigator9626478975aishvarya1396@gmail.com