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
- Health Condition 1: D761- Hemophagocytic lymphohistiocytosis
- Registration Number
- CTRI/2021/03/032323
- Lead Sponsor
- one
- Brief Summary
Not available
- Detailed Description
Not available
Recruitment & Eligibility
- Status
- ot Yet Recruiting
- Sex
- Not specified
- Target Recruitment
- 0
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. Fever
b. 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
- Secondary Outcome Measures
Name Time Method