Health Condition 1: D761- Hemophagocytic lymphohistiocytosis
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 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 d. Hypertriglyceridemia (>=265 mg/dL) and/or hypofibrinogenemia ( 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)
Exclusion criteria
Exclusion criteria: 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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Clinical, Epidemiological and Hematological profile of Hemophagocytic lymphohistiocytosis in children less than 12 years in a tertiary health centerTimepoint: Assessment of Participants from the time of admission for the entire period of hospital stay | — |
Secondary
| Measure | Time frame |
|---|---|
| Rate of fall of ferritin as a prognostic markerTimepoint: Every 5 days once S.Ferritin will be repeated until normalises | — |
Countries
India
Contacts
Institute of Child Health and Hospital for Children, Madras Medical College