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Clinical Trials/CTRI/2021/03/032323
CTRI/2021/03/032323
Not yet recruiting
未知

Clinical, Epidemiological and Hematological profile of Hemophagocytic Lymphohistiocytosis in Children less than 12 years and Rate of decline of Ferritin as a prognostic marker for disease outcome.

one0 sites0 target enrollmentTBD
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Overview

Phase
未知
Intervention
Not specified
Conditions
Not specified
Sponsor
one
Status
Not yet recruiting
Last Updated
4 years ago
OverviewInvestigatorsEligibility CriteriaOutcomesSimilar Trials

Overview

Brief Summary

No summary available.

Registry
who.int
Start Date
TBD
End Date
TBD
Last Updated
4 years ago
Study Type
Observational

Investigators

Sponsor
one

Eligibility 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:
  • •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)

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

Outcomes

Primary Outcomes

Not specified

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