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临床试验/CTRI/2021/03/032323
CTRI/2021/03/032323尚未招募未知

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.

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试验速览

阶段
未知
状态
尚未招募
发起方
one

研究概览

简要总结

暂无简介。

研究设计

研究类型
Observational

入排标准

入选标准

  • 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)

排除标准

  • 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

研究者

发起方
one

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