A Randomized Controlled Study of High-dose Cyclophosphamide Induction Therapy in Adult Patients With HLH
Hemophagocytic Syndrome
About this trial
This is an interventional treatment trial for Hemophagocytic Syndrome focused on measuring Hemophagocytic syndrome(HLH)
Eligibility Criteria
Inclusion Criteria: Age range from 18 to 65 years old (including the critical value), gender is not limited; According to the diagnostic criteria of HLH-2004, HLH can be diagnosed if any of the following two criteria are met: Molecular diagnosis is consistent with HLH: Currently known HLH related pathogenic genes exist, such as PRF1, UNC13D, STX11, STXBP2, Rab27a, LYST, SH2D1A, BIRC4, ITK, AP3β1, MAGT1, CD27 (cluster of differentiation antigen 27 )and other pathological mutations. Meet 5 or more of the following 8 indicators: Fever: body temperature > 38.5 ℃, continuous > 7 d; ② Splenomegaly; Hemocytopenia (involving two or three peripheral blood lines) : hemoglobin < 90 g/L (< 4 weeks infant, hemoglobin < 100 g/L), platelet < 100×109/L, neutrophils < 1.0×109/L and not caused by reduced hematopoietic function of bone marrow; ④ High triglyceride (TG) sepsis and/or low fibrinogenemia: triglyceride > 3 mmol/L or 3 standard deviations above the same age, fibrinogen < 1.5g /L or less than 3 standard deviations for the same age; (5) Hematophagy was found in bone marrow, spleen, liver or lymph nodes; The activity of NK cells is decreased or absent; ⑦ Serum ferritin increase: ferritin ≥500 μg/L; Elevated sCD25 (soluble interleukin-2 receptor). (3) Those who can understand the research content, agree to comply with the research plan, and voluntarily sign the informed consent. Exclusion Criteria: HLH caused by treatable infectious causes (such as bacteria, fungi, viruses (except Epstein-Barr virus), protozoa, etc.); Have a history of allergy or contraindications to the drugs involved in the program; Organ damage caused by long-term chronic diseases; Extreme physical weakness, unstable vital signs and inability to tolerate large doses of cyclophosphamide; Severe and/or uncontrolled co-morbidivities (e.g., uncontrolled diabetes, pulmonary hypertension, etc.) that the investigator believes may pose an unacceptable safety risk or interfere with protocol compliance; Mental instability or history of severe mental illness Other factors determined by the researcher that subjects are not suitable to participate in this study.
Sites / Locations
- The Second affiliated Hosptial of Chongqing medical UniversityRecruiting
Arms of the Study
Arm 1
Arm 2
Experimental
Active Comparator
CTX(Cytoxan) group
Normal treatment group