hemophagocytic lymphohistiocytosis
Conditions
Interventions
eltrombopag:eltrombopag 50mg po qd
rhTPO:rhTPO 15000iu ih qd
Sponsors
Beijing Friendship Hospital Affiliated to Capital Medical University
Eligibility
Sex/Gender
All
Age
18 Years to No maximum
Inclusion criteria
Inclusion criteria: 1. meet the diagnostic criteria of HLH-2004: Hemophagocytic syndrome can be diagnosed when either of the following two criteria is met: (1) Molecular diagnosis is consistent with hemophagocytic syndrome: pathological mutations have been found in known genes related to hemophagocytic syndrome, such as PRF1, UNC13D, STX11, STXBP2, Rab27a, LYST, SH2D1A, BIRC4, ITK, AP3B1, MAGT1, CD27, etc. (2) Five of the following eight criteria are ful- filled: 1) Duration of fever more than 7 days, and temperature higher than 38.5 degree C; 2) Splenomegaly; 3) Cytopenia (affecting =2 of 3 lineages in the peripheral blood): hemoglobin =3.0 mmol/L or >3 stan- dard deviations (SD) of the normal value of triglycerides in the same age group. Fibrinogen=500 ug/L; 8) Increase of soluble CD25(soluble IL-2 receptor); 2. Platelet count =18 years; 4. Patients have good adherence to planned treatment and follow-up, can understand the study process and sign informed consent.
Exclusion criteria
Exclusion criteria: ? Patients with hypercoagulable state, or had thrombosis recently. ? Patients with liver function impairment (Child-Pugh score = 5). ? Pregnant or lactating women and women of childbearing age who refused to take appropriate contraceptive measures during this trial. If the patient was male, who refused to use adequate contraceptive methods or intends to donate semen during the study and within 3 months after receiving the last cycle of drug study. ? Those who are allergic to rhTPO or eltrombopag and patients with severe allergic constitution. ? HIV infected (HIV antibody positive). ? Patients already participated in other clinical research. ? Patients who were not suitable to participate in the study determined by researchers.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Absolute number of platelet elevation;Effection odds ratio; | — |
Secondary
| Measure | Time frame |
|---|---|
| The recovery time of platelets;Proportion of patients with platelet count higher than 50x10^9/L;The time of platelet count maintained at or above 50x10 ^ 9/L;Platelet transfusion;The assessment of the efficacy of treatment of HLH;Incidence of bleeding events(WHO1-2);Incidence of bleeding events(WHO3-4); | — |
Countries
China
Contacts
Public ContactZhao Wang
Beijing Friendship Hospital Affiliated to Capital Medical University
Outcome results
None listed