Pilot Study of Etoposide-based Therapy and Hematopoietic Cell Transplantation for Hemophagocytic Lymphohistiocytosis (HELA2012)

This is an interventional treatment trial for Hemophagocytic Lymphohistiocytosis focused on measuring Secondary hemophagocytic lymphohistiocytosis of adults Read More

Inclusion Criteria:

• Patients whose clinical findings satisfy 5 or more criteria out of the following 8 ones

  1. Fever ≥ 38.5 ℃ for ≥ 7 days
  2. Splenomegaly ≥ 3 FB below left subcostal margin
  3. Cytopenias affecting ≥ 2 of 3 lineages in PB Hb < 9 g/L Platelet < 100 x 109 /L ANC < 1.0 x 109 /L
  4. Hypertriglyceridemia and/or hypofibrinogenemia (fasting triglycerides ≥ 265 mg/dL, fibrinogen ≤ 1.5 g/L)
  5. Hemophagocytosis in BM or spleen or LN
  6. Low or absent NK-cell activity ( according to local laboratory reference)
  7. Serum-ferritin ≥ 500 mcg/L
  8. Soluble CD25(sIL-2 receptor) ≥ 2,400 U/ml
• 18 years of age and over.
• All patients (or his/her family when the patient cannot sign the consent form because of his/her general conditions) give written informed consent according to guidelines at institution's committee on human research.

Exclusion Criteria:

• HLH from malignancy (such as lymphoma, myeloma, leukemia, and other solid tumor)
• HLH from rheumatic disorder (such as SLE, AOSD, antiphospholipid antibody syndrome)
• Patients with psychiatric disorder or mental deficiency severe as to make compliance with the treatment unlike, and making informed consent impossible
• Nursing women, pregnant women, women of childbearing potential who do not want adequate contraception
• Male patient who reject the methods of avoiding pregnancy via methods such as abstinence, barrier method (condom etc).
• Patients with a diagnosis of prior malignancy unless disease-free for at least 5 years following therapy with curative intent (except curatively treated nonmelanoma skin cancer, in situ carcinoma, or cervical intraepithelial neoplasia)