Table 1 Diagnostic criteria for adult lymphoma associates hemophagocytic syndrome (LAHS)

2 Anemia (Hb < 9 g/dl) or thrombocytopenia (platelet < 100,000 μ/l)

3 a) LDH ≥ 2 × upper limit

   b) Hyperferritinemia (≥ 1,000 ng/dl)

   c) Hepatosplenomegaly on CT, US or MRI

   d) FDP ≥ 10 μg/ml

4 Hemophagocytosis in bone marrow, spleen or liver

5 No evidence of infection

6 Histopathologically confirmed malignant lymphoma

A diagnosis of LAHS requires that all of the above conditions are fulfilled.

Of the item 3, at least two of the four sub-items (a~d) should be fulfilled.

When item 1 to item 5 are present for 2 weeks and glucocorticoid or γ-globulin therapy is not effective, a diagnosis of probable LAHS can be made and chemotherapy against malignant lymphoma can be started.