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

From: Gastric T-cell lymphoma associated with hemophagocytic syndrome

1 High fever for more than a week (peak 38.5°C)
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.