From: Gastric T-cell lymphoma associated with hemophagocytic syndrome
1 High fever for more than a week (peak 38.5°C) |
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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. |