Table 1.

Diagnostic Criteria for Adult LAHS²⁷

Criteria
1. High fever for more than 1 week (peak ≥ 38°C)
2. Anemia (Hb 9 g/dL) or thrombocytopenia (plt < 1,000,000/μL)
3. At least two of four subitems: LDH ≥ 2× upper limit of normal Hyperferritinemia (≥ 1,000 ng/dL) Hepatosplenomegaly on CT, US, or MRI FDP ≥ 10 μg/mL
4. Hemophagocytosis in bone marrow, spleen, or liver
5. No evidence of infection
6. Histopathologically confirmed malignant lymphoma

NOTE. A diagnosis of LAHS requires all of the items listed in this table to be fulfilled. When items 1 through 5 are present for 2 weeks and glucocorticoids or γ-globulin therapy are not effective, a diagnosis of probable LAHS can be made and chemotherapy against malignant lymphoma can be started.

Abbreviations: CT, computed tomography; FDP, fibrin degradation products; Hb, hemoglobin; LAHS, lymphoma-associated hemophagocytic syndrome; LDH, lactate dehydrogenase; MRI, magnetic resonance imaging; plt, platelets; US, ultrasonography.