What are 2 scoring systems for diagnosing hemophagocytic lymphohistiocytosis (HLH)?

By William Aird

diagnostic criteria
Known underlying immunosuppressionNA0 (no) or 18 (yes)HIV positive or receiving long‐term immunosuppressive therapy (e.g., glucocorticoids, cyclosporine).
Fever0 (<38.5) or 1 (38.5)0 (39.4)Persistent or recurrent high fever. Nearly universal in untreated HLH.
Hepatomegaly NA0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegalyAbnormal liver tests reported in about 80% of adults with HLH (hypoalbuminemia detected in most adults with HLH).
Splenomegaly 0 (no) or 1 (yes)0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegaly)Splenomegaly may be caused by infiltration by activated lymphocytes and histiocytes or it by an underlying condition triggering HLH.
Cytopenias 0 (one lineage) or 1 (two or three lineages)*0 (one lineage), 24 (two lineages), or 34 (three lineages)Especially thrombocytopenia and anemia in early stages; results primarily from severe cytokine-mediated myelosuppression.
High Ferritin level (ng/ml) 0 (<500) or 1 (500)0 (6,000)No specific ferritin level determined to be specific for HLH. However, the higher the ferritin the likelier the diagnosis of HLH.
Elevated triglyceride level NA0 (4)Hypertriglyceridemia is detected in 42%-85% of adults.
Low Fibrinogen level NA0 (>2.5) or 30 (<2.5)Hypofibrinogenemia detected in about 50% of adults with HLH.
Elevated triglyceride level or low fibrinogen level Triglycerides > 265 mg/dl and/or fibrinogen </= 15 mg/dL (1)NA
ASTNA0 (<30) or 19 (30)LDH and bilirubin may also be increased.
Hemophagocytosis features on bone marrow aspirate 0 (no) or 1 (yes)Hemophagocytosis in bone marrow 0 (no) or 1 (yes)If the bone marrow specimen is not conclusive, material may be obtained from other organs.
Elevated soluble IL-2 receptor 1 (sIL-2R ≥ 2,400 units/mL)NAsIL-2R (sCD25), a marker of T cell activation; not available at most medical centers, their utility in real-time diagnosis and monitoring remains limited.
Low or absent NK-cell activity1 (Yes)NANot available at most medical centers; their utility in real-time diagnosis and monitoring remains limited.

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