Diagnostic criteria for hemophagocytic lymphohistiocytosis

Calculator

When to use

Genetic mutation associated with HLH

Yes

Fever

Yes

Splenomegaly

Yes

Cytopenias affecting ≥ 2 of 3 lineages in the peripheral blood: hemoglobin < 90 g/L, platelets < 100×10**9 cells/L, neutrophils < 1.0×10**9 cells/L

Yes

Fasting triglycerides ≥ 3.0 mmol/L [≥ 265 mg/dL] and/or fibrinogen < 1.5 g/L

Yes

No evidence of malignancy

Yes

Low or absent NK-cell activity

Yes

Ferritin ≥ 500 mcg/L

Yes

Soluble CD25/interleukin-2 receptor ≥ 2,400 U/mL

Yes

Hemophagocytosis in bone marrow/spleen/lymph nodes

Yes

Calculation

Please enter all the required inputs.

The calculator in question is the Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis (HLH). This scoring system is used in the clinical setting to diagnose patients suspected of having HLH, a rare but potentially fatal immune disorder. The criteria are applicable to patients of all ages who present with symptoms suggestive of HLH, such as fever, splenomegaly, cytopenias, and neurological symptoms. The clinical utility of this calculator is to aid in the early detection and diagnosis of HLH, which is crucial for initiating appropriate treatment and improving patient outcomes. The criteria include parameters like fever, splenomegaly, cytopenias affecting at least two of three lineages in the peripheral blood, hypertriglyceridemia and/or hypofibrinogenemia, hemophagocytosis in bone marrow or spleen or lymph nodes, low or absent natural killer cell activity, ferritin >500 ng/mL, and soluble CD25 (i.e., soluble IL-2 receptor) >2400 U/mL. There are no specific exclusion criteria for the use of this calculator. However, it should be noted that the diagnosis of HLH can be challenging due to the overlap of symptoms with other conditions, and the criteria should be

Reference

Jan-Inge Henter, Annacarin Horne, Maurizio Arico et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb;48(2):124-31.

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