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Volume 22, Number 6—June 2016

Hemophagocytic Lymphohistiocytosis and Progressive Disseminated Histoplasmosis

Kenice Ferguson-Paul, Spencer Mangum, Ashley Porter, Vasiliki Leventaki, Patrick Campbell, and Joshua WolfComments to Author 
Author affiliations: St. Jude Children’s Research Hospital, Memphis, Tennessee, USA (K. Ferguson-Paul, S. Mangum, A. Porter, V. Leventaki, P. Campbell, J. Wolf); University of Tennessee Health Sciences Center, Memphis (K. Ferguson-Paul, S. Mangum, A. Porter, J. Wolf)

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Diagnostic criteria for HLH*

Criterion HLH reference Patient in this report
Fever ≥38.5°C 40°C†
Cytopenia, 2 of 3 lineages
ANC, cells/mm3 <1,000 1,500
Hemoglobin, g/dL <9 5.9†
Platelet count, × 103/μL
Low fibrinogen, mg/dL <150 78†
High triglycerides, mg/dL
>265 fasting
Hemophagocytosis Present Present†
NK cell activity
Low or absent
Elevated ferritin, ng/mL >500 317 (max 1,218)†
Soluble IL-2 receptor, pg/mL >2,400 21,530†

*ANC, absolute neutrophil count; HLH, hemophagocytic lymphohistiocytosis; IL-2, interleukin-2; NK, natural killer.
†Criteria met by this patient. The HLH-2004 diagnostic criteria require either a molecular/genetic diagnosis consistent with HLH or fulfillment of 5 of the 8 criteria shown. Adapted from (5).

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Page created: May 17, 2016
Page updated: May 17, 2016
Page reviewed: May 17, 2016
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