Volume 22, Number 6—June 2016
Letter
Hemophagocytic Lymphohistiocytosis and Progressive Disseminated Histoplasmosis
Kenice Ferguson-Paul, Spencer Mangum, Ashley Porter, Vasiliki Leventaki, Patrick Campbell, and Joshua Wolf
Table
Diagnostic criteria for HLH*
| Criterion | HLH reference | Patient in this report |
|---|---|---|
| Fever | ≥38.5°C | 40°C† |
| Splenomegaly |
Present |
Present† |
| Cytopenia, 2 of 3 lineages | ||
| ANC, cells/mm3 | <1,000 | 1,500 |
| Hemoglobin, g/dL | <9 | 5.9† |
| Platelet count, × 103/μL |
<100 |
11† |
| Low fibrinogen, mg/dL | <150 | 78† |
| or | ||
| High triglycerides, mg/dL |
>265 fasting |
378† |
| Hemophagocytosis | Present | Present† |
| NK cell activity |
Low or absent |
Normal |
| 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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