Case Report
Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis
| Molecular diagnosis consistent with primary HLH (gene mutations) | Homozygosity or compound heterozygosity is required (PRAF1, STX11, RAB27A, STXBP2, UNC13D, SH2D1A, BIRC4) |
| Below criteria are for secondary HLH (at least 5 of 8 criteria must be present) | Fever | ≥38.5 C | Splenomegaly | Spleen palpated >3 cm below the left costal margin | Hemophagocytosis | In bone marrow, spleen, lymph nodes, or liver | NK-cell activity | Low or absent according to local laboratory references | Soluble CD25 | (i.e., soluble IL-2 receptor) ≥ 2,400 U/ml | Ferritin | ≥500 μg/L | Cytopenias (affecting ≥2 of 3 lineages) | Hemoglobin <9 g/dL | Platelets <100 × 109/L | Neutrophils <1 × 109/L | Hypertriglyceridemia and/or hypofibrinogenemia | Fasting triglycerides >265 mg/dL | Fibrinogen <150 mg/dL |
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