Case Report

Disseminated Mycobacterium tuberculosis: An Unusual Presentation with Associated Hemophagocytic Lymphohistiocytosis

Table 1

HLH 2004 criteria [14].

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
SplenomegalySpleen palpated >3 cm below the left costal margin
HemophagocytosisIn bone marrow, spleen, lymph nodes, or liver
NK-cell activityLow 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 hypofibrinogenemiaFasting triglycerides >265 mg/dL
Fibrinogen <150 mg/dL