Tuberculosis-associated hemophagocytic lymphohistiocytosis misdiagnosed as systemic lupus erythematosus: A case report

Table 3 The HScore system

Parameter	No. of points (criteria forscoring)	Our case
Known underlyingImmunosuppression1	0 (no) or 18 (yes)	Yes (steroid therapy): 18
Temperature (°C)	0 (< 38.4), 33 (38.4–39.4), or 49 (> 39.4)	Temperature > 39.4°C: 49
Organomegaly	0 (no), 23 (hepatomegaly or splenomegaly), or 38 (hepatomegaly and splenomegaly)	No: 0
No. of cell lines involved in cytopenia2	0 (1 lineage), 24 (2 lineages), or 34 (3 lineages)	2 lineages involved in cytopenia (HGB <90 g/L; platelet <100 × 109/L): 24
Ferritin (μg/L)	0 (< 2000), 35 (2000-6000), or 50 (> 6000)	Ferritin 679.93 ng/mL: 0
Triglyceride (mmol/L)	0 (< 1.5), 44 (1.5-4), or 64 (> 4)	Triglyceride 2.6 mmol/L: 44
Fibrinogen (g/L)	0 (> 2.5) or 30 (≤ 2.5)	Fibrinogen 1.26 g/L: 30
Aspartate aminotransferase (U/L)	0 (< 30) or 19 (≥ 30)	Aspartate aminotransferase 99 U/L: 19
Hemophagocytosis on bone marrow aspirate	0 (no) or 35 (yes)	Yes (hemophagocytosis on bone marrow aspirate): 35

¹HIV positive or receiving long-term immunosuppressive therapy (i.e., glucocorticoids, cyclosporine A, azathioprine).

²Defined as a hemoglobin level of 9.2 g/L and/or a leukocyte count ≤ 5 x 10⁹/L and/or a platelet count ≤ 110 x 10⁹/L.

Our patient had an HScore of 219 (steroid therapy: 18, temperature > 39.4°C: 49, 2 lineages involved in cytopenia: 24, TG level of 2.6 mmol/L: 44, FIB level of 1.26 g/L: 30, AST level of 99 U/L: 19, hemophagocytosis in bone marrow aspirate: 35), which is much higher than the threshold score of 169.