Cytomegalovirus-Induced Hemophagocytic Lymphohistiocytosis in an Immunocompromised Patient with Inflammatory Bowel Disease
Table 1
The HLH-2004 consensus diagnostic criteria and the HScore are validated for the screening and diagnosis of HLH.
HLH-2004 diagnostic criteria4 (five of eight needed for diagnosis)
Presence in study patient
Fever
Yes, 40°C
Splenomegaly
No
Cytopenia (affecting 2 or more lineages in peripheral blood):
Yes Hemoglobin 83 g/L Neutrophils 0.56 × 109/L
(i) Hemoglobin <90 g/L
(ii) Platelets <100 × 109/L
(iii) Neutrophils <1.0 × 109/L
Hypertriglyceridemia and/or hypofibrinogenemia:
Yes Fibrinogen 0.67 g/L
(i) Fasting triglycerides ≥3.0 mmol/L
(ii) Fibrinogen ≤1.5 g/L
Hemophagocytosis in bone marrow, spleen, or lymph nodes
Yes
Low or absent NK-cell activity
Not assessed
Ferritin ≥500 μg/L
Yes
Ferritin 13106 μg/L
Soluble CD25 ≥ 2400 U/mL
Result pending
Variables assessed in HScore5
Presence in study patient
Underlying immunosuppression
Yes
Fever
Yes
Hepatosplenomegaly
No
Cytopenia
Yes
Ferritin
Yes
Hypertriglyceridemia
No
Hypofibrinogenemia
Yes
Elevated aspartate aminotransferase
Yes
Hemophagocytosis features on bone marrow aspirate
Yes
The superscripts here refer to the number 4 and 5 papers cited in my bibliography. The normal values for these two validated scores were adapted from those works and reproduced in this table.