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 criteria^4 (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 × 10^9/L
(i) Hemoglobin <90 g/L
(ii) Platelets <100 × 10^9/L
(iii) Neutrophils <1.0 × 10^9/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 HScore^5	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.