Table 1.
Clinical, Labs Characteristics and Underlying Conditions of Adult Patients with HLH
| Clinical and Biological Characteristics | Number of Patients (n = 52) |
|---|---|
| Fever | 51 (98%) |
| Splenomegaly | 35 (67%) |
| Cytopenia (at least two lineages) | 48 (92%) |
| Anemia (Hb <9 g/dl) | 38 (73%) |
| Neutropenia (ANC <1000/µL) | 26 (50%) |
| Thrombocytopenia (<100 000/ µL) | 48 (92%) |
| Ferritin ≥500 μg/L | 52 (100%) |
| Hemophagocytosis | 52 (100%) |
| Hepatomegaly | 14 (27%) |
| Hypertriglyceridemia | 51 (98%) |
| Hypofibrinogenemia | 25 (48%) |
| Renal involvement, acute renal failure | 6 (14.2%) |
| Neurological symptoms | 3 (7.1%) |
| DIC* | 16 (30.7%) |
| Pulmonary involvement, respiratory insufficiency | 1 (2.3%) |
| Peripheral lymphadenopathy | 12 (28.5%) |
| Underlying conditions | |
| Malignancy | 22 (42%) |
| Solid cancer | 1 (1.9%) |
| Hematological disease | 21 (40%) |
| B-cell lymphoma | 10 (19.2%) |
| T-cell lymphoma | 7 (13.5%) |
| Multicentric Castleman disease | 1 (1.9%) |
| Acute leukemia | 3 (5.8%) |
| Infections | 20 (38.5%) |
| EBV | 8 (15%) |
| CMV | 2 (3.8%) |
| HIV | 1 (1.9%) |
| HSV-1 | 2 (3.8%) |
| Dengue | 2 (3.8%) |
| Parvovirus | 1 (1.9%) |
| Hepatitis A | 1 (1.9%) |
| Toxoplasmosis | 1 (1.9%) |
| Tuberculosis | 1 (1.9%) |
| Autoimmune diseases | 7 (13.5%) |
| Systemic lupus erythematosus | 1 (1.9%) |
| Adult-onset still diseases | 3 (5.8%) |
| Shulman disease | 1 (1.9%) |
| Scleroderma | 1 (1.9%) |
| Overlap syndrome | 1 (1.9%) |
| Pregnancy | 1 (1.9%) |
| Idiopathic | 3 (3.8%) |
Note: *ISTH criteria of DIC was used (DIC was considered if score ≥ 5).
Abbreviations: ANC, Absolute Neutrophil Count; DIC, Disseminated Intravascular Coagulation; EBV, Epstein-Barr virus; CMV, Cytomegalovirus; HSV, Herpes Simplex Virus.