Characteristics of each of 3 patients with hemophagocytic syndrome*
. | Patient no. . | . | . | ||
|---|---|---|---|---|---|
. | 1 . | 2 . | 3 . | ||
| Sex/age, y | M/70 | M/58 | F/67 | ||
| Underlying diseases | DIC‡ | HIV/AIDS§ | DLBCL∥ | ||
| Clinical findings | |||||
| Nonremitting high fever | + | + | + | ||
| Hepatosplenomegaly | + | + | + | ||
| Peripheral blood | |||||
| White cells (neutrophils), × 109/L | 2.7 (1.6) | 0.8 (0.5) | 1.0 (0.6) | ||
| Hemoglobin, g/L | 85 | 65 | 61 | ||
| Platelet, × 109/L | 96 | 31 | 6 | ||
| Biochemistry¶ | |||||
| Triglycerides, mg/dL | ND | 120 | 106 | ||
| Fibrinogen, mg/dL | 97 | 78 | 481 | ||
| Ferritin, μg/L | 1746 | ND | 2861 | ||
| sIL-2R, U/mL | 3870 | ND | 6280 | ||
| FDP, μg/mL | 53.2 | 38.6 | 8 | ||
| Hemophagocytosis in bone marrow† | + | + | + | ||
| Outcome | Remission | Rapidly fatal | Remission | ||
. | Patient no. . | . | . | ||
|---|---|---|---|---|---|
. | 1 . | 2 . | 3 . | ||
| Sex/age, y | M/70 | M/58 | F/67 | ||
| Underlying diseases | DIC‡ | HIV/AIDS§ | DLBCL∥ | ||
| Clinical findings | |||||
| Nonremitting high fever | + | + | + | ||
| Hepatosplenomegaly | + | + | + | ||
| Peripheral blood | |||||
| White cells (neutrophils), × 109/L | 2.7 (1.6) | 0.8 (0.5) | 1.0 (0.6) | ||
| Hemoglobin, g/L | 85 | 65 | 61 | ||
| Platelet, × 109/L | 96 | 31 | 6 | ||
| Biochemistry¶ | |||||
| Triglycerides, mg/dL | ND | 120 | 106 | ||
| Fibrinogen, mg/dL | 97 | 78 | 481 | ||
| Ferritin, μg/L | 1746 | ND | 2861 | ||
| sIL-2R, U/mL | 3870 | ND | 6280 | ||
| FDP, μg/mL | 53.2 | 38.6 | 8 | ||
| Hemophagocytosis in bone marrow† | + | + | + | ||
| Outcome | Remission | Rapidly fatal | Remission | ||
sIL-2R indicates soluble interleukin-2 receptor; FDP, fibrin degeneration products; DIC, disseminated intravascular coagulation syndrome; HIV, human immunodeficiency virus; AIDS, acquired immunodeficiency syndrome; DLBCL, diffuse large B-cell lymphoma; ND, not determined.
According to the diagnostic guidelines developed by the Histiocyte Society, the diagnosis of hemophagocytic lymphohistiocytosis (HLH) requires the presence of all 5 major criteria: (1) fever, (2) splenomegaly, (3) cytopenia involving 2 or more cell lines, (4) hypertriglyceridemia or hypofibrinogenemia, (5) hemophagocytosis. Either criterion (a) low or absent natural killer cell activity or a combination of criteria, (b) ferritin above 500 mg/L, and (c) sIL-2R above 2400 U/mL may substitute for 1 of the major criteria.25
Details are shown in Table 3
Chronic DIC after surgery for aortic abdominal aneurysm
Diagnosed as AIDS at acute onset of Pneumocystis carinii pneumonia. Cytomegalovirus antigenemia assay was also positive
DLBCL with bone marrow invasion. Some cases of DLBCL have a prominent background of reactive T cells and histiocytes
Reference ranges for normal values are 30 to 150 mg/dL for triglycerides, 160 to 360 mg/dL for fibrinogen, below 234 μg/L for ferritin, 135 to 483 U/mL for sIL-2R, and less than 10 μg/mL for FDP