Table I. Characteristics at Presentation.
| Data | Units | Pt 1 | Pt 2 | Pt 3 | Pt 4 | Pt 5 |
|---|---|---|---|---|---|---|
| Age | yrs | 6 | 16 | 9 | 15 | 7 |
| Ethnicity | NA | Hispanic | Caucasian | Hispanic | Pakistani | Anglo |
| Signs & Symptoms | NA | Fever, organomegaly, liver failure | Fever, hepatomegaly, adenopathy | Fever, adenopathy, h/o osteomyelitis | Fever, splenomegaly, adenopathy | Fever, bilateral knee swelling |
| Hgb | g/dl | 8.3 | 9.4 | 9.6 | 7.6 | 8.5 |
| WBC | 103/ml | 2.8 | 1.2 | 61.9 | 0.47 | 7.5 |
| Platelets | 103/ml | 54 | 22 | 116 | 91 | 87 |
| Triglycerides | mg/dl | 366 | 178 | 413 | 284 | 200 |
| Fibrinogen | mg/dl | 88 | 184 | 541 | 243 | 287 |
| NK-Activity | Lytic Units | NA | Decreased | Normal | Absent | Normal |
| Ferritin | ng/ml | 63,919 | 9,640 | 7,718 | 1,542 | 30,486 |
| Soluble CD-25 | pg/ml | NA | 17,697 | 79,060 | 4,470 | 9,572 |
| Hemophagocytosis | NA | Bone Marrow - positive | Bone Marrow - positive | Liver - positive | Bone Marrow - negative | Bone Marrow - positive |
| Blood Pressure | mm Hg | 100/50 | 92/51 | 94/49 | 78/32 | 99/44 |
| AST/ALT | U/L | 4736/1406 | 761/430 | 77/22 | 570/102 | 319/226 |
| PT/PTT | sec | 24.1/80.8 | 18.2/55.8 | 15.7/41.3 | 16.3/64.4 | 16.7/40.1 |
| Serum Cr | mg/dl | 0.6 | 0.9 | 0.8 | 0.7 | 0.5 |
| CNS Disease at Diagnosis | NA | Unknown | Positive – based on MRI | Positive – based on MRI | Negative | Negative |
| Baseline MRI | NA | NA | 4 mm left parietal lesion | Scattered small white matter lesions in frontal lobes | Cerebral volume loss | Cerebral volume loss |
| Baseline Lumbar Puncture | Cells/mm3 | NA | 0 WBC 10 RBC | 0 WBC 157 RBC | 10 WBC 15 RBC | NA |