Table 1.
Comparison of the present case with previously published cases of ALCL-associated hemophagocytic syndrome.
| Present case | Krenova et al. [11] | Sovinz et al. [12] | Sevilla et al. [13] | Cho et al. [14] | ||
|---|---|---|---|---|---|---|
| Age, gender | 20 years, M | 17 years, F | 15 years, M | 16 years, F | 5 years, F | 25 years, M |
| History and physical exam | FUO, facial swelling, LAD, and uveitis | FUO, LAD, and splenomegaly | FUO, neck swelling, and splenomegaly | FUO, splenomegaly | FUO, abdominal pain, and headache | FUO, myalgia, weakness, jaundice, LAD, and splenomegaly |
|
| ||||||
| Labs | ||||||
| WBC (cells/L) | 2.0 × 109 | — | 5.0 × 109 | 1.4 × 109 | 5.2 × 109 | 1.6 × 109 |
| ANC (cells/L) | 0.7 × 109 | 0.4 × 109 | — | — | — | — |
| Hemoglobin (g/dL) | 9.5 | 4.4 | 10.1 | 7 | 8.9 | 8.8 |
| Platelets (cells/L) | 145 × 103 | 46 × 103 | 167 × 103 | 84 × 103 | 213 × 103 | 88 × 109 |
| Triglycerides (mg/dL) | 284 | 316 | 218 | — | — | 113 |
| Fibrinogen (mg/dL) | 246 | 70 | 210 | 320 | 91 | 150 |
| Ferritin (ng/mL) | 13500 | 20 (g/L) | 9270 | 5166 | — | 2240 |
| Imaging studies | Soft tissue swelling, splenomegaly, fat stranding, and lung nodules | — | Cervical LAD | Mediastinal LAD, splenomegaly, and lung nodules | Splenomegaly, mediastinal and hilar LAD | Cervical, mediastinal, and abdominal LAD, hepatomegaly, and splenomegaly |
| Initial diagnosis | IAHS | Hepatitis | IAHS | IAHS | IAHS | ALCL |
|
| ||||||
| Biopsy | ||||||
| Lymph node | — | ALCL | HPS; ALCL | ALCL | ALCL | ALCL |
| Soft tissue | Necrosis, HPS | — | — | — | — | |
| Bone marrow | HPS | HPS; ALCL | HPS | HPS | HPS | HPS |
| Lung nodule | Nonspecific | — | — | ALCL | — | — |
| Final diagnosis | ALCL | ALCL | ALCL | ALCL | ALCL | ALCL |
Abbreviations: M: male; F: female; FUO: fever of unknown origin; LAD: lymphadenopathy; WBC: white blood cells; ANC: absolute neutrophil count; ALCL: anaplastic large cell lymphoma; HPS: hemophagocytic syndrome; IAHS: infection-associated hemophagocytic syndrome.