Case Report
Autoimmune Lymphoproliferative Syndrome and Epstein-Barr Virus-Associated Lymphoma: An Adjunctive Diagnostic Role for Monitoring EBV Viremia?
Table 1
Clinical and laboratory profiles of ALPS patients with EBV lymphoma and/or EBV viremia.
| Patient (reference; pedigree if available) | Country | Sex | Age of ALPS manifestations | Adeno-pathy | Spleno-megaly | Anemia | Thrombo-cytopenia | % DNTC* | EBV+ Lymphoma | Lymphoma onset (years after ALPS onset) | EBV viremia | FAS mutation | Outcome |
|
1† | Canada | M | 4 years | + | + | + | − | 15 | Composite lymphoma (follicular lymphoma, diffuse large B-cell lymphoma, and classic Hodgkin’s lymphoma) | 30 | + (progressively increasing) | 784AT | Treated with chemotherapy. Alive. |
| 2 [3] (26-II-4) | USA | M | 2 years | + | + | + | + | 0.7 | Omental Burkitt’s lymphoma | 48 | NR | 973AT | Treated with chemotherapy. Survived. |
| 3 [3] (26-IV-5) | USA | M | 10 months | + | + | + | NR | 2.1 | Classic Hodgkin’s lymphoma (mixed cellularity) | 7 | NR | 973AT | Treated with chemotherapy; survived. Diagnosed with histiocytic sarcoma (at age 13); died from pulmonary hemorrhage after bone marrow transplant |
| 4 [3] (45-III-2) | USA | M | 5 years | + | + | + | + | 11.7 | Hodgkin’s lymphoma | 6 | NR | 779del11 | Treated with chemotherapy. Outcome NR. |
| 5 [3] (G3-III-4) | Germany | F | NR | + | + | − | + | 75 | T-cell lymphoma | 2 | NR | 1009AG | Treated with chemotherapy. Survived |
| 6 [8] | Japan | M | 3 months | + | + | − | + | 10.4 | NR | NR | + | IVS8 + 5GT | Alive at 15 years old. |
| 7 [8] | Japan | M | 1 | + | + | + | − | 20.3 | NR | NR | + | 1020CT | Alive at 14 years old. |
| 8 [8] | Japan | M | 2 | + | + | − | + | 12.5 | NR | NR | + | 1020CT | NR |
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“+”: presence. “−”: absence. NR: not reported. *DNTC: double negative T cells = TCR α/β CD4- CD8-T cells. †Current case; [3] Straus et al. Blood, 2001. [8] Nomura et al. Int J Hematol, 2011.
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