| Patient | Age (years)/sex | Type of PID | Lymphoma | Stage | Involved sites | EBVc in tumor | EBV in blood (DNA) | Treatment | Outcome |
| 1 | 0.6/F | SCIDd, atypical | DLBCLe | III | Lungs, pleura, chest soft tissues* | POSf | NEGg | A# + R –5 courses R-EPOCH, 2/3 dosage | CRh 1, alive disease-free for 4.8 years |
| 2 | 2.2/F | SCID, atypical | DLBCL, immunoblastic | IV | CNSj (massive brain* tumor), mediastinum, lung* | POS | POS | Individual 5 courses with HD MTX or ARA-C + R | Progression after the 5th course, died of disease at 6 months after the diagnosis |
| 3 | 1.4/F | CIDk, NSl | PTCLm, CD8+, NOSn | III | Peripheral LNo*, tonsils, mediastinum, lungs, inguinal LN, ileac LN, spleen, retroperitoneal LN | NDp | ND | AA#, 2/3 dosage | Partial response, death of sepsis after the 1st course |
| 4 | 1.2/M | CID, NS | DLBCL, centroblastic | III | Peripheral LN*, mediastinum, lungs, abdominal LN, retroperitoneal LN, hepar*, small and large intestines | POS | POS | Dexamethasone only | Sepsis and generalized EBV infection initially, death after 7 days of therapy |
| 5 | 0.7/F | CID, NS | DLBCL, immunoblastic | III | Peripheral LN*, mediastinum, lungs, salivary glands, spleen, abdominal LN | POS | POS | Dexamethasone only | Sepsis and generalized EBV infection initially, death after 5 days of therapy |
| 6 | 1.1/M | CID, NS | DLBCL, immunoblastic | III | Lungs*, hepar, spleen, cervical LN*, abdominal LN | POS | POS | R-EPOCH, 3/4 dosage | Partial response, sepsis initially, death of sepsis after the 1st course of therapy |
| 7 | 8.3/M | CID, NS | T-LLr (pro-T) | IV | Peripheral LN*, abdominal LN, retroperitoneal LN, hepar, spleen, BMs | ND | ND | NHL-BFM 95, TG1, full dosage | Early relapse (BM) during maintenance, death of sepsis in CR 2 at 2 years after the diagnosis |
| 8 | 15.5/F | CID, NS | ALCLt, cutaneous, ALKu - negative | I | Skin* of the left upper eyelid | ND | ND |
Brachytherapy only | CR 1, alive disease-free for 2.0 years |
| 9 | 6.3/M | WASw | Burkitt | III | Small intestine*, abdominal LN* | ND | ND | AA–BB–AA–BB–AA–BB#, full dosage | Second malignancy (B-mature leukemia) in 15 years, died at 15.1 years after the first diagnosis |
| 10 | 10.9/F | NBSx | DLBCL, T-cell/rich | III | Peripheral LN*, mediastinum, lungs, spleen, abdominal LN, retroperitoneal LN | ND | ND | A–B#, full dosage, stop therapy in unconfirmed remission because of parent's refusal | Second NHL in 4.5 years, no treatment, died of disease at 4.6 years after the first diagnosis |
| 11 | 5.6/M | NBS | T-LL (mature CD8+) | III | Mediastinum, lung, peripheral LN* | NEG | ND | NHL-BFM-95, TG1: I phase I protocol, HR1-HR2-HR3-HR1-HR2-HR3 (full dosage), nonmyeloablative conditioning, HSCT, unrelated | CR 1, alive disease-free for 1.4 years |
| 12 | 16.9/M | NBS | PTCL, small cell type, CD8+, high grade | III | Mediastinum (bulky disease), peripheral LN* | NEG | ND | CHOP–CHOP–ICE–DHAP–DHAP–DexaBEAM, full dosage | Progression after the 5th course, died of disease at 5 months after the diagnosis |
| 13 | 14.2/M | NBS | T-LL (cortical) | III | Mediastinum* (bulky disease) | ND | ND | NHL-BFM 95, TG1, ImRG, protocol I, full dosage | Death of sepsis in partial remission after 7 weeks of therapy |
| 14 | 9.1/F | NBS | ALCL | III | Peripheral LN*, mediastinum, abdominal LN, retroperitoneal LN, ileac LN | ND | NEG | A# (no MTX) | Partial response, died of invasive pulmonary aspergillosis after the 1st course |
| 15 | 15.9/M | Bloom syndrome | ALCL, ALK-negative | III | Peripheral LN*, mediastinum, lungs, abdominal LN, retroperitoneal LN, ileac LN | NEG | NEG | ALCL99, HRG, full dosage | Secondary hemophagocytic syndrome during maintenance, death of sepsis at 1.3 years after the diagnosis |
| 16 | 6.0/M | XLPS-1y | Burkitt | III | Mesentery, jejunum*, abdominal LN*, bladder, abdominal wall | NEG | ND | NHL-BFM 95, TG2, R2, full dosage, +3 doses of R | CR 1, alive disease-free for 5.7 years |
|
|
Primary immune deficiency disease, bnon-Hodgkin lymphoma, cEpstein-Barr virus, dsevere combined immunodeficiency, ediffuse large B-cell lymphoma, fpositive, gnegative, hcomplete remission, jcentral nervous system, kCombined immunodeficiency, lNot specified, mPeripheral T-cell lymphoma, nNot other specified, oLymph node, pno data, rlymphoblastic lymphoma, sbone marrow, tanaplastic large cell lymphoma, uanaplastic lymphoma kinase, wWiskott-Aldrich syndrome, xNijmegen Breakage syndrome, yХ-linked lymphoproliferative syndrome-1, *site of biopsy, and #chemotherapy course, see [5] (Reiter et al. Blood, 1999).
|