Diagnostic details of lymphoma in ALPS-FAS (16 patients and 2 HMPRs)
| Family number . | Age at lymphoma diagnosis (years) . | Gender (male [M]/female [F]) . | Lymphoma HL/NHL . | Stage . | Immunosuppressive medications prior to lymphoma diagnosis . | EBV status by testing for EBER . | Histopathology details . | Outcome . |
|---|---|---|---|---|---|---|---|---|
| 3.3 | 25.0 | M | NHL | IV | No | Not tested | T-cell and histiocyte rich large B-cell lymphoma (LBCL) | Deceased lymphoma |
| 3.4 | 25.0 | M | HL | IIIA | No | Not tested | Nodular lymphocyte predominant HD (NLPHL) with recurrence ×2 | Alive |
| 24.1 | 19.0 | M | HL | IA | Yes | Not tested | NLPHL | Alive |
| 26.1 | 6.9 | M | HL | IIIB | Yes | Positive | CHL, MC, interfollicular, mixed cellularity | |
| 26.1 | 13.0 | M | Histiocytic sarcoma | IV | Yes | Not tested | Second malignancy with history of sinus histiocytosis with massive lymphadenopathy (SHML) | Deceased histiocytic sarcoma |
| 26.9 | 50.0 | M | NHL | IIIB | No | Positive | Burkitt lymphoma | Alive |
| 30.16 | 17.0 | F | NHL | NA | No | Negative | Burkitt lymphoma | Alive |
| 45.1 | 13.2 | M | HL | IV | No | Positive | CHL, MC, interfollicular, mixed cellularity | Deceased unrelated to malignancy |
| 55.1 | 17.0 | M | NHL | IIIB | No | Not tested | follicular lymphoma (atypical) | Alive |
| 64.1 | 25.7 | M | NHL | IIIB | No | Negative | Burkitt lymphoma | Alive |
| 89.1 | 17.1 | F | HL | IA | No | Not tested | NLPHL in the background of PTGC | Alive |
| 141.23 | 38.5 | F | HL | IIIB | No | Positive | CHL, MC | Alive |
| 149.1 | 7.8 | M | HL | IVB | Yes | Positive | CHL, MC | Deceased transplant related |
| 149.3 | 7.0 | F | HL | II | No | Not tested | CHL, MC, a diagnosis of mixed cellularity HL favored, though some lacunar variant cells suggest nodular sclerosing subtype | Alive |
| 215.1 | 17.3 | M | HL | IVB | No | Positive | CHL, MC, classic by bone marrow and liver biopsy | Deceased transplant related |
| 230.3 HMPR | 52.0 | M | HL | IIIB | No | Positive | CHL, lymphocyte rich | |
| 230.3 HMPR | 53.5 | M | NHL | NA | No | Not tested | Second malignancy PTCL, follicular variant | Deceased lymphoma |
| 230.8 HMPR | 60.0 | F | NHL | IA | No | Not tested | Cutaneous mantle zone lymphoma | Alive |
| 255.1 | 37.0 | M | NHL | IVB | Yes | Not tested | T-cell/histiocyte-rich LBCL | Alive |
| 294.1 | 5.0 | M | HL | IVB | No | Positive | CHL, MC | Alive |
| Family number . | Age at lymphoma diagnosis (years) . | Gender (male [M]/female [F]) . | Lymphoma HL/NHL . | Stage . | Immunosuppressive medications prior to lymphoma diagnosis . | EBV status by testing for EBER . | Histopathology details . | Outcome . |
|---|---|---|---|---|---|---|---|---|
| 3.3 | 25.0 | M | NHL | IV | No | Not tested | T-cell and histiocyte rich large B-cell lymphoma (LBCL) | Deceased lymphoma |
| 3.4 | 25.0 | M | HL | IIIA | No | Not tested | Nodular lymphocyte predominant HD (NLPHL) with recurrence ×2 | Alive |
| 24.1 | 19.0 | M | HL | IA | Yes | Not tested | NLPHL | Alive |
| 26.1 | 6.9 | M | HL | IIIB | Yes | Positive | CHL, MC, interfollicular, mixed cellularity | |
| 26.1 | 13.0 | M | Histiocytic sarcoma | IV | Yes | Not tested | Second malignancy with history of sinus histiocytosis with massive lymphadenopathy (SHML) | Deceased histiocytic sarcoma |
| 26.9 | 50.0 | M | NHL | IIIB | No | Positive | Burkitt lymphoma | Alive |
| 30.16 | 17.0 | F | NHL | NA | No | Negative | Burkitt lymphoma | Alive |
| 45.1 | 13.2 | M | HL | IV | No | Positive | CHL, MC, interfollicular, mixed cellularity | Deceased unrelated to malignancy |
| 55.1 | 17.0 | M | NHL | IIIB | No | Not tested | follicular lymphoma (atypical) | Alive |
| 64.1 | 25.7 | M | NHL | IIIB | No | Negative | Burkitt lymphoma | Alive |
| 89.1 | 17.1 | F | HL | IA | No | Not tested | NLPHL in the background of PTGC | Alive |
| 141.23 | 38.5 | F | HL | IIIB | No | Positive | CHL, MC | Alive |
| 149.1 | 7.8 | M | HL | IVB | Yes | Positive | CHL, MC | Deceased transplant related |
| 149.3 | 7.0 | F | HL | II | No | Not tested | CHL, MC, a diagnosis of mixed cellularity HL favored, though some lacunar variant cells suggest nodular sclerosing subtype | Alive |
| 215.1 | 17.3 | M | HL | IVB | No | Positive | CHL, MC, classic by bone marrow and liver biopsy | Deceased transplant related |
| 230.3 HMPR | 52.0 | M | HL | IIIB | No | Positive | CHL, lymphocyte rich | |
| 230.3 HMPR | 53.5 | M | NHL | NA | No | Not tested | Second malignancy PTCL, follicular variant | Deceased lymphoma |
| 230.8 HMPR | 60.0 | F | NHL | IA | No | Not tested | Cutaneous mantle zone lymphoma | Alive |
| 255.1 | 37.0 | M | NHL | IVB | Yes | Not tested | T-cell/histiocyte-rich LBCL | Alive |
| 294.1 | 5.0 | M | HL | IVB | No | Positive | CHL, MC | Alive |
Stage with A or B suffix denotes absence or presence of systemic B symptoms. Most of the patients had documented systemic B symptoms and/or advanced stage IV disease. NA, not available; NHL, non-Hodgkin lymphoma; HL, Hodgkin lymphoma; CHL, classical HL; MC, mixed cellularity; PTGC, progressive transformation of germinal center; PTCL, peripheral T-cell lymphoma. Atypical lymphomas noted in the 2 older HMPRs belonged to the same family. Patients 3.3, 3.4, 26.9, 30.16, and 55.1 were also included in the earlier report.6