Clinical and Histological Features in 16 Patients WithFas-Mutated Lymphomas
| Case No. . | Histology . | Fas Mutation . | Primary Localization . | Localization at Progression . | Disease Status at Biopsy . | Autoimmune/ Paraneoplastic Phenomena . | Age . | Sex . | Survival From Biopsy (mo) . |
|---|---|---|---|---|---|---|---|---|---|
| 193 | B-CLL | A-1T3-150 | Blood, bone marrow | Skin | Progression | Maculo-papular skin rash | 78 | M | 6 |
| 17 | FCC | E256K | Waldeyers’ ring, lymph nodes | Parotoid gland, lymph nodes | Progression | None | 43 | M | 48 |
| 49 | FCC + DLC-B (TRB) | 598 Ins T | Spleen | Lymph nodes | Diagnosis | NA | 63 | M | 63 |
| 67 | MALT-type | IVS8nt + 5g → c | Orbit, bone marrow, liver/spleen | Skin, lymph nodes | Progression | Arthralgy, bursitis with noduli rheumatici, conjunctivitis | 43 | M | 132+ |
| 145 | MALT-type | N248K | Thyroid gland, regional lymph nodes | — | Diagnosis | Myxoedematous symptoms before lymphoma, elevated TSH | 93 | F | 17 |
| 146 | MALT-type | IVS8nt + 5g → a | Paranasal sinus, nasal cavity | Salivary gland, orbit | Progression | Recurrent pleural effusions. “Sjögren-like” | 70 | M | 39+ |
| 69 | DLC-B | L199X | Lymph node | Lymph nodes | Progression | NA | 70 | F | 1 |
| 86 | DLC-B | D244V | Skin, lymph node | — | Diagnosis | None | 90 | F | 3 |
| 92 | DLC-B | L208X | Thyroid gland | — | Diagnosis | Goiter Myxoedema, elevated TSH | 82 | F | 30 |
| 93 | DLC-B | L262F | Tonsil | Lymph node | Diagnosis | None | 80 | M | 30 |
| 128 | DLC-B | L164F | Skeletal muscle | Pancreas, stomach, liver/spleen | Diagnosis | Pancreatitis | 60 | F | 15 |
| 141 | DLC-B | K283N | Mediastinum (thymus) | — | Diagnosis | Monoclonal serum IgM, neuropathy | 54 | M | 1 |
| 156 | DLC-B | N248K | Skin | — | Diagnosis | Monoclonal serum IgGκ and IgMλ, neuropathy | 55 | F | 144+ |
| 225 | DLC-B | IVS7nt − 2a → g | Thyroid gland | Stomach | Diagnosis | Hashimoto’s thyroiditis | 82 | F | 6 |
| 157 | DLC-B (TRB) | T182I | Salivary gland | Lymph nodes | Diagnosis | SLE, Sjögren syndrome | 67 | F | 6 |
| 72 | ALCL, null | P167L | Skin | Lymph nodes | Diagnosis | NA | 62 | M | 6 |
| Case No. . | Histology . | Fas Mutation . | Primary Localization . | Localization at Progression . | Disease Status at Biopsy . | Autoimmune/ Paraneoplastic Phenomena . | Age . | Sex . | Survival From Biopsy (mo) . |
|---|---|---|---|---|---|---|---|---|---|
| 193 | B-CLL | A-1T3-150 | Blood, bone marrow | Skin | Progression | Maculo-papular skin rash | 78 | M | 6 |
| 17 | FCC | E256K | Waldeyers’ ring, lymph nodes | Parotoid gland, lymph nodes | Progression | None | 43 | M | 48 |
| 49 | FCC + DLC-B (TRB) | 598 Ins T | Spleen | Lymph nodes | Diagnosis | NA | 63 | M | 63 |
| 67 | MALT-type | IVS8nt + 5g → c | Orbit, bone marrow, liver/spleen | Skin, lymph nodes | Progression | Arthralgy, bursitis with noduli rheumatici, conjunctivitis | 43 | M | 132+ |
| 145 | MALT-type | N248K | Thyroid gland, regional lymph nodes | — | Diagnosis | Myxoedematous symptoms before lymphoma, elevated TSH | 93 | F | 17 |
| 146 | MALT-type | IVS8nt + 5g → a | Paranasal sinus, nasal cavity | Salivary gland, orbit | Progression | Recurrent pleural effusions. “Sjögren-like” | 70 | M | 39+ |
| 69 | DLC-B | L199X | Lymph node | Lymph nodes | Progression | NA | 70 | F | 1 |
| 86 | DLC-B | D244V | Skin, lymph node | — | Diagnosis | None | 90 | F | 3 |
| 92 | DLC-B | L208X | Thyroid gland | — | Diagnosis | Goiter Myxoedema, elevated TSH | 82 | F | 30 |
| 93 | DLC-B | L262F | Tonsil | Lymph node | Diagnosis | None | 80 | M | 30 |
| 128 | DLC-B | L164F | Skeletal muscle | Pancreas, stomach, liver/spleen | Diagnosis | Pancreatitis | 60 | F | 15 |
| 141 | DLC-B | K283N | Mediastinum (thymus) | — | Diagnosis | Monoclonal serum IgM, neuropathy | 54 | M | 1 |
| 156 | DLC-B | N248K | Skin | — | Diagnosis | Monoclonal serum IgGκ and IgMλ, neuropathy | 55 | F | 144+ |
| 225 | DLC-B | IVS7nt − 2a → g | Thyroid gland | Stomach | Diagnosis | Hashimoto’s thyroiditis | 82 | F | 6 |
| 157 | DLC-B (TRB) | T182I | Salivary gland | Lymph nodes | Diagnosis | SLE, Sjögren syndrome | 67 | F | 6 |
| 72 | ALCL, null | P167L | Skin | Lymph nodes | Diagnosis | NA | 62 | M | 6 |
Abbreviations: ALCL, anaplastic large cell lymphoma; FCC, follicle center cell lymphoma; NA, not available.
Numbering refers to the last residue of the signal peptide.