Presentation, Characteristics and Features of Lymphoma in a Retrospective Case Series of Patients with Sjogren’s Disease
Abstract
1. Introduction
2. Materials and Methods
3. Results
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Case/Sex/ Ethnicity | Age at SjD Diagnosis | Anti-Ro/SSA Anti-La/SSB | Time (Years) from SjD Diagnosis | Lymphoma Subtype | Presentation and Site of Lymphoma | Glandular SjD Manifestations | Extraglandular SjD Manifestations | Comorbidities |
---|---|---|---|---|---|---|---|---|
1/F/Hispanic | 41 | Positive/Positive | 1 | MALT | Asymmetrical enlargement of parotid gland | XF, XS, PGE | Polyarthritis | None |
2/F/Caucasian | 32 | Positive/Positive | 4 | MALT | Bilateral cervical lymphadenopathy | XS, PGE, parotitis | Anemia, bronchiectasis, polyarthritis, RP | SLE, IBM, sarcoidosis, GERD |
3/F/AA | 67 | Positive/Negative | 7 | NSHL stage IVB | B-symptoms, supraclavicular LN, para-aortic LN | XS, PGE | Neuropathy | Hypothyroidism |
4/F/Caucasian | 51 | Negative/Negative | 21 | MZL | B-symptoms, monoclonal gammopathy, inguinal LN | XF, XS | Bronchiectasis, interstitial fibrosis, nephrocalcinosis | IBD |
5/M/Caucasian | 32 | Positive/Negative | 23 | DLBCL, stage III | B-symptoms, parotid gland enlargement, cervical LN, mediastinal LN, axillary LN, inguinal LN, retroperitoneal LN | XF, XS, PGE | VL, RP | Hypothyroidism |
6/F/AA | 42 | Positive/Positive | 17 | MALT | Conjunctival mass, ocular inflammation, weight loss/lacrimal gland | XF, XS | Bronchiectasis, pulmonary fibrosis, synovitis, neuropathy | SLE, cryoglobulinemia GN |
7/F/Caucasian | 40 | Positive/Positive | 14 | MALT | Intraglandular palpable mass followed by diffuse lymphadenopathy/Axillary LN | XF, XS, sialadenitis | Neuropathy, VL | AH |
8/F/Caucasian | 56 | Positive/Negative | 11 | DLBCL, stage IV | Upper abdominal pain, anorexia, weight loss, LUQ mass | XS, XF, PGE | Polyarthritis | HLD, HTN, breast cancer |
9/F/Caucasian | 46 | Positive/Negative | 25 | DLBCL, stage 1AE, in CR | LUQ pain, gastric mass on EGD | XS, XF, Parotid abscess | Tubulointerstitial nephropathy/RTA, nephrocalcinosis | SCAD with vfib arrest, melanoma |
10/F/Asian Indian | 42 | Positive/Positive | 20 | Pulmonary MALT lymphoma | Incidental finding on CT chest, RML mass and RLL nodule | XS, XF | Polyarthritis | HLD, GERD, vasovagal syncope |
Case 1/F | Case 2/F | Case 3/F | Case 4/F | Case 5/M | Case 6/F | Case 7/F | Case 8/F | Case 9/F | Case 10/F | |
---|---|---|---|---|---|---|---|---|---|---|
Persistent enlargement of parotid glands | + | + | − | − | + | + | + | + | + | − |
Peripheral neuropathy | − | − | + | − | − | + | + | − | − | − |
Palpable purpura | − | − | − | − | + | − | − | − | − | − |
Splenomegaly | − | − | − | − | + | − | − | − | − | − |
ANA positivity | + | + | + | + | + | + | + | + | + | + |
Rheumatoid factor positivity | + | NA | − | + | + | NA | + | + | − | + |
Lymphopenia 1 | − | + | − | + | − | + | + | − | + | − |
Monoclonal proteins in serum and/or urine | − | − | + | + | − | − | − | − | + | − |
Low C4 | − | + | NA | − | + | + | − | − | − | − |
Elevated immunoglobulins | − | + | + | − | + | + | + | − | − | + |
Cryoglobulinemia | − | − | − | − | NA | + | − | − | − | − |
Case/Sex | Age at Lymphoma Diagnosis | Lymphoma Subtype | Initial Induction Therapy | Duration | Response to Therapy | Age at Relapse (Years) | Number of Relapses/ Location | Treatment at Relapse | Response to Therapy | Follow-Up Period (mo) | Disease Evolution |
---|---|---|---|---|---|---|---|---|---|---|---|
1/F | 42 | MALT | Rituximab | 4 weeks | PR | 43 | 1/parotid gland and lip nodule | Rituximab | CR | 202 | Free of disease |
2/F | 36 | MALT | 3000 cGY to right neck over 15 fractions | 2 weeks | CR | 41, 58 | 1/parotid gland and 2/parotid gland | 1/radiation and 2/resection of gland and rituximab | CR | 312 | Death unrelated to disease progression |
3/F | 74 | NSHL stage IVB | R-CHOP | 6 months | CR | 88 | 1/intrathoracic lymph nodes | Prednisone | PR | 264 | On maintenance treatment |
4/F | 72 | MZL | Surveillance | - | - | - | - | - | - | 12 | Death unrelated to disease progression |
5/M | 55 | DLBCL, stage III | CHOP | 1 month | NR | - | - | - | - | 2 | Death by disease progression |
6/F | 59 | MALT | Rituximab | weeks | CR | 59 | 1/lacrimal | Rituximab | CR | 24 | Death unrelated to disease progression |
7/F | 54 | MALT | Surveillance | - | - | - | - | - | - | 165 | Free of disease |
8/F | 67 | DLBCL stage IV | RCHOP | 4 months | CR | - | - | - | CR | 30 | Free of disease |
9/F | 71 | DLBCL, stage 1AE | RCHOP | 6 months | CR | - | - | - | - | 21 | Death unrelated to disease progression |
10/F | 65 | Pulmonary MALT lymphoma | Surveillance | - | - | - | - | - | - | 22 | Under surveillance |
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Behbodikhah, J.; Balistreri, L.; Carsons, S.E. Presentation, Characteristics and Features of Lymphoma in a Retrospective Case Series of Patients with Sjogren’s Disease. Rheumato 2024, 4, 153-162. https://doi.org/10.3390/rheumato4030012
Behbodikhah J, Balistreri L, Carsons SE. Presentation, Characteristics and Features of Lymphoma in a Retrospective Case Series of Patients with Sjogren’s Disease. Rheumato. 2024; 4(3):153-162. https://doi.org/10.3390/rheumato4030012
Chicago/Turabian StyleBehbodikhah, Jennifer, Lisa Balistreri, and Steven E. Carsons. 2024. "Presentation, Characteristics and Features of Lymphoma in a Retrospective Case Series of Patients with Sjogren’s Disease" Rheumato 4, no. 3: 153-162. https://doi.org/10.3390/rheumato4030012
APA StyleBehbodikhah, J., Balistreri, L., & Carsons, S. E. (2024). Presentation, Characteristics and Features of Lymphoma in a Retrospective Case Series of Patients with Sjogren’s Disease. Rheumato, 4(3), 153-162. https://doi.org/10.3390/rheumato4030012