Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis
Introduction. The association between Sjögren’s syndrome (SS) and the development of lymphoma is well known. The prevalence of lymphoma in patients with SS is 5%. Mucosa-associated lymphoid tissue (MALT) lymphoma is the most common lymphoma type in patients with SS. It is common for MALT lymphoma to...
Saved in:
| Main Authors: | , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade
2024-01-01
|
| Series: | Vojnosanitetski Pregled |
| Subjects: | |
| Online Access: | https://doiserbia.nb.rs/img/doi/0042-8450/2024/0042-84502400039B.pdf |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1850163231178358784 |
|---|---|
| author | Božić Ksenija Živojinović Dragan Đenić Ljubinko Atanasković Lavinika |
| author_facet | Božić Ksenija Živojinović Dragan Đenić Ljubinko Atanasković Lavinika |
| author_sort | Božić Ksenija |
| collection | DOAJ |
| description | Introduction. The association between Sjögren’s syndrome (SS) and the development of lymphoma is well known. The prevalence of lymphoma in patients with SS is 5%. Mucosa-associated lymphoid tissue (MALT) lymphoma is the most common lymphoma type in patients with SS. It is common for MALT lymphoma to develop in the stomach, while it is extremely rare in the thymus. Case report. We present a 61-year-old Caucasian male patient with primary SS, cutaneous vasculitis, and thymic MALT lymphoma. The patient had a two-year history of diffuse cutaneous palpable purpura on legs, intermittently enlarged left parotid gland, and dry mouth. The results of Schirmer’s test were positive, labial salivary glands biopsy revealed a focus score ≥ 1, serology testing showed positive anti-Ro/SS-A and anti- La/SS-B antibodies, while skin biopsy findings revealed leukocytoclastic vasculitis. Diagnosis of primary SS with extraglandular cutaneous manifestations was confirmed. Cryoglobulinemia (Cg) and monoclonal gammopathy (MG) were detected, which increased the suspicion of hematological malignancy, and additional diagnostic procedures were performed. Computed tomography of the chest revealed an enlarged, multicystically altered anterior mediastinal mass. A thymectomy was performed through video-assisted thoracic surgery. Histological findings of the tissue confirmed the presence of tumor tissue consistent with MALT lymphoma in the thymus. Induction therapy with pulse doses of glucocorticoids was applied for three days, which was continued with medium doses of the drug. The doses were gradually reduced, and hydroxychloroquine was introduced. This has shown to be an effective therapy against features of SS. Postoperative local radiotherapy was performed. Conclusion. In SS patients with CV and in the presence of Cg and MG, attention should also be paid to the eventual development of MALT lymphoma, including the rare localization in the thymus. |
| format | Article |
| id | doaj-art-f0875a5c75b34d61bfc9e40c60762a5f |
| institution | OA Journals |
| issn | 0042-8450 2406-0720 |
| language | English |
| publishDate | 2024-01-01 |
| publisher | Ministry of Defence of the Republic of Serbia, University of Defence, Belgrade |
| record_format | Article |
| series | Vojnosanitetski Pregled |
| spelling | doaj-art-f0875a5c75b34d61bfc9e40c60762a5f2025-08-20T02:22:20ZengMinistry of Defence of the Republic of Serbia, University of Defence, BelgradeVojnosanitetski Pregled0042-84502406-07202024-01-0181851952310.2298/VSP230713039B0042-84502400039BThymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitisBožić Ksenija0Živojinović Dragan1Đenić Ljubinko2Atanasković Lavinika3https://orcid.org/0000-0002-4975-6867University of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia + Military Medical Academy, Clinic for Rheumatology, Belgrade, SerbiaMilitary Medical Academy, Institute of Pathology, Belgrade, SerbiaMilitary Medical Academy, Clinic for Thoracic Surgery, Belgrade, SerbiaUniversity of Defence, Faculty of Medicine of the Military Medical Academy, Belgrade, Serbia + Military Medical Academy, Clinic for Hematology, Belgrade, SerbiaIntroduction. The association between Sjögren’s syndrome (SS) and the development of lymphoma is well known. The prevalence of lymphoma in patients with SS is 5%. Mucosa-associated lymphoid tissue (MALT) lymphoma is the most common lymphoma type in patients with SS. It is common for MALT lymphoma to develop in the stomach, while it is extremely rare in the thymus. Case report. We present a 61-year-old Caucasian male patient with primary SS, cutaneous vasculitis, and thymic MALT lymphoma. The patient had a two-year history of diffuse cutaneous palpable purpura on legs, intermittently enlarged left parotid gland, and dry mouth. The results of Schirmer’s test were positive, labial salivary glands biopsy revealed a focus score ≥ 1, serology testing showed positive anti-Ro/SS-A and anti- La/SS-B antibodies, while skin biopsy findings revealed leukocytoclastic vasculitis. Diagnosis of primary SS with extraglandular cutaneous manifestations was confirmed. Cryoglobulinemia (Cg) and monoclonal gammopathy (MG) were detected, which increased the suspicion of hematological malignancy, and additional diagnostic procedures were performed. Computed tomography of the chest revealed an enlarged, multicystically altered anterior mediastinal mass. A thymectomy was performed through video-assisted thoracic surgery. Histological findings of the tissue confirmed the presence of tumor tissue consistent with MALT lymphoma in the thymus. Induction therapy with pulse doses of glucocorticoids was applied for three days, which was continued with medium doses of the drug. The doses were gradually reduced, and hydroxychloroquine was introduced. This has shown to be an effective therapy against features of SS. Postoperative local radiotherapy was performed. Conclusion. In SS patients with CV and in the presence of Cg and MG, attention should also be paid to the eventual development of MALT lymphoma, including the rare localization in the thymus.https://doiserbia.nb.rs/img/doi/0042-8450/2024/0042-84502400039B.pdfcryoglobulinemialymphomab-cellmarginal zonesjogren’s syndromethymus neoplasmsvasculitisleucocytoclasticcutaneous |
| spellingShingle | Božić Ksenija Živojinović Dragan Đenić Ljubinko Atanasković Lavinika Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis Vojnosanitetski Pregled cryoglobulinemia lymphoma b-cell marginal zone sjogren’s syndrome thymus neoplasms vasculitis leucocytoclastic cutaneous |
| title | Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis |
| title_full | Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis |
| title_fullStr | Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis |
| title_full_unstemmed | Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis |
| title_short | Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis |
| title_sort | thymic mucosa associated lymphoid tissue lymphoma in a patient with sjogren s syndrome with cutaneous vasculitis |
| topic | cryoglobulinemia lymphoma b-cell marginal zone sjogren’s syndrome thymus neoplasms vasculitis leucocytoclastic cutaneous |
| url | https://doiserbia.nb.rs/img/doi/0042-8450/2024/0042-84502400039B.pdf |
| work_keys_str_mv | AT bozicksenija thymicmucosaassociatedlymphoidtissuelymphomainapatientwithsjogrenssyndromewithcutaneousvasculitis AT zivojinovicdragan thymicmucosaassociatedlymphoidtissuelymphomainapatientwithsjogrenssyndromewithcutaneousvasculitis AT đenicljubinko thymicmucosaassociatedlymphoidtissuelymphomainapatientwithsjogrenssyndromewithcutaneousvasculitis AT atanaskoviclavinika thymicmucosaassociatedlymphoidtissuelymphomainapatientwithsjogrenssyndromewithcutaneousvasculitis |