Primary thyroid marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type: Clinical manifestation and outcome of a rare disease - Consortium for improving survival of lymphoma study

Sung Yong Oh, Won Seog Kim, Jin Seok Kim, Seok Jin Kim, Suee Lee, Dae Ho Lee, Hye Jin Kang, Moo Kon Song, Hyo Jung Kim, Jung Hye Kwon, Jae Yong Kwak, Byeong Bae Park, Young Rok Do, Seong Hyun Jeong, Cheolwon Suh

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Abstract

Purpose: Primary thyroid marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type (pTY-MZL) is an extremely uncommon form of lymphoma. Due to its rarity, the natural history and optimal treatment modality for this disease have yet to be clearly established. Methods: A total of 27 patients with histologically confirmed pTY-MZL were retrospectively analyzed. Results: The median age of our subjects was 53 years (range 25-82). This study involved 17 females (63.0%) and 10 males (37.0%). Twenty-four out of 27 patients (88.9%) initially presented with localized disease, defined by Ann Arbor stage I/II. Bone marrow involvement was detected in 8.3% of the patients (2 patients), and 91.7% of the patients (25 of 27) were categorized into the low or low-intermediate risk group, according to the International Prognostic Index criteria. Accompanying Hashimoto's thyroiditis was detected in 72% of the patients, whereas thyroglobulin antibody levels were elevated in 70% of the patients. Twenty-six patients were treated with surgery, radiotherapy or chemotherapy, and 25 patients achieved complete remission. During the follow-up period, only 2 patients evidenced progression, and no deaths occurred over the course of the study. Conclusion: pTY-MZL tends to be an indolent disease. However, unlike other mucosa-associated lymphoid tissue site MZLs, pTY-MZL was well controlled via several treatment modalities, and the patients' responses were sustained for a prolonged period.

Original languageEnglish
Pages (from-to)100-104
Number of pages5
JournalActa Haematologica
Volume127
Issue number2
DOIs
Publication statusPublished - 2012 Feb

All Science Journal Classification (ASJC) codes

  • Hematology

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    Oh, S. Y., Kim, W. S., Kim, J. S., Kim, S. J., Lee, S., Lee, D. H., Kang, H. J., Song, M. K., Kim, H. J., Kwon, J. H., Kwak, J. Y., Park, B. B., Do, Y. R., Jeong, S. H., & Suh, C. (2012). Primary thyroid marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type: Clinical manifestation and outcome of a rare disease - Consortium for improving survival of lymphoma study. Acta Haematologica, 127(2), 100-104. https://doi.org/10.1159/000333113