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Dermatomyositis Associated With High-Grade B-Lymphoproliferative Disorders: A Case Report and Literature Review
Jun Yen Ng1, Mridula Mokoonlall1, Hana Kawatu1
1Canberra Hospital, ACT, Australia.
Summary
Idiopathic inflammatory myopathies (IIMs), like dermatomyositis, are linked to cancers. This case highlights the crucial association between IIM and lymphoproliferative disorders (LPDs), emphasizing vigilant cancer screening.
Area of Science:
- Rheumatology
- Oncology
- Hematology
Background:
- Idiopathic inflammatory myopathies (IIMs), including dermatomyositis (DM) and polymyositis, are autoimmune disorders.
- IIMs are associated with various malignancies, predominantly solid tumors, but also hematological malignancies.
Purpose of the Study:
- To present a case of DM associated with diffuse large B-cell lymphoma.
- To review the literature on the pathogenesis, clinical course, treatment, and prognosis of IIM-associated lymphoproliferative disorders (LPDs).
- To discuss diagnostic and management challenges of LPDs in IIM patients and highlight the importance of cancer screening.
Main Methods:
- Case report presentation.
- Comprehensive literature review focusing on IIM and LPD association.
- Discussion of diagnostic and management strategies.
- Analysis of current cancer screening guidelines for IIM patients.
Main Results:
- A case of dermatomyositis co-occurring with diffuse large B-cell lymphoma was identified.
- Literature review confirmed a significant association between IIM and LPDs, though less common than solid tumors.
- Challenges in diagnosing and managing underlying LPDs in IIM patients were identified.
Conclusions:
- Vigilance for LPDs is essential in patients diagnosed with IIM.
- The International Guideline for IIM-Associated Cancer Screening provides a framework for proactive screening.
- Further research is needed to refine cancer screening protocols and address knowledge gaps in IIM-associated cancers.

