DOI: 10.1097/md.0000000000051176 ISSN: 0025-7974

Relapsed Waldenström macroglobulinemia presenting as bilateral parotid gland enlargement with multiple cystic lesions

Hsin-Yao Hung, Yu-Cheng Chang, Tsung-Yu Yen

Rationale:

Waldenström macroglobulinemia (WM) is a rare lymphoplasmacytic malignancy, and extramedullary disease involving the parotid glands is an exceptionally uncommon presentation.

Patient concerns:

We report the case of a 79-year-old man with a history of WM who had achieved stable disease for several years following multiple courses of chemotherapy. He subsequently presented with bilateral parotid gland enlargement suspicious for relapsed WM.

Diagnoses:

Imaging studies revealed multiple cystic lesions, and relapsed WM was considered the most likely diagnosis based on markedly elevated serum immunoglobulin M (IgM) levels and polymerase chain reaction analysis of fine-needle aspirates demonstrating immunoglobulin heavy chain (IgH) gene rearrangement.

Interventions:

Given his poor clinical condition, the patient received palliative care.

Outcomes:

The patient died due to sepsis 4 months after the presentation of parotid enlargement.

Lessons:

This case highlights the importance of considering relapsed WM in the differential diagnosis of parotid gland enlargement, particularly in patients with a prior history of lymphoplasmacytic malignancy.

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