Age / Sex : 39 / F

Chief complaint: Weight loss, night fever and multiple enlarged lymph nodes of both axillas

What is your impression?

Two weeks later, you can see the final diagnosis with a brief discussion of this case.

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Courtesy: Yi Jaehyuck, Kyungpook National University Hospital

Castleman’s disease variant of POEMS syndrome

Discussion

Findings:

1) Multiple enlarged lymph nodes of both axillas

2) Large effusion of right hemithorax

3) Splenomegaly

4) Multiple sclerotic nodules of the vertebrae

Differential Diagnosis: lymphoma, myeloma, sarcoidosis

Diagnosis: Castleman’s disease variant of POEMS syndrome

Discussion:

1. POEMS syndrome

•       Paraneoplastic syndrome due to an underlying plasma cell disorder (PCD)

•       Polyradiculoneuropathy

•       Organomegaly

•       Endocrinopathy

•       Monoclonal PCD: myeloma, MGUS…

•       Skin changes

2. Castleman disease variant of POEMS

•       Angiofollicular LN hyperplasia

•       Monocentric / multicentric

•       Simple B symptom ~ frank POMES manifestation

•       Peripheral neuropathy: more subtle than that of POEMS syndrome with osteosclerotic myeloma

References:

1. Angela D. et al. POMES syndrome: 2014 update on diagnosis, risk-stratification, and management. American Journal of Hematology 2014;89(2):213-223