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Premature vascular calcification in a case of POEMS syndrome
1Department of Diagnostic Radiology, University of South Manchester, U.K.
Insights
This case study highlights extensive arterial calcification in POEMS syndrome, a rare disorder. The findings suggest a link between POEMS syndrome, myeloproliferative disorders, and early-onset vascular calcification.
Area of Science:
- Hematology
- Vascular Medicine
- Oncology
Background:
- POEMS syndrome is a rare multisystem disorder.
- Myeloproliferative disorders can be associated with POEMS syndrome.
- Arterial calcification is not a typical feature of POEMS syndrome.
Observation:
- A patient with POEMS syndrome and a myeloproliferative disorder presented with early-onset extensive arterial calcification.
- Skeletal radiographs showed sclerotic foci and new bone formation.
- Histological examination revealed an osteosclerotic solitary plasmacytoma and Castleman's disease.
Findings:
- The patient exhibited dermal mast cell infiltration.
- These findings suggest a potential association between POEMS syndrome, myeloproliferative disorders, and premature arterial calcification.
- Osteosclerotic plasmacytoma and Castleman's disease were confirmed, aligning with known POEMS syndrome pathology.
Implications:
- This case expands the understanding of POEMS syndrome manifestations.
- It highlights the importance of considering vascular complications in POEMS syndrome patients.
- Further research is warranted to elucidate the mechanisms linking POEMS syndrome to arterial calcification.
Abstract:
A patient with classical POEMS syndrome associated with a myeloproliferative disorder is presented, in whom extensive arterial calcification was seen at an early age in the absence of other predisposing factors. Radiographs also demonstrated several small discrete sclerotic foci elsewhere in the skeleton and areas of new bone formation around the spine which were described in previous cases. Histological studies in this patient demonstrated an osteosclerotic solitary plasmacytoma of bone and Castleman's disease of lymph nodes, both of which have previously been causally associated with POEMS syndrome. In addition, dermal infiltration by mast cells was demonstrated.