Osteosclerotic Myeloma
Definition
- Plasmacytoma with osteosclerosis accompanied by clinical POEMS syndrome and lymph node changes resembling plasma cell variant of Castleman disease
Alternate/Historical Names
- POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes)
- Multicentric Castleman disease
- Crowe-Fukase syndrome
Diagnostic Criteria
- Osteosclerotic plasmacytoma
- Plasma cells entrapped in fibrosis
- Thickened trabecular bone
- Usually lambda light chain restricted
- Usually does not meet criteria for classical myeloma
- Bone marrow plasmacytosis usually <5-10%, if present
- Serum M protein usually <3.5 g/dL, if present
- Bence Jones protein usually <1 g/24hr, if present
- Lymph nodes may show changes of multicentric plasma cell type Castleman disease
- Reactive follicular hyperplasia
- Interfollicular plasmacytosis
- Some follicles with hyaline vascular changes
- Clinical findings of POEMS syndrome present
- Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes
- Serum VEGF often markedly elevated
- (Under consideration as a diagnostic criterion)
Dita Gratzinger MD PhD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342
Initial posting/updates : 9/1/07, 4/15/10