• Information
  1. Constituents of Syndrome
  2. Characteristics
  3. Symptoms
  4. Treatment

A. Constituents of Syndrome

1. Polyneuropathy
2. Organomegaly
3. Endocrinopathy
4. Monoclonal Gammopathy
5. Skin Changes

B. Characteristics

1. Related to osteosclerotic myeloma (may be same disease)
2. Rare disorder, ~1-5% of all patients with plasma cell dyscrasia
3. Median Age 51
4. Paraproteinemic neuropathy may develop, particularly with osteosclerotic myelomas [3]

C. Symptoms

1. Polyneuropathy - combined sensory and motor [3]
2. Osteosclerotic bone lesions (82%)
3. Skin abnormalities (58%) - hyperpigmentation, hypertrichosis, angiomas
4. Lymphadenopathy (42%)
5. Papilledema (37%)
6. Hepatomegaly and/or Splenomegaly (~25%)
7. Ascites 11%
8. Abnormal serum and/or urine M protein (IgA or IgG)
9. Scleroderma like changes can occur
10. Thrombocytopenia is not rare [2]

D. Treatment

1. Plasmapheresis for acute symptoms
2. Glucocorticoids
3. Alkylating Agents
   1. Cyclophosphamide
   2. Melphalan or Busulfan
   3. Chlorambucil
4. Bortezombi (Velcade®) has shown efficacy in refractory disease [4]
5. Treatment similar to that for multiple myeloma

References

1. Miralles GD, O'Fallon JR, Talley NJ. 1992. NEJM. 327(27):1919
2. Soubrier MJ, Dubost JJ, Sauvezie BMJ. 1994. Am J Med. 97(6):543
3. Ropper AH and Gorson KC. 1998. NEJM. 338(22):1601
4. Richardson PG, Barlogie B, Berenson J, et al. 2003. NEJM. 348(26):2609