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Osteosclerotic Myeloma

Synonyms: POEMS Syndrome

Definition

This is a type of plasma cell dyscrasia with sclerotic bone lesions and progressive demyelinating polyneuropathy. Multiorgan involvement including organomegaly, endocrinopathy, skin changes (POEMS syndrome). Multicentric Castleman disease may be associated in some cases.

Histopathology

  • Bone marrow aspirate usually contains <10% plasma cells
  • Plasma cell proliferation usually evident as a plasmacytoma in:
    • sclerotic lesions (Figs 1 and 2
      An osteosclerotic bone lesion from a patient with osteosclerotic myeloma.

      Fig. 1: An osteosclerotic bone lesion from a patient with osteosclerotic myeloma.

      An osteosclerotic bone lesion from a patient with osteosclerotic myeloma. High magnification of the marrow in the specimen in Fig. 1. This area shows a large number of plasma cells that were λ light chain restricted on immunohistochemical study.

      Fig. 2: An osteosclerotic bone lesion from a patient with osteosclerotic myeloma. High magnification of the marrow in the specimen in Fig. 1. This area shows a large number of plasma cells that were λ light chain restricted on immunohistochemical study.

      )
    • lymph nodes
  • Marrow from areas uninvolved by sclerotic process may show:
    • typical myeloma cell infiltration
    • increased large megakaryocytes with hyperlobulated nuclei (Fig. 3
      Marrow biopsy from a nonsclerotic area from a patient with osteosclerotic myeloma with POEMS syndrome. There was a thrombocytosis. The marrow in this area shows an infiltration by immature plasma cells. There was also an increase in large megakaryocytes, two of which are at right.

      Fig. 3: Marrow biopsy from a nonsclerotic area from a patient with osteosclerotic myeloma with POEMS syndrome. There was a thrombocytosis. The marrow in this area shows an infiltration by immature plasma cells. There was also an increase in large megakaryocytes, two of which are at right.

      )
  • Involved lymph nodes:
    • may show angiofollicular hyperplasia with a parafollicular infiltration of monoclonal plasma cells6

Differential Diagnosis

Select up to 2 differential diagnoses to compare with Osteosclerotic Myeloma

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Genetics

TypeNameComment
chromosome11, 13, 14 and 17t(11;14) and del of 13q14 and 17p13

Prognosis

  • Median survival 97 months:
    • in contrast with 30–35 months for typical myeloma

References

1 Case record of the Massachusetts General Hospital; Case 39–1992. N Engl J Med. 1992;327:1014–1021.

2 Diego Miralles G, O'Fallon JR, Talley NJ. Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N Engl J Med. 1992;327:1919–1923.

3 Imawari M, Akatsuka N, Beppu H, Suzuki H, Ishibashi M. Syndrome of plasma cell dyscrasia, polyneuropathy, and endocrine disturbances. Ann Intern Med. 1974;81:490–493.

4 Miralles GD, O'Fallon JR, Talley NJ. Plasma-cell dyscrasia with polyneuropathy. The spectrum of POEMS syndrome. N Engl J Med. 1992;327:1919–1923.

5 Takatsuki K, Sanada I. Plasma cell dyscrasia with polyneuropathy and endocrine disorder. Clinical and laboratory features of 109 reported cases. Jpn J Clin Oncol. 1983;13:543–556.

6 Bitter MA, Komaiko W, Franklin WA. Giant lymph node hyperplasia with osteoblastic bone lesions and the POEMS (Takatsuki's) syndrome. Cancer. 1985;56:188–194.

7 Soubrier MJ, Dubost J-J, Sauvezie BJH. POEMS syndrome. A study of 25 cases and a review of the literature. Am J Med. 1994;97:543–553.

Last updated: 14 Mar 2006

Osteosclerotic Myeloma

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