POEMS syndrome

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Classification according to ICD-10
D47.7 Other specified neoplasms of unsafe or unknown behavior of lymphatic, hematopoietic and related tissue - histiocyte tumor of unsafe or unknown behavior
ICD-10 online (WHO version 2019)

The POEMS syndrome , acronym for P olyneuropathie , O rganomegalie , E ndokrinopathie , M onoklonale gammopathy and skin changes ( S kin) is a very rare congenital disease with the eponymous main features.

It occurs more frequently paraneoplastically , often in osteosclerotic plasmacytoma , in up to 23% in the plasma cell type of Castleman's disease .

Synonyms are: POEMS complex; Crow-fukase syndrome; PEP syndrome; Shimpo syndrome; Takatsuti syndrome

The names refer to the author of the first description of the full symptoms by the English doctor R. S. Crow in 1956 and the description of the first Japanese patient by M. Fukase and colleagues in 1968.

The acronym was proposed by PA Bardwick et al . In 1980.

distribution

The frequency and cause are not known, the frequency peak is in the 5th to 6th decade of life.

Clinical manifestations

Clinical criteria are:

diagnosis

The x-ray shows more often single sclerotic , less often mixed sclerotic- lytic bone changes, spicula formation on small vertebral joints, enthesopathy .

Differential diagnosis

The easily confused chronic inflammatory demyelinating polyneuropathy must be distinguished .

therapy

For interdisciplinary treatment, radiation, corticosteroids, chemotherapy and stem cell transplantation are all possible.

history

The first description of a patient comes from 1939 by the German doctor Scheinker .

literature

  • A. Birkenbach, F. Kühlhorn, M. Grube, H. Helbig, MA Gamulescu: POEMS syndrome as a rare cause of bilateral papillary edema. In: Der Ophthalmologe: Journal of the German Ophthalmological Society. Vol. 114, No. 3, March 2017, pp. 262-265, doi: 10.1007 / s00347-016-0287-8 , PMID 27364634 .
  • SA Braun, P. Albrecht, A. Methner, S. Hanneken: POEMS syndrome. An interdisciplinary clinical challenge. In: The dermatologist; Journal of Dermatology, Venereology, and Allied Fields. Vol. 62, No. 10, October 2011, pp. 722-725, doi: 10.1007 / s00105-011-2239-1 , PMID 21915731 .

Individual evidence

  1. ↑ S. Kuwabara, A. Dispenzieri et al. a .: Treatment for POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome. In: Cochrane database of systematic reviews. No. 4, 2008, p. CD006828, ISSN  1469-493X . doi: 10.1002 / 14651858.CD006828.pub2 . PMID 18843731 . (Review).
  2. ↑ a b c d POEMS syndrome. In: Orphanet (Rare Disease Database).
  3. ↑ a b c Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  4. ↑ RS CROW: Peripheral neuritis in myelomatosis. In: British medical journal. Vol. 2, No. 4996, October 1956, pp. 802-804, PMID 13364332 , PMC 2035359 (free full text).
  5. ↑ M. Fukase, T. Sunematsu, A. Tanaka: [Autoimmunity in hematological diseases with special reference to autoimmune hemolytic anemia]. In: Naika. Internal medicine. Vol. 21, No. 2, February 1968, pp. 256-264, PMID 5685612 .
  6. ↑ PA Bardwick, NJ Zvaifler, GN Gill, D. Newman, GD Greenway, DL Resnick: Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes: the POEMS syndrome. Report on two cases and a review of the literature. In: Medicine. Vol. 59, No. 4, July 1980, pp. 311-322, PMID 6248720 .
  7. ↑ Encyclopedia Dermatology
  8. ↑ I. Scheinker: About a hitherto not described polyneuritis associated with peculiar skin changes in a plasmacellular myeloma of the sternum. In: Deutsche Zeitschrift für Nervenheilkunde , Vol. 147, pp. 247-273, 1939.

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