Successful treatment of POEMS syndrome in a young male with stem cell transplantation

Authors

  • Jyotsna Oak Consultant Physician & Rheumatologist, Kokilaben Dhirubhai Ambani Hospital, Andheri, Mumbai 400053 INDIA
  • Ishita Shah
  • Amit Pandey
  • Trideep Das
  • Sameer Tulpule

DOI:

https://doi.org/10.15305/ijrci/v8i1/334

Keywords:

POEMS syndrome, inflammatory demyelination polyneuropathy, osteosclerotic myeloma, stem cell transplantation

Abstract

POEMs syndrome is a rare paraneoplastic disorder related to plasma cell dyscrasia. The present study deals with an unusual case of POEMs syndrome in a young male who came with ascending demyelinating polyneuropathy, resulting in complete quadriparesis. The diagnosis was based on clinical findings of gynecomastia, hyperpigmentation and hypertrichosis of skin and lymphadenitis. Detailed laboratory investigations and imaging confirmed POEMS syndrome. In view of the rapidly progressing polyneuropathy, the patient was treated with chemotherapy followed by stem cell transplantation. The patient recovered completely after the treatment.



References

Arima F, Dohmen K, Yamano Y, et al. Five cases of Crow-Fukase syndrome. Fukuoka Igaku Zasshi Fukuoka Acta Medica 1992;83(2):112–20.

Bardwick,P.A.,Zvaifler,N.J.,Gill,G.N.,etal 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 Medicine 1980;59;311-322

Dispenzieri,A,Kyle ,R.A.,Lacy,M.Q.,et al POEMS syndrome :definitions and longterm outcome Blood 2003,101,2496-2506

Belec,L.,Authier,F.J.,Mohamed,A.S.,et al Antibodies to human herpes virus 8 in POEMS syndrome associated multicentric Castleman’s disease.Blood 1999b;93,3643-3653

Emile,C.,Danon,F.,Fermand,J.P.,et al Castleman disease in POEMS syndrome with elevated interleukin -6 Cancer 1993;71,874

Gherardi,R.K.,Belec,L.,Soubrier,M.,et al Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome .Blood 1996;87,1458-1465

Soubrier,M.J.,Dubost,J.J.Sauvezie,B.J. POEMS syndrome :a study of 25 cases and a review of literature.French Study Group on POEMS Syndrome .American Journal Of Medicine 1994;97,543-553

Soubrier,M.,Dubost,J.J.,Serre,A.F.,et al Growth factors in POEMS syndrome:evidence for a marked increase in circulating vascular endothelial growth factor.Arthritis and rheumatism 1997;40,786-787

Arimura K. Increased vascular endothelial growth factor (VEGF) is causative in Crow-Fukase syndrome. Rinsho Shinkeigaku 1999;39(1):84–5 [in Japanese].

Soubrier M, Sauron C, Souweine B, et al. Growth factors and proinflammatory cytokines in the renal involvement of POEMS syndrome. American Journal of Kidney Disease 1999;34(4):633–638.

Crow,R.Peripheral neuritis in myelomatosis Brit Med J 1956;2,802-804

Brazis,P.W.,Liesegang,T.J.,Bolling,J.P.,et al When do optic disc edema and peripheral neuropathy constitute Poetry Survey of Opthalmology 1990;35,219-225

Nakanishi,T.,Sobue,I.,Toyokura,Y.,et al The Crow-Fukase syndrome:a study of 102 cases in Japan Neurology1984;34,712-720

Attal,M.,Harousseau,J.L.,Stoppa,A.M.,et al.A prospective, randomized trial of autologous bone marrow transplantation and chemotherapy in multiple myeloma.Intergroupe francais du Myelome. N Engl J Med 1996;335,91-97

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Published

18-05-2020

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Case Studies