Non-familial systemic amyloidosis

Authors

  • Margarida Ascensão Interna do Internato Complementar de Medicina Interna, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • Helena Figueiredo Assistente Hospitalar de Medicina Interna, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • José Pontes Assistente Hospitalar de Gastrenterologia, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • Ana Paiva Interna do Internato Complementar de Nefrologia, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • Paula Pimenta Assistente Hospitalar de Medicina Interna, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • Francisco Parente Assistente Hospitalar de Medicina Interna, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • José Feio Farmacêutico Hospitalar, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • Borges Alexandrino Assistente Graduado de Medicina Interna, Serviços de Medicina II dos Hospitais da Universidade de Coimbra
  • Políbio Serra e Silva Director de Serviço, Serviços de Medicina II dos Hospitais da Universidade de Coimbra

Keywords:

systemic amyloidosis, potassium per­ manganate test, classification, diagnosis, clinical manifestations

Abstract

The authors present the causes of non familial sys­temic amyloidosis seen in a department of Internal Medicine. The work comprises 21 patients with a di­agnosis of amyloidosis. The diagnostic distribution varied between primary (AL), secondary (AA) and b -microglobuün (b2m). The study group was comprised­ of 13 on women (62%) and 8 men (38%) with an average age of 61.5 years ( min. 31/max. 85).

ln this group the authors studied some aspects wich highlight the causes that lead to the investigation of amyloidosis, the associated pathologies, the clinical­ laboratorial syndromes the distribution of the seve­ral types of amyloidosis, the biopsy locations, the evo­lution of the diseases and the patients survivaL

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References

Robert C, Aractingi S, Prost C et al. Bullous amyloidosis - report of 3 cases and review of the literature. Medicine 1993; 72:38-44.

Husby G. Nomenclature and classification of amyloid and amyloi­dosis. J Intern Med 1992; 232:511-512.

Kyle RA. Amyloidosis. J lnt Med 1992; 232:507-508.

Kisilevsky. Proteoglycans, glycosaminoglicans, amyloid-enhancing factor, and amyloid deposition. J Int Med 1992; 232:515-516.

Pepys MB. Amyloid P component and the diagnosis of amyloido­sis. J lnt Med 1992; 232:519-521.

Buxbaum J. Mechanisms of disease: monoclonal immunoglobulin deposition. Hematol Oncol Clin North Am 1992; 6:323-345.

Vogelgesang S, Klipple GL. The many guises of amyloidosis. Postgrad Med 1994; 96:119-127.

Wong CK, Wang WL. Systemic amyloidosis - a report of 19 cases. Dermatology 1994; 189:47-51

Gertz MA, Kyle RA. Secondary systemic amyloidosis: response and survival in 64 patients. Medicine 1991; 70:246-256.

Yakar S, Livneh A, Kaplan B, Pras M. The molecular basis of reactive amyloidosis. Semin Arthritis Rheum 1995; 24:255- 261

Lopes S, Costa A, Afonso A, Vedes J, Alexandrino B, Silva PS. Amiloidose AL ou AA - a propósito de um caso clínico. O Médico 1990; 123.165-166

Hawkins PN, Pepys MB. Amyloidosis. ln Malpas JS, Bergsagel DF, Kyle RA (eds.). Myeloma. Oxford: Oxford University Press. 1995:477-506.

Kyle RA, Gertz MA. Primary systemic amyloidosis: clinical and laboratory features in 474 cases. Semin Hematol 1995; 32:45-

Kyle RA. Primary systemic amyloidosis. J Int Med 1992; 232:523- 524.

Gabriel J, Pimenta P, Parente F et ai. Amiloidose sistémica e síndrome da veia cava superior em hemodialisado crónico. O Médico 1991; 125:386-390.

Kyle HA, Greipp PR, O'Fallon M. Primary systemic amyloido­sis: multivariate analysis for prognostic factors in 168 cases. Blood 1986; 68:220-224.

Gouveia D, Carranca J, Lousada N et al. Amiloidose cardíaca: revisão da literatura. Rev Port Cardiol 1996; 15(2):657-664

Duston MA, Skinner M, Shirahama T, Cohen AS. Diagnosis of amyloidosis by abdominal fat aspiration. Am J Med 1987; 82:412-414.

Gertz MA, Kyle RA. Myopathy in primary systemic amyloidosis. J Neurol Neurosurg Psychiatry 1996; 60:655-660.

Glenner GG. Amyloid deposits and amyloidosis: the beta-fibri­lloses. N Engl J Med 1980; 3021283-1292, 1333-134.3.

Cohen AS,Jones LA. Advances in amyloidosis. Curr Opin Rheu­ matol 1993; 5:62-76.

Gertz MA, Kyle RA. Amyloidosis: prognosis and treatment. Semin Arthritis Rheum 1994; 24:124-138.

Hawkins PN. Diagnosis and monitoring of amyloidosis. Baillie­ res Clin Rheumatol 1994; 8:635-659.

Maulin L, Hachulla E, Facon T et ai. Évaluation de l'amylose primitive (AL) par scintigraphie au composant sérique amylo'i­ de P: du diagnostic au prognostic. Rev Med Interne 1993; 14962.

Hachulla E, Deveaux M, Duquesnoy B, Marchandise X. Carto­ graphie de l'amylose par scintigraphie au composant sérique amyloide P marqué à !'iode 123: mythe ou réalité' Rev Med Interne 1994; 15:238-239

Hawkins PN, Richardson S, MacSweeney JE et al. Scintigraphic quantification and serial monitoring of human visceral amyloid

deposits provide evidence for turnover and regression. QJ Med 1993; 86:365-.174.

Hachulla E, Wechsler B, Deveaux M et al. Amylose localisée ou systémique? lntérêt et limites de la scintigraphie au composant amylo'ide P marqué à !'iode 123, place de la hiopsie de glandes salivaires accessoires. Rev Med Interne 1994; 15:182-185.

Hachulla E, Deveaux M, Duquesnoy B, Marchandise X. Scinti­ graphie au composant amylo'ide P marqué à !'iode 123: une nouvelle méthode d'évaluation de l'amylose. La Presse Médi­cale 1994; 23:348.

Stone MJ. Amyloidosis: a final common pathway for protein deposition in tissues. Blood 1990; 75:531-541

Additional Files

Published

1997-09-30

How to Cite

1.
Ascensão M, Figueiredo H, Pontes J, Paiva A, Pimenta P, Parente F, Feio J, Alexandrino B, Serra e Silva P. Non-familial systemic amyloidosis. RPMI [Internet]. 1997 Sep. 30 [cited 2025 Mar. 28];4(3):156-62. Available from: https://revista.spmi.pt/index.php/rpmi/article/view/2225

Issue

Vol. 4 No. 3 (1997): Julho/ Setembro

Section

Original Articles

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