Hemofilia adquirida - A propósito de um caso clínico

Autores

  • Ana Cabral Serviço de Medicina I do Hospital de Egas Moniz, Lisboa
  • Teresa Romão Serviço de Medicina I do Hospital de Egas Moniz, Lisboa
  • J. A. Monteiro Serviço de Medicina I do Hospital de Egas Moniz, Lisboa
  • L. Sousa Uva Serviço de Medicina I do Hospital de Egas Moniz, Lisboa

Palavras-chave:

hemofilia adquirida, inibidor de factor VIII, discrasia hemorrágica

Resumo

O desenvolvimento espontâneo de auto-anticor­pos contra o factor VIII da coagulação em do­entes não hemofílicos é uma entidade rara, mas clinicamente significante pelas suas complica­ções hemorrágicas.

A associação da hemofilia adquirida com do­enças benignas e malignas esta descrita, mas na maioria dos casos não é possível identificar qualquer causa precipitante.

Os autores apresentam uma revisão sumária desta patologia, ilustrando-a com um caso clíni­co de um doente de 73 anos, do sexo feminino, internado por quadro de discrasia hemorrági­ca grave.

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Referências

Green D. Lechner K. A survey of 215 non-hemophilic patient with inhibitors to factor Vlll.Thromh Haemost 1981;45:200- 20.1.

Lusher JM. Acquired inhihitors to factor VIII in non-hemophilic patients. ln: Craij Kessler, ed,. Acquired Hemophilia 2nd ed. Princeton. Excerpta Medica Inc. 1995:1-8.

Kessler CM: An introduction to factor VIII inhibitors: the detec­tion and quantitation. AmJ Med 199l;vol 91.suppl 5A: 1-5.

Green D. Acquired Factor VIII Inhibitors and immunosuppressi­on of autoantibodies. ln: Craij Kessler, eds. Acquired Hemo­ philia 2nd ed. Princeton.Excerpta Medica lnc. l995;25-40.

Hoyer LW, Scandella D: Factor VIII inhibitors :structure and func­tion in autoantibody and hemophilia A patient. Seminars in Hematology 1994; 2(2) 4:1-5.

Hltin MB: Acquired inhihitors in malignant and nonmalignant disease states. Am J Med 1991:vol 91.suppl 5A:9-13.

Garvey MH: lncidence and management of patients with acqui­red factor VIII inhibitors: The practical experience of a tertiary care Hospital. ln: Craij Kessler, eds. Acquired Hemophi­lia 2nd ed. Princeton. Excerpta Medica lnc. 1995;91-111.

Ludbm CA, Morrison AE, Kessler C. Treatment of acquired he­mophilia. Seminars in Hematology 1994; 2(2) 4:16-19.

Kasper CK. Lahoratory diagnosis of factor VIII inhibitors. ln: Craij Kessler, eds. Acquired Hemophilia 2nd ed. Princeton. Ex­cerpta Medica lnc. 1995:9-23.

Green D: Cytotoxic suppression of acquired factor VIIl:C inhi­bitors. Am.J Med 1991; 91(suppl 5A) 14-19.

Kasper CK. Raising factor VIII leveis in patients with acquired factor VIII inhibitors. ln: Craij Kessler, ecb. Acquired Hemo­philia 2nd ed. Princeton. Excerpta Medica lnc. 1995;41-69.

Kernoff PBA. Rationale and evolution of therapy with porcine factor VIII:C. Am J Med 1991;9l(suppl 5A):20-22.

Kernoff PBA. Thomas ND, Lilley PA et ai. Clinicai experience with polyelectrolyte-fractionated porcine factor VIII concen­trate in the treatment of hemophiliacs with antibodies to fac­tor VIII. Blood 1984; 63:31-41.

Hay CRM, Bolton-Maggs P: Porcine factor VIII in the manage­ment of patients with factor VIII inhihitors. Transfusion Med Rev 1991; vol V(2):145-151.

Kessler CM. The treatment of acquired factor VIII inhibitors: Worldwide experience with porcine factor VIII. ln: Craij Kes­sler, eds. Acquired Hemophilia 2nd ed. Princeton. Excerpta Medica Inc. 1995;71-89.

Negrier C. The use of porcine factor VIII in France. Seminars in Hematology 1994; 2 (2) 4:29-32.

Kessler CM. Factor VIII inhihitors-an algorithmic approach to treatment. Seminars in Hematology 1994; 2(2) 4:33-36.

Lusher JM. Use of factor VIII bypassing agents to control blee­ding in patients with acquired factor VIII inhib itors. ln: Craij Kessler, eds. Acquired Hemophilia 2nd ed. Princeton. Ex­cerpta Medica Inc. 1995; 115-129.

Lusher JM. l'erspectives on the use of factor IX complex conentrates in the treatment of bleeding in persons with acqui­red factor VIII inhibition. Am.J Med 1991;91Csuppl 5A):30-34.

Schwartz RS, Gabriel DA, Aledort LM et ai. A prospective study of treatment of acquired (autoimmune) factor VIII inhibi­tors with intravenous gammaglobulin. Blood 1995; 86:797- 804.

Sultan Y, Maisonneuve P, Kazatchkine MD, Nydegger UE: Anti­ idiotypic suppression of autoantibodies to factor VIII (antihaemophilic factor) by high-dose intravenous gam­maglohulin. Lancet 1984; 2:765-768.

Sultan Y, Kazatchkine MD e al. Intravenous immunoglohulin in the treatment of spontaneously acquired factor VIII:C inhi­bitors. Am J Med 1991;91(suppl 5A):35-39.

Sultan Y, Kazatchkine et al. The use of immunoglobulins in the treatment of factor VIII inhibitors. Seminars in Hematology 1994;2(2) 4:65-66.

Green D. lmmunosuppression of patients with acquired factor VIII inhibitors. Seminars in Hematology 1994;2(2) 4:60-61.

Lian ECY, Larcada AF, Chiu AYZ. Combination immunisuppres­sive therapy after factor VIII infusion for acquired factor VIII inhibitor. Ann lntern Med 1989; 110: 774-778.

Kasper CK. Algorithm for management of patients with acqui­ red hemophilia. ln: Craij Kessler, eds. Acquired Hemophilia 2nd ed. Princeton. Excerpta Medica Inc. 1995;149-153.

Hoyer LW. Future approaches to factor VIII inhibitors therapy. AmJ Med 1991;91(suppl SA):40-44.

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Publicado

31-03-1998

Como Citar

1.
Cabral A, Romão T, Monteiro JA, Sousa Uva L. Hemofilia adquirida - A propósito de um caso clínico . RPMI [Internet]. 31 de Março de 1998 [citado 14 de Novembro de 2024];5(1):53-9. Disponível em: https://revista.spmi.pt/index.php/rpmi/article/view/2118

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