Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature
Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include seve...
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| Format: | Article |
| Language: | English |
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Wiley
2017-01-01
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| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2017/2057019 |
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| _version_ | 1832558765621116928 |
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| author | Quentin Binet Catherine Lambert Laurine Sacré Stéphane Eeckhoudt Cedric Hermans |
| author_facet | Quentin Binet Catherine Lambert Laurine Sacré Stéphane Eeckhoudt Cedric Hermans |
| author_sort | Quentin Binet |
| collection | DOAJ |
| description | Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled. Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment. |
| format | Article |
| id | doaj-art-f7a4befd323641778bd8fa4dacdf74c3 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-f7a4befd323641778bd8fa4dacdf74c32025-02-03T01:31:39ZengWileyCase Reports in Hematology2090-65602090-65792017-01-01201710.1155/2017/20570192057019Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the LiteratureQuentin Binet0Catherine Lambert1Laurine Sacré2Stéphane Eeckhoudt3Cedric Hermans4Hemostasis and Thrombosis Unit, Division of Hematology, Cliniques Universitaires Saint-Luc, 1200 Brussels, BelgiumHemostasis and Thrombosis Unit, Division of Hematology, Cliniques Universitaires Saint-Luc, 1200 Brussels, BelgiumDivision of Dermatology, Cliniques Universitaires Saint-Luc, 1200 Brussels, BelgiumHemostasis Laboratory, Division of Biological Chemistry, Cliniques Universitaires Saint-Luc, 1200 Brussels, BelgiumHemostasis and Thrombosis Unit, Division of Hematology, Cliniques Universitaires Saint-Luc, 1200 Brussels, BelgiumBackground. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled. Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.http://dx.doi.org/10.1155/2017/2057019 |
| spellingShingle | Quentin Binet Catherine Lambert Laurine Sacré Stéphane Eeckhoudt Cedric Hermans Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature Case Reports in Hematology |
| title | Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature |
| title_full | Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature |
| title_fullStr | Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature |
| title_full_unstemmed | Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature |
| title_short | Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature |
| title_sort | successful management of acquired hemophilia a associated with bullous pemphigoid a case report and review of the literature |
| url | http://dx.doi.org/10.1155/2017/2057019 |
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