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Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report
BACKGROUND: Acquired Hemophilia A (AHA) is a rare hematological disorder that exhibits an incidence of approximately 1.5 cases per million patients a year. It is characterized by the development of autoantibodies against circulating Factor VIII coagulation proteins which, in turn, which in turn lead...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5583976/ https://www.ncbi.nlm.nih.gov/pubmed/28870236 http://dx.doi.org/10.1186/s13104-017-2767-6 |
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author | Rivera Cora, Nalyssa I. Irizarry Delgado, Freddie Merle Ramírez, Santa M. Vera Quiñones, Jorge |
author_facet | Rivera Cora, Nalyssa I. Irizarry Delgado, Freddie Merle Ramírez, Santa M. Vera Quiñones, Jorge |
author_sort | Rivera Cora, Nalyssa I. |
collection | PubMed |
description | BACKGROUND: Acquired Hemophilia A (AHA) is a rare hematological disorder that exhibits an incidence of approximately 1.5 cases per million patients a year. It is characterized by the development of autoantibodies against circulating Factor VIII coagulation proteins which, in turn, which in turn lead to potentially life-threatening hemorrhagic episodes. The incidence of AHA increases with age; with 80% of the affected patient population encompassing men and women that are 65 years or older. Some of the challenges that are highlighted in managing this disorder relate to the delayed diagnosis of this condition due to the rarity of the latter, the difficulty in establishing reliable hemostasis, and the secondary complications that are found when using immunosuppressive and hemostatic treatments in tandem with the elderly population afflicted with this disease. CASE PRESENTATION: A 90-year-old female of Hispanic origin presented with a 2-week history of generalized weakness, dizziness, shortness of breath and extensive purpuric formations that involved the left arm towards the lateral aspect of the thorax with the inclusion of a small right lateral neck hematoma formation. Upon initial laboratory screening, a hemoglobin level of 7.9, a hematocrit level of 21.9 and a PTT value of 70.9 were discovered. Despite conventional hemostatic treatment approaches, the patient did not show marked improvement of the laboratory values. Ongoing specialized laboratory reports, combined with the clinical presentation of the patient, led to the diagnosis of Acquired Hemophilia A. Treatment with recombinant porcine Factor VIII was initiated, which led to rapid improvement of clinical symptoms and laboratory values. The patient was discharged with current treatment plan and emergent follow/up with a hematologist was scheduled. CONCLUSION: Acquired Hemophilia A is an elusive bleeding disorder that has been seldom encountered in the demographics of Puerto Rico. The prompt detection of this diagnosis based on the clinical presentation alone is paramount to prevent the occurrence of grave hemorrhagic episodes. General knowledge and awareness of the treatment options available is key to ameliorate the prognosis of this ailment. |
format | Online Article Text |
id | pubmed-5583976 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-55839762017-09-06 Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report Rivera Cora, Nalyssa I. Irizarry Delgado, Freddie Merle Ramírez, Santa M. Vera Quiñones, Jorge BMC Res Notes Case Report BACKGROUND: Acquired Hemophilia A (AHA) is a rare hematological disorder that exhibits an incidence of approximately 1.5 cases per million patients a year. It is characterized by the development of autoantibodies against circulating Factor VIII coagulation proteins which, in turn, which in turn lead to potentially life-threatening hemorrhagic episodes. The incidence of AHA increases with age; with 80% of the affected patient population encompassing men and women that are 65 years or older. Some of the challenges that are highlighted in managing this disorder relate to the delayed diagnosis of this condition due to the rarity of the latter, the difficulty in establishing reliable hemostasis, and the secondary complications that are found when using immunosuppressive and hemostatic treatments in tandem with the elderly population afflicted with this disease. CASE PRESENTATION: A 90-year-old female of Hispanic origin presented with a 2-week history of generalized weakness, dizziness, shortness of breath and extensive purpuric formations that involved the left arm towards the lateral aspect of the thorax with the inclusion of a small right lateral neck hematoma formation. Upon initial laboratory screening, a hemoglobin level of 7.9, a hematocrit level of 21.9 and a PTT value of 70.9 were discovered. Despite conventional hemostatic treatment approaches, the patient did not show marked improvement of the laboratory values. Ongoing specialized laboratory reports, combined with the clinical presentation of the patient, led to the diagnosis of Acquired Hemophilia A. Treatment with recombinant porcine Factor VIII was initiated, which led to rapid improvement of clinical symptoms and laboratory values. The patient was discharged with current treatment plan and emergent follow/up with a hematologist was scheduled. CONCLUSION: Acquired Hemophilia A is an elusive bleeding disorder that has been seldom encountered in the demographics of Puerto Rico. The prompt detection of this diagnosis based on the clinical presentation alone is paramount to prevent the occurrence of grave hemorrhagic episodes. General knowledge and awareness of the treatment options available is key to ameliorate the prognosis of this ailment. BioMed Central 2017-09-04 /pmc/articles/PMC5583976/ /pubmed/28870236 http://dx.doi.org/10.1186/s13104-017-2767-6 Text en © The Author(s) 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Rivera Cora, Nalyssa I. Irizarry Delgado, Freddie Merle Ramírez, Santa M. Vera Quiñones, Jorge Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report |
title | Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report |
title_full | Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report |
title_fullStr | Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report |
title_full_unstemmed | Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report |
title_short | Acquired Hemophilia A in an advanced age patient of hispanic origin: a case report |
title_sort | acquired hemophilia a in an advanced age patient of hispanic origin: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5583976/ https://www.ncbi.nlm.nih.gov/pubmed/28870236 http://dx.doi.org/10.1186/s13104-017-2767-6 |
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