Acquired hemophilia A (AHA) is a bleeding disorder that occurs from aberrant production of autoantibodies that target factor VIII. The underlying cause of AHA is unclear but can present postpartum. Very few cases have reported instances of AHA coexisting with other hematological disorders, such as sickle cell trait (SCT). Although rare and no direct correlation between the two, critical situations involving intractable bleeding can intensify the severity of these disorders. A 31-year-old pregnant female with a medical history significant for SCT presented to the hospital for a C-section. Shortly after the procedure she experienced intractable bleeding from multiple sites. Initial lab work yielded an isolated increase in activated partial thromboplastin time (aPTT). Further investigation showed abnormal mixing studies, reduced factor VIII activity and the presence of factor VIII antibodies. The patient was diagnosed with postpartum AHA (PAH) and treated with activated Factor VII and prednisone. PAH is an uncommon bleeding disorder that commonly occurs one to four months postpartum and presents as excessive bleeding elevated aPTT, abnormal mixing studies, and reduced factor VIII levels with abnormally high inhibitor levels. Despite an unknown identifiable etiology, treatment hinges upon establishing hemostasis and eradicating the aberrant generated factor VIII inhibitors. The association of AHA and other hematological disorders is not yet elucidated.
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http://dx.doi.org/10.55729/2000-9666.1382 | DOI Listing |
Transfus Apher Sci
December 2024
Department of Hematology and Blood Banking, Faculty of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran. Electronic address:
Background: Hemophilia B, or Christmas disease, is a hemorrhagic inherited disorder. Previous studies have reported measurement discrepancies in factor VIII activity between clot-based and chromogenic assays in approximately one-third of patients with non-severe hemophilia A. However, similar discrepancies in hemophilia B have been less extensively studied.
View Article and Find Full Text PDFHematol Rep
November 2024
Department of Pediatrics, Faculty of Medicine, Beni-Suef University, Beni-Suef 62521, Egypt.
: Hemophilia A is an X-linked recessive illness produced by a deficiency of coagulation factor VIII. This study aimed to evaluate serum vitamin D in hemophilic pediatric patients and its correlation with joint health and quality of life. : This case-control study was performed on ninety children under the age of 18 years old and separated into two groups: study group of 45 children with hemophilia A and control group of 45 healthy children at an outpatient pediatric hematology clinic at the Beni-Suef University hospitals.
View Article and Find Full Text PDFBMJ Open
December 2024
Department of Paediatrics, Nara Medical University, Kashihara, Japan.
Introduction: Emicizumab prophylaxis is approved for people of all ages with haemophilia A (HA) including infants and children. Although previous studies have demonstrated the efficacy and tolerability of emicizumab in infants with HA, real-world data on emicizumab use in infants are limited. The Haemophilia A in Infancy and NewbOrns: multi-instituional prospective observational study to assess the efficacy anD safety of Emicizumab (HINODE) study aims to evaluate the coagulation potential and safety of emicizumab prophylaxis in infants with congenital HA from birth to <12 months of age.
View Article and Find Full Text PDFAnn Hematol
December 2024
Shandong Blood Center, Shandong Hemophilia Treatment Center, Jinan, China.
Hemophilia A (HA) is an X-chromosome-linked recessive genetic disorder. Female carriers may have bleeding symptoms, but rarely have moderate or severe disease. We identified a female patient with moderate HA by pedigree tracking and genetic testing in a HA family involving consanguineous marriage.
View Article and Find Full Text PDFOchsner J
January 2024
Department of Hematology and Hematopoietic Cell Transplantation, City of Hope National Medical Center, Irvine, CA.
Prolongation of the activated partial thromboplastin time (aPTT) may signify an intrinsic factor deficiency or the presence of an inhibitor of coagulation, potentially placing a patient at increased risk for bleeding. However, a contact factor (ie, factor XII, prekallikrein, and high molecular weight kininogen) deficiency, which may also cause a prolonged aPTT, is not associated with clinical bleeding. A 71-year-old female had an isolated prolonged aPTT discovered during preoperative laboratory testing.
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