Importancia de la orientación diagnóstica en hemofilia A adquirida

Abstract

Acquired hemophilia A (AHA) is a rare and life-threatening autoimmune hemorrhagic disorder where autoantibodies are developed against factor VIII. An early diagnosis is challenging and mandatory: an immediate hemostatic control is required to reduce morbidity and mortality. Laboratory features of AHA are: presence of autoantibodies against factor VIII, prolonged activated partial thromboplastin time (with normal prothrombin time and thrombin time) and decreased factor VIII levels. In some cases, the results of laboratory tests may be incorrect due to errors in analysis, blood extraction or manipulation of samples; also worth of consideration are limitations in the measurement range and low sensitivity of the tests. This review highlights the importance of adequate screening in patients with suspected AHA to make an adequate diagnosis and reduce overall fatal outcomes.

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“…Regarding dental manifestations, it is common to find spontaneous mucosal bleeding, episodic, prolonged, spontaneous or traumatic gingival bleeding. There is also hemarthrosis of the temporomandibular joint and pseudotumors of hemophilia [19] . It manifests as excessive bleeding in the joints, pain and edema, ecchymosis, urinary tract and digestive tract hemorrhages.…”

“…Once the defect has been identified, other family members will require less testing for diagnosis [31,32,33] . Tests included are partial thromboplastin time, prothrombin time, Factor VIII dosage, Factor IX dosage, complete blood count and platelet count [19] . Accurate and timely diagnosis is important and essential for the effective management of patients [22] .…”

“…Receiving clotting factor at home means that bleeds can be treated more quickly, which means less severe bleeding and side effects. Other treatment options include desmopressin acetate, epsilon aminocaproic acid, among other medications [19] . The main treatment for severe hemophilia is to receive specific clotting factor replacement.…”

“…It is important to inform patients and parents of children about the risks of local oral trauma before the anesthetic wears off. Buccal infiltration can be used without the need for factor replacement [19] , it will anesthetize the entire upper dentition as well as the anterior lower dentition and premolars. The use of Articaine as a local anesthetic is recommended, several studies have shown that alveolarinferior block could be avoided, and buccal infiltration of the mandible can be used as an alternative to inferior alveolar blocks [13] .…”

Hemofilia A adquirida: un diagnóstico de infarto

“…La hemofilia A adquirida es una enfermedad infrecuente, con una incidencia aproximada de 1 a 1.5 casos por millón de habitantes 1 . Se distribuye de forma bimodal, con un pico inicial de presentación en mujeres jóvenes durante el parto y el puerperio y un segundo pico en adultos mayores frágiles o con condiciones comórbidas 2 .…”