An unusual case of acute postpartum bleeding in a mother and her newborn

Acquired hemophilia A (AHA) is a rare autoimmune disease due to acquired IgG1-4 autoantibodies against coagulation factor VIII (FVIII). This causes patients, who have negative family and personal histories, to present with a sudden onset of heavy bleeding.1 –3 Although rare, the presence of antibodies against factors V and IX is possible.4
Source: Pediatrics and Neonatology - Category: Perinatology & Neonatology Authors: Tags: Letter to the Editor Source Type: research

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Authors: Menegatti M, Biguzzi E, Peyvandi F Abstract Autoantibodies toward clotting factors may develop in people suffering from autoimmune or neoplastic diseases, after drug intake or even in subjects without apparent conditions. They are more commonly directed against factor VIII (FVIII) or von Willebrand factor leading to acquired hemophilia A or acquired von Willebrand syndrome, respectively. Rarely, autoantibodies develop against other clotting factors, such as fibrinogen, FII, FV, FVII, FX, FXI, and FXIII. The clinical picture of an acquired bleeding disorder includes a wide spectrum of clinical manifestation...
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
We report a 68-year-old Thai woman diagnosed and treated for bullous pemphigoid (BP) for 11 months who recently presented with a 3-day history of extensive hemorrhagic bullae and large intra-oral buccal hematoma. Laboratory investigations confirmed a prolonged activated partial thromboplastin time, a low factor VIII level, a high factor VIII inhibitor level, and elevated anti-BPAG180 and anti-BPAG230 titers, confirming the diagnosis of BP associated with AHA. Immunosuppressive therapy with systemic corticosteroids and cyclophosphamide combined with bypassing agents for bleeding control resulted in significant clinical impr...
Source: Case Reports in Dermatology - Category: Dermatology Source Type: research
Roser Calvo* Patient Safety, Safety Science, AstraZeneca Pharmaceuticals, Gaithersburg, MD, United States Immune-related hematological adverse events are amongst the rare but potentially life-threatening complications of immune checkpoint inhibitors. The spectrum of these toxicities is broadening as the number of patients exposed to these agents is increasing. Yet, they are still relatively unknown to many clinicians, possibly due to a lack of specific diagnostic criteria, which poses a challenge for their recognition and proper reporting, and partly due to their low incidence, often too low to be noted in most c...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
We report a rare case of a compartment syndrome of the forearm in a 30-year-old woman 2.5 months postpartum as the presentation of pregnancy-associated AHA.
Source: Blood Coagulation and Fibrinolysis - Category: Hematology Tags: CASE REPORT Source Type: research
CONCLUSIONS: Rituximab appears to be an effective and well-tolerated treatment for autoimmune acquired haemophilia. However, its place remains to be specified. PMID: 30904179 [PubMed - as supplied by publisher]
Source: Revue de Medecine Interne - Category: Internal Medicine Tags: Rev Med Interne Source Type: research
We report the case of a 78-year-old a male patient presenting with symptoms of a hematoma after a fall three days previously. He is medically followed for multiple myeloma and bullous pemphigoid. Laboratory investigations revealed isolated and recent increased of activated partial thromboplastin time (80,6/32,1s) and a markedly low FVIII activity (
Source: Annales de Biologie Clinique - Category: Biochemistry Authors: Tags: Ann Biol Clin (Paris) Source Type: research
We report a 35-year-old postpartum female with acquired hemophilia A who initially presented with pleural hemorrhage. Diagnoses: In this patient activated prothrombin time (PT) and activated partial thromboplastin time (APTT) were found, and the factor VIII activity was 12.6%, furthermore Bethesda assay showed a FVIII antibody titer of 7.4 Bethesda units (BUs). Interventions: The treatment requires a 2-pronged approach: treatment of the bleeding and elimination of the inhibitor. Outcomes: After hemostatic agents were used and inhibitors were eradicated, the patient achieved complete remission without relapse. Les...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
This article gives a comprehensive overview of the current knowledge about the pathophysiology, diagnosis, and treatment of AHA.
Source: Drugs - Category: Drugs & Pharmacology Source Type: research
Acquired hemophilia A (AHA) is a rare acquired bleeding disorder caused by autoantibodies against autologous factor VIII (FVIII). It is a disease that most commonly affects the elderly, but it has been described in children and during the post-partum period. It is idiopathic in 50% of cases and is associated with autoimmune disease, malignancy, pregnancy, infection or certain medications in the other 50%. The diagnosis should be suspected in patients with an isolated prolonged aPPT without previous personal or familial bleeding history.
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
Conclusion: AHA is an autoimmune disease and can co-exist with immune cytopenia besides connective tissue disease (CTD). Joint hemarthrosis is not specific to congenital hemophilia and mainly related to the extent of prolonged aPTT and weight loading of joint in AHA. When the first-line therapy of cyclophosphamide combined with prednisone is not enough to eradicate the inhibitor, especially for a higher inhibitor titer, anti-CD20 monoclonal antibody could play an important role.
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
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