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Acquired hemophilia A is a rare disease. The incidence has been estimated to be 1.3-1.5 cases per 1 million persons per year. The etiology of acquired hemophilia A varies. It may develop in patients with autoimmune disorders, hematologic and solid cancers or in women during pregnancy or following childbirth. In about half of the cases no underlying disease can be found. The clinical picture is dominated by severe soft tissue hematomas especially in the cases of pregnancy-related acquired hemophilia A. Unlike congenital hemophilia A, bleeding into joints is rare. Pregnancy-related acquired hemophilia A may develop following any pregnancy but is observed more often in primigravidas. In most cases it arises in the postpartum period, most commonly 1-4 months after delivery. If factor VIII inhibitors develop during pregnancy or labor, they are frequently associated with severe uterine bleeding. The prognosis of pregnancy-related acquired hemophilia A is good with a high percentage of spontaneous remissions especially if the inhibitor was detected postpartum. Patients with acquired inhibitors do not usually have personal or family history of bleeding tendency, thus it is the presence of bleeding at multiple sites with prolonged activated partial thromboplastin time not corrected by incubation with normal plasma (mixing study) that raises the suspicion of inhibitor. Prompt diagnosis and treatment achieved by close collaboration among gynecologists and hematologists may improve the prognosis and prevent severe bleeding.
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Peptic ulcer disease is the most common cause of acute gastrointestinal bleeding, followed by variceal bleeding, Mallory-Weiss syndrome, and malignancy. On the contrary, acquired hemophilia A is a ver...
Almost all of what is known about neurologic and cognitive development in hemophilia derives from the Hemophilia Growth and Development Study, conducted during an era when treatment regimens and comor...
Cardiovascular conditions are now the leading cause of maternal death in both the US (Petersen EE et al MMWR Morb Mortality Wkly Rep 2019; 68:762-765) and the UK (Knight M et al Saving Lives, Improvin...
We previously developed the Tilburg Pregnancy Distress Scale (TPDS). The aim of the current study was to further assess its test-retest reliability, internal consistency, and construct and concurrent ...
The purpose of this prospective study is to determine the efficacy and safety of Rituximab plus Bortezomib in patients with newly diagnosed acquired hemophilia A.
Prenancy and specialy delivery for hemophilia mother and her children possibly hemophilia is risked. Diagnostical of hemophilia pregnan is the first difficulty but patients with hemorrhag...
Purpose: To evaluate the efficacy when administering steroid combined with small dose rituximab to eliminate the antibody in acquired hemophilia A patients compared to treatment using ste...
This is a real-world study of the safety of the treatments used for people with hemophilia. The study will follow people with hemophilia A or B from across the country for about 4 years as...
The relevance of the study will be in establishing whether Health Related Quality of Life (HRQoL) measures obtained repeatedly during hemophilia care are sensitive to significant changes i...
A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)
The classic hemophilia resulting from a deficiency of factor VIII. It is an inherited disorder of blood coagulation characterized by a permanent tendency to hemorrhage.
Condition in which the plasma levels of homocysteine and related metabolites are elevated (>13.9 μmol/l). Hyperhomocysteinemia can be familial or acquired. Development of the acquired hyperhomocysteinemia is mostly associated with vitamins B and/or folate deficiency (e.g., PERNICIOUS ANEMIA, vitamin malabsorption). Familial hyperhomocysteinemia often results in a more severe elevation of total homocysteine and excretion into the urine, resulting in HOMOCYSTINURIA. Hyperhomocysteinemia is a risk factor for cardiovascular and neurodegenerative diseases, osteoporotic fractures and complications during pregnancy.
A hereditary deficiency of blood coagulation factor XI (also known as plasma thromboplastin antecedent or PTA or antihemophilic factor C) resulting in a systemic blood-clotting defect called hemophilia C or Rosenthal's syndrome, that may resemble classical hemophilia.
Proteins produced by organs of the mother or the PLACENTA during PREGNANCY. These proteins may be pregnancy-specific (present only during pregnancy) or pregnancy-associated (present during pregnancy or under other conditions such as hormone therapy or certain malignancies.)
Women's Health - key topics include breast cancer, pregnancy, menopause, stroke Follow and track Women's Health News on BioPortfolio: Women's Health News RSS Women'...
Autoimmune disorders are conditions that occurs when the immune system mistakenly attacks and destroys healthy body tissue. There are more than 80 different types of autoimmune disorders. Normally the immune system's white blood cells help protect ...
Obstetrics and gynaecology
Fertility Menopause Obstetrics & Gynaecology Osteoporosis Women's Health Obstetrics and gynaecology comprises the care of the pregnant woman, her unborn child and the management of diseases specific to women. Most consultant...