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PREGNANCY RELATED ACQUIRED HEMOPHILIA A.

07:00 EST 1st March 2019 | BioPortfolio

Summary of "PREGNANCY RELATED ACQUIRED HEMOPHILIA A."

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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This article was published in the following journal.

Name: Harefuah
ISSN: 0017-7768
Pages: 184-186

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Medical and Biotech [MESH] Definitions

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.)

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