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  • Platelet transfusion refractoriness


    Platelet transfusion refractoriness is the repeated failure to achieve the desired level of blood platelets in a patient following a platelet transfusion. The cause of refractoriness may be either immune or nonimmune based. Among immune-related refractoriness, antibodies against HLA antigens are the primary cause. Non-immune causes include splenomegaly (enlargement of the spleen), fever, and sepsis.

  • Bernard–Soulier syndrome


    Bernard–Soulier syndrome (BSS), also called hemorrhagiparous thrombocytic dystrophy, is a rare autosomal recessive bleeding disorder that causes a deficiency of glycoprotein Ib (GpIb), the receptor for von Willebrand factor. The incidence of BSS is estimated to be less than 1 case per million persons, based on cases reported from Europe, North America, and Japan. BSS is a giant platelet disorder, meaning that it is characterized by abnormally large platelets.

  • HELLP syndrome


    HELLP syndrome is a complication of pregnancy characterized by hemolysis, elevated liver enzymes, and a low platelet count. It usually begins during the last three months of pregnancy or shortly after childbirth. Symptoms may include feeling tired, retaining fluid, headache, nausea, upper right abdominal pain, blurry vision, nosebleeds, and seizures. Complications may include disseminated intravascular coagulation (DIC), placental abruption, and kidney failure. The cause is unknown. Usually it occurs in association with preeclampsia or eclampsia. Other risk factors include previously having the syndrome, a mother older than 25 years, and being white. Diagnosis is generally based on blood tests finding signs of red blood cell break down (LDH greater than 600 U/L), an AST greater than 70 U/L, and platelets of less than 100x109/L. If not all the criteria are present the condition is incomplete. Treatment generally involves delivery of the baby as soon as possible. This is particularly true if the pregnancy is beyond 34 weeks of gestation. Medications may be used to decrease blood pressure and blood transfusions may be required. Corticosteroids may be used to speed development of the baby's lungs, if it is early in pregnancy. HELLP syndrome occurs in about 0.7% of pregnancies and affects about 15% of women with eclampsia or severe preeclampsia. Death of the mother is uncommon. Outcomes in the baby are generally related to how premature they are at birth. The syndrome was first named in 1982.

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