Features family thrombocytosis

Content

  • The concept of family thrombocytosis
  • The mechanism of development of thrombocytosis
  • Treatment and prevention of thrombocytosis



  • The concept of family thrombocytosis

    Family thrombocytosis have been described in children's partiesage. This disease refers to primary thrombocytosis, or related to an abnormality in the genetic apparatus or any other as yet unknown reason. Transfer of family thrombocytosis can be autosomal dominant, autosomal recessive, or be associated with the X chromosome. In autosomal dominant type of inheritance for the development of the disease is enough to inherit the mutant allele from one parent. In autosomal recessive type of inheritance of the disease is manifested only in homozygotes - organism in the hereditary set of homologous chromosomes which carry the same form of the gene. When inheriting associated with the X chromosome the abnormal gene, linked to the X chromosome, is passed from parents to children. In this case, the disease is almost always seen in men, women only receive the mutant gene and pass it on to their offspring.



    The mechanism of development of thrombocytosis

    The evolution of these family thrombocytosis yetlittle known. However, this embodiment is different from the primary thrombocytosis thrombocytosis considerably smaller haemostatic complications (thrombotic or hemorrhagic), mainly in children.



    Treatment and prevention of thrombocytosis

    Features family thrombocytosisTreatment Indications need to discuss with eachfamily member afflicted with this disease, depending on the presence of additional risk of thrombosis (vascular disease, heart disease, the presence of indications for bed rest, as well as biological factors predisposing to thrombosis). Many important role in the treatment of thrombocytosis plays platelet count and tolerance to the treatment of disease.

    Treatment for mielosanom thrombocytosis,mielobromolom and other cytotoxic drugs in the weeks leading up to the normalization of platelet count. When violations in the microvasculature is prescribed antiplatelet agents (aspirin, trental and others.). Antiplatelet agents - agents which inhibit platelet aggregation process and erythrocytes, i.e. their ability to reduce the bonding and adhesion to the vascular wall. By reducing the surface tension of the red blood cell membranes, they facilitate their deformation during the passage through the capillaries and improve the "flow" of blood. Antiplatelet agents are able to not only prevent aggregation, but also cause disaggregation has aggregated blood platelets. Apply them to prevent the formation of blood clots.

    In some cases, a long period of time can thrombocytosisoccur without marked symptomatic external (with or without small thrombocytosis thrombohemorrhagic complications), wherein the treatment is not required. Forecast in this case is relatively favorable.

    Introduction aspirin prophylaxis of thrombosis requires discussion for each patient depending on platelet aggregation disorders.

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