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Diseases of platelets

 
, medical expert
Last reviewed: 23.04.2024
 
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Platelets, or blood platelets, play a key role in the processes of vascular-platelet hemostasis - the initial stage of thrombus formation.

Platelets are denuclearized blood cells of 1-4 μm in size (young forms of platelets are larger) and a life span of 7-10 days. 1/3 of the platelet is in the spleen and 2/3 - in the bloodstream. The number of platelets in the human peripheral blood varies between 150 000-400 000 / mm 3. The average volume of the platelet is 7.1 femoliters (1x10 15 l).

With a decrease in the number of platelets or a violation of their function, bleeding may occur. The most typical bleeding from damaged skin and mucous membranes: petechiae, purpura, ecchymosis, nasal, uterine, gastrointestinal bleeding, hematuria. Intracranial hemorrhages are rare.

The most common cause of thrombocytopenia is the immune destruction of blood platelets by auto-, allo- or drug-induced antibodies.

trusted-source[1], [2], [3], [4], [5], [6]

Pathophysiological classification of thrombocytopenic conditions

Increased destruction of platelets (with normal or increased number of megakaryocytes in the bone marrow - megakaryocytic thrombocytopenia).

Immune thrombocytopenia

  • Idiopathic:
    • ETC.
  • ♦ Secondary:
    • are induced by infections, including viral (HIV, cytomegalovirus [CMV], Epstein-Barr, herpes, measles rubella, measles, parotitis, parvovirus B19) and bacterial (tuberculosis, typhus);
    • drug-induced;
    • posttransfusion purpura;
    • autoimmune hemolytic anemia (Fischer-Evans syndrome).
    • systemic lupus erythematosus;
    • hyperthyroidism;
    • lymphoproliferative diseases;
    • allergy, anaphylaxis.
  • Congenital immune thrombocytopenia:
    • congenital trans-symmetric thrombocytopenia;
    • congenital alloimmune thrombocytopenia;
    • fetal erythroblastosis - Rh-incompatibility.

Non-immune thrombocytopenia

  • Caused by increased consumption of platelets:
    • microangiopathic hemolytic anemia;
    • DIC-syndrome;
    • chronic recurrent schizocytic hemolytic anemia;
    • hemolytic-uremic syndrome;
    • TTP;
    • the Kazabaha-Merritt syndrome (giant hemangioma);
    • "Blue" heart defects.
  • Caused by increased destruction of platelets:
    • medicinal (ristocetin, protamine sulfate, bleomycin, etc.);
    • stenosis of the aorta;
    • infection;
    • cardial (artificial valves, repair of intracardiac defects, etc.);
    • malignant hypertension.

Violation of the distribution and deposition of platelets

  • Hyperplenism (portal hypertension, Gaucher's disease, congenital "blue" heart defects, neoplasms, infections, etc.)
  • Hypothermia.

Decreased platelet production (decrease or absence of megakaryocytes in the bone marrow - amygakaryocytic thrombocytopenia)

Hypoplasia or depression of megakaryocytes 1

  • Medicinal (chlorothiazides, estrogens, ethanol, tolbutamide, etc.).
  • Constitutional:
    • thrombocytopenia with TAR-syndrome (congenital absence of radial bones);
    • congenital platelet hypoplasia without abnormalities;
    • congenital hypoplastic thrombocytopenia with microcephaly;
    • rubella embriofetopathy;
    • trisomy 13,18;
    • Fanconi anemia.
  • Ineffective thrombocytopoiesis:
    • megaloblastic anemia (deficiency of vitamin B 12 and folic acid);
    • severe iron deficiency anemia;
    • hereditary thrombocytopenia;
    • paroxysmal nocturnal hemoglobinuria.
  • Disorders of regulation of thrombocytopoiesis:
    • insufficiency of thrombopoietin;
    • intermittent dysgenesis of platelets;
    • cyclic thrombocytopenia.
  • Metabolic disorders:
    • methylmalonic acidemia;
    • ketone glycineemia;
    • deficiency of carboxyl synthetase;
    • isovaleric acidemia;
    • idiopathic hyperglycinemia;
    • Newborns from mothers with hypothyroidism.
  • Hereditary diseases of platelets 2:
    • the Bernard-Soulier syndrome;
    • the May-Heglin anomaly;
    • Wiskott-Aldrich syndrome;
    • linked to the sex of thrombocytopenia;
    • Mediterranean thrombocytopenia.
  • Acquired aplastic diseases:
    • idiopathic;
    • drug-induced (overdose of antitumor drugs, organic and inorganic arsenic, mezantoin, trimethine, antithyroid, anti-diabetic, antihistamines, butadione, insecticides, gold preparations, idiosyncrasy to levomycetin);
    • radiation;
    • virus-induced (hepatitis, HIV, Epstein-Barr virus, etc.).

Infiltrative processes in the bone marrow

  • Benign:
    • osteopetrosis;
    • accumulation diseases.
  • Malignant:
    • neoplasms: leukemia, myelofibrosis, Langer- cell-cell histiocytosis.
    • secondary: lymphomas, neuroblastoma, metastasis of solid tumors.

With a decrease in the number of megakaryocytes in the bone marrow, in addition to aspiration, bone marrow biopsy is required to prevent typical errors. These conditions are associated with a normal or increased amount of megakaryocytes in the bone marrow.

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