Tricocytopenia types


  • Thrombocytopenia
  • Hemorrhagic thrombocytopenia
  • Thrombotic thrombocytopenic purpura (TTP) (Moshkovitz disease)
  • Hemolytic-uremic syndrome
  • Idiopathic thrombocytopenic purple

  • Thrombocytopenia

    Platelet pathology is the cause of bleeding in almost 80% of cases. At the same time, thrombocytopenia is the most frequent manifestation. It is believed that normally the number of platelets should be within (150-400) × 109 / L. In normal physiological conditions, the number of blood platelets can fluctuate. It depends on the physical exertion, digestion, hormonal background, in women from the menstrual cycle - in the first days of menstruation it can decrease by 30-50%. Lifetime of platelets - 9-10 days. About 1/3 of blood plates deposited in a normal spleen. In cases where the number of platelets is less than 150 × 109 / l, the thrombocytopenia should be aspage. The frequency of this phenomenon is large enough - from 10 to 130 new cases per 1 million people.

    Distinguish acute and chronic thrombocytopenia. The latter is diagnosed when it exceeds it 6 months. In persons with platelet levels above 50×109 / l extremely rarely bleeding. In this regard, many believe that in order to ensure a full hemostasis, there are enough platelets in the range of 30×109 / l, 15 and even 10×109 / l full platelet can provide effective hemostasis. It is probably why the thrombocytopenia is most often found in medical practice only as a laboratory phenomenon, causing surprise of doctors, because in no way manifests itself clinically. At the same time, a decrease in platelets is below 100×109 / l should attract serious attention to the doctor, since this symptom of thrombocytopenia may accompany a serious pathology. In this regard, the cause of each case of thrombocytopenia must be found out.

    Hemorrhagic thrombocytopenia

    Hemorrhagic thrombocytopenia was one of the first identified human hemorrhagic diseases. Distinguish several types of thrombocytopenia. Very often they are secondary, being a symptom of some disease. However, thrombocytopenia syndrome can often be independent, and its pathogenesis is different. Thrombocytopenia may be a consequence of the inferiority of platelet production, their increased destruction due to immune and non-immune mechanisms, as well as the redistribution in the body from the blood into individual bodies, more often a spleen, or their cluster in thrombotic masses with a massive intravascular blood coagulation. Frequent cases of combination of these reasons.

    Tricocytopenia typesHowever, the lack of clear criteria to confirm a certain
    The pathogenetic mechanism makes it use for practical
    the doctor is not very desirable, since most doctors are trying
    identify a specific nosological form, and then already
    comprehend its etiology and pathogenesis. In this regard, it seems to us
    It is advisable to immediately inform practical doctors information
    Regarding the possibility of thrombocytopenia as a separate
    secondary syndrome with some other disease.

    First of all, the disease should be excluded,
    able to cause coagulopathy consumption. These include all
    infectious diseases in T.C. HIV infection; Neoplastic processes
    any localization, especially the tumors of the liver, gastrointestinal tract, leukemia, etc.;
    systemic autoimmune diseases primarily well as well as
    Obstetric pathology due to a premature detachment of the placenta,
    intrauterine fetal death, emboline by oily waters; Massive
    Tissue damage as a result of injuries. Diseases such as
    Drug disease, Auglobulinemia, lymphoproliferative
    Diseases, inherited and acquired myelodysplasias also often
    accompanied by thrombocytopenia.

    Pathological deserves special attention
    States accompanied by an increase in spleen. Most often that
    Observed with portal hypertension of any genesis (liver cirrhosis,
    Other disorders of the outflow on a muster vein), accumulation diseases
    (Thezaurismos): Nimanne-Peak's disease, Hend-Shuller-Chrischena, Goshe,
    Gemochromatosis and other., as well as with many of the states listed above
    - infections, diffuse diseases of connective tissue and blood disease.
    The occasionally splenomegaly is noted with a stagnant cardiac
    Insufficiency. As mentioned earlier, in a normal spleen
    Focus from 1/3 to 1/4 of all platelets. Therefore
    Any increase in the spleen leads to an increase in the delay in it
    platelets, which causes the thrombocytopenia phenomenon. At all
    listed states will be more fair to assume
    Possible presence of several pathogenetic mechanisms. An exception
    or confirmation of many of the listed diseases often requires
    big differential diagnostic work.

    Secondary thrombocytopenia are due
    Impact on the organism of radial energy, being a symptom of radiation
    diseases, various toxic substances, in t.C. salts of heavy metals,
    alcohol and other., Entering the symptom complex of pancytopenia. Likely such
    The mechanism of thrombocytopenia in patients with uremia. It is possible that violation
    The regulation of hemopower with vitamins and hormones has certain
    Features for thrombocytopower, however thrombocytopenia
    pernicious anemia should be considered only as a symptom,
    Sometimes the main thing.

    Primary thrombocytopenia, t.E. Those diseases,
    Clinical manifestations of which are fully related to thrombocytopenia and
    in which other pathology is not observed, also have different
    Pathogenetic mechanisms, but these diseases deserve special

    Thrombotic thrombocytopenic purpura (TTP) (Moshkovitz disease)

    Thrombotic thrombocytopenic purpura (TTP) (Moshkovitz disease) was
    First described by the author at a 16-year-old girl in 1924. The disease had
    Acute start and manifested by fever, impaired kidney function and CNS,
    Candular deficiency phenomena with severe thrombocytopenia. On the
    An autopsy revealed a lot of blood clots in capillaries and small arteriols,
    Almost entirely consisting of platelets and a small fibrin
    Component. For the disease, a combination of pronounced
    plateitopenia with intravascular hemolysis (with many
    fragmented erythrocytes) and neurological manifestations - from
    unwitting disruption of consciousness, motor and sensitive functions
    Sailor and coma. These clinical manifestations in 70-90% of cases are
    one-time, and only 10-30% of patients they recur at
    Pregnancy, various infections, chemotherapy. In individual patients
    They can recur through regular intervals. This allows
    assume that TTP syndrome is heterogeneous and combines several
    various diseases. Practical measures: Plasma Plasma Replacement
    Patient on poor platelets Freshly frozen plasma of healthy
    allow today to save up to 90% of patients recently doomed on

    Hemolytic-uremic syndrome

    Hemolytic-uremic syndrome many
    considered as a variety of TTP. However, the absence of pathology
    nervous system, persistent arterial hypertension and pronounced renal
    Pathology with progressive renal failure as well
    The possibility of inheritance is forced to consider it
    Self. Thrombocytopenia is pronounced, accompanied by
    intravascular hemolysis - direct consequence of thrombotic occlusion

    Drug thrombony persecution. Described pretty
    Many cases of thrombocytopencies that follow therapeutic measures.
    Initially, attention paid only on cases of thrombocytopenia,
    accompanying treatment with radioactive drugs. Medicinal
    Preparations can cause thrombocytopenia in various ways. Cytostatics
    Caught to catch a thrombocytopoese in bone marrow. Hinidin,
    Sulfonamides, Salicylates, Dipyridamol, Gold preparations, Cefalotin,
    Timetoprix, A-Methyldop and others may include immune mechanisms.
    They provoke thrombocytopenia, settled on platelets and stimulating
    Education of antibodies to this complex. Other medicines form
    complexes with plasma protein, which is then connected to
    platelet membrane and leads to the development of antibodies to this
    formation. Third cause the destruction of platelets themselves and thereby
    contribute to the formation of antibodies directly to the last.

    Recently, great attention is attracted
    thrombocytopenia caused by heparin, or as some
    Researchers, thrombocytopenia associated with heparinotherapy.
    Almost 10% of patients receiving intravenously heparin, moderately reduced
    The amount of platelets, which usually occurs within 1-2 days
    After administration, the level of reduction rarely is less than 100 x 10nine/ L. Today it is already known that heparins are able to cause thrombocytopenia of two types.

    The first type described above is due to direct
    interaction of heparin with platelets. Its connection with the surface
    platelets depends on the molecular weight of heparins, their sulfatization
    and the degree of activation of platelet caused by these interaction.
    Noted that the degree of thrombocytopenia decreases in parallel with
    Reducing the molecular weight of the introduced heparin. Heparinniducan
    Thrombocytopenia of the 1st type arises quickly after its introduction, sometimes
    After a few hours, due to the change in platelet membranes,
    which provide aggregation of platelets. This type of thrombocytopeny
    quickly passes and danger for the patient does not represent.

    Heparinniduced, or associated,
    The thrombocytopenia of the 2nd type occurs between the 4-20th day after the introduction
    Heparin with a maximum of severity on the 10th day in patients who received
    Heparinotherapy for the first time. When re-introducing heparin, it can
    Arrive in the first days after the introduction of the drug. This
    Thrombocytopenia is due to immune mechanisms provoked
    heparins responsible for the production of specific antibodies to
    Thrombocyt. These antibodies are detected by almost 7.5% of patients,
    receiving an unficing heparin produced from products,
    obtained from pigs, and only in 2.5% of those who received
    Low molecular weight heparins.

    The mechanism of this thrombocytope differs from other
    immune thrombocytopenia in that in this case the antibodies are not
    Strengthening the phagocytosis of platelets by the system of macrophages, and stimulate
    intravascular aggregation of thrombocyte. From other immune
    Thrombocytopenia it also differs in that it is practically
    There are no hemorrhagic complications, despite the fact that it
    develops against the background of anticoagulant therapy. This thrombocytopenia is
    The essence reflects the consumption of platelets in the formation of microtrombov,
    which can increase to large sizes, determining
    Thrombotic phenomena. The latter are dangerous and in cases where
    Heparinotherapy does not stop, may be the cause of death
    in 20-30% of cases.

    Idiopathic thrombocytopenic purple

    Tricocytopenia typesAlmost in 95% of cases based on thrombocytopenia lies idiopathic
    Thrombocytopenic purpura (ITP). It is assumed and diagnosed,
    when platelet reduction is not directly connected with any
    cause or condition capable of calling this reduction. Annually
    10-125 cases of idiopathic
    thrombocytopenic purpura for every million people. Idiopathic
    Thrombocytopenic purple is considered
    acute if less than 6 months lasts, a longer existence
    should be regarded as chronic idiopathic
    Thrombocytopenic purple. Acute form idiopathic
    Thrombocytopenic purpura more often
    occurs in children and ends with complete recovery in 75%
    Sicklaby. Adults more often suffer from chronic idiopathic version
    thrombocytopenic purpura, while
    Up to 5% of them die from bleeding, mainly from hemorrhage
    in the brain. Analysis of a large number of patients with thrombocytopenia, which
    Passed a full-fledged examination, including bone marrow research,
    showed that the causes of thrombocytopenia other than idiopathic
    Thrombocytopenic purpura, were only 4%
    Examined. However, due to the fact that specific criteria for
    diagnostics idiopathic
    Thrombocytopenic purpura today is absent, its diagnosis
    Based only at the exception of other causes of thrombocytopenia. At
    this to properly determine the nature of the disease equally
    Anamnesis data, physical characteristics of the patient, as well as important
    Clinical picture and laboratory and instrumental results
    Research. History can help first of all when finding out
    Inherited pathology. In the presence of anomalies of development near the nearest
    Relatives of the surveyed can be suspected and reveal inherited

    Inherited and congenital thrombocytopenia. At
    the absence of a radiy (radial) bone to think about inherited
    pathology - tar-syndrome (thrombocytopathy in the absence of radial
    bones), which is also characterized by a defect release dense
    platelet and thrombocytopathy. Thrombocytopenia combined with
    Defect for hair pigmentation, leather, retina, obliges thinking about
    In the patient inherited pathology - Chediak Higashi syndrome, which
    It is also characterized by a defect release of platelet granules and, as
    Corollary, thrombocytopathy. The presence of eczema and tendency to infectious
    Diseases will always assume inherited pathology -
    Syndrome Viscott-Aldrich, also with a defect release of dense granules
    platelet and thrombocytopathy due to a platelet aggregation defect
    Adrenaline. The presence in the blood smear of giant platelets gives
    reason to assume both inherited Maya-Hegglin syndrome and
    Bernard Sulie. Their difference is that when syndrome
    Maya-Hegglin can be found in the blood abnormal granulocytes with large
    Inclusions, and during the disease Bernara Sulie, a defect is detected
    LB glycoprotein, which determines platelet adhesion to factor
    Willebranda. It is possible that some thrombocytopenia of newborns
    Causes inherited thrombooetin deficiency.

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