Types of thrombocytopenia

Content

  • thrombocytopenia
  • Hemorrhagic thrombocytopenia
  • Thrombotic thrombocytopenic purpura (TTP) (Moshkovits disease)
  • Hemolytic-uremic syndrome
  • Idiopathic thrombocytopenic purpura



  • thrombocytopenia

    platelet pathology is the causebleeding in nearly 80% of cases. This thrombocytopenia - the most frequent manifestation of it. It is believed that normal platelet count should be between (150-400) × 109 / L. Under normal physiological conditions in the blood platelet count can vary. It depends on the physical activity, digestion, hormonal levels in women of menstrual cycle - in the first days of menstruation it may decrease by 30-50%. The lifespan of platelets - 9-10 days. About 1/3 of platelets deposited in a normal spleen. In cases when the platelet count of less than 150 × 109 / L, should be stated thrombocytopenia. The frequency of this phenomenon sufficiently high - from 10 to 130 new cases per 1 million people.

    There are acute and chronic thrombocytopenia. The latter is diagnosed when its duration exceeds 6 months. In patients with platelet counts above 50 × 109 / L is extremely rare bleeding disorders. In this regard, many believe that to ensure full haemostasis enough platelets in the range of 30 × 109 / L, 15 or even 10 × 109 / L platelets can provide full effective hemostasis. Perhaps that is why most of thrombocytopenia occurs in medical practice only as a laboratory phenomenon, causing the surprise of doctors, as does not manifest itself clinically. At the same time decrease in platelets below 100 × 109 / L should attract serious attention of the doctor, because he may be the symptom of thrombocytopenia accompanied by serious pathology. Due to these reasons, in each case of thrombocytopenia need to find out.


    Hemorrhagic thrombocytopenia

    Hemorrhagic thrombocytopenia was one ofFirst identified hemorrhagic diseases of humans. There are several types of thrombocytopenia. Very often, they are secondary, as a symptom of a disease. However, thrombocytopenia syndrome may often be independent, but its pathogenesis different. Thrombocytopenia may result from an inferiority generation platelet destruction due to their increased immune and non-immune mechanisms, and redistribution in the body from the blood to individual organs, usually the spleen, or their accumulation in the thrombotic masses with massive intravascular coagulation. There are many cases a combination of these reasons.

    Types of thrombocytopeniaHowever, the lack of clear criteria for the confirmation of certain
    pathogenetic mechanism makes its use for practical
    the doctor is not very desirable, since most doctors try
    identify the specific forms, and then
    comprehend its etiology and pathogenesis. In this regard, it seems to us
    immediately inform appropriate practical information to doctors
    regarding the possibility of thrombocytopenia as a separate
    syndrome secondary at some other disease.

    The first step is to exclude disease
    can cause coagulopathy consumption. These include all
    infectious diseases, including HIV infection; neoplastic processes
    any localization, especially tumors of the liver, gastrointestinal tract, leukemia, etc .;
    systemic autoimmune diseases, especially SLE and
    obstetric pathology caused by premature detachment of the placenta,
    intrauterine fetal death, amniotic fluid embolism; massive
    tissue damage as a result of injuries. Diseases such as
    drug disease, auglobulinemiya, lymphoproliferative
    diseases, inherited and acquired myelodysplasia often
    accompanied by thrombocytopenia.

    Particularly noteworthy pathological
    State, accompanied by enlargement of the spleen. Most often it is
    observed in portal hypertension of any origin (cirrhosis of the liver,
    other violations of the outflow through the portal vein), storage diseases
    (Tezaurismozy): Niemann-Pick disease, Henda-Shyullera-Christian, Gaucher,
    hemochromatosis et al., as well as many of the conditions listed above
    - Infection, diffuse disease of connective tissue and blood diseases.
    Occasionally observed splenomegaly and in congestive heart
    failure. As previously mentioned, in normal spleen
    concentrated by 1/3 to 1/4 of the platelet. Consequently
    any increase in the spleen leads to increased delay in it
    platelets, which results in the phenomenon of thrombocytopenia. when all
    these conditions will be more fair assumption
    the possible presence of multiple pathogenic mechanisms. An exception
    or confirmation of many of these diseases often requires
    a great differential diagnostic work.

    Secondary thrombocytopenia are the result of
    Radiation effects on the energy beam being a symptom
    disease, various toxic substances, including salts of heavy metals,
    alcohol, etc.., entering the symptom of pancytopenia. The likelihood of such a
    the mechanism of thrombocytopenia in patients with uremia. It is possible that a violation
    regulation of hematopoietic vitamins and hormones has certain
    especially for trombotsitopoeza, however thrombocytopenia with
    pernicious anemia should only be considered as a symptom,
    sometimes major.

    Primary thrombocytopenia, ie those diseases
    the clinical manifestations of which are fully associated with thrombocytopenia and
    in which a pathology is not observed, also have different
    pathogenetic mechanisms, but these diseases deserve special
    descriptions.


    Thrombotic thrombocytopenic purpura (TTP) (Moshkovits disease)

    Thrombotic thrombocytopenic purpura (TTP) (Moshkovits disease) It was
    first described by the author, 16-year-old girl in 1924. The disease was
    acute onset and manifested by fever, renal and central nervous system functions,
    symptoms of heart failure with severe thrombocytopenia. On
    autopsy revealed a lot of blood clots in capillaries and small arterioles,
    almost entirely composed of platelets and fibrin small
    component. For the disease characterized by a combination of severe
    thrombocytopenia with intravascular hemolysis (with a lot of
    fragmented erythrocytes) and neurological manifestations - from
    unsharp disturbance of consciousness, motor and sensory functions to
    convulsions and coma. These clinical symptoms in 70-90% of cases are
    single-entry, and in only 10-30% of patients when they recur
    pregnancy, various infections, chemotherapy. For some patients
    they can recur at regular intervals. This allows
    suggest that TTP syndrome is heterogeneous and combines several
    various diseases. Practical measures: plasmapheresis, plasma replacement
    patient on the platelet poor plasma, fresh frozen healthy
    allow to save up to 90% of patients today, recently condemned to
    death.


    Hemolytic-uremic syndrome

    Hemolytic-uremic syndrome many
    considered as a kind of TTP. However, the absence of pathology
    nervous system, resistant hypertension and severe renal
    pathology with progressive renal insufficiency and
    the possibility of inheriting the disease are forced to consider it
    independent. Thrombocytopenia is severe, accompanied by
    intravascular hemolysis - the direct result of thrombotic occlusion
    vessels.

    Medicinal Trombone persecution. described pretty
    many cases of thrombocytopenia, which follow the therapeutic measures.
    Initially, attention was paid only to the cases of thrombocytopenia,
    accompanying treatment of radioactive drugs. Drugs
    drugs can cause thrombocytopenia in various ways. cytostatics
    trombotsitopoez able to inhibit the bone marrow. quinidine,
    sulfonamides, salicylates, dipyridamole, gold preparations, cephalothin,
    trimethoprim, a-methyldopa and may include other immune mechanisms.
    They provoke thrombocytopenia, settling on platelets and stimulating
    antibodies to this complex. Other drug form
    complexes with plasma proteins, which then connects to already
    platelet membrane and leads to the production of antibodies to the
    formation. Others cause the destruction of platelets themselves, and thus
    contribute to the formation of antibodies directly to the latter.

    In recent years, attracted a lot of attention
    thrombocytopenia caused by heparin, or, as some suggest
    researchers, thrombocytopenia, associated with heparin.
    Almost 10% of patients receiving intravenous heparin and moderately reduced
    platelet count, which usually occurs within 1-2 days
    after administration, and rate reduction are rarely less than 100 x 109/ L. Today it is known that heparin can cause thrombocytopenia in two types.

    The first type, described above, is due to direct
    Platelet interaction with heparin. Communicate it to the surface
    platelets depends upon the molecular weight heparins, and their sulfation
    and the extent of platelet activation caused by this interaction.
    Note that the degree of thrombocytopenia in parallel with reduced
    decrease in molecular weight heparin is administered. Geparinindutsirovannaya
    thrombocytopenia type 1 occurs quickly after administration, sometimes
    within a few hours, due to changes in the platelet membrane,
    and provides that the aggregation of platelets. This type of thrombocytopenia
    It passes quickly and the risk to the patient is not.

    Geparinindutsirovannaya, or associated,
    thrombocytopenia type 2 occurs between 4-20 th day after administration
    heparin with a maximum severity on day 10 in patients receiving
    heparin for the first time. Repeated administration of heparin, it can
    occur in the first days after drug administration. This
    thrombocytopenia caused by immune mechanisms, provoked
    heparin, responsible for the production of specific antibodies to
    platelets. These antibodies are detected in almost 7.5% of patients
    receiving unfractionated heparin produced from the products
    derived from pigs, and only 2.5% of those treated with
    low molecular weight heparins.

    The mechanism of thrombocytopenia differs from other
    immune thrombocytopenia fact that in this case the antibody does not cause
    amplification of platelet phagocytosis by macrophages system and stimulate
    intravascular platelet aggregation. From other immune
    thrombocytopenia it also differs in that it is practically at
    no hemorrhagic complications despite the fact that it
    develops on the background of anticoagulant therapy. This thrombocytopenia on
    essentially reflects the consumption of platelets in the formation mikrotrombov,
    which can grow to a large size, and defining
    thrombotic events. Recent dangerous in those cases where
    Heparin does not stop, can be the cause of death
    in 20-30% of cases.


    Idiopathic thrombocytopenic purpura

    Types of thrombocytopeniaAlmost 95% of the basis of thrombocytopenia is idiopathic
    thrombocytopenic purpura (ITP). It suggests and diagnose,
    When platelet reduction has no direct connection with any
    cause or condition that can cause this decline. Annually
    identify cases of idiopathic 10-125
    thrombocytopenic purpura per million population. Idiopathic
    thrombocytopenic purpura considered
    acute if lasts less than 6 months, a longer its existence
    It should be regarded as chronic idiopathic
    thrombocytopenic purpura. Acute idiopathic form
    thrombocytopenic purpura often
    in children and ends with complete recovery at 75%
    ill. Adults often suffer from chronic idiopathic option
    thrombocytopenic purpura, wherein
    up to 5% of them are killed by bleeding, bleeding mainly from
    in the brain. Analysis of a large number of patients with thrombocytopenia which
    We underwent a complete examination, including bone marrow examination,
    has shown that the causes of thrombocytopenia other than idiopathic
    thrombocytopenic purpura, was only 4%
    surveyed. However, due to the fact that the specific criteria for
    diagnosis of idiopathic
    thrombocytopenic purpura today absent, her diagnosis
    based only on the exclusion of other causes of thrombocytopenia. at
    This right to determine the nature of the disease is equally
    important data history, physical characteristics of the patient, as well as
    the clinical picture and the results of laboratory and instrumental
    study. History can help in the first place at clarifying
    inherited disease. In the presence of anomalies in the next
    relatives of the subject may be suspected and to identify inherited
    thrombocytopenia.

    Hereditary and congenital thrombocytopenia. at
    lack of patient radiation (radial) bone think of inherited
    Pathology - TAR syndrome (thrombocytopathy in the absence of the radial
    bone), which is also characterized by the release of dense defect
    granules and platelet thrombocytopathy. Thrombocytopenia, combined with
    defective pigmentation of hair, skin, retina, oblige to think about the presence of
    the patient has an inherited disease - Chediak-Higashi syndrome, which
    also characterized by defective platelet granule release, and as
    consequently, thrombocytopathy. The presence of eczema and a tendency to infectious
    disease causes always assume the inherited pathology -
    Wiskott-Aldrich syndrome, also defective release of dense granules
    and platelet trombotsitopaty of platelet aggregation defect
    adrenaline. The presence in the blood smear giant platelet yields
    suggests as an inherited syndrome Meia Hegglina and
    Bernard-Soulier disease. Their difference lies in the fact that the syndrome
    Meia Hegglina can detect abnormal blood granulocytes with major
    inclusions, and a defect is detected while the disease Bernard-Soulier
    glycoprotein lb, which determines the platelet adhesion factor
    Willebrand. It is possible that some of thrombocytopenia newborns
    It is an inherited deficiency of thrombopoietin.

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