Thrombocytopenic purpura

Thrombocytopenic purpura (synonym disease Verligofa) - a disease characterized by multiple hemorrhages in the skin and bleeding from the mucous membranes caused by thrombocytopenia (see). More common among women. The essence of the disease is low development of platelets by megakaryocytes, the bone marrow due to their functional inferiority. Emerging thrombocytopenia, as well as violation of the permeability of the vascular wall lead hemorrhage and bleeding.
Currently the pathogenesis of some of thrombocytopenia associated with education in the body (mostly in the spleen) and the accumulation in the blood of antiplatelet ISO - or antibodies that destroy the platelets patient (immune form). The immune nature of thrombocytopenia established on the basis of appropriate laboratory tests (detection of antiplatelet antibodies).
Obura thrombocytopenicClinical picture. Bleeding in the skin and bleeding from the mucous membranes develop spontaneously or under the influence of minor injuries. Most often bleeding from the nose, gums, and mouth, sometimes of internal organs; women predominate uterine bleeding. The lien is not enlarged. Blood picture in the stage of remission or in cases occurring among subcutaneous hemorrhages, normal, except for thrombocytopenia. Blood loss is developing posthemorrhagic anemia, neutrophilic leucocytosis. The most characteristic in this period of profound thrombocytopenia, severe disturbance of retraction (reduction) of a blood clot, a sharp increase in bleeding time (up to 5 minutes or more). In most cases the positive symptoms of tug and pinch (see Konchalovsky - Tiller - Leede symptom). Kostnomozgove blood characterized by hyperplasia of megakaryocytes; in progressive anemia - cell hyperplasia of the red Rostock, hyperplasia of the red bone marrow in the flat bones and development of centres of red bone marrow in diatezah tubular bones. For thrombocytopenic purpura cyclical, with periods of recurrence are followed by remission. The question on continuation of the pregnancy in patients thrombocytopenic purpura is solved individually. In severe with frequent bleeding maintaining a pregnancy is not shown, calm infections during pregnancy can be saved. The patient should be not only under the supervision of a midwife and physician-hematologist.
Diagnosis thrombocytopenic purpura put on the basis of clinical and hematological picture. The forecast in relation to life in the most favorable cases, however, it can be fatal result in massive bleeding and extensive haemorrhages in vital organs.
Treatment. In the period of bleeding appoint local (see Thrombin and Hemostatic sponge) and General hemostatic means: 0.5-1 g of ascorbic acid 10 ml of 10% solution of calcium chloride intravenous vitamin R-citrine 0.05 g and rutin 0,025 g 3-4 times a day, a blood transfusion (for the purposes and under the supervision of a doctor) at doses of 100-150 ml or transfusion trombotsitnoy estate (on 40-60 ml). When the immune form thrombocytopenic purpura treated with corticosteroids (by prescription). Radical method of treatment thrombocytopenic purpura is splenectomy (see), the indication for which are resistant thrombocytopenic bleeding. Symptomatic thrombocytopenic purpura found in various pathological conditions (anemia, leukemia, infectious diseases and other).

Thrombocytopenic purpura (synonym: benign thrombocytopenia, disease Verligofa) - a disease characterized by multiple hemorrhages in the skin and bleeding from the mucous membranes caused by thrombocytopenia. Occurs more often in women in the perestroika period of the neuroendocrine system - in 15-20 and 40-50 years. The known cases, the family of diseases. The frequency of P. T. takes the first place among the various forms of hemorrhagic diathesis.
Etiology. There are disease Verligofa as independent nosological form and symptom complex Verligofa caused by various etiological factors. To disease Verligofa is called idiopathic, often family thrombocytopenic purpura, with a relatively benign, chronic relapsing course. Syndrome Verligofa may occur as the disease acquired in various pathological conditions.
Classification. In accordance with the etiology and pathogenesis, and clinical course there are the following options thrombocytopenic purpura. 1) actually disease Verligofa - chronic idiopathic" P. T.; 2) izoimmunnaya P. I. on the ground of a repeated blood transfusions (trombotsitnoy estate) or pregnancies; 3) P. T. newborn related passed through the placenta maternal auto - and strombo-agglutinins; 4) autoimmune thrombocytopenia, often combined with other autoaggressive diseases with autoimmune hemolytic anemia (so-called tromboliticescoe P. T. syndrome Fischer - Evans), systemic lupus red and other; 5) symptomatic thrombocytopenia (syndrome Verligofa) for the most part non-immune, which can be observed when Vitamin B12 deficiency, Hypo - and aplastic anemia, acute and chronic radiation sickness, systemic lesions of the blood (leukemia - retikulez), metastatic bone-blood system malignant neoplasms, infectious diseases, when taking certain medicines. Thrombocytopenic purpura may complicate typhoid fever, malaria, visceral leishmaniasis, persistent septic endocarditis and other
Drug-induced thrombocytopenia occurs shortly after administration of medications, usually 1 to 12 weeks, more rarely, and usually ends of spontaneous recovery. Known cases of drug thrombocytopenic purpura in connection with the use of barbiturates, in particular sutormina (allyl-isopropyl-acetyl-carbamide), sulfanilamidov, quinine, quinidine, preparations bismuth, arsenic (noversana), mercury, gold, antibiotics, anti-toxic (tetanus) serum. The comparative rarity of such complications indicates the value of individual predisposition.
Radiation thrombocytopenia occur in people exposed to radiant energy,radiologists, radiologists, as well as in patients receiving x - ray and radiation. Although the decline in the number of blood platelets in these cases usually does not reach the critical numbers (30 000) and is not accompanied haemorrhagic phenomena, it should serve as an alarm signal, as a further application of x-ray exposure (or radioactive isotopes) can lead to severe hemorrhagic diathesis, meaning transition bone marrow in Hypo - or aplastic state.
Pathogenesis. Observations showed, thrombocytopenia when illness Verligofa depends on the functional lesions of megakaryocytes, expressed in a broken process of "adsorbance" blood platelets (Fig. 1).

Fig. 1. Mature megakaryocyte in punctate bone marrow when thrombocytopenic purple (up to splenectomy). Giant cell morphologically normal, but functionally active (no formation of blood platelets).

Currently, the pathogenesis of most functional thrombocytopenia (not associated with fossil bone marrow aplasia) consider, in the light of immunohaematology as an autoimmune disease, rarely izoimmunnaya nature. A number of authors highlighted when thrombocytopenic purple autoimmune antiplatelet antibodies among of them are: 1) thromboangiitis; 2) incomplete antiplatelet antibodies are detected using the sample Kumbsa; 3) "allergic" antiplatelet antibodies that are valid only in the presence of drugs or other substances to which sensitized patient.
Immunoallergical character thrombocytopenia established on the basis of appropriate laboratory tests (detection of antiplatelet antibodies, a positive test Kumbsa). In favor of immunological nature of the disease and says the favorable effect of treatment with steroids.
Bleeding when thrombocytopenic purple is due primarily thrombocytopenia and related changes in biological properties of blood and vascular wall, leading to the increase in the duration of capillary bleeding, a violation of clotting and blood clot retraction.
In Genesis thrombocytopenic bleeding should be considered common factors leading to increased permeability of the capillary wall, in particular With-deficiency disease.
Pathological anatomy. At showdown died from thrombocytopenic bleeding find multiple bleeding of mucous and serous membranes, skin, adipose tissue, parenchymatous organs, the membranes of the brain and the substance of the brain.
In profuse bleeding into the cavity and the abdominal organs (abdominal, chest cavity, small pelvic cavity, gastrointestinal tract) they find a large number streamed blood. If the patient died from massive blood loss, the autopsy found anemia bodies along with hyperplasia of the red bone marrow in the flat bones and development of centres of red bone marrow in diatezah bone (femur). There is also a moderate enlargement of the spleen at the expense of pulp. Microscopically in the bone marrow hyperplasia megakaryocytes (the latter is absent in the gipoplasticheskaya States blood).

The clinical picture. The main clinical symptoms thrombocytopenic purpura are bleeding in the skin and bleeding from the mucous membranes that develop as though spontaneously, under the influence of tiny, barely visible injuries. Bleeding in the skin are different by size (from a pinhead to saucers), and color: depending ago hemorrhage changes its initial purple color, getting consistently purple, blue, green, brown, or yellow, simultaneously becoming paler.
Favorite places of location of Achkasov and petechiae on the skin is the front surface of the body and extremities; rarely, bleeding occur on the face.
Bleeding from the mucous membranes is one of the characteristic features thrombocytopenic purpura. Most often nosebleeds, which sometimes have intensive and lead to dramatic development
anemia. The second most frequently occupy bleeding from gums and oral cavity; they do not reach high-intensity, unless caused by trauma. Especially dangerous extraction of teeth and tonsillectomy (known cases of fatal bleeding after these operations), meanwhile larger surgical intervention - appendectomy or splenectomy is not pose too much bleeding.
Often when thrombocytopenic purple bleeding from internal organs (gastrointestinal, pulmonary, renal and bleeding in the pleura, the abdominal cavity, the brain, the sclera and the retina.
Women in the clinical picture of the disease is often dominated by uterine bleeding Meno - and metrorrhagia. Bleeding in the peritoneum due to ovulation can mimic the picture of ectopic pregnancy. Girls thrombocytopenic purpura often found with the advent of the first month, which character of profuse bleeding.
The tendency to bleeding from the genitals in women with thrombocytopenic purpura, due to the decline in the number of platelets during menstruation. If in healthy women for 1-2 day of menstruation, the number of platelets in the blood is reduced by 30 - 50%, in patients thrombocytopenic purpura in the days of menstruation plates almost disappear, clot becomes retractile and bleeding time dramatically lengthened. At the same time or several days before the onset of menstruation found common haemorrhagic phenomenon (bruises on the body, nasal and other bleeding).
The lien is not enlarged. In rare cases enlarged spleen due transferred malaria or any other chronic infections; enlargement of the spleen cannot be associated with thrombocytopenic purpura. The great size of the spleen in hemorrhagic diathesis and thrombocytopenia have to think about leukemia, Gaucher disease, etc.
Blood picture. In remission, and also in cases, manifested only subcutaneous (or submucosal) hemorrhages without external or internal bleeding, blood picture normal. Due to blood loss develops posthemorrhagic anemia. Prolonged and repeated bleedings anemia gets gipomagniemii and even gipoplasticheskaya character. From the side of the white blood cells in the ordinary course of the disease is not observed changes; blood loss - neutrophilic leucocytosis to 10 000; with the development of gipomagniemia (gipoplasticheskaya) of the blood is marked leukopenia (due granulotsitopenii).
Haemorrhagic syndrome. Thrombocytopenia is the most constant symptom. In periods of bleeding the number of blood platelets in the product falls to units and even to zero. In periods of remission number of records increased, but, as a rule, does not reach the standards.
Retraction of a blood clot sharply broken; at the height thrombocytopenic bleeding clot completely lost the ability to retraction and remains loose serum is not separated. In periods of remission retraction of the clot becomes normal.
Bleeding time in acute thrombocytopenic purpura sharply extended (up to 30 minutes or more). Blood clotting can be delayed due to the deficit of the third tromboplasticheskoy factor plates; in remission, and after splenectomy the clotting of blood becomes normal.
Symptom harness in most cases, thrombocytopenic purpura positive. With great constancy when thrombocytopenic purple observed symptom pinch - to place a light pinch in a few minutes found growing ekstrawazat.
Over. Disease Verligofa has a cyclical nature. During remission in the number of blood platelets in the blood increases, reaching in some cases, the lower limit of normal (100 000 to 1 mm3) or the full rate. Relapse thrombocytopenic purpura may be triggered by infection, surgery, nutritional intoxication-With-vitamin deficiencies.
Diagnosis and differential diagnosis. Thrombocytopenic purpura is diagnosed on the basis of typical clinical picture and the characteristic hemorrhagic syndrome in complete safety and even hyperplasia megakaryocytes apparatus bone marrow (Fig. 2 and 3). Particular difficulties may represent differential diagnosis thrombocytopenic purpura with aplastic and systemic diseases of blood. So, acute leukemia often begins with phenomena thrombocytopenic purpura, which has been the cause of incorrect diagnoses and even the direction of patients to the operation splenectomy. In these cases, an important diagnostic importance of the following symptoms: 1) increased dense spleen - the trait is more characteristic of the system diseases of the blood (leukemia), than for P. T.; 2) anemia, especially macrocytic type, is not adequate blood loss.

Fig. 2. Punctat bone marrow when thrombocytopenic purple. Hyperplasia giant cells.
Fig. 3. Punctat bone marrow when thrombocytopenic purple. The megakaryocytes in the counting chamber.

Differential diagnosis with other forms of hemorrhagic diathesis - hemophilia, hemorrhagic vasculitis, scurvy, gastrointestinal bleeding in tromboliticescoy splenomegaly with gepatrombina - is not difficult considering the research data hemorrhagic syndrome.
Uterine bleeding in young girls in the first place should be differentiated disease Verligofa from discoverylink (so-called juvenile bleeding.
Forecast in respect of life in the vast majority of cases favourable. The case fatality rate is about 4% after a single (primary) acute attacks and 8% in cases of multiple (re) attacks. Death comes as a result of a brain hemorrhage or severe anemia on the ground bleeding.