Bleeding tendency

Introduction Bleeding tendency refers to a clinical manifestation of bleeding on the skin or when tiny blood vessels are slightly traumatized, and is caused by hemostasis and coagulopathy.

Cause

First, abnormal blood vessel wall disease

Hereditary

Hereditary hemorrhagic telangiectasia (HHT), Ehlers-Danlos syndrome.

2. Acquired

Allergic reaction: allergic purpura, autologous erythrocyte sensitizing purpura, self-deoxyribonucleic acid (DNA) allergic purpura, drug-induced vascular purpura.

Non-allergic: Infectious purpura (such as sepsis, subacute infective endocarditis, meningococcal bacteremia, scarlet fever, epidemic hemorrhagic fever, leptospirosis, typhoid, etc.), toxic purpura (chemistry) Drug or drug poisoning, snake bite, bee venom poisoning, uremia), mechanical purpura (local mechanical compression, long steric position), abnormal globulinemia (macroglobulinemia, cryoglobulinemia) , multiple myeloma, vitamin C deficiency, senile purpura. The cause is unknown: simple purpura.

Second, abnormal platelet diseases

Thrombocytopenia

Congenital: Fanconi syndrome, congenital megakaryocyte-free thrombocytopenia, hereditary thrombocytopenia.

Immunity: idiopathic thrombocytopenic purpura (acute and chronic), drug-induced immune purpura, post-transfusion purpura, autoimmune diseases (SLE, hyperthyroidism, autoimmune hemolytic anemia, etc.), newborns Immunological thrombocytopenic purpura, human immunodeficiency virus (HIV) infection, anti-human lymphocyte globulin therapy.

Non-immune: infection (virus, bacteria), aplastic anemia, bone marrow infiltration (leukemia, bone marrow metastasis, myelofibrosis, tuberculosis, etc.), megakaryocyte aplastic anemia, ionizing radiation, drugs (cytotoxic drugs, estrogen, thiazide Drugs, interferon, etc., malnutrition, paroxysmal nocturnal hemoglobinuria, periodic thrombocytopenia, renal failure, DIC, blood loss, hemodialysis, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, hypothermia Wait.

2. Thrombosis

Primary hemorrhagic thrombocytosis, other myeloproliferative diseases (chronic myeloid leukemia, primary polycythemia).

3. Platelet function defects

Congenital: giant platelet syndrome (Bernard-Soulier syndrome), platelet weakness (Glanzmann disease), platelet storage disease (alpha particle deficiency and dense particle deficiency), primary platelet release response defect, platelet third factor ( PF3) defects, other congenital abnormalities with thrombocytopenia (Wiscott-Aldrich syndrome, May-Hegglin abnormalities, Chediak-Higashi syndrome), cyclooxygenase and TXA2 synthetase deficiency.

Acquired: uremia, myeloproliferative diseases, abnormal proteinemia, liver disease, DIC, drugs [aspirin, dipyridamole (dipyridamole), indomethacin (indomethacin), dextran, caffeine, aminophylline, etc. .

Third, coagulation, anticoagulant abnormal disease

1. Coagulation factor deficiency

Congenital: common hemophilia A (factor VIII deficiency), hemophilia B (factor IX deficiency), factor XI deficiency, vascular pseudohemophilia (Von Willebrand disease), rare factors I, II , V, VII, XIII deficiency, kallikrein and high molecular weight bradykinin deficiency, congenital multifactor deficiency.

Acquired: vitamin K deficiency, liver disease, amyloidosis, nephrotic syndrome, Gaucher's disease, DIC, fibrinolysis.

2. Increased anticoagulant substances

Mostly found in acquired diseases such as liver disease, uremia, rheumatism, tumors, acute leukemia, lymphoma, radiation sickness, cardiopulmonary bypass, and hemophilia A patients with repeated blood transfusions.

3. Fibrinolytic disease

Primary fibrinolysis (severe trauma, burns, amniotic fluid embolism, early placental ablation, hemolytic transfusion reaction, acute leukemia, cancer metastasis, hypothermic anesthesia, lung, uterus, pancreas, etc., streptokinase and urokinase excess) . Secondary fibrinolysis (DIC, etc.).

Fourth, the comprehensive factors

DIC, liver disease, uremia, rheumatism, acute leukemia, malignant tumors.

an examination

First, medical history

1. Characteristics of bleeding: By understanding the location of bleeding, clinical features, and methods of hemostasis, it can be clinically distinguished into two categories: hemorrhage caused by abnormal blood vessels and platelets and abnormal bleeding of coagulation function.

2. Age and gender: those who have a tendency to hemorrhage after birth or at an early age are more likely to be congenital bleeding disorders. In children and young adults, it is considered to be primary thrombocytopenic purpura, allergic purpura, or hereditary hemorrhagic telangiectasia. Adults should be considered as acquired hemorrhagic purpura. The elderly have cyanosis and ecchymoses, most of which belong to the elderly with purpura; young women repeatedly appear under the ecchymosis, often simple purpura. Male patients with joint bleeding and deformity are considered to be hemophilia A.

3. Family history: Most of the hereditary bleeding disorders have a positive family history. For example, hemophilia A is a hereditary association, in which male members of the family are present and females are carriers. Autosomal dominant inheritance, in both male and female members of the family can have bleeding, such as hereditary hemorrhagic telangiectasia, vascular pseudohemophilia.

4. Inducing factors: There is a history of medication [especially aspirin, phenylbutazone, indomethacin (indomethacin) and other drugs], anticoagulant therapy history, radiation or chemical exposure history should consider bleeding related to the above factors.

5. Concomitant diseases: those with associated diseases are mostly acquired bleeding diseases. Patients with severe anemia should be considered as aplastic anemia, leukemia, and renal failure; those with jaundice and liver dysfunction are suggestive of liver disease; severe infection, shock, obstetric accident, malignant tumor, etc. are accompanied by extensive severe bleeding. Prompt disseminated intravascular coagulation.

Second, physical examination

First, the characteristics of the bleeding should be observed, including the location, distribution and morphology of the bleeding; whether the degree of anemia is consistent with the amount of bleeding. Focus on the lymph nodes, liver, spleen and skin. Swelling of the gums, bleeding and bleeding around the hair follicles suggest a lack of vitamin C. A bit or spotted telangiectasia in the lips, tongue, nose, face, fingers, and back of the hand is characteristic of hereditary telangiectasia. Symptoms of bilateral lower limbs with urticaria should first be considered as allergic purpura. If there is severe anemia and bleeding is not obvious, acute leukemia and aplastic anemia should be suspected. Deep tissue hemorrhage, hematoma or joint cavity bleeding suggest hemophilia. Splenomegaly with skin purpura, ecchymosis should consider hypersplenism. Malignant lymphoma should be considered for systemic lymphadenopathy with hemorrhage. Hemorrhage with congenital malformations suggests a congenital disease. Others such as severe liver disease, chronic kidney disease, SLE, etc. may have corresponding abnormal signs found for identification.

Third, laboratory inspection

1. Blood routine examination: It is helpful for the diagnosis of acquired bleeding disorders. Such as complete blood cell reduction should consider aplastic anemia, leukemia, PNH, hypersplenism and so on. Red blood cells and platelets are simultaneously reduced and white blood cell counts are normal, suggesting Evans syndrome. Eosinophilia suggests allergic purpura. Normal blood tests may be abnormal platelet function or abnormal blood vessel wall disease.

2. Urine routine examination: urine protein positive more suggestive of kidney disease. Urine lysin positive should be considered as multiple myeloma or light chain disease.

3. Coagulation examination: It is an important basis for diagnosing the cause of bleeding. There are many inspection items, and the relevant items should be selected according to clinical characteristics. Generally, the following steps can be taken.

(1) Simple screening test: check the project and judge the result.

(2) Classification test: According to the screening test, the possible causes of bleeding tendency are provided, and then the classification-specific test, such as the reduction of platelet count, should be further examined by bone marrow to observe the number of megakaryocytes and platelet formation. There are no abnormal cells to help identify possible causes of thrombocytopenia. Platelet function tests should be performed for suspected platelet dysfunction. Patients suspected of having hemophilia may be further tested for simple thromboplastin production (STGT), Biggs thromboplastin test and corrective test to determine the type of hemophilia; factor VIII, XI, IX coagulation activity may also be performed. (VIII: C, IX: C, XI: C). In the case of prolonged prothrombin time, the prothrombin correction test and the coagulation activity of the relevant factors should be determined to identify factors II, V, VII, X or anticoagulant substances. If the test reveals prolonged thrombin time, fibrinogen determination is performed to identify a decrease in fibrinogen or a qualitative abnormality. Diagnostic tests for disseminated intravascular coagulation include platelet count, prothrombin time and plasma fibrinogen determination, plasma protamine subcoagulation test (3P test), ethanol gel test and FDP test. The latter three tests can be used as Identification of primary fibrinolysis.

There are many laboratory tests for hemorrhagic diseases, and the test methods are easily affected by factors such as ambient temperature, reagent quality, and skill in operation, which may cause errors. Therefore, the analysis of the experimental results should be judged correctly in combination with clinical manifestations.

Fourth, other inspections

Such as bone marrow examination, liver and kidney function tests, immunological tests, genetic tests and histopathological examinations can be selected according to the needs of specific patients.

Differential diagnosis

This type of hemorrhage is fundamentally different from small and medium blood vessels due to trauma, surgery, ulcers, tumor necrosis and other injuries and varicose veins and hemangiomas. The bleeding tendency is an autoimmune disease. Anti-platelet antibodies are present in the blood of more than 60% of patients, mostly IgG, and a few are C3. Most of the disease occurs in children and young people. There are scattered or dense purpura, ecchymosis, gum bleeding and nasal discharge in the skin and mucous membranes of the limbs. In women, menstrual abnormalities increase, and the spleen is generally not enlarged. The number of bone marrow megakaryocytes is increased or normal, and the formation of platelet function is poor, and the increase of platelet-associated antibody (PAIgG) is the main basis for the diagnosis of this disease.

The disease needs to be differentiated from secondary immune thrombocytopenic purpura, which has a history of medication, blood transfusion, malignant lymphoma, and connective tissue disease. ITP is divided into acute type and chronic type, and the identification of the two is shown in Table 1. Acute type needs to be differentiated from non-immune thrombocytopenia caused by viral infection. The latter is mostly in the acute phase of infection. The thrombocytopenia recovers for about 2 weeks. The nucleus of bone marrow megakaryocytes has degenerative changes. The cytoplasm has vacuoles and megakaryocytes. The number reduction can be identified with the ITP. In a small number of chronic patients, the number of megakaryocytes in bone marrow is reduced or even absent, and attention should be paid to the identification of megakaryocyte aplastic anemia.

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