What are Common Bleeding Disorders?

Bleeding diseases are a group of diseases caused by abnormal hemostatic mechanisms. Hemorrhagic diseases can be divided into two categories: hereditary and acquired. Clinical manifestations are mainly bleeding in different parts. There are many types of hemorrhagic diseases, and the pathogenesis is different. Clinical treatment should be given according to different causes and pathogenesis.

Basic Information

English name

hemorrhagic disorder

Visiting department

surgical

Common locations

Skin, submucosa

Common causes

Congenital or hereditary, allergic purpura, drug allergic purpura, autoimmune purpura, etc., vitamin C deficiency, mechanical purpura, etc.

Common symptoms

Bleeding in different parts

Causes of bleeding disorders

Abnormal vessel wall

(1) Congenital or hereditary

Such as hereditary telangiectasia, familial simple purpura, giant cavernous hemangioma, diffuse diffuse vascular keratosis, ataxia telangiectasia and so on.

(2) Acquirability

1) Immunity such as allergic purpura, drug allergic purpura, autoimmune purpura, etc.

2) Non-immunity such as vitamin C deficiency, mechanical purpura, simple purpura, infectious purpura, corticosteroid purpura, senile purpura and orthostatic purpura.

2. Platelet abnormalities

(1) Platelet count abnormal

1) Heritability of reduced platelet production : such as Wiskott-Aldrich syndrome, Trousseau syndrome, Mediterranean platelet deficiency with giant platelets, Alport syndrome, Chediak-Higashi syndrome, Fanconi anemia, platelet deficiency with radius deficiency syndrome, etc. .

Acquired: such as aplastic anemia, tumorous bone marrow infiltration such as leukemia, physical and chemical biological factors caused by megakaryocytes and platelet inhibition (such as radiation, drug, infectious, etc.).

2) Excessive platelet consumption or destruction Immunity such as immune thrombocytopenic purpura, drug-induced immune thrombocytopenic purpura, connective tissue disease (such as systemic lupus erythematosus, etc.). Non-immunity such as diffuse intravascular coagulation, thrombotic thrombocytopenic purpura, heparin thrombocytopenia, drug-induced non-immune thrombocytopenic purpura and so on.

3) Thrombocytosis

Such as primary thrombocytosis and other myeloproliferative diseases, some patients may appear bleeding.

(2) abnormal platelet quality

1) Heritability such as platelet weakness, Bernard-Soulier syndrome, etc.

2) Acquired by antiplatelet drugs, infections, uremia, dysglobulinemia, liver disease, bone marrow proliferative diseases, etc. can cause abnormal platelet quality and cause bleeding symptoms.

3. Abnormal quantity and quality of coagulation factors

Can also be divided into two major categories of hereditary and acquired.

(1) Hereditary

Such as hemophilia A, B and hereditary , , , X, , , factor and fibrinogen deficiency.

(2) Acquirability

Such as vitamin K-dependent coagulation factor deficiency, abnormal coagulation factors caused by liver disease, acquired coagulation factor inhibitors and so on.

4. Anticoagulant and fibrinolytic abnormalities

Such as excessive use of anticoagulants or thrombolytic drugs, snake bites, rat poisoning and so on.

Clinical manifestations of bleeding disorders

The clinical manifestations of hemorrhagic diseases are mainly bleeding in different parts. When making a preliminary assessment of bleeding disorders, ask the patient's bleeding history, family history, symptoms, and carefully check the patient's bleeding signs, etc. For the diagnosis of the patient is very important when collecting the history, pay attention to the patient's gender, age at bleeding, and frequency of bleeding , Drugs, surgery, trauma history, no family history, etc.

Clinical manifestations often vary depending on the pathogenesis.

Subcutaneous mucosal bleeding

Various bleeding diseases, especially vascular and platelet diseases, the most common and most easily found symptoms and signs are bleeding from the skin and submucosa. The manifestations are as follows depending on the degree, extent, and location of the bleeding.

(1) Bleeding point refers to bleeding within 2mm in diameter on the skin, as much as the size of a needle, usually not higher than the skin surface, and does not fade when pressed. It was dark red in the early stage and was completely absorbed within 1 to 2 weeks. Bleeding points can be scattered throughout the body, more common in the extremities, and the lower part of the trunk is more common.

(2) Purpura is a subcutaneous hemorrhage with a diameter of 3 to 5mm. It is not higher than the skin surface and does not fade.

(3) The ecchymosis is a subcutaneous patchy hemorrhage with a diameter of 5mm or more, and the distribution site is the same as the bleeding point and purpura. Single and multiple small patches are generally indicated by vascular or platelet disease; large patches are commonly associated with severe thrombocytopenia or functional dysfunction and severe coagulopathy.

(4) Blood blister Oral mucosal blood blister is often the manifestation of severe thrombocytopenia.

(5) Nosebleed platelet disease, hereditary telangiectasia are common. However, under high temperature and dry climate, normal people may also experience nosebleeds. If there is bleeding from only one nasal cavity, local vascular factors are more likely than coagulopathy.

(6) Gingival bleeding is a common symptom of platelet disease and vascular disease.

2. Deep tissue bleeding

Deep tissue hemorrhage is common in deeper subcutaneous, muscle, joint cavity, and serosal cavity.

(1) Hematoma deeper subcutaneous, muscle and other soft tissue bleeding. Large hematoma can cause swelling and pain, compressing adjacent tissues and organs causing pain and dysfunction. Mild trauma or spontaneous hematomas are common in disorders of the coagulation mechanism, such as hemophilia.

(2) Joint bleeding is common in weight-bearing joints such as knee, ankle, elbow, wrist, and hip joints. Swelling and pain in the joints can be seen early, and joint puncture can extract old blood that is not easy to coagulate. Repeated joint bleeding can lead to permanent joint deformities and severe dysfunction. Joint bleeding is common in disorders of the coagulation mechanism, such as hemophilia.

(3) Serous hemorrhage is mainly seen in the abdominal cavity, pleura, pericardium, and testicular sheath. Unexplained or spontaneous serous hemorrhage is more common in disorders of the coagulation mechanism, such as hemophilia.

(4) Fundus hemorrhage is more common in patients with severe thrombocytopenia and severe vascular disease, and other hemorrhagic diseases are rare.

3. Visceral bleeding

Visceral hemorrhage can be clinically manifested as hemoptysis, vomiting blood, blood in the stool, hematuria, guided bleeding, and central nervous system bleeding, and the amount of bleeding is large. In addition to the corresponding organ and system symptoms, it can also be accompanied by symptoms such as blood circulation disorders and shock. Mainly seen in severe thrombocytopenia and coagulation factor deficiency.

Examination for bleeding disorders

Based on the patient's medical history and physical examination, to determine whether there is a hemostatic dysfunction, and to analyze possible causes, laboratory tests are important for the diagnosis of bleeding disorders.

Screening test

Including capillary fragility test, platelet count, start time, clotting time, partially activated thrombin time, prothrombin time, thrombin time, etc.

2. Confirmation test

(1) Vascular abnormalities include capillary endoscopy and vWF measurement.

(2) Platelet abnormal platelet adhesion and aggregation test.

(3) Coagulation abnormalities include antigen and activity determinations of various coagulation factors, thrombin generation, and correction tests.

(4) Anticoagulant abnormalities include antithrombin III antigen and activity or thrombin-antithrombin complex, protein C, lupus anticoagulant determination, etc.

(5) Fibrinolytic abnormalities include protamine paracoagulation test, fibrinogen degradation products, D-dimer, plasminogen measurement, etc.

3. Special inspection

For some hereditary diseases and some rare bleeding diseases, special examinations such as protein structure analysis, genetic testing and immunopathological examinations are needed to confirm the diagnosis.

Treatment of bleeding disorders

There are many types of hemorrhagic diseases, and the pathogenesis is different. Clinical treatment should be given according to different causes and pathogenesis.

1. Treatment of bleeding disorders caused by vascular factors

In addition to etiological treatment, bleeding caused by purely vascular factors is generally treated with drugs that reduce vascular fragility and permeability (such as rudin, carlosulfonate, phenothelamine, vitamin C, blood clotting tablets, and adrenal corticosteroids. According to the amount of savings, vasoconstrictor drugs (such as pituitary lutein, ephedrine, etc.) are used for treatment.

2. Treatment of bleeding disorders caused by platelet factors

(1) Drugs that promote thrombopoiesis

1) Thrombopoietin (TPO) TPO participates in the entire process of megakaryocyte proliferation, differentiation, maturation and division to form functional platelets.

2) IL-11 acts on primordial hematopoietic stem cells in bone marrow cells, causing an increase in megakaryocyte progenitor cell ploidy, promoting megakaryocyte maturation, and increasing the number of peripheral platelets.

(2) Drugs that enhance platelet function

Batroxobin may promote platelet activation and induce platelet aggregation.

(3) adrenal cortex hormones

Mainly by inhibiting the production of platelet antibodies, blocking macrophage Fc receptors, reducing the destruction of platelets with antibodies or immune complexes in the mononuclear phagocyte system, reducing the retention of platelets in the spleen, and increasing the number of peripheral platelets. It is mainly used to treat immune thrombocytopenic purpura. Prednisone is generally used.

(4) Immunosuppressive agents

Immunosuppressants such as vincristine, cyclophosphamide, azathioprine, and cyclosporine can reduce platelet antibody production by suppressing immunity.

(5) Splenectomy

Drug therapy is not effective or the platelet reduction caused by hypersplenism is significant, and splenectomy can be considered to reduce platelet destruction sites.

(6) Platelet transfusion

In principle, it should only be used for severe bleeding caused by platelet quantity or qualitative abnormalities caused by various reasons. When the platelet count is less than 20 × 109 / L, it is often accompanied by extensive and severe bleeding, such as hemoptysis, gastrointestinal bleeding, and intracranial bleeding. Prophylactic and therapeutic platelet transfusions are the most effective treatments. When the platelet count is greater than 20 × 109 / L, the bleeding is generally light, and platelet transfusion is usually not needed to avoid the production of the same anti-platelet antibody after repeated platelet transfusions, and the efficacy is reduced when platelet transfusion is needed in the future.

3. Treatment of bleeding disorders caused by coagulopathy

According to the different pathogenesis, coagulation factor deficiency diseases can be treated with vitamin K (prothrombin, F, F, FXX deficiency) and plasma and blood products (congenital coagulation factor deficiency).