Life-and-death rescue at the "Hell" Border 思维导图模板

case 1

Basic information

Gender:Male

Age:73 years old

living habits:good lifestyle habits, no smoking and drinking, regular exercise, no hypertension and heart disease

Cardinal symptom

there is a lump in the neck, which affects eating, the skin is purple-black

diagnosis,at the first hospital 

Examination

CT

Blood routine at the beginning of the disease

Blood routine for hemorrhagic diseases:1.Main manifestations: Changes in platelet count, which may be accompanied by abnormal levels of red blood cells and hemoglobin. 2.Content of changes: If the platelet count (PLT) is significantly below the normal range, it may indicate the presence of a bleeding disorder. Due to blood loss, red blood cells and hemoglobin levels will decrease, leading to anemia. Many bleeding disorders can cause an increase in monocytes, such as leukemia, primary immune thrombocytopenia.It may be accompanied by infections or inflammatory reactions, leading to changes in white blood cell counts.

Disease

the hematoma

1.Definition: Blood from blood vessels (usually capillaries) leaks out of the blood vessels due to various abnormal reasons, accumulates in the skin and under the skin, forming a hematoma.                                                            2.Categories: A diameter ≤2mm is called a petechia, a diameter of 3~5mm that does not fade upon pressure is called purpura, a diameter greater than 5 millimeters, mostly caused by trauma, is called ecchymosis, and a diameter greater than 10 millimeters, with local elevation or a sense of fluctuation, is a subcutaneous hematoma. 3. Causes of formation:① Trauma ② Coagulation dysfunction ③ Abnormal vascular wall function ④ Platelet abnormalities ⑤ Local inflammation ⑥ Tumor

Treatment

hemostatic drugs were used

Common anticoagulant mechanisms:1.Vitamin K: It participates in the process of liver synthesis of coagulation factors II, VII, IX, and X. When vitamin K is deficient, the liver can only synthesize coagulation factors II, VII, IX, and X without coagulation activity, leading to coagulation disorders, prolonged prothrombin time, and bleeding. 2.Fibrinolytic inhibitors: They competitively inhibit plasminogen activators, preventing plasminogen from converting into plasmin, thereby inhibiting the dissolution of fibrin and producing a hemostatic effect. 3.Coagulation factor preparations: (1) Prothrombin complex: This is a mixed preparation containing coagulation factors II, VII, IX, and X, obtained from the venous blood of healthy individuals. The coagulation effects of these four coagulation factors all depend on the presence of vitamin K. (2) Fibrinogen: Infusion can rapidly increase the concentration of fibrinogen in the blood, which, under the action of thrombin, is converted into fibrin, achieving the purpose of promoting blood coagulation and hemostasis. (3) Thrombin: It directly acts on fibrinogen in the blood, converting it into fibrin and exerting a hemostatic effect. In addition, it promotes mitosis of epithelial cells and accelerates wound healing.

blood transfusions were performed

Result:the symptoms worsened

case 2

Medical history

He had thrombocytopenia and megakaryocytosis, and the diagnosis was considered as immune thrombocytopenia purpura (misdiagnosis). Platelet count returned to normal after treatment

Reason: Thrombocytopenia occurred, and bone marrow aspiration showed an increase in megakaryocytes

(I) Overview and Principle of Bone Marrow Aspiration Function: Bone marrow aspiration can be used to diagnose various blood diseases, such as leukemia, aplastic anemia, myelodysplastic syndrome, etc., and to evaluate bone marrow hematopoietic function and immune status. It is of great significance for clarifying the diagnosis of blood diseases, guiding treatment, and judging prognosis. (II) Bone Marrow Aspiration Skills and Precautions 1. Puncture Site: Usually, the sternum, iliac bone, tibia, and other areas rich in bone marrow are selected for puncture, avoiding areas with bone marrow lesions or infection. 2. Body Position: Patients usually adopt a prone, supine, or lateral position to facilitate the puncture operation. The sternum puncture adopts a supine position, and the iliac bone puncture adopts a lateral or prone position. 3. Disinfection and Anesthesia: Before puncture, the puncture site should be conventionally disinfected and locally anesthetized with an anesthetic to reduce the patient's pain. At the same time, attention should be paid to the dose and injection site of the anesthetic to avoid inadequate anesthesia or overdose. 4. Puncture Skills: ① Choose a puncture needle with appropriate length, diameter, and wall thickness, commonly 16-18G bone marrow aspiration needle ② Insert the puncture needle into the bone marrow cavity, gently rotate and forcefully aspirate to obtain sufficient bone marrow fluid. When aspirating bone marrow fluid, avoid excessive force or sudden changes in the puncture direction to prevent damage to the bone marrow tissue.

Treatment with glucocorticoids, which have an immunosuppressive effect

Disease development

Multiple and large ecchymosis and hematomas throughout the body

hematuria

1. Mechanism of hematuria: （1）. Glomerular hematuria: Glomerular hematuria results from damage to the glomerular filtration barrier, causing red blood cells to leak into the urine. Causes are diverse, including inflammation, immune damage and mechanical factors. （2）. Non-glomerular hematuria: Non-glomerular hematuria originates from bleeding in other parts of the urinary tract, such as the renal pelvis, ureter, bladder and urethra. May be caused by infection, stones, tumors, etc. （3）. Systemic diseases:  Blood diseases such as hemophilia may lead to systemic bleeding due to coagulation disorders, including the urinary system  Autoantibodies produced by autoimmune diseases such as SLE attack the glomeruli, causing inflammation and bleeding                      （4）.Exercise hematuria 2."The patient's urine is all red" This condition occurred in the patient due to systemic bleeding caused by coagulation dysfunction

Mild coma 2 days later Shortness of breath, rapid heart rate Long-term failure to eat leads to electrolyte disorders

Examination

Blood routine: Hemoglobin decreased, centripetal granulocyte percentage increased, monocyte percentage decreased

Coagulation test: PT was normal, APTT was significantly prolonged, D-dimer increased, and fibrinogen increased

coagulation function test PT： It is one of the indicators of exogenous coagulation pathways. The length of PT is mainly related to the quality and quantity of fibrinogen, prothrombin, FV, FVII and FX. The patient's PT is normal, which reflects that the patient's exogenous coagulation function is normal APTT： Reflects whether the endogenous coagulation pathway is abnormal and whether anticoagulant substances are present in the blood. APTT extension: (1) Hemophilia: APTT can sensitively detect mild defects in FVIII and FIX. APTT prolongation gradually increased in mild, moderate and severe patients. (2) Von Willebrand disease (vWD): Patients with vWF deficiency reduce the stability of FVIII, resulting in prolonged APTT. Different types of vWD patients have different degrees of reduction in FVIII activity, so the degree of APTT prolongation is also different. TT： TT is a screening indicator that reflects the conversion of fibrinogen to fibrin in plasma. The prolongation of TT mainly reflects reduced fibrinogen concentration or abnormal function and the presence of related anticoagulant substances in the blood.

Platelet aggregation function test

case 3

Examination

Chest CT identified the cause of chest tightness: bleeding caused a large amount of pleural effusion

The autoimmune antibody series were all negative, and the humoral immune series showed no abnormalities

The autoimmune antibody series：1. Systemic autoimmune disease-associated autoantibodies ANA, anti-ENA antibody profile, anti-dsDNA antibody, AnuA, anti-Clq antibody, anti-phospholipid antibody profile, ANCA, AECA, RF, ACPA 2. Autoantibodies associated with autoimmune liver disease AIH and PBC-related ANA profiles, AIH-1 and AIH-2 related autoantibodies, anti-mitochondrial antibodies (AMA) 3. Central nervous system autoimmune disease-associated antibodies Anti-N-methyl-D-aspartate receptor (NMDAR) antibodies, anti-aquaporin 4 (AQP4) antibodies, autoantibodies related to other central nervous system diseases, anti-acetylcholine receptor antibodies, anti-voltage-gated potassium ion channel (VGKC) complex antibodies, anti-voltage-gated calcium ion channel receptor, anti-a-amino-3-hydroxy-5-methylisoxazole-4-propionic acid (AMPA) receptor antibodies, Anti-gamma-aminobutyric acid-B (GABAB) receptor antibody, anti-glycine receptor antibody, anti-myelin basic protein (MBP) antibody, anti-myelin oligodendrocyte glycoprotein (MOG) antibody 4. other autoantibodies Thyroid-associated autoantibodies, pancreas-associated autoantibodies, celiac disease (CD)-associated autoantibodies, reproductive associated autoantibodies                                                                                                   Humoral immunity: IgA, IgM, IgG, C3, C4..

There were no tests for vwf and factor VIII antibodies in there

Treat

Hemostasis, infusion of plasma and cryoprecipitate

Poor effect and no improvement in factor VIII activity

Possible causes of decreased coagulation factor VIII: 1. Haemophilia A 2. Cirrhosis, hepatitis 3. Infectious shock, sepsis 4. Vitamin K deficiency 5. Abnormalities of the fibrinolytic system 6. Acute promyelocytic leukaemia 7. Autoimmune diseases

Fresh frozen plasma infusion is suitable for: 1. Supplement for simple coagulation factor deficiency: Because the coagulation factors contained in FFP are very limited, only It is suitable for hemophilia patients with mild bleeding. 2. Bleeding caused by excessive oral anticoagulants: Drugs plus FFP should be used for treatment, while drugs alone require It takes longer. 3. Acquired coagulation disorders in patients with liver disease: Patients with acute liver failure need to replenish all bleeding Coagulation factor, which is the best indication for using FFP. Indications for FFP use in patients with chronic liver disease are unclear Indeed, most scholars currently believe that patients with chronic liver disease with active bleeding can be infused with FFP without active bleeding. There is no indication for the use of FFP even if there is an abnormal coagulation factor. 4. Coagulation disorders caused by large amounts of blood transfusions: This condition often mainly causes a large decrease in platelets.

Definition: Plasma cryoprecipitate is a white precipitate from fresh frozen plasma that is insoluble under low temperature conditions. It mainly contains coagulation factor VIII, fibrinogen, von Willebrand factor, etc. Indications: treatment of hemophilia and coagulation disorders, assistance to hemostasis in trauma and surgery, supportive treatment of liver disease, and exploration of treatment of autoimmune diseases.

Recombinant human activated factor VIII

APTT still not restored，PT shortening

PT shortening Hypercoagulable states such as early DIC, myocardial infarction, cerebral infarction, deep vein thrombosis, multiple myeloma, etc., but with poor sensitivity and specificity. Normal PT, prolonged APTT Bleeding disorders caused by defects in endogenous coagulation pathways. PT prolonged, APTT normal Bleeding disorders caused by defects in exogenous coagulation pathways.

plasma exchange

treatment effect is good

Plasma Replacement: A treatment that helps remove disease-causing substances from the body and purifies the blood. Removes harmful substances from the plasma, such as autoantibodies, immune complexes, and toxins.

final estimated

Acquired Hemophilia A（AHA）

An acquired bleeding disorder in which the activity of FⅧ (FⅧ:C) is reduced due to the presence of autoantibodies to anticoagulant factor VIII (FⅧ) in the circulating blood. Characteristics: no previous history of bleeding, no positive family history, spontaneous bleeding or abnormal bleeding during surgery, trauma, or invasive tests, isolated prolonged APTT Pathogenesis: immune dysregulation, inflammatory activity associated with autoimmune diseases; altered immune status of the body due to pregnancy; malignancy; drugs and surgery. Treatment: active management of the primary disease, removal of the causative agent and treatment of the underlying disease; Haemostatic therapy; inhibitor removal

Prognosis: Based on the GTH study, the 2020 International Recommendation for AHA Diagnosis and Treatment divides the IST efficacy of AHA patients into good group and poor group. The good group is F VIII: C≥1% and inhibitor titer ≤ 20BU/ml; the poor group is F VIII: C<1% or inhibitor titer> 20BU/ml. The first-line plan for the good group recommends taking glucocorticoid alone, and the first-line plan for the poor group recommends taking glucocorticoid in combination with cyclophosphamide or rituximab. If there is no response within 3 to 4 weeks, a second-line regimen will be given. When recommended conditions permit, an IST plan (level 2B) will be formulated based on prognosis stratification and patient physical status. For patients in both groups who fail to meet the partial response criteria under the first-line regimen, but continue to improve FVIII: C and inhibitor titers, it is recommended to appropriately extend the current treatment regimen to 4 to 6 weeks (level 2B).                                            Judgment of inhibitor clearance efficacy: The recommended criteria in this guideline are as follows: complete remission: inhibitor titer <0.6 BU/ml, F VIII: C≥50%, immunosuppressants are discontinued or restored to pre-onset doses. Partial response: F VIII: C≥50%, inhibitor titer ≥0.6 BU/ml, and no new bleeding 24 hours after the end of hemostatic treatment. Ineffective: F VIII: C<50%, inhibitor titer ≥0.6 BU/ml, with or without active bleeding. Recurrence: During follow-up, patients with complete or partial remission experienced FVIII: C<50% and inhibitor titer ≥0.6 BU/ml. Treatment process monitoring: It is recommended to test FVIII: C, inhibitor titer, blood routine, etc. once a week during IST to evaluate the efficacy and evaluate possible complications of IST. Monitoring of other concomitant diseases depends on the needs of clinical evaluation, minimizing tests that may cause bleeding.

Life-and-death rescue at the &quot;Hell&quot; Border

Life-and-death rescue at the "Hell" Border