MindMap Gallery Hematological-purpuric diseases
The mind map of blood system-purpura diseases shares the content of Henoch-Schonlein purpura, primary immune thrombocytopenia, and thrombotic thrombocytopenic purpura. Let’s take a look at it together.
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This is a mind map about bacteria, and its main contents include: overview, morphology, types, structure, reproduction, distribution, application, and expansion. The summary is comprehensive and meticulous, suitable as review materials.
This is a mind map about plant asexual reproduction, and its main contents include: concept, spore reproduction, vegetative reproduction, tissue culture, and buds. The summary is comprehensive and meticulous, suitable as review materials.
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Hematological-purpuric diseases
【concept】
1) Purpura
a Vascular purpura: caused by abnormalities in the structure or function of the blood vessel wall
b Platelet purpura: caused by platelet disease
2) Clinically, skin and mucous membrane bleeding is the main manifestation
Section 1 Henoch-Schonlein Purpura
【Allergic purpura】
1) It is a common vascular allergic disease
2) Due to the body's allergic reaction to certain allergenic substances, capillary fragility and permeability increase, blood extravasation, resulting in purpura, mucous membranes and bleeding in certain organs.
3) May be accompanied by other allergic symptoms such as angioedema and urticaria
【Cause】
(1) Infection
I Bacteria
II viruses
III Others: Parasitic infection
(2) Food
It is mainly caused by allergies caused by animal proteins, such as fish, shrimp, crab, milk, etc.
(3) Drugs
1) Antibiotics
2) Antipyretic and analgesic drugs
3) Other drugs
4) Others: pollen, dust, etc.
【Pathogenesis】
1) The mechanism is unknown
2) Various stimulating factors activate the T cells of patients with genetic susceptibility, causing dysfunction, causing polyclonal activation of B cells, secreting large amounts of IgA, IgE, TNF-α, IL-6 and other inflammatory factors to form IgA immune complexes , triggering abnormal immune response, leading to systemic vasculitis, causing tissue and organ damage
3) Pathological changes are mainly systemic small vessel vasculitis
[Clinical manifestations]
I Simple allergic purpura (purpura type)
1) Most common
2) Mainly manifested as skin purpura, limited to the limbs
3) Purpura often appears repeatedly in batches, is symmetrically distributed, and varies in size. In severe cases, it can merge into bullae with hemorrhagic necrosis in the center.
4) May be accompanied by skin edema, urticaria, etc.
II Abdominal Henoch-Schonlein Purpura
1) In addition to skin purpura, the gastrointestinal mucosa and peritoneal visceral capillaries are affected
2) Symptoms include abdominal pain, diarrhea, vomiting, and blood in the stool. Abdominal pain is the most common, often paroxysmal colic.
3) In children, intussusception may be induced due to intestinal wall edema and intestinal peristalsis disorder.
III articular allergic purpura
1) In addition to skin purpura, due to blood vessel involvement in the joints
2) Symptoms such as joint swelling, pain, tenderness and dysfunction
3) Symptoms of migratory and recurrent attacks, which heal after a few days without leaving any joint deformity.
IV renal type Henoch-Schonlein purpura
1) The most serious
2) On the basis of skin purpura, hemorrhagic glomerular capillary loop inflammatory reaction
3) Symptoms include hematuria, proteinuria and cast urine, and occasionally edema, hypertension and renal failure.
V Mixed allergic purpura
Skin purpura combined with more than two of the above clinical manifestations
VI Others
A few cases may also involve the eyes, brain and meningeal blood vessels, causing optic atrophy, iritis, retinal hemorrhage and edema, as well as central nervous system symptoms and signs.
【Laboratory examination】
(1) Routine examination of blood, urine and stool
I Routine blood tests
II Routine examination of urine and stool
III Capillary fragility test: half positive
(2) Platelet function and coagulation related tests
Bleeding time (BT) can be prolonged
(3) Serological examination
【Diagnosis and Differential Diagnosis】
I Diagnostic points
1) There is often a history of low-grade fever, sore throat, general fatigue or upper respiratory tract infection 1 to 3 weeks before the onset of illness
2) Typical purpura on the skin of the limbs, which may be accompanied by abdominal pain, joint swelling and pain, and hematuria
3) Platelet count, function and coagulation-related tests are normal
4) Exclude vasculitis and purpura caused by other causes
II Differential Diagnosis
1) Hereditary telangiectasia
2) Simple purpura
3) Primary immune thrombocytopenia
4) Rheumatoid arthritis
5) Glomerulonephritis
6) Systemic lupus erythematosus
7) Surgical acute abdomen, etc.
【Prevention】
(1) Eliminate disease-causing factors
1) Prevent infection
2) Clear local lesions
3) Get rid of intestinal parasites
4) Avoid foods and drugs that may cause allergies
(2) General treatment
I General processing
Bed rest and fasting in case of gastrointestinal bleeding
II antihistamines
Promethazine hydrochloride, chlorpheniramine (chlorpheniramine)
III Drugs that improve vascular permeability
Vitamin C, troxerutin
(3) Glucocorticoids
1) Mainly used for patients with joint swelling and pain, severe abdominal pain combined with gastrointestinal bleeding, and patients with severe kidney disease such as rapidly progressive nephritis or nephrotic syndrome.
2) Inhibit antigen-antibody reactions, reduce inflammatory exudation, and improve vascular permeability
3) The general course of treatment does not exceed 30 days, but may be extended as appropriate for renal-type purpura.
(4) Symptomatic treatment
1) Abdominal pain: atropine or anisodamine
2) Joint pain: use painkillers as appropriate
3) For severe vomiting: antiemetics
4) People who vomit blood or bloody stools: Omeprazole, etc.
(5) Others
I Immunosuppressants: azathioprine, cyclosporine, etc.
II Anticoagulant Therapy: Suitable for Renal Type Patients
III Chinese medicine: mainly cooling blood, detoxifying, activating blood circulation and removing blood stasis, suitable for patients with chronic recurring attacks and kidney type
[Course and prognosis]
The course of the disease is about 2 weeks
Section 2 Primary immune thrombocytopenia
[Primary immune thrombocytopenia (ITP)]
1) It is a complex acquired autoimmune disease involving multiple mechanisms.
2) Due to the patient's immune intolerance to self-platelet antigens, excessive platelet destruction and inhibition of platelet production mediated by humoral immunity and cellular immunity occur, resulting in thrombocytopenia with or without skin and mucosal bleeding.
3) Divided into acute type and chronic type, the former is more common in children
4) Features
a Extensive bleeding of skin, mucous membranes and internal organs
b Peripheral blood thrombocytopenia
c Bone marrow: Megakaryocyte development and maturation disorder
d PLT autoantibodies appear and PLT survival time is shortened
[Cause and pathogenesis]
I Excessive destruction of platelets mediated by humoral and cellular immunity
II Abnormal quantity and quality of megakaryocytes mediated by humoral immunity and cellular immunity, and insufficient platelet production
[Clinical manifestations]
I Symptoms
1) Insidious onset
2) Often manifested by repeated bleeding of skin and mucous membranes such as petechiae, purpura, ecchymosis, and difficulty in stopping bleeding after trauma, etc.
3) Fatigue is another common clinical symptom of ITP
4) Excessive bleeding or long-term menorrhagia may cause blood loss anemia
II signs
1) Skin purpura or ecchymosis, most common on the distal ends of the limbs
2) Mucosal bleeding is more common with nose bleeding, gum bleeding or oral mucosal blood blisters.
3) Generally there is no enlargement of liver, spleen or lymph nodes
【Laboratory examination】
I Routine blood tests
1) Decreased platelet count
a Acute type usually <20×10^9/L
b Chronic type usually <50×10^9/L
2) The average platelet volume is too large
II Coagulation and platelet function tests
1) Normal coagulation function
2) Prolonged bleeding time
III bone marrow examination
Bone marrow megakaryocyte count is normal or increased
megakaryocyte maturation disorder
IV serology test
【Diagnosis and Differential Diagnosis】
(1) Key points in diagnosis
1) Check the platelet count at least twice, and there is no abnormality in the blood cell morphology.
2) Physical examination shows that the spleen is generally not enlarged
3) The number of megakaryocytes in bone marrow examination is normal or increased, and there is maturity disorder.
4) Exclude other secondary thrombocytopenias
(2) Differential diagnosis
1) Pseudothrombocytopenia
2) Secondary thrombocytopenia: aplastic anemia, hypersplenism, leukemia, etc.
(3) Classification and staging
1) Newly diagnosed ITP: refers to ITP patients within 3 months after diagnosis
2) Persistent ITP: refers to ITP patients with sustained thrombocytopenia 3 to 12 months after diagnosis.
3) Chronic ITP: refers to ITP patients whose thrombocytopenia lasts for more than 12 months
4) Severe ITP: refers to platelets <10×10∧9/L, and there are bleeding symptoms that require treatment at the time of treatment or new bleeding symptoms occur during routine treatment, and other platelet-raising drugs or drugs that increase existing treatments need to be used. dose
5) Refractory ITP
a Ineffectiveness or recurrence after splenectomy
b Still needs treatment to reduce the risk of bleeding
c Except for thrombocytopenia caused by other causes, ITP is diagnosed
【treat】
ITP is an autoimmune disease. There is currently no cure. The purpose of treatment is to increase the patient's platelet count to a safe level and reduce the mortality rate.
(1) General treatment
1) PLT<20×10^9/L, strict bed rest to avoid trauma
2) Application of hemostatic drugs and local hemostasis
a Common hemostatic drugs: hemostatic sensitive, hemostatic aromatic acid
b Local hemostasis: gauze packing for nosebleeds
(2) Observation
If the platelet count is higher than 30×10^9/L, there is no surgery or trauma, and the patient is not engaged in work or activities that increase the risk of bleeding, generally no treatment is required, and observation and follow-up are possible.
(3) First-line treatment for newly diagnosed patients
I Glucocorticoids
Generally the first choice treatment
1) Prednisone
2) High-dose dexamethasone
II Intravenous infusion of immunoglobulin (IVIg)
Indications
1) Emergency treatment of ITP
2) Patients who cannot tolerate glucocorticoid treatment
3) Preparation before splenectomy
4) Before pregnancy or childbirth
(4) Second-line treatment of ITP
For patients whose first-line treatment is ineffective or who require larger doses of glucocorticoids (>15 mg/d) for maintenance, second-line treatment can be selected.
I Medication
1) Platelet-promoting drugs
2) Anti-CD20 monoclonal antibody (rituximab)
3) Other second-line drugs
immunosuppressant
a Vinblastines: most commonly used
b Cyclosporine: Mainly used for the treatment of refractory ITP
c Others: azathioprine, cyclophosphamide, etc.
Danazol
II Splenectomy
Before splenectomy, the diagnosis of ITP must be re-evaluated
1) Indications
a Glucocorticoid therapy is ineffective after 4 to 6 weeks
b Glucocorticoid treatment is effective, but relapses after dose reduction or discontinuation, or requires maintenance at a larger dose (30 mg/d) or more
c Those who have contraindications to the use of glucocorticoids
d Those with increased Cr51 radiation index in the spleen area
2) Contraindications
a Age less than 2 years old
b pregnancy period
c Those who cannot tolerate surgery due to other diseases
(5) Emergency treatment
I Platelet transfusion
PLT<20×10^9/L
II Intravenous infusion of gamma globulin (IVIg)
III High dose methylprednisolone
IV thrombopoietic drugs
rhTPO, eltrombopag, etc.
V Recombinant human activated factor VII (rhF VIIa)
Section 3 Thrombotic Thrombocytopenic Purpura