MindMap Gallery Blood system-hemolytic anemia
Blood system - Hemolytic anemia is an anemia caused by an increase in the destruction rate of red blood cells (shortened lifespan) and exceeding the compensatory capacity of bone marrow hematopoiesis. Let's take a look at the clinical manifestations, laboratory and other examinations, treatments and other knowledge.
Edited at 2023-04-13 19:53:24El cáncer de pulmón es un tumor maligno que se origina en la mucosa bronquial o las glándulas de los pulmones. Es uno de los tumores malignos con mayor morbilidad y mortalidad y mayor amenaza para la salud y la vida humana.
La diabetes es una enfermedad crónica con hiperglucemia como signo principal. Es causada principalmente por una disminución en la secreción de insulina causada por una disfunción de las células de los islotes pancreáticos, o porque el cuerpo es insensible a la acción de la insulina (es decir, resistencia a la insulina), o ambas cosas. la glucosa en la sangre es ineficaz para ser utilizada y almacenada.
El sistema digestivo es uno de los nueve sistemas principales del cuerpo humano y es el principal responsable de la ingesta, digestión, absorción y excreción de los alimentos. Consta de dos partes principales: el tracto digestivo y las glándulas digestivas.
El cáncer de pulmón es un tumor maligno que se origina en la mucosa bronquial o las glándulas de los pulmones. Es uno de los tumores malignos con mayor morbilidad y mortalidad y mayor amenaza para la salud y la vida humana.
La diabetes es una enfermedad crónica con hiperglucemia como signo principal. Es causada principalmente por una disminución en la secreción de insulina causada por una disfunción de las células de los islotes pancreáticos, o porque el cuerpo es insensible a la acción de la insulina (es decir, resistencia a la insulina), o ambas cosas. la glucosa en la sangre es ineficaz para ser utilizada y almacenada.
El sistema digestivo es uno de los nueve sistemas principales del cuerpo humano y es el principal responsable de la ingesta, digestión, absorción y excreción de los alimentos. Consta de dos partes principales: el tracto digestivo y las glándulas digestivas.
Blood system-hemolytic anemia
【definition】
Hemolytic anemia (HA)
The normal life span of red blood cells is about 120 days. Anemia only occurs when the life span of red blood cells is shortened to 15-20 days.
Anemia occurs due to the increased destruction rate of red blood cells (shortened life span) and exceeding the compensatory capacity of bone marrow hematopoiesis.
hemolytic state
If the red blood cell destruction rate is within the compensatory range of the bone marrow, hemolysis will occur but anemia will not occur.
hemolytic jaundice
The presence or absence of jaundice depends on the degree of hemolysis and the liver's ability to process bilirubin.
Hemolytic anemia does not necessarily mean jaundice
[Hemoglobin metabolism process]
[Clinical classification of HA]
I According to onset and condition
1) Acute hemolysis
2) Chronic hemolysis
See clinical manifestations
II Press the hemolyzed area
1) Intravascular hemolysis
2) Extravascular hemolysis
3) In situ hemolysis: young red blood cells are destroyed in the bone marrow
See pathogenesis
III According to the cause
(1) HA caused by abnormalities in red blood cells themselves
I Abnormalities in red blood cell membranes
1) Hereditary red blood cell membrane abnormalities
2) Acquired blood cell membrane glycophosphatidylinositol (GPI) anchor chain membrane protein abnormalities: such as PNH
II Hereditary red blood cell enzyme deficiency
1) Pentose phosphate pathway enzyme defects: such as glucose-6-phosphate dehydrogenase (G-6-PD) deficiency
2) Anaerobic glycolysis pathway enzyme defects: such as pyruvate kinase deficiency
III Inherited disorders of globin production
1) Abnormal globin peptide chain structure: such as dyshemoglobinopathies
2) Abnormal number of globin peptide chains: such as globin production disorder anemia (thalassemia)
(2) HA caused by external factors of red blood cells
I immune HA
1) Autoimmune HA
2) Alloimmune HA: blood group incompatibility transfusion reaction
II Vascular HA
1) Microangiopathic HA
2) Valvular disease
3) The blood vessel wall is repeatedly squeezed
III biological factors
Snake venom, malaria, kala-azar, etc.
IV physical and chemical factors
Extensive burns, changes in plasma osmotic pressure, and poisoning by chemical factors such as phenylhydrazine and nitrites can cause hemolysis due to acquired methemoglobinemia.
【Pathogenesis】
(1) Increased destruction of red blood cells
I Intravascular hemolysis
1) Definition
Refers to the destruction of red blood cells in blood circulation and the release of free hemoglobin to form hemoglobinemia.
2) Seen in blood type incompatibility transfusion, PNH, mechanical hemolytic anemia
3) Features
a Severe destruction of red blood cells
b damage in blood vessels
c More common in acquired red blood cell damage
4) Classification
Rapid intravascular hemolysis: The amount of hemoglobin exceeds the reabsorption capacity of the proximal convoluted tubule, resulting in hemoglobinuria
Slow intravascular hemolysis: Iron is deposited in the renal tubular epithelial cells in the form of ferritin or hemosiderin, and is excreted in the urine as the epithelial cells shed, forming hemosiderinuria.
II extravascular hemolysis
1) Definition
Refers to the phagocytosis and digestion of red blood cells by the monocyte-macrophage system such as the spleen, and the released hemoglobin is decomposed into globin and heme, which is further decomposed into bilirubin.
2) Seen in autoimmune hemolytic anemia, globin production disorder anemia, etc.
3) Features
a Abnormal red blood cells (mainly intrinsic defects)
b Red blood cells are destroyed by macrophages in the liver, spleen, and bone marrow.
c More common in congenital red blood cell defects
III in situ hemolysis
1) The red blood cells in the bone marrow have been destroyed in the bone marrow before being released into the blood circulation, which may be accompanied by jaundice.
2) Its essence is extravascular hemolysis
3) Common in megaloblastic anemia, etc.
(2) Compensatory hyperplasia of the erythroid system
[Clinical manifestations]
I Acute HA
Mostly intravascular hemolysis, onset is sudden
1) Headache, vomiting, high fever
2) Soreness in lower back, limbs and abdominal pain
3) Soy sauce color urine
4) Pale complexion and jaundice
5) Severe cases include peripheral circulatory failure, oliguria, and anuria
II chronic HA
Mostly extravascular hemolysis, with slow onset and mild symptoms.
1) Characteristics: anemia, jaundice, splenomegaly
2) Can be complicated by cholelithiasis and liver function damage
3) Extramedullary hematopoiesis can cause liver and splenomegaly
4) Expansion of the bone marrow cavity, thinning of the bone cortex, and bone deformation
【Laboratory examination】
(1) Examination for increased red blood cell destruction
I Intravascular hemolysis
1) Urine: Positive urine occult blood (hemoglobinuria), positive urine protein, negative red blood cells
2) Serum free hemoglobin intravascular hemolysis >40mg/L
3) Serum haptoglobin is significantly reduced or disappears
4) Positive Rous test (hemosiderinuria)
II extravascular hemolysis
1) Urine: increased urobilinogen, strongly positive, bilirubin negative
2) Serum bilirubin: Mainly increased serum free bilirubin (indirect bilirubin)
3) Increased fecal bilegenin
(2) Examination of compensatory hyperplasia of erythroid system
I Reticulocyte count
increase height
II bone marrow
1) Obvious proliferation of red blood cells
2) Hao-peribodies and Kapos rings may appear
III peripheral blood
nucleated red blood cells
(3) Examination of red blood cell defects and external abnormalities
I Red blood cell survival time is shortened
It is the most direct and conclusive evidence
II lactate dehydrogenase
increase
【Diagnosis and Differential Diagnosis】
(1) Diagnosis
Diagnostic steps
1) First determine whether it is hemolytic anemia
2) Determine the site of hemolysis
3) Determine the cause and type of hemolysis
4) Select relevant tests to exclude or confirm one by one
I Medical history
II Clinical Manifestations
III One of the following situations exists
1) Patients with both excessive destruction of red blood cells and compensatory proliferation of immature red blood cells
2) Those with hemoglobinuria or other signs of intravascular hemolysis
(2) Differential diagnosis
1) Anemia with reticulocytosis: such as early recovery from hemorrhagic, iron deficiency or megaloblastic anemia
2) Non-bilirubinuric jaundice: such as familial non-hemolytic jaundice
3) Myelocytic anemia with mild reticulocytosis: such as bone marrow metastasis
【treat】
I Cause treatment
1) Discontinue medication
2) Control infection
II Symptomatic treatment
1) Adrenocortical hormone
Indications: immune hemolytic anemia, incompatible post-transfusion hemolysis, PNH
2) Splenectomy
Indications: Hereditary spherocytosis, refractory autoimmune hemolytic anemia, etc.
3) Immunosuppressants: cyclosporine A, cyclophosphamide, etc.
4) Blood transfusion: Autoimmune hemolytic anemia and PNH require transfusion of washed red blood cells.
5) Other treatments: prevention and treatment of acute renal failure, shock and electrolyte imbalance in acute hemolysis
[Autoimmune hemolytic anemia (AIHA)]
[Paroxysmal nocturnal hemoglobinuria (PNH)]