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Blood system - aplastic anemia
Blood system - mind map of aplastic anemia, aplastic anemia (AA): It is a bone marrow hematopoietic failure disease that may be caused by different causes and mechanisms. Let’s take a look at the clinical manifestations, laboratory and other examinations, and treatments. Wait for knowledge.
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Blood system - aplastic anemia
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Blood system - aplastic anemia
【concept】
1) Aplastic anemia (AA): It is a bone marrow hematopoietic failure disease that may be caused by different causes and mechanisms.
2) AA mainly manifests as low bone marrow hematopoietic function, pancytopenia, and resulting anemia, bleeding, and infection syndrome.
3) Classification
According to the condition, blood picture, bone marrow picture and prognosis
Heavy (SAA)
Non-heavy duty (NSAA)
Intermediate
Lightweight
According to the cause
congenital/hereditary
acquired/acquired
Secondary
primary
【Epidemiology】
1) It can occur in all age groups, with a higher incidence among young people and the elderly.
2) There is no significant difference in the incidence rates between men and women
[Cause and pathogenesis]
Cause
1) Viral infection, especially hepatitis virus, parvovirus B19, etc.
2) Chemical factors, especially chloramphenicol antibiotics, sulfa drugs, etc.
3) Long-term exposure to X-rays, radium and radionuclides can affect DNA replication, inhibit cell mitosis, interfere with bone marrow cell production, and lead to a reduction in the number of hematopoietic stem cells.
Pathogenesis
1) Hematopoietic stem and progenitor cell defects (seed theory)
2) Abnormal hematopoietic microenvironment (soil theory)
3) Immunity abnormality (bug theory)
[Clinical manifestations]
(1) Severe aplastic anemia (SAA)
1) Anemia: mostly progressively worsening
2) Infection: Most patients have fever, and some patients suffer from uncontrollable high fever from onset to death, often accompanied by sepsis.
3) Bleeding: Bleeding occurs in all cases. In addition to skin and mucous membranes, there is often visceral bleeding, and even intracranial bleeding can lead to death.
(2) Non-severe aplastic anemia (NSAA)
1) Anemia: milder than SAA
2) Infection: Mild and easy to control
3) Bleeding: Mild, visceral bleeding is rare
【Laboratory examination】
I blood picture
1) Severe pancytopenia
2) The absolute value of reticulocytes decreases
3) Relative increase in lymphocytes
II bone marrow image
1) Low hematopoietic function in at least one part
2) Reduction of megakaryocytes
III Pathogenesis and other related examinations
1) Decreased CD4 cell:CD8 cell ratio
2) Increased ratio of Th1:Th2 type cells, etc.
【Diagnosis and Differential Diagnosis】
(1) Diagnosis
IAA diagnostic criteria
1) Pancytopenia, reticulocyte percentage <0.01, and increased lymphocyte ratio
2) Generally no liver or splenomegaly
3) The proliferation of multiple parts of the bone marrow is reduced (<50% of normal) or severely reduced (<25% of normal), hematopoietic cells are reduced, the proportion of non-hematopoietic cells is increased, and the bone marrow granules are empty.
4) Exclude other diseases that cause pancytopenia, such as paroxysmal nocturnal hemoglobinuria, immune-related pancytopenia, etc.
Diagnostic criteria for II AA classification
1)SAA-I/AAA
a Absolute value of reticulocytes <15 ×10^9/L
b Neutrophils <0.5 ×10^9/L
c Platelets <20 ×10^9/L
The blood picture has two out of three items
d Extensive and severe reduction in bone marrow proliferation
2)NSAA/CAA
Refers to AA that does not meet the diagnostic criteria for SAA-I type
3)SAA-II
When the condition of NSAA worsens, and the clinical, blood and bone marrow images meet the diagnostic criteria for SAA-I type
(2) Differential diagnosis: pancytopenia
I Paroxysmal nocturnal hemoglobinuria (PNH)
II Myelodysplastic Syndrome (MDS)
III Autoantibody-Mediated Pancytopenia
IV acute leukemia (AL)
V Acute hematopoietic arrest
VI Others: Reactive hemophagocytic syndrome
【treat】
(1) Supportive treatment
A protective measures
1) Prevent infection
2) Avoid bleeding
3) Eliminate exposure to various risk factors
4) Provide preventive antifungal treatment as appropriate
5) Necessary psychological care
B Symptomatic treatment
1) Correct anemia
2) Control bleeding
3) Control infection
4) Liver protection treatment
5) Iron removal treatment
6) Vaccination
(2) Treatment targeting the pathogenesis
A Immunosuppressive treatment
1) Antilymphoid/thymocyte globulin: mainly used in SAA
2) Cyclosporine: Applicable to all AA
mechanism
a Selectively blocks Th cell-dependent immune responses
b Inhibit the transcriptional activity of IL-2, IL-4, TNF-α, IFN-γ and other genes in T cell activation
c Prevent the expression of IL-2 receptor to exert its immunosuppressive effect, thereby restoring normal hematopoiesis
Adverse reactions
a Gingival hyperplasia
b hairy skin
c Both hands tremble slightly
d Gastrointestinal reactions
3) Others: CD3 monoclonal antibodies, etc.
B Treatment to promote hematopoiesis
1) Androgens: Applicable to all AA
mechanism
a Stimulates hematopoiesis by promoting the release of hematopoietic cell growth factors: EPO is produced by the kidneys and GM-CSF is produced by monocytes and macrophages
b Directly acts on hematopoietic cells to promote hematopoiesis: 5β-dihydrotestosterone
2) Hematopoietic growth factors: applicable to all AA, especially SAA
a Granulocyte-monocytic colony-stimulating factor (GM-CSF) or granulocytic colony-stimulating factor (G-CSF)
b Erythropoietin (EPO)
c Eltrombopag: platelet receptor agonist
d Thrombopoietin (TPO)
C Hematopoietic stem cell transplantation
For SAA patients under 40 years old, without infection or other complications, and with a suitable donor, allogeneic hematopoietic stem cell transplantation can be considered first
[Effectiveness Standards of AA]
I basic cure
II remission
III significant progress
IV is invalid
【prevention】
【Prognosis】