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“The Missed Chain”, image, image, image, image, image - Coggle Diagram

- - - - This leads to unregulated MYC expression resulting in uncontrolled divisions
  - - - This results in over-expression of BCL2 which prevents the cell from dying
- - - - ie. breakdown of aged RBCs
  - - - types
        
        Alveolar
        
        lungs
        
        Reticular
        
        spleen
        
        Mesangial
        
        kidney
        
        Kupffur
        
        liver
- - - - Peritoneum
    - - Ant.: Stomach, Pancreas
      - Post. Diaphragm (Ribs)
      - Inf. : Left colic flexure
  - - - Hypertrophy
      - Infection
      - Lymphoma causing lymphadenopathy
    - - Splenectomy
        
        Complications include
        
        Less IgG/M ; Less splenic B cells
        
        Increased RBC abnormalities
        
        Increased Thrombocytosis
- - - - Haptoglobin-hemoglobin complex formation:
        
        The liver produces a carrier protein called haptoglobin, which has the capacity to bind free hemoglobin
        
        The haptoglobin-hemoglobin complex is then recognized by a receptor on macrophages (CD163) and internalized for degradation
      - Heme-hemopexin complex formation
        
        Hemopexin: a glycoprotein with high affinity for heme.
        
        hemopexin binds to met-heme-> recognized by LDL receptor (D91) o macrophage for endocytosis
        
        If hemopexin is saturated, excess met-heme bind to Albumin : methemalbumin (Met-heme-Albumin complex)
        
        causes
        
        brown discoloration of plasma
- - - - in thalassemia
        
        Microcytic cells (low MCV)
        
        Hypochromic cells.
        
        (anisocytosis and poikilocytosis)
        
        Increased percentage of reticulocytes.
        
        Target cells.
        
        Heinz bodies.
  - - - a test that measures the different types of hemoglobin in the blood
    - - the principles of gel electrophoresis to separate the various types of hemoglobin
- - - - is
        
        • Genetic disorder that is caused by a decrease in the production of the globin chains needed for hemoglobin
      - Divided into
        
        α-Thalassemia
        
        Includes
        
        One Gene Deleted
        
        Three Genes Deleted : HbH Disease
        
        Two Genes Deleted : Trans and cis
        
        Four Genes Deleted :Hb Bart’s hydrops fetalis.
        
        is
        
        Deletions in the genes that encodes for alpha chains
        
        β - Thalassemia
        
        is
        
        mutations in the genes that encodes for Beta Chain
        
        Includes
        
        ẞ thalassemia minor
        
        ẞ thalassemia major
        
        ẞ thalassemia intermediate
- - - - Hemoglobinopathies
        
        Sickle Cell Disease
        
        HbC (glutamic acid replaced by lysine)
        
        Thalassemia
        
        Prognosis
        
        for alpha thalassemia
        
        Silent carriers +Thalassemia trait -- very good prognosis
        
        HgH disease (loss of 3 genes) -- survive until adulthood with lifelong blood transfusions
        
        Untreated alpha thalassemia major (loss of all genes) -- intrauterine death with hydrops fetalis
        
        for beta thalassemia
        
        Untreated beta thalassemia major -- fatal by age of 5 years in 85% of patients -- Due to infections
        
        Treated beta thalassemia major -- excellent prognosis if iron chelators start early, can live to 60s
        
        Beta thalassemia minor has excellent prognosis
      - Enzymopathies
        
        Pyruvate Kinase Deficiency
        (Extravascular)
        
        Glucose-6-Phosphate Dehydrogenase
        deficiency
      - Membranopathies
        
        Hereditary spherocytosis
        
        Hereditary elliptocytosis
    - - Microangiopathic Hemolytic anemia
        (Intravascular)
      - RBC Infections
        (eg. Babesia & malaria)
      - Autoimmune Hemolytic anemia
        (extravascular)
        
        warm
        
        cold
- - - - Chelation therapy.
        
        Three medicines are used for iron chelation therapy:
        
        Deferasirox
        
        Deferiprone
        
        Deferoxamine
      - Stem cell transplant.
        
        Replaces blood-forming stem cells that aren’t working properly with healthy donor cells.
        
        A stem cell transplant is the only treatment that can cure thalassemia
      - Frequent blood transfusions.
        
        Occasional blood transfusions
        
        may be needed for people who have hemoglobin H disease or beta thalassemia intermedia.
        
        Regular blood transfusions
        
        (every 3 to 4 weeks) may be needed for people who have beta thalassemia major.
      - Folic acid
        
        Folic acid can help red blood cells develop. Treatment with folic acid is usually done in addition to other therapies.