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Hematology (Myelodysplastic Syndromes (Polycythemia Vera (mutation (JAK 2)…

- - - - Allogenic HCT, or hypomethylating chemotherapy (azacytidine, decitabine)
  - - - Granulocytic leukocytosis (>50,00is present on CBC, and earlier myeloid forms such as metamyelocytes, myelocytes, and less than 5% blasts are present on peripheral blood smear review, release of early progenitor cells (blasts) and basophils!
    - - translocation of 9;22 - BCR-Abl- philledelphia chromosome
      - first line therapy
        
        TKI (imintinab, nilotinib, or dasatinib
        
        must watch out for which side effects
        
        prolongation of QT interval and fluid retention
  - - - JAK 2
    - - hemoglobin level greater than 18.5 g/dL (185 g/L) in men or greater than 16.5 g/dL (165 g/L) in women after secondary causes are excluded.
    - - disorders mediated by hypoxia, ectopic EPO production (RCC, hepatocellcular), people living at high altitudes
    - - pruritus after a warm bath or shower; intermittent heat, redness, and pain of the palms and soles (erythromelalgia); hepatosplenomegaly; a concomitant elevation of the leukocyte and platelet counts; and a propensity for unusual thromboses.
    - - portal/splanchnic/Budd chiari
    - - Low dose ASA
        Phelbotomy (goal Hct < 45%, hydroxyurea
  - - - plt count greater than 600,000 1 month apart, excluding 2/2 causes
    - - IDA, infection, inflammation
    - - digital ischemia, erythromelalgia, transient ischemic attack, visual disturbances, venous thromboembolism, or bleeding (due to dysfunctional platelets) may be presen
    - - can be observed, but high risk, with older age, previous thrombosis, and low leukocyte count, get ASA and hydroxyurea
    - - 1 mil
- - - - pancytopenia, with associated neutropenia, anemia, and thrombocytopenia, and a severely hypocellular bone marrow (<10%).
    - - sulfa-antibiotics, antieplepic drugs, psychotropic drugs, NSAIDS
    - - autoreactive T cells attack pleuripotent HSC
      - treatment
        
        cyclosporine, antithymocyte globulin
      - how common?
        
        most common reason in adults
    - - 10% risk in 10 years
  - - - erythrocytes lack membrane proteins CD55 and CD59 required to stabilize complement, leading to episodic intravascular hemolysis
    - - PIG-A gene, GPI anchor
    - - Eculizumab, a monoclonal antibody that binds C5 and inhibits activation of the terminal component of the complement cascade
  - - - predominantly T cell autoimmunity (pregnancy, thymoma, malignancy) or direct toxicity to erythrocyte precursors (viral, medications).
    - - large granular lymphocytosis
        
        diagnosed by
        
        bone marrow with lymphocyte CD 57 positive
    - - Prednisone, either administered alone or with cyclosporine or cyclophosphamide
  - - - Acute HIV, cytomegalovirus, and Epstein-Barr virus infection
    - - NSAIDs, carbamazepine, phenytoin, propylthiouracil, cephalosporins, trimethoprim-sulfamethoxazole, and psychotropic drugs
    - - GM-CSF
    - - seen with SLE or RA (Felty)
    - - <1500 ANC
    - - RA, splenomegaly, and neutroenia
- - - - blood tests
        
        microcyctic anemia
        ferritin low (<12)
        serum fe low
        TIBC high
        % transferrin sat - less than 15%
      - blood tests compared to anemia of chronic disease
        
        look at the ferritin - above 100 in ACD and low TIBC
    - - mechanism
        
        low EPO production when GFR gets less than 30
        
        what do you measure
        
        nothing, EPO measurement doesn't help
  - - - B12 def - increased methylmalonic acid and homocysteine levels
      - blood cells
        
        hypersegmented (> 6 lobes)
      - indefinite
        
        if between 200-300, you have to follow up methyl/homocysteine, if less than 200, you are good
      - labs for pernicious
        
        anti-parietal cell/ anti-intrisinc factor
      - what needs to be ruled out before treating folate def
        
        Cobalamin deficiency should be excluded before initiating therapy, because large doses of folic acid may lead to improved hematopoiesis despite cobalamin deficiency, thus leaving patients vulnerable to central and peripheral nervous system injury from vitamin B12 deficiency.
  - - - mechanism
        
        Mutations causing deficiencies or dysfunction in five erythrocyte membrane proteins (α-spectrin, β-spectrin, ankyrin, band 3, and protein 4.2) have been identified
        
        test
        
        the proteins reduce surface to volume ratio, making them osmotically fragile - osmotic fragility test along with increased MCHC or eosin 5 -maleimide
      - inheritance
        
        AD
    - - Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis
    - - mechanism
        
        enzyme G6PD, the first enzyme in the pentose phosphate pathway, a crucial step in nicotinamide adenine dinucleotide phosphate reduction (NADP to NADPH), which is essential for cells to counterbalance oxidative stress
      - inheritance
        
        X-linked
      - known triggers
        
        sulfonamides, some antimalarials, rasburicase), naphthalene in mothballs, fava beans, and infection
      - characteristic cells
        
        Heiz bodies, bite cells (denatured oxidized hemoglobin)
    - - Warm
        
        type of antibody
        
        IgG
        
        mechanism
        
        usually secondary to some other autoimmune process
        
        treatment
        
        blood transfusion only if absolutely necessary, glucocorticoids
      - Cold
        
        antibody
        
        IgM
        
        characteristic precipitants
        
        Mycoplasma pneumoniae or Epstein-Barr virus.
      - hallmark
        
        positive direct antiglobulin Coombs
    - - shear damage to erythrocytes as the result of endothelial cell activation or endothelial cell damage. The hallmark of disease is the presence of schistocytes on the peripheral blood smear, typically accompanied by an elevated LDH level and other clues of intravascular hemolysis
    - - heart valves (especially mechanical heart valves), intracardiac tumors, or rupture of either chordae tendineae or an aneurysm of the sinus of Valsalv
    - - repetitive trauma to the soles of the feet or palms of the hands, positive Prussian blue stain for urine hemosiderin
    - - Babesia microti
        
        Clostridum perfinges
        
        bartonella bacilliformis