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Hematology-Pathology

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drnieves's version from 2017-06-21 13:46

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Paroxysmal nocturnal hemoglobinuriaIncreased complement mediated RBC lysis.
Impaired synthesis of GPI anchor for decay accelerating factor that protects RBC membrane from complementParoxysmal nocturnal hemoglobinuria
Paroxysmal nocturnal hemoglobinuria causesAcquired mutation in hematopoietic stem cells, increased incidence of acute leukemias.
Coombs - hemolytic anemia, pancytopenia, venous thrombosis. CD55/59Paroxysmal nocturnal hemoglobinuria
PNH txEculizumab (terminal complement inhibitor)
Sickle cell anaemiaHbS pont mutation causes a single aa replacement in B chain.
Glutamic acid for valineSickle cell
Sickle cell pathlow O2, high altitude or acidosis precipitate sickling (deoxygenated sickle cell polymerizes).
Heterozygotes have malaria RSicke cell.
Sickle cell complicationAplastic crisis, autosplenectomy (Howell jolly bodies), splenic sequestration, salmonella osteomyelitis, painful crises: dactylics, acute chest syndrome, avascular necrosis,s stroke, renal papillary necrosis.
Hemoglobin electrophoresisSickle cell dx
Sickle cell txhdyroxyurea (increase HbF), hydration
Polycythemia veraIncreased Hct. Ass with JAK 2 mutation. 2ry due to increased EPO.
Non R tyrosine kinase mutationpolycythemia vera
Polycythemia vera sxShower itching (basophils), erythromelalgia, red blue coloration due to episodic blood clots in vessels.
Increased marrow sensitivity to GFPolycythemia vera
AML M3t (15;17). Contains all trans retinoid acid.
Microvascular hemolytic anemiadecreased haptoglobin and increased bilirubin and LDH.
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