Lecture 28 - Normocytic Anemia/Intrinsic Hemolysis Flashcards
For Normocytic anemias, you can determine if the bone marrow (BM) is responding by measuring the ______ count. If it is elevated, the BM is responding, and the anemia is due to blood loss (keep in mind blood loss could result from acute hemorrhage or ______, the later of which is broken down into Intrinsic or Extrinsic.) If the count is depressed, the BM is failing to respond, which typically indicates _____ anemia. Just keep in mind BM failure anemia may also be Macrocytic.
Reticulocyte count
Hemolysis
Aplastic Anemia
Intrinsic causes (problem with the RBCs, themselves) of hemolytic anemia are usually _____, while Extrinsic causes (problem outside of RBCs) are all ______.
Inherited
Acquired
What are the characteristic cells/shapes that help distinguish Extravascular hemolysis (mediated by macrophages) and Intravascular Hemolysis? Remember that these are NOT the same as Extrinsic and Intrinsic Hemolysis!!!
Extravascular –> Spherocytes with decreased central pallor (from incomplete phagocytosis)
Intravascular –> Schistocyte (RBC fragment)
______ is the term that describes increased Reticulocytes in peripheral blood (PB).
Polychromasia
With intravascular Hemolysis, if haptoglobin and hemopexin are depleted, ______ may be observed as Hb in filtrate exceeds amount that can be reabsorbed.
This and what other two key features help distinguish between intra and extravascular Hemolysis?
Hemoglobinuria
Hemoglobinuria along with increased Free Plasma Hb and Urine hemosiderin.
Hereditary Spherocytosis is an example of a _____ (vertical or horizontal?) defect leading to Intrinsic Hemolytic anemia. The defect disrupts the linkage between transmembrane proteins and ________ proteins –> leads to spherocyte formation.
Vertical
Cytoskeletal proteins
Hereditary Elliptocytosis is an example of a _____ (vertical or horizontal?) defect leading to Intrinsic Hemolytic anemia. The defect disrupts linkage between ONLY the Cytoskeletal proteins –> leads to fragmentation and elliptocyte formation.
Horizontal defect
What is a characteristic feature in PB of ALL hemolytic anemias?
Increased Retics (aka Polychromasia)
_____ ______ Hemoglobinuria is the ONLY acquired membrane defect causing chronic INTRAvascular hemolysis. Hematopoietic stem cells acquire a mutation in the _____ gene on the X-Chromosome. This affects ALL blood cells, including platelets. RBCs become susceptible to lysis by Complement with the loss of CD____ and CD____, which are GPI-anchored proteins that normally protect against complement. Be on the lookout for a triad of symptoms including Hemolytic anemia, Pancytopenia, and ____ thrombosis to identify this disease.
Paroxysmal Nocturnal Hemoglobinuria
PIGA gene
CD55 and CD59
Venous
______ is an Anti-C5 monoclonal Ab used to treat Paroxysmal Nocturnal Hemoglobinuria, as it blocks the Complement C5 molecule.
Eculizumab
What is the most common RBC enzyme deficiency worldwide, predominantly found in individuals of Mediterranean or African descent? Keep in mind it’s X-linked, and can lead to hemolytic anemia under oxidant stress (e.g. bc of drugs - “PAIINS”, infections, or consumption of ____ beans).
Which drug does the P represent and what is it used to treat?
G6PD (G6P dehydrogenase)
Fava beans
Primaquine –> used to treat Malaria. These patients should be screened for G6PD deficiency before treatment.
_____ bodies (denatured Hb) and Bite cells are characteristic features of G6PD deficiency. The former result from oxidant stress leading to oxidation of Hb (so iron is +3).
Heinz Bodies
Pyruvate Kinase deficiency can lead to hemolytic anemia and is distinguished by presence of ____ cells (bumpy edges around the RBC) and _____ depletion which causes rigidity of the RBCs.
Burr cells
ATP depletion
