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69 Cards in this Set
- Front
- Back
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what is MCV
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size of the corpuscle ( RBC)
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what is Hgb
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hemoglobin content
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what is Hct
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RBC count X MCV
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what is MCH
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mean corpuscular hemoglobin
Hgb/RBC = # of hemoglobin per RBC |
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ref range for RBC counts (median)
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Hgb = 15.5 m, 13.5 w
Hct= 44 m, 38 w RBC= 5 m, 4 f MCH= 30 MCHC= 30 |
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wht is MCHC
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Hgb/Hct = Hgb concentration per RBC
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anisocytosis
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dif in size
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hypochromia
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dec MCH
central pallor |
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poikilocytosis
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dif in shape
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polychromatophilia
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young RBC c bluish tint
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acanthocyte
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cell with long projections
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schistocyte
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fragmented cell - irregular shape
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stomatocyte
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slit like central pallor
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basophilic stippling
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RNA aggregates- blue dots in RBC
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Heinz body
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Hgb precipitate (blue dot)
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howell- jolly body
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round, basophilic nuclear remnant
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pappenheimer body
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multiple, iron containing, granular blue dots
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rouleaux
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stack of RBCs
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siderocyte
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RBC with iron
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what is the normal myeloid to erythroid ratio
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1:1
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what are the 3 mechs of anemia
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blood loss
impaired production increased distruction |
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clinical features of anemia
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pale, weak, malaise
doe brittle nails fatty change in liver, heart and kidney headache, faint, dim vision |
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what are the major features of hemolytic anemia
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premature destruction of RBCs
accumulation of Hgb catabolism byproducts inc in erythropoesis |
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where do hemolytic anemias usually happen
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extravascular hemolysis
macs in the spleen |
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if it does happen, what are the features of intravascular hemolysis
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hemoglobinuria
janudice hemosidenuria |
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elevated bilirubin levels in the blood lead to the formation of (other than jaundice)
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gallstones
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what is the typical genetic defect in spherocytosis
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deficiency of ankyrin protein (cytoskeletal component of RBCs)
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is spherocytosis extra or intravascular
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mostly extravascular
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clinical factors for hereditary spherocytosis
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anemia, splenomegally, jaundice
parvovirally induced aplastic crisis gallstones |
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treatment for spherocytosis
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splenectomy
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what pathway is G6PD crucial for and what is its fxn
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pentose phosphate pathway
restores NADPH supplies this NADPH reduces glutathione which inhibits oxidative stress on the cell |
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what are the 2 G6PD variants that cause hemolysis
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G6PD-A
G6PD - Mediterranean |
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what are the positive effects of G6PD deficiencies
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antimalarial measure by the body
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what is the inheritance pattern of G6PD deficiencies
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X-linked
Males- all erythrocytes effected Females mono or hetrozygous for it |
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what drugs can induce anemia in people with a G6PD deficiency
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Primaquine and chloroquine
sulfas, nitrofurantoins |
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what type of veg should G6PD deficient people avoid
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fava beans
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what typy of hemolysis occures in individuals with G6PD deficiency
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intra and extravascular hemolysis
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what might you see in a RBC of someone with a G6PD deficiency
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heinz bodies- Hgb precipitates
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why do you see extravascular hemolysis in G6PD deficiency
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the heinz bodies inhibit deformability of the RBC and the splenic macks bite into them, then englulf them later
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which RBC are vulnerable to lysis from oxidant injury in a G6PD deficiency
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the old cells are far more vulnerable
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which Hgb chain is effected by sickle cell
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the B-globin chain
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what causes the defect in B-globin in a sickle cell person
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substitution of a valine for a glutamic acid at the 6th place
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what happens to the MCHC in a sickled cell
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it increases
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what Hgb molecule inhibits the polymerization of HbS and is used in sickle cell therapy
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HbF
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which Hgb molecule aggregates more readily with HbS
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HbC
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what does dehydration do to sickle cell pts
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induces sickling by increasing the MCHC
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what body sites are most affected by sickle cell
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bone marrow
spleen peripheral vascular beds |
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what types of hemolysis do you see in sickle cell
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intravascular and extravascular
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what skull effect is common in sickle cell and B-thalassemia
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the crew cut look on xray due to bone marrow hyperplasia
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what are the morphological effects of sickle cell
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anemia
bone marrow hyperplasia hyperbiliruibinemia, jaundice pigment gallstones capillary stasis and thrombosis (infarctions and ulcers) |
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what happens to the spleen of sickle cell pts?
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enlarged in children
continued thrombosis, infarction, and scarring leads to progressive shrinking autosplenectomy by adolescence/adulthood |
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clinical presentation of Sickle cell
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severe anemia
vaso-occlusive crises (very painful) chronic hyperbilirubinemia painful bone crisis in children (hard to distinguish from osteomyelitis or hand-foot and mouth synd) lung crisis CNS hypoxia (stroke, seizures) leg ulcers parvo induced aplastic crisis sequestration crisis (children c splenomegaly- hypovolumic/shock) |
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treatment of sickle cell
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hydroxiuria- inc HbF concentration, inc RBC volume, anti-inflamm, NO producer
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what is the difference btw B0 and B+ thalassemias
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B0 has no B-globin chains in the homozygous state
B+ has a reduced amount of B-globin chains in the homozygous state |
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how does impared B-globin snthesis contribte to anemia
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inadequate HbA formation makes MCHC low and cells are hypochromic
the imbalance affects the RBC's survival -free chains aggrigateand form inclusions that damage the cell membrane - apoptotic death of RBCs in the bone marrow (ineffective erythropoesis) - inclusions in the RBCs, that make it to the blood, cause them to be destroyed in the spleen |
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what happens to the bone marrow in B-thalassemia pts
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it expands and becomes hypererythropoetic
-crew cut appearance of the skull on xray |
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why is systemic iron overload a problem for B-thalassemics
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ineffective erythropoesis leads to an increase in iron absorption in the diet and repeated blood transfusions add to the iron pool.
thalassemia pts receiving blood transfusions must be on iron chelators |
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what happens to the liver and spleen in thalassemia pts
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they enlarge due to extramedullary hematopoesis and also splenomegally due to RBC destruction
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what are the effects of B-thalassemia major
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severe anemia c all of the conditions previously discussed
transfusions are required (death in childhood w/o it) HbA dec, HbF inc peripheral smear: anicytosis, microcytic hypochromic cells, target cells facial distortion and is large cardiac disease |
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ethnic predisposition for B-thalassemia
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mediterranean
African Southeast Asia |
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effects of B-thalassemia minor
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more common, heterozygotes
malarial protection asymptomatic/mild anemia hypochromia, microcytosis, and target cells in blood smear HbA2 inc, HbF norm |
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what are gamma4 tetramers called and what is it associated with?
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barts hemoglobin
a-thalassemia |
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what are B-globin tetramers called and what are they associated with
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HbH
a-thalassemia |
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what is true of a-thalassemia disease as compared to B-thalassemia
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a-thal is much less severe than B-thal
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what are the 4 diff a-Thalassemia classifications
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Silent -a/aa
trait --/aa or -a/-a HbH disease --/-a hydrops fetalis --/-- |
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clinical symptoms of silent a-thalassemia
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no anemia
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clinical symptoms of a-thalassemia
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minimal or no anemia
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clinical symptoms of HbH thalassemia
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typically effects asians
HbH has extreme O2 affinity, bad for tissue perfusion Older RBCs with HbH precipitate are removed by spleen moderately severe anemia |
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clinical symptoms of hydrops fetalis
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Barts Hbg forms
intrauterine death in the past intrauterine transfusions can save the infant |
