Internal Medicine Simple Cases 19-36
With Complete Solutions.
anemia def - ✔✔men: Hg< 13.5
women: Hg< 12.5
over 12.5 = requirement for blood donation
Drug-mediated hemolysis can be secondary to (4) - ✔✔1 - hapten-mediated immune
rxns (i.e., penicillin);
2 - autoantibody formation (i.e., alpha methyldopa, L-dopa);
3- thrombotic microangiopathy (i.e., clopidrogel, cyclosporine);
4- oxidant injury in pts w enzyme deficiencies such as G6PD (i.e., dapsone, some
sulfonamides).
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RBCs w many diff shapes?
sizes? - ✔✔shapes = poikilocytosis
sizes = anisocytosis
alpha thalassemia - ✔✔production of alpha globulin chains = controlled by 4 genes.
Mut of all 4 -- hydrops fetalis -- not compatible w life.
Mut of 3 = excess beta chains forming tetramers (hemoglobin H).
mut of 2 = microcytosis + mild anemia (alpha thalassemia trait).
Mut of only 1 = neither anemia nor microcytosis.
beta thalassemia - ✔✔In beta thalassemia trait or beta thalassemia minor, 1 of the 2 beta
globulin chains is mut. usually asymptomatic w hypochromic, microcytic, mild anemia.
In homozygous state of beta thalassemia - accum of excess alpha globulin - highly
insoluble + result in severe hemolytic anemia. spectrum of disease severity. beta
thalassemia major require intensive transfusion support for survival, whereas beta
thalassemia intermedia have milder phenotype. In severe , secondary to hemolysis,
there is stim of EPO, bone marrow stim, and extramedullary hematopoiesis.
Clinical manifestations = "chipmunk faces" (secondary to maxillary marrow hyperplasia
+ frontal bossing), pathological fractures, hepatosplenomegaly, leg ulcers + gallstones.
8 causes of macrocytic anemia - ✔✔A. B12 deficiency
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B. Folate deficiency
C. Liver disease
D. Hydroxyurea
E. Myelodysplasia
F. Reticulocytosis
G. Hypothyroidism
H. Alcohol
best test to ID iron deficiency - ✔✔ferritin
other iron studies (serum iron, total iron binding capacity, transferrin saturation) do not
reliably distinguish between iron deficiency and anemia of chronic disease.
how do you check folate levels - 2 ways. which way to use?! - ✔✔2 tests are available to
evaluate for folic acid deficiency.
A serum folate level can be checked; however, because folate levels can change quickly
in response to dietary changes, serum folate can be low when folate stores are normal.
Similarly, serum folate levels can quickly return to normal in the malnourished patient
after eating, while overall stores remain low.
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Erythrocyte (red cell) folate is 30-fold more concentrated than serum folate, and a low
erythrocyte folate is a strong indicator of depleted folate stores
Hg electrophoresis - ✔✔used to dx thalassemia.
In pts w beta-thalassemia minor, inc in Hg A2 (alpha2lamda2); however, there are no
electrophoretic abnormalities in pts w alpha-thalassemia minor.
When a patient is anemic, you need to calculate the ______ to assess whether the bone
marrow is responding appropriately to the anemia - ✔✔reticulocyte production index
(RPI)
when B12 def is suspected... what 2 things can be checked - ✔✔serum methylmalonic
acid and homocysteine levels
*(they will both be elevated!)
However, incr methylmalonic acid can also been seen in renal insufficiency (secondary
to decreased urinary excretion of methylmalonic acid).
Of note, folic acid is also needed to convert homocysteine to methionine; therefore, pts
w folic acid deficiency will also have elevated plasma homocysteine.
hypersegmented neutrophils seen in what - ✔✔B12 def
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