First Aid 392-396 Heme/Onco Flashcards
Which heme disorder is assoc with hypersegmented neutrophils?
Megaloblastic anemia
Which three drugs are associated with folate deficiency?
Methotrexate, Trimethoprim, Phenytoin
B12 def is associated with inc in what metabolic intermediate?
L-mm CoA, aka methylmalonic acid
Where do you see subacute combined degeneration of the spino cerebellar tract?
B12 def - along with lat corticospinal tract, dorsal column dysfunction
How do you differentiate Orotic aciduria from Ornithine transcarbamylase def?
There is no hyperammonemia with Orotic aciduria
Most common causes of macrocytic anemia that is not megaloblastic?
Alcoholism, lIver disease, hypothyroidism, reticulocytosis
Defective enzyme in Orotic aciduria? Substrate?
Orotic Acid –> UMP, done by UMP synthase
Short stature, thumb and radial defects?
Diamond Blackfan anemia and Fanconi anemia
Two heme diseases with inc HbF?
B thalassemia major, Diamond blackfan anemia
dec haptoglobin, inc LDH, target cells seen in ?
intravascular hemolysis
Urine test shows Hgb, hemosiderin, and urobilinogen?
Intravacular hemolysis
Inc risk of gallstones and BM hyperplasia with what type of hematological disordeR?
extravascular hemolysis
Iron, TIBC, ferritin - inc or dec in ACD (anemia of chronic disease)?
Dec FE, TIBC (indicator of transferrin) Inc Ferritin
What differentiates Diamond Blackfan from Fanconi Anemia?
Fanconi has cafe au lait spots
Is retic # inc or dec with aplastic anemia?
Dec
Most common congenital cause of Aplastic anemia?
Fanconi anemia
MCV and MCHC in heriditary spherocytosis
?
Inc MCHC (bc no central pallor) and normal to low MCV
Dec ATP production is a part of the pathogenesis of what heme disorder?
Pyruvate kinase deficiency - dec ATP will lead to rigid RBCs –> extravasc hemolysis
What amino acid mutation in HbC disease?
glutamic acid to lysine in B globin chain