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
CD marker in Paroxysmal nocturnal hemoglobinuria?
CD 55/59
Tx for Paroxysmal nocturnal hemoglobinuria?
Eculizumab - complement inhibitor
Pathogenesis of PNH?
Impaired synthesis of GPI anchor for decay accelerating factor that protects RBC from complement
Why are newborns normally asymptomatic in sickle cell?
They have higher hbF and lower HbS
Why is there a crew cut skull in thalassemia major and Sickle cell?
Marrow expansion due to inc erythropoiesis
What renal issue in Sickle cell and why?
Renal papillary necrosis - due to dec pO2 in papilla
Why give hydroxyurea in Sickle cell?
Inc the amount of HbF
Sickle cell osteomyelitis is assoc with which bact?
Salmonella
What Ig is seen in Warm AIHA and Cold AIHA?
IgG and IgM
Which 3 pathologies are associated with cold agglutination AIHA?
Mycoplasma pneumoniae, Infectious Mono, CLL
Drug assoc with Warm AIHA?
methyldopa (alpha)
Microangiopathic hemolytic anemia seen in which diseases? (6)
DIC, TTP, HUS, SLE, Malig HTN, HELLP
Two diseases associated wtih macroangiopathic anemia?
Prosthetic heart valves and aortic stenosis
What is mixed with patients blood in Direct coombs test?
anti Ig antibody
What in patients serum will yield a positive result in indirect coombs test?
If serum has anti RBC surface Ig, RBC will agglutinate = pos result
What is the difference in Labs between ACD and Hemochromatosis?
Both have low TIBC and and high ferritin, but Hemochromatosis has high Fe levels and also high %transferrin sat’n therefore.
Neutropenia cut off point?
Eosinophilia cut off point?
Which drug causes neutrophilia, inspite of eosinopenia and lymphopenia?
Corticosteroids (dec activation of neutrophil adhesion molecules, dec migration out, stay in blood - However, tend to keep eosinophils in l.n and cause lymphocyte apoptosis )
What is a Left shift?
shift to a more immature cell in maturation process - i.e. inc neutrophil precursors, like band cells and metamyelocytes in periph blood. In infection and inflammation, neutrophils are rapidly used up (neutrophils now, macs later) so need to make more. Produce them so fast, end up releasing more immature cells in our hurry.
Enzymes def in lead poisoning?
ALA DH, and Ferro chelatase - “Dont eat DAH Fucking Lead!”
5 Ps of Acute intermittent porphyrias?
Painful stomach, Port wine colored urine, Polyneuropathy, Psych issues, Precipitated by drugs, etc
Tx for AIP? (2) Why?
Glucose and Heme, they inhibit ALA synthase
Enzyme def in AIP?
PBG deaminase
Which HIV drugs are CI with Acute Intermittent Porphyria?
NNRTIs - Nevirapine, Efavirenz (p450 inducer)
Def enzyme in Porphyria cutanea tarda?
UPG decarboxylase
Which is the most common Porphyria?
PCTarda
Which pathology is assoc with def in ALA synthase?
Sideroblastic anemia - S = synthase and sideroblastic
Which porphyria is assoc with photosensitivity?
PCT
