Heme Flashcards
preferred anticoagulant for immediate anticoagulation
heparin
long-term anticoagulation
warfarin
what allows RBC to change shape in vessels
spectrin
lead poisoning
basophilic stippling
G6PD deficiency
Heinz bodies and bite cells
DIC
schistocytes
abetalipoproteinemia
acanthocytes (spur cells)
asplenia
howell-jolly bodies and target cells
liver disease
acanthocytes and target cells
bone marrow infiltration (myelofibrosis)
teardrop cell
Target cells
THAL
Thallasemia, HbC disease, Asplenia, Liver disease
absence of HGPRTase
Lesch-Nyhan
deficiency of aldolase B
fructose intolerance
deficiency of cystathionine synthase
homocystineuria
galactose-1-phophate uridyl transferase deficiency -> intellectual disability, HSM, cataracts
galactosemia
deficiency of tyrosinase
albinism
location of fetal erythropoiesis
Young livers synthesize blood yolk sac liver spleen bone marrow
different causes of polycythemia
increased EPO (tumors)
chronic hypoxia
polycythemia vera
trisomy 21
lead poisoning
hematologic: microcytic anemia, basophilic stippling, ringed sideroblasts
non-hematologic: encephalopathy, memory loss, HA, foot/wrist drop, lead lines, abdominal colick, renal failure
lead poisoning affects which enzymes
ferrochelatase and ALA dehydratase
leads to accumulation of protoporphyrin and d-ALA
acute intermittent porphyria
inhibits porphobilinogen deaminase (uroporphyrinogen-1-synthase)
accumulates porphobilinogen, d-ALA, and uroporphyrin
5 P’s:
painful abdomen, port-wine urine, polyneuropathy, psychological disturbances, precipitated by drugs
treatment of acute intermittent porphyria
glucose and heme (inhibits ALA synthase)
porphyria cutanea tarda
uroporphyrinogen decarboxylase
accumulates uroporphyrin
blistering cutaneous photosensitivity, hypertrichosis, facial hyperpigmentation, Hep C and alcoholism
RLS of Heme synthesis
d-ALA synthase (d-aminolevulinic acid)
diagnose beta-thalassemia minor
HbA2 on electrophoresis
lab findings of iron def:
decreased iron and ferritin, increased TIBC
what should be ruled out in 50 yo pt with new-onset iron deficiency anemia
colon cancer
microcytic anemia + swallowing difficulty + glossitis
Plummer-Vinson syndrome
microcytic anemia + >3.5% HbA2
B-thalassemia minor
megaloblastic anemia not correctable by B12 or folate
orotic aciduria
megaloblastic anemia along with peripheral neuropathy
B12 deficiency
microcytic anemia + basophilic stippling
lead poisoning
microcytic anemia reversible with B6
sideroblastic anemia
HIV-positive patient with macrocytic anemia
Zidovudine induced
Normocytic anemia and elevated creatinine
chronic renal disease
causes of hypochromic, microcytic anemias
iron deficiency, thalassemia, lead poisoning
hypersegmented neutrophils
megaloblastic anemia (B12 or folate)
skull x-ray shows a “hair-on-end” appearance
marrow hyperplasia
basophilic stippling of RBC
lead poisoning
findings associated with hereditary spherocytosis
anemia, jaundice, pigmented gallstones, splenomegaly, spherocytes
+ osmotic fragility test
(-) Coombs test
Ham’s test
Paroxysmal nocturnal hemoglobinuria
cells lyse when placed in acid (now use flow cytometry to look for no CD55/CD59)
Heinz bodies
G6PD deficiency
painful cyanosis of fingers and toes, with hemolytic anemia
cold autoimmune hemolytic anemia
red urine in the morning, and fragile RBC
paroxysmal nocturnal hemoglobinuria