Hemeonc MSK Flashcards
Anisocytosis
varying sizes
Poikilocytosis
varying shapes
thrombocytopenia/platelet dysfunction results in
petechiae
Cell surface marker for Macrophages?
MC14
What causes eosinophilia?
DNAAACP Drugs Neoplasms Atopy/allergy Addisons Acute interstitial neph Collagen vascular dx Parasites- helminths - Churgg Strauss
funtion of a mast cell?
binds to the Fc portion of IgE to membrane
- cross linking of IgE causes degranulation releasing histamine, heparin and eosinophil chemotactic factors
only APC that can activate NK cells?
Dendritic cells- acts as a linke between innate and adaptive immune system
CD28
marker of T cells, necessary for t cell activation
what enzyme does warfarin inhibit?
epoxide reductase which usually activates vitamin K to make mature clotting factors
Role of kallikrein?
activates bradykinin and complement cascade (via activation of plasmin)
C1 esterase inhibitor?
inhibits kallikrein > brakykinin, so a deficiency causes a buildup of bradykinin, leading to angioedema
role of plasmin?
decreases the fibrin clot
What does the endothelial cell make in terms of thrombogenesis?
vWF
thromboplastin
tPA
PGI2
when do you see a acanthocyte?
abetalipoproteinemia
When do you see target cells?
HALT HbC Asplenia Liver disease Thalassemia
> 3.4% HbA2
diagnostic confirmation fo B Thal minor
what are the genetics behind beta thal?
- point mutations in splice sites and promoter sequences decreasing B globin synthesis
(Alpha thal = gene deletions)
crew cut on skull xray, chipmunk facies
beta thal major- homozygotes, stop codon, increased HbF
what are the aggregates seen in basophilic stipling?
rRNA aggregates
where in the bone does lead target?
epiphysis of distal femur and proximal tibia
Pathogenesis of sideroblastic anemia
X linked defect in d-ALA synthase gene
- also seen in alcohol, lead and INH deficiency
tx of sideroblastic anemia?
pyridoxine- B6 is a cofactor for ALA synthase
findings of intravascular hemolysis
decreased haptoglobin
increased LDH
hemoglobinuria
Extravascular hemolysis findings
increased LDH
increased UCB > jaundice
What viruses can cause Aplastic anemia?
Parvo B19
EBV
HCV
HIV
how does pyruvate kinase deficiency cause anemia?
defective pyruvate kinase causes decreased ATP > rigid RBCs
- hemolytic anemia in newborn
whats the defect behidn HbC?
glutamic acid to lysine mutation in B globin gene
treatment of PNH?
eculizumab
genetics of HbS?
substitution of glutamic acid with val in Beta chain
What drug could cause Warm agglutinin autoimmune hemolytic anemia?
alpha-methyldopa
What enzymes does Lead inhibit in heme synthesis? what builds up?
- Ferrochelatase
- ALA dehydrogenase
Buildup: protoporphyrin and delta-ALA
Acute intermittent porphyria: affected enzyme and accumulated substrate
Enzyme: porphobilinogen deaminase
Substrate: prophobilinogen, delata-ALA, uroporphyrin
abdominal pain, burgundy colored urine, polyneuropathy, psych disturbances
Acute intermittent porphyria
Porphyria cutanea tarda: affected enzyme and accumulated substrate
Enzyme: uroporphyrinogen decarboxylase
Accumulated substrate: uroporphyin (tea colored urine)
blistering cutaneous photosensitivty and tea colored urine
Porphyria cutanea tarda
what disease is a combination of AIP with photosensitivity?
Coroporphyrinogen III oxidase deficiency
what is the treatment for Acute intermittent porphyria?
glucose and heme (inhibit ALA synthase)
what are the signs of a platelet disorder versus coagulation disorder?
Coagulation: bleeding into joints easy bruising
Platelet: epistaxis, petechiae, purpura
Bernard soulier syndrome
decreased GpIb leading to defect in paltelet to vWF adhesion
Glanzmanns thrombasthenia
decreased GpIIb/IIIa, defect in platelet to platelet aggregation
- blood smear shows no platelet clumping
- normal platelet count
Idiopathic thrombocytopenic purpura
anti-GpIIb/IIIa antibodies, splenic macs eat platelet/antibody complex
TTP
deficiency of ADAMTS13 (vWF metalloprotease) > decrease of vWF multimers
- neurologic, renal sx, fever, thrombocytopenia, and microangiopathic hemolytic anemia
How is heme degraded?
by Heme oxygenase- turning it into CO2 and biliverdin (which gives bruises their green color)
inheritance of vWF disease
Autosomal Dominant