Pages 387-391 Flashcards
Ristoecetin DX test for which pathologies? Mechanism?
Baernard Soulier syndrome, Von Willebrand Disease
Normally ristocetin activates vWF to bind Gp IB. Failure of agglutination with ristocetin Ab’s is confirmatory test for both of the pathologies
Aspirin is used in platelet aggregation by blocking formation of TXA2? Why is it contraindicated in Asthma patients?
Aspirin irreversibly inhibits COX enzymes. The AA pathway shifts to increase lipoxygenase products especially LTC4, LTD4, LTE4. Together they form a group of molecules called slow reactive substances of anaphylaxis (SRS-A) which increase bronchial tone that is unfavorable in asthmatics
VWF is stored in which cells?
1) Weibel Palade bodies in Endothelial cells
2) Alpha granules in Platelets
How would you differentiate b/w VW disease and BS syndrome after a positive ristocetin test?
BS syndrome: inc BT, PT and PTT (N)
VW Disease: Inc BT, PT (N), Inc PTT, (vWF stabilizes Factor VIII therefore deficiency increase PTT)
Deficiency of Gp IIb/IIa?
Glanzmann Thrombasthenia
Name the Pathology associated with peripheral blood smear presentation: Degmacyte
G6PD deficiency (Degmacyte = Bite cell)
Name the Pathology associated with peripheral blood smear presentation: Basophilic stippling
Lead poisoning
Name the Pathology associated with peripheral blood smear presentation: Dacrocyte
Myelofibrosis (Dacrocyte = Teardrop cell)
Name the Pathology associated with peripheral blood smear presentation: hyper-segmented neutrophils
Megaloblastic Anemia
Name the Pathology associated with peripheral blood smear presentation: Schistocyte
DIC, TTP, HUS, Mechanical hemolysis (Schistocyte =Helmet cell)
Name the Pathology associated with peripheral blood smear presentation: Target cell
HALT
HbC disease, Asplenia, Liver Disease, Thalassemia
Name two heme pathologies that show an X linked inheritance pattern?
G6PD Deficiency, Sideroblastic Anemia
Copper deficiency can lead to this heme pathology?
Microcytic Sideroblastic Anemia
Clinical presentation of a pt with Iron Deficiency Anemia
Conjunctival Pallor, Spooned Nails (koilonychia)
Clinical presentation of Plummer Vinson Syndrome (Triad)?
Iron Def Anemia + Dysphagia + Esophageal Webs
Describe the genetic defect in Alpha thalassemia? Which mutation is affiliated with which population?
Deletion in alpha globin gene (Ch 16). Cis deletion prevalent in Asians, Trans deletion prevalent in Africans
Describe the genetic defect in Beta thalassemia? Which mutation is affiliated with which population?
Point mutation in the beta globin gene promoter and splice sites (Ch 11). Prevalent in Mediterranean populations
Clinical presentation of Beta Thalassemia
Crew cut on skull XR, Chipmunk Facies, Extramedullary hematopoiesis
Which virus can induce aplastic crisis (Clinical Presentation)? Name the heme pathologies associated with it.
Parvovirus B19 infection leads to slap cheek appearance in pts
Patients with aplastic crisis: SCA, B Thalassemia
What is basophilic stippling?
Remnants of rRNA in RBCs due decreased degradation
Clinical Presentation of Lead Poisoning?
Lead lines on gums (Burton lines) and long bone metaphyses
Encephalopathy
Abdominal colic
Dropping of wrist and foot
Name the enzymes inhibited by Lead
Ferrochelatase, ALA Dehydratse
DOC for Lead poisoning in Adults?
Dimercaprol, EDTA
DOC for Lead poisoning in Children?
Succimer
What is the most common cause of Sideroblastic Anemia ? Is it Irreversible?
Alcoholism which is reversible if given B6 as Tx
