Pages 387-391

Question 1

Ristoecetin DX test for which pathologies? Mechanism?

Answer 1

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

Question 2

Aspirin is used in platelet aggregation by blocking formation of TXA2? Why is it contraindicated in Asthma patients?

Answer 2

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

Question 3

VWF is stored in which cells?

Answer 3

1) Weibel Palade bodies in Endothelial cells

2) Alpha granules in Platelets

Question 4

How would you differentiate b/w VW disease and BS syndrome after a positive ristocetin test?

Answer 4

BS syndrome: inc BT, PT and PTT (N)

VW Disease: Inc BT, PT (N), Inc PTT, (vWF stabilizes Factor VIII therefore deficiency increase PTT)

Question 5

Deficiency of Gp IIb/IIa?

Answer 5

Glanzmann Thrombasthenia

Question 6

Name the Pathology associated with peripheral blood smear presentation: Degmacyte

Answer 6

G6PD deficiency (Degmacyte = Bite cell)

Question 7

Name the Pathology associated with peripheral blood smear presentation: Basophilic stippling

Answer 7

Lead poisoning

Question 8

Name the Pathology associated with peripheral blood smear presentation: Dacrocyte

Answer 8

Myelofibrosis (Dacrocyte = Teardrop cell)

Question 9

Name the Pathology associated with peripheral blood smear presentation: hyper-segmented neutrophils

Answer 9

Megaloblastic Anemia

Question 10

Name the Pathology associated with peripheral blood smear presentation: Schistocyte

Answer 10

DIC, TTP, HUS, Mechanical hemolysis (Schistocyte =Helmet cell)

Question 11

Name the Pathology associated with peripheral blood smear presentation: Target cell

Answer 11

HALT

HbC disease, Asplenia, Liver Disease, Thalassemia

Question 12

Name two heme pathologies that show an X linked inheritance pattern?

Answer 12

G6PD Deficiency, Sideroblastic Anemia

Question 13

Copper deficiency can lead to this heme pathology?

Answer 13

Microcytic Sideroblastic Anemia

Question 14

Clinical presentation of a pt with Iron Deficiency Anemia

Answer 14

Conjunctival Pallor, Spooned Nails (koilonychia)

Question 15

Clinical presentation of Plummer Vinson Syndrome (Triad)?

Answer 15

Iron Def Anemia + Dysphagia + Esophageal Webs

Question 16

Describe the genetic defect in Alpha thalassemia? Which mutation is affiliated with which population?

Answer 16

Deletion in alpha globin gene (Ch 16). Cis deletion prevalent in Asians, Trans deletion prevalent in Africans

Question 17

Describe the genetic defect in Beta thalassemia? Which mutation is affiliated with which population?

Answer 17

Point mutation in the beta globin gene promoter and splice sites (Ch 11). Prevalent in Mediterranean populations

Question 18

Clinical presentation of Beta Thalassemia

Answer 18

Crew cut on skull XR, Chipmunk Facies, Extramedullary hematopoiesis

Question 19

Which virus can induce aplastic crisis (Clinical Presentation)? Name the heme pathologies associated with it.

Answer 19

Parvovirus B19 infection leads to slap cheek appearance in pts

Patients with aplastic crisis: SCA, B Thalassemia

Question 20

What is basophilic stippling?

Answer 20

Remnants of rRNA in RBCs due decreased degradation

Question 21

Clinical Presentation of Lead Poisoning?

Answer 21

Lead lines on gums (Burton lines) and long bone metaphyses
Encephalopathy
Abdominal colic
Dropping of wrist and foot

Question 22

Name the enzymes inhibited by Lead

Answer 22

Ferrochelatase, ALA Dehydratse

Question 23

DOC for Lead poisoning in Adults?

Answer 23

Dimercaprol, EDTA

Question 24

DOC for Lead poisoning in Children?

Answer 24

Succimer

Question 25

What is the most common cause of Sideroblastic Anemia ? Is it Irreversible?

Answer 25

Alcoholism which is reversible if given B6 as Tx