March 19 test 2

Question 1

What is the cause of hereditary spheocytosis?

Answer 1

Defect in proteins interacting with RBC membrane

• Ankyrin
• Band 3
• Protein 4.2
• Spectrin

Premature removal by the spleen–so tx is splenectomy

Question 2

Pt presents with mucocutaneous bleeding and mild petechial hemorrhages with an isolated thrombocytopenia. What is the dz and what is causing it?

Answer 2

Immune thrombocytopenia–Anti-GpIIb/IIIa antibodies–splenic macrophage consumption of platelet-antibody complex

Commonly due to viral illness

Question 3

What abnormal labs are seen with von Willebrand disease?

Answer 3

Increased PTT– vWF acts to carry/protect factor VIII

Increased bleeding time

Question 4

What leukemia is associated with down syndrome and has massive increase in lymphoblasts?

Answer 4

ALL

Has an acute onset

Question 5

What is the cause of Patau syndrome?

Answer 5

Maternal nondisjunction –trisomy 13

Question 6

What type of lymphoma is characterized by Reed-Sternberg cells?

Answer 6

Hodgkin Lymphoma

Question 7

Majority of non-Hodgkin lymphomas involve ____ cells?

Answer 7

B

Question 8

What is associated with hyperhsegmented PMNs?

Answer 8

Vit B12 deficiency –pernicious anemia

Question 9

Pt with a1 antitrypsin deficiency with cirrhosis will show what on biopsy?

Answer 9

Intracellular PAS + globules

Question 10

Pt presents with hyperthyroid symptoms in an ovarian tumor composed of thyroid tissue. Dx?

Answer 10

Struma ovarrii

Question 11

What is the distinguishing feature of focal segmental glomerulosclerosis?

Answer 11

Large focal hyaline deposits on biopsy

Question 12

What dose EF2-a do?

Answer 12

Complexes with guanosine triphosphate and binds the ribosome, initiating the translocation step of protein synthesis