Pathology 2

Question 1

Decreased Haptoglobin, Increased LDH, schistocytes and increased reticulocytes, and Urobilinogen in urine

Answer 1

Intravascular Hemolysis

Question 2

Spherocytes in peripheral smear, increased LDH and UCB causing jaundice

Answer 2

Extravascular Hemolysis

Question 3

Anemia of Chronic Dx

Answer 3

Increase in hepcidin and inhibits iron transport, decreases release of iron from macrophages

Question 4

Aplastic Anemia

Answer 4

caused by failure or destruction of myeloid stem cells due to radiation, drugs, viruses (B19, EBV, HIV, HCV), Fanconi anemia, idiopathic

Question 5

Defect in ankyrin, band 3, protein4.2, spectrin

Answer 5

Hereditary Spherocytosis

Question 6

Osmotic Fragility Test

Answer 6

Hereditary Spherocytosis

Question 7

Sulfa drugs, antimalarials, infections, fava beans

Answer 7

Acute hemolytic anemia from G6PD deficiency

Question 8

back pain, hemoglobinuria a few days after oxidant stress

Answer 8

G6PD deficiency

Question 9

G6PD deficiency Resistance to

Answer 9

P. falciparum

Question 10

Hemolytic anemia in a newborn masked by 2,3 BPG

Answer 10

Pyruvate Kinase Deficiency

Question 11

Glutamic Acid to Lysine Mutation at residue 6 in beta-globin

Answer 11

HbC defect

Question 12

Impaired synthesis of GPI anchor for decay-accelerating factor that protects RBC membrane from complement

Answer 12

Paroxysmal Nocturnal Hemoglobinuria

Question 13

Coomb negative hemolytic anemia, pancytopenia, and venous thrombosis, CD55/59 negative RBCs on flow cytometry

Answer 13

Paroxysmal Nocturnal Hemoglobinuria

Question 14

Tx for Paroxysmal Nocturnal Hemoglobinuria

Answer 14

Eculizumab

Question 15

Salmonella osteomyelitis

Answer 15

Sickle cell anemia

Question 16

Dactylitis

Answer 16

Painful vaso-occlusive crisis in bones associated with sickle cell disease

Question 17

Renal Papillary Necrosis

Answer 17

due to low oxygen in papilla; also seen in heterozygotes and microhematuria (medullary infarcts)

Question 18

Tx of Sickle Cell Anemia

Answer 18

Hydroxyurea to increase fetal hemoglobin

BM transplant

Question 19

Warm Agglutinin

Answer 19

IgG, Coombs (+), chronic anemia seen in SLE, CLL or with alpha-methyldopa

Question 20

Cold Agglutinin

Answer 20

IgM, acute anemia triggered by cold; seem om CLL, Mycoplasma pneumonia, infectious mononucleosis

Question 21

Direct Coombs

Answer 21

anti-Ig antibody added to patient’s blood. RBCs agglutinate if RBCs are coated with Ig

Question 22

Indirect Coombs

Answer 22

normal RBCs added to patient’s serum. If serum has anti-RBC surface Ig, RBCs agglutinate when anti-Ig antibodies added

Question 23

ADAMTS13 deficient

Answer 23

TTP

Question 24

Causes of Eosinopenia

Answer 24

Cushing Syndrome, corticosteroids

Question 25

Enzyme affected in Acute Intermittent Porphyria

Answer 25

Porphobilinogen deaminase

Question 26

Painful abdomen, Port wine colored urine, Polyneuropathy, Psychological disturbances, Precipitated by drugs, alcohol and starvation

Answer 26

Acute Intermittent Porphyria

Question 27

Deficient in Porphyria cutanea tarda

Answer 27

Uroporphyrinogen decarboxylase

Question 28

Blistering cutaneous photosensitivity

Answer 28

Porphyria cutanea tarda

Question 29

Tx for Acute Intermittent Porphyria

Answer 29

Glucose and Heme, they inhibit ALA synthase

Question 30

Enzyme deficient in lead poisoning

Answer 30

delta-aminolevulinic acid dehydratase

Question 31

Defect in Factors I, II, V, VII, and X

Answer 31

increases PT

Question 32

Tx for Hemophilia A

Answer 32

Recombinant Factor VIII

Question 33

Warfarin on PT and PTT

Answer 33

both increase

Question 34

Defect in platelet-to-platelet aggregation

Answer 34

Glanzmann Thrombasthenia

Question 35

Decreased GpIIb/IIIa

Answer 35

Glanzmann Thrombasthenia

Question 36

Defect in platelet to vWF adhesion

Answer 36

Bernard-Soulier Syndrome

Question 37

anti-GpIIb/IIIa antibodies, may be triggered by viral illness

Answer 37

Immune Thrombocytopenia

Question 38

vWF metalloprotease

Answer 38

Thrombotic Thrombocytopenic purpura

Question 39

Neurologic and renal symptoms, fever, thrombocytopenia, and microangiopathic hemolytic anemia

Answer 39

Thrombotic Thrombocytopenic purpura

Question 40

Tx of TTP

Answer 40

transfusion and steroids

Question 41

Diseases with decreased Plt Count and increased Bleeding time

Answer 41

Bernard-Soulier Syndrome, Immune Thrombocytopenia, Thrombotic Thrombocytopenic purpura

Question 42

igG + Heparin + Platelet Factor 4

Answer 42

HIT

Question 43

Tx of HIT

Answer 43

Direct Thrombin Inhibitors

Question 44

Osler-Weber-Rendu

Answer 44

hereditary hemorrhagic telangiectasia

Question 45

Idiopathic Thrombocytopenic Purpura

Answer 45

Female of reproductive years, plts opsonized by anti-plt Ig

Question 46

Hemolytic Anemia, Thrombocytopenia, Renal Failure

Answer 46

HUS

Question 47

Tx of von Willebrand Dx

Answer 47

DDAVP which releases vWF stored in endothelium

Question 48

Widespread activation of clotting leads to a deficiency in clotting factors, which creates a bleeding state

Answer 48

DIC

Question 49

Best screening test for DIC

Answer 49

D-dimers

Question 50

Decreased ability to inactivate factors V and VIII

Answer 50

Protein C or S deficiency

Question 51

Most common hypercoagulability in whites

Answer 51

Factor V Leiden

Question 52

Virchows Triad

Answer 52

Disrupt blood flow, endothelial cell damage, hypercoaguable state

Question 53

Packed RBCs

Answer 53

increases Hb and oxygen carrying capacity | Used in acute blood loss and severe anemia

Question 54

Platelet Transfusion

Answer 54

to increase plt count what <5000, used to stop significant bleeding

Question 55

Fresh Frozen Plasma

Answer 55

Increases coagulation factor levels | used in DIC, cirrhosis, warfarin overdose, exchange transfusion in TTP/HUS

Question 56

Cryoprecipitate

Answer 56

Contains fibrinogen, factor VIII, factor XIII, vWF and fibronectin To treat coagulation factor deficiencies involving fibrinogen and factor VIII

Question 57

Open

Answer 57

Meow