BLI 9 - Normocytric Anemia II (Siddiqui)

Question 1

Usually asymptomatic but may have difficulty concentrating urine

Answer 1

Hb SA (sickle cell trait)

Question 2

In sickle cell disease, hemolysis is mostly _______

Answer 2

extravascular

Question 3

Sickle cell disease point mutation

Answer 3

Glutamic acid to valine at 6th position

Question 4

RIsk due to autosplenectomy

Answer 4

risk of infection from encapsulated organisms Spleen has fibrosed due to ischemia and infarction –> autosplenectomy

Question 5

Spherocytosis w/ positive coombs test

Answer 5

AIHA

Question 6

O157:H7

Answer 6

HUS Verotoxin causes endothelial cell damage –> platelet microthrombi –> schistocytes

Question 7

Organism responsible for osteomyelitis in the a patient with sickle cell disease

Organism responsible for osteomyelitis in normal pt

Answer 7

Salmonella typhi S. aureus

Question 8

Clinical manifestations for TTP/HUS

Answer 8

FAT RN

Fever

Anemia

Thrombocytopenia

Renal insufficiency – more HUS

Neurologic abnormalities – more TTP

Question 9

Homozygous Hb S

Answer 9

Sickle cell disease

Question 10

vaso-occlusive crisis

Answer 10

Sickle cell disease – sickled RBCs obstruct microvasculature

Question 11

Schistocytes

Answer 11

MAHA

Question 12

Hb C disease mutation

Answer 12

Glutamic acid –> Lysine at 6th position

Question 13

Pancytopenia

Low RI

Paroxysmal Nocturnal Hemolysis

Answer 13

Aplastic Anemia

Question 14

Hb SS vaccine

Answer 14

Pneumococcal

Question 15

Heterozygous Hb S

Answer 15

Sickle cell trait

Question 16

S. pneumo H. flu

Answer 16

Encapsulated organisms with increased risk of infection after autosplenectomy

Question 17

Aplastic crisis

Stroke

Acute chest syndrome

Autosplenectomy

Aseptic necrosis

Skin infections/ulcers

Answer 17

Sickle cell disease

Question 18

Detects antibody-coated RBCs

Answer 18

Direct Coombs test

Question 19

Target cells Hb crystals

Answer 19

Hb C disease

Question 20

Best screening test for DIC

Answer 20

D-dimer

Question 21

IgM mediated AIHA: hemolysis

Answer 21

Intravascular hemolysis

Question 22

Sepsis

Abruptio placentae

Trauma/burns

Acute promyelocytic leukemia

Answer 22

Etiologies of DIC

Question 23

Sickle cell disease effect on bone marrow

Answer 23

Necrosis

Question 24

Tx of TTP/HUS includes plasmaphoresis and steroids, but never ________

Answer 24

platelet transfusion

Question 25

Hematopoietic stem cell defect

Answer 25

Aplastic Anemia

Question 26

Like thalassemia, shows crewcut appearance on xray with chipmunk facies and hepatomegaly

Answer 26

Sickle cell disease

Question 27

SLE, RA CLL PCN, quinidine, methyldopa

Answer 27

Warm agglutinin AIHA diseases

Question 28

IgM (complement-fixation): Cold or warm Extravascular or intravascular

Answer 28

Cold Intravascular

Question 29

Pulmonary risk of sickle cell disease

Answer 29

Acute chest syndrome

Question 30

Initial presentation of sickle cell disease \>6 mo

Answer 30

Dactylitits

Question 31

Stroke/TIA sequelae Hypersplenism

Answer 31

Chronic complications of sickle cell disease

Question 32

IgG mediated AIHA: hemolysis

Answer 32

Extravascular hemolysis

Question 33

Sickle cell disease confirmatory test

Answer 33

Hemoglobin electrophoresis

Question 34

Sickle cell disease inheritance pattern

Answer 34

Autosomal recessive

Question 35

3 deoxygenated states that cause Hb S polymerization

Answer 35

Hypoxemia Acidosis Dehydration

Question 36

Components of Microangioapthic Hemolytic Anemia (MAHA)

Answer 36

TTP/HUS & DIC

Question 37

Mycoplasma pneumoniae Infectious Mononucleosis

Answer 37

Cold agglutinin AIHA diseases

Question 38

ADAMTS13

Answer 38

TTP Decreased ADAMTS13 = failure to cleave large vWF multimers --\> platelet microthrombi --\> schistocytes

Question 39

IgG (Opsonization): Cold or warm Extravascular or intravascular

Answer 39

Warm Extravascular

Question 40

In sickle cell disease, this form of hemoglobin is protective against sickling

Answer 40

Hb F

Question 41

Detects antibodies in serum

Answer 41

Indirect Coombs test

Question 42

Hydroxyurea

Answer 42

Increased Hb F --\> protective against sickling