RBC disorders

Question 1

Anemia due to underproduction is caused by

Answer 1

Parvovirus B19

Aplastic anemia

Question 2

Autosplenectomy leads to increased suseptability to

Answer 2

Capsulated organism:

1. S. pneumoniae
2. H. influenzae
3. Salmonella

Question 3

Cis deletion in two gene α-Thalassemia found in

Answer 3

Asians

Question 4

Clinical features of Iron deficiency

Answer 4

1. Anemia
2. Koilonychia
3. Pica
4. Plummer-Vinson

Question 5

Clinical findings of Folate deficiency

Answer 5

Glossitis

Macrocytic RBC and hypersegmented PMN

Question 6

Clinical in Hemolytic anemia

Answer 6

1. Anemia with splenomegaly
2. Jaundice due to unconjungated bilirubin
3. Marroy hyperplasia with corrected RC

Question 7

Hb Bart see in

Answer 7

Four gene deletion in α-Thalassemia

Usually leads to hydrops fetalis

Question 8

HbH is seen in

Answer 8

Three gene deleted α-Thalassemia

Question 9

Heinz bodies seen in

Answer 9

G6PD deficiency

Question 10

IgG-mediated IHA chrx

Answer 10

Occurs extravascular

Occurs in warm temperatures

Question 11

IgM-mediated IHA chx

Answer 11

Occurs intravascular

Occurs in cold temperatures

Associated with Mycoplasma pneumoniae

Question 12

Immune hemolytic anemia (IHA) is

Answer 12

AB mediated IgG or IgM destruction of RBCs

Question 13

Irreversible sickling leads to

Answer 13

1. Dactylitis: swollen hands and feet
2. Autosplenectomy: Howell-jolly bodies on blood smear
3. Acute chest syndrome
4. Pain
5. Renal papillary necrosis

Question 14

Lab findings in Anemia of Chronic disease

Answer 14

1. Increase ferritin
2. Decrese TIBC
3. Decrease serum iron
4. Decrease % saturation
5. Increase Free erythrocyte protoprophyrin (FEP)

Question 15

Lab findings in folate deficiency

Answer 15

Increase serum homocysteine

Decrese serum folate

Normal methylmalonic acid

Question 16

Lab findings in Iron deficiency

Answer 16

1. Microcytic, hypochromic RBC
2. Decrease ferritin
3. Increase TIBC
4. Decrease serum iron
5. Decrease % saturation

Question 17

Lab resuts of β-Thalassemia minor

Answer 17

Microcytic, hypochromaic RBC with target cells

Question 18

Macrocytic anemia is most commonly due to

Answer 18

Folate or Vit B12 deficiency

Question 19

MCV < 80

Answer 19

Microcytic anemia

Question 20

Microcytic anemias are due to

Answer 20

Decreased produciton of hemoglobin

Extra devision in BM to maintain hemoglobin concentration

Question 21

Microcytic anemias include

Answer 21

1. Iron deficiency anemia
2. Anemia of chronic disease
3. Sideroblastic anemia
4. Thalassemia

Question 22

Paroxysmal nocturnal hemogloninuria (PNH) is due to

Answer 22

acquired defect in myeloid stem cells resulting in absent GPI, renders cells susceptible to destruction by complements

Question 23

Parvovirus B19 does what

Answer 23

Infects progenitor red cells and temporarily halts erythropoiesis

Question 24

Reticulocytes are

Answer 24

young RBCs released from the BM

Identified as larger cells with bluish cytoplasm

Question 25

Serum ferritin reflects

Answer 25

Iron stores in macrophages and liver

Question 26

Sickle cell is due to

Answer 26

replacement of normal **glutamic acid** with **valine**

Question 27

Sideroblastic anemia is due to

Answer 27

Defective **protoporphyrin synthesis**

Question 28

Sideroblastic anemia lab findings

Answer 28

1. Increase ferritin 2. Decrease TIBC 3. Increase serum iron 4. Increase % saturation

Question 29

Thalassemia protective against

Answer 29

Plasmodium flaciparum

Question 30

Total iron-binding capacity (**TIBC**) measures

Answer 30

Transferring molecules in the blood

Question 31

Trans deletion in two gene α-Thalassemia found in

Answer 31

Africans

Question 32

Vit. B12 clinical

Answer 32

Glossitis Subacute combind degeneration of SC due to increased methylmalonic acid (peripheral neuropathy)

Question 33

What causes IgG-mediated IHA

Answer 33

1. SLE 2. Drugs: 1. Penicillin 2. Cephalosporin 3. Methyldopa

Question 34

What complications arise from PNH

Answer 34

1. Iron deficiency anemia 2. AML

Question 35

What increases risk of sickling in HbS

Answer 35

1. Hypoxemia 2. Dehydration 3. Acidosis

Question 36

Where are β genes pressent in β-Thalassemia

Answer 36

chromosome 11

Question 37

α-Thalassemia is due to

Answer 37

Gene deletion of alpha gene on chromosome 16

Question 38

β-Thalassemia major clinical

Answer 38

Massive erythroid hyperplasia causing: 1. Expansion of hematopoiesis into skull (**crewcut** appearance) 2. Expansion of hematopoiesis into facial bone (**chipmunk** face) 3. Extramedullary hematopoiesis with **hepatosplenomegaly** 4. Risk of aplastic crisis with **B19** **infection**

Question 39

β-Thalassemia major (**β⁰/β⁰**) is

Answer 39

1. Most severe β-Thalassemia that presents with severe anemia a few months after brith 2. α tetramers aggregate and damage RBC resulting in ineffective erythropoiesis and extravascular hemolysis

Question 40

β-Thalassemia major smear

Answer 40

Microcytic, hypochrmic RBC with **target cells and nucleated RBC**

Question 41

β-Thalassemia minor (**β/β⁺**) is

Answer 41

Mildest form of β-Thalassemia usually asymptomatic with an increased RBC count

Question 42

β-Thalassemia usually due to

Answer 42

gene mutation