Chapter 12 - RBC disorders

Question 1

Where do I have EMH and so head-on-appearance and HSM?

Answer 1

1. β thal major

2. SCD (severe)

Question 2

Where do I have increased RDW?

Answer 2

1. Iron deficiency
2. β thal major
3. HS

Question 3

Sideroblastic anemia is present in ~ …% of chronic alcoholics

Answer 3

30. Alcoholism is the MCC of sideroblastic anemia

Question 4

Which enzymes does lead denaturate?

Answer 4

1. Ferrochelatase
2. ALAD
3. Ribonuclease

Question 5

Which is the MC anemia in alcoholism?

Answer 5

ACD

Question 6

2 conditions where RBC osmotic fragility is increased

Answer 6

1. HS

2. Hereditary Elliptocytosis

Question 7

In sickle cell anemia, normal Glutamic Acid is hydroph… and Valine, which replaces it, is hydroph…

Answer 7

Glu: hydrophilic
Val: hydrophobic

Question 8

People with Sickle Cell Trait do not normally have anemia. HbS

Answer 8

Peritubular capillaries in renal medulla. There the O2 tension is normally low enough to induce sickling in not only disease but also in trait.

Microinfarctions are induced leading to microhematuria. (*always order a sickle cell screen in any black person with unexplained hematuria)

Renal papillary necrosis may occur-> resulting in loss of concentration and dilution of urine.

Question 9

MC hemoglobinopathy in AFRICANS (highest prevalence : 30-40%, in sub-Saharan Africa)

Answer 9

SCD

Question 10

In SCD, what kind of hemolysis do we have?

Answer 10

Both extravascular (dominant) and intravascular

Question 11

At which position of the β-globin chain does the missense point mutation occur?

Answer 11

6th

Question 12

Percent of SC trait in black population

Answer 12

10%

Question 13

3 factors that increase the concentration of deoxyHb and increase the risk for sickling

Answer 13

1. Acidosis
2. Volume depletion/dehydration
3. Hypoxemia

Question 14

Which drug increases the synthesis of HbF and so is used therapeutically for SCD?

Answer 14

Hydroxyurea

Question 15

MCC of death in SCD young adults

Answer 15

Acute chest syndrome

Question 16

MCC of death in SCD children

Answer 16

Infection with encapsulated organisms (eg. S.pneumoniae sepsis) due to dysfunctional spleen due to autosplenectomy

Question 17

The people with SCD have increased risk for osteomyelitis. The MCC of osteomyelitis -in general- is S. aureus. But in this situation is …

Answer 17

Salmonella paratyphi

Question 18

A common cause of stroke in children 2-5y is…

Answer 18

SCD. 70%recurrence rate

Question 19

What kinds of cells do we have in a blood smear of a patient with SCD (HbSS)?

Answer 19

1. Sickle cells
2. Target cells
3. Nucleated RBCs
4. Howell-Jolly bodies

Question 20

Anemia with predominantly IV hemolysis but also mild to moderate component of extravascular hemolysis as well

Answer 20

G6PD def

Question 21

Clinical presentation of G6PD def and why

Answer 21

Sudden onset of back pain with hemoglobinuria 2-3 days after an oxidant stress. That is why Hb is nephrotoxic.
Also jaundice may be present.

Question 22

Which anemia is present at birth with jaundice and splenomegaly?

Answer 22

PK def

Question 23

PK def produces IV or EV hemolysis?

Answer 23

EV

Question 24

Which anemia is related to syphilis?

Answer 24

Paroxysmal cold hemoglobinuria

Question 25

Paroxysmal cold hemoglobinuria has IV or EV hemolysis?

Answer 25

IV

Question 26

Anemias associated with Reynaud phenomenon?

Answer 26

1. PCH

2. AIHA (cold types)

Question 27

Which Plasmodium is the MCC of malaria worldwide?

Answer 27

P. vivax!

Question 28

Which is the most lethal plasmodium?

Answer 28

falciparum

Question 29

Which plasmodium is associated with nephrotic s?

Answer 29

malariae