#26 - RBC disorders

Question 1

What reticulocyte index indicates insufficient marrow response?

Answer 1

less than 2.0

Question 2

Which results in a bigger increase in reticulocytes: blood loss or hemolysis?

Answer 2

hemolysis.

Question 3

acquired hyperproliferatve anemia diseases - 4

Answer 3

• autoimmune hemolytic anemia
• TTP-HUS
• DIC
• Malaria

Question 4

inherited hyperproliferative anemia diseases - 3

Answer 4

• spherocytosis
• sickle cell
• thalassemia

Question 5

microcytic anemias- 3

Answer 5

• iron deficiency
• thalassemic disorders/ hemoglobinopathies
• anemia of chronic disease (also normocytic)

Question 6

normocytic anemias - 4

Answer 6

• acute blood loss
• iron deficiency (early)
• anemia of inflammation/ chronic disease. (also microcytic
• bone marrow suppression

Question 7

macrocytic anemias - 4

Answer 7

• alcohol abuse
• folate, B12 deficiency
• hemolytic anemia
• bone marrow disorders such as MDS (myelodysplastic syndromes)

Question 8

diagnostic testing for iron deficiency anemia

Answer 8

• iron saturation will go down

- ferritin will go down (ferritin correlates with blood iron levels)

Question 9

How can inflammation interfere with an anemia diagnosis?

Answer 9

Ferritin rises as an acute phase reactant in inflammation. This can mask the low level expected in iron deficiency. Iron saturation can also be borderline. Ferritin should drop though!!

Question 10

If a person has iron deficiency without anemia, what will labs look like?

Answer 10

hemoglobin and serum iron will be normal, but ferritin will be low!

Question 11

Vit B12 deficiency anemia - associations

Answer 11

• vegans
• autoimmune history
• neurologic symptoms
• gait ataxia
• sensory loss/parasthesia

Question 12

Folate deficiency anemia - associations

Answer 12

-malnourished
-alcoholic
NOT associated with autoimmune tendency
*NO neurologic signs**

Question 13

You see a patient which you think has B12 or folate deficiency anemia but you aren’t sure. What do you do?

Answer 13

test for methylmalonate and homocysteine can confirm either diagnosis.

Question 14

When should methylmalonate and homocysteine testing be done

Answer 14

When you see a patient which you think has B12 or folate deficiency anemia but you aren’t sure.

Question 15

T/F - Anemia of chronic disease is always inflammatory

Answer 15

True. Thought to be cytokines interfering with action of EPO

Question 16

Labs for anemia of chronic disease

Answer 16

• low iron
• low TIBC
• low reticulocyte count.
• normal RBC morphology.
• iron deficiency may also be present.
• may see kidney damage (decreased EPO)

Question 17

What is the most common cause of anemia in the Western world?

Answer 17

iron deficiency

Question 18

3 key features of iron deficiency anemia

Answer 18

• hypochromia (pale RBCs)

- microcytosis (MCV

Question 19

anisocytosis

Answer 19

RBCs of different sizes. present in iron deficiency anemia.

Question 20

Therapy for B12 deficiency

Answer 20

lifelong cobalamin therapy (monthly B12 injections)

Question 21

Therapy for folate deficiency

Answer 21

folate + cobalamin

Question 22

Schistocytes on smear - likely diagnosis?

Answer 22

TTP

Question 23

Spherocytes on smear - likely diagnosis?

Answer 23

-autoimmune condition

Question 24

bite cells on peripheral blood smear

Answer 24

-G6PD deficiency

Question 25

Case: Patient with dark urine, jaundice. What is the next step/likely diagnosis?

Answer 25

Get a smear - likely to be hemolytic anemia. | bleeding = free hemoglobin damages kidney, jaundice = indirect bilirubin

Question 26

malaria = what type of anemia

Answer 26

hemolytic

Question 27

TTP - HUS = what type of anemia

Answer 27

Hemolytic

Question 28

``` Case: young woman -fatigue for several years -family history of anemia -MCV 60 -targetoid cells on smear. Diagnosis? ```

Answer 28

Thalassemia

Question 29

labs in thalassemia

Answer 29

abnormal -hemoglobin electrophoresis | -microcytic anemia

Question 30

Associated symptoms - thalassemia

Answer 30

skeletal changes/ abnormalities - hepatomegaly - splenomegaly - iron overload.

Question 31

Treatment of thelassemias

Answer 31

**chronic blood transfusions!!!!: 2 units every 2-5 weeks to keep target Hb of 9-10 . Usually started in first decade of life. Bone marrow transplant is curative, but not always done.

Question 32

sickle cell substitution

Answer 32

valine in place of glutamic acid on B globin gene - chromosome 11

Question 33

sickle cell disease =

Answer 33

recessive inherited, chronic hemolytic anemia

Question 34

Manifestations of sickle cell

Answer 34

- RBCs only survive 10-25 days - hemolystic anemia - multiorgan involvement. - chest syndrome, pulmonary fibrosis - vascular occlusion, organ ischiemia, vasoconstriction

Question 35

Treatment for sickle cell

Answer 35

mainly supportive - hydration and analgesia for painful crisis. - RBC transfusions sometimes indicated

Question 36

hallmark of sickle cell disease

Answer 36

painful crisis