CIS: RBC Disorders

Question 1

If on a deserted island and you have anemia, what is the one test that you would order?

Answer 1

The reticulocyte count

-represents immature blood cells

Question 2

RBC’s live in blood how long?

Answer 2

120 days

Question 3

What is a RBC 1-2 days before it is fully mature?

Answer 3

A reticulocyte

-we can test for reticulin

Question 4

Aplastic anemia

Answer 4

The congenital kind is Fanconi anemia

• most cases are secondary to other causes
• EBV, HIV, radiation, chloramphenicol, quinacrine

Question 5

What is chloramphenicol?

Answer 5

An antibiotic

• risk is 1:30,000 for aplastic anemia
• bone marrow replacement is a good treatment for it

Question 6

What do people who sniff glue have increased risk for?

Answer 6

Aplastic anemia

Question 7

What has aplastic anemia as a hallmark of the disease?

Answer 7

PNH

Question 8

What will we see on a blood smear in chronic kidney disease

Answer 8

• spikes cells… Acanthocytes

- nor chromic normacytic anemia

Question 9

Megaloblastic anemias

Answer 9

95% due to deficiency of either B12 or folic acid

-rest are due to drugs

Question 10

What does the neuro symptoms with B12 patients look like?

Answer 10

• tertiary syphillis related tabes dorsalis
• so always check B12 levels
• the difference is that in tabes, there will be more urinary incontinence symptoms

Question 11

What lab test do you do for folic acid deficiency?

Answer 11

Folate levels!

Question 12

Tx of folate deficiency

Answer 12

Supplemental folate… 1mg/day

Question 13

what is B12 deficiency due to ?

Answer 13

• impaired absorption
• pernicious anemia
• gastrectomy
• Zollinger-Ellison syndrome
• blind loop syndrome
• tapeworm infestation
• being a vegan….

Question 14

What is pernicious anemia

Answer 14

-auto antibodies directs against PARIETAL CELLS, not Intrinsic factor itself

Question 15

Iron deficiency anemia

Answer 15

• dietary deficiency: most infants have problems

- if bleeding from GI tract, it’s cancer until proven otherwise

Question 16

What is Asthenia?

Answer 16

• pale

- ppl look like their just a bag of bones

Question 17

What is the result of an altered expression of nRAMP2 gene?

Answer 17

• that encodes the DCT1 transport protein

- iron deficiency anemia

Question 18

What is myoglobin

Answer 18

It’s in the muscles and gives them a store of Oxygen

-this is why iron deficient people have sore muscles

Question 19

What do iron deficient people like to eat a lot?

Answer 19

• dirt (pica)

- ice

Question 20

Is iron supplementation effective in anemia of chronic disease?

Answer 20

No

-if anemia is that severe, transfusions may be helpful to keep Hb in check

Question 21

What is a myelopthisic anemia?

Answer 21

Anemia due to infiltration of the bone marrow

-like, by cancer or something

Question 22

What are the ice cravings seen in iron deficiency anemia called?

Answer 22

Pagophagia

Question 23

Clinical triad of diabetes

Answer 23

Polyuria, polydipsia, and something else

Question 24

What the hallmark disorder for enzyme deficiency blood disorder

Answer 24

G6PD deficiency

• X-linked
• fava beans!!! Favism

Question 25

What are Heinz bodies?

Answer 25

Collection of denatured hemoglobin | -spleen removes this and results in "bite cells"

Question 26

March hemoglobinuria

Answer 26

- feet striking the ground repeatedly causes damage/lysis of RBCs in capillaries on plantar surface of feet - common in marathon runners - treat with well padded footwear and reassurance - originally described in soldiers who developed hemoglobinuria after long marches

Question 27

What was some of the chemical induced hemolytic anemias caused by?

Answer 27

Lead! -slows production of RBC's in marrow Copper

Question 28

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

Answer 28

Malaria - backwater fever - their urine gets REALLY dark

Question 29

Bartonellosis

Answer 29

- Bartonellosis bacilli forms - does not infect, but adheres to RBC membrane - hemolysis is the initial stage of the infection (Oroya fever) - transmitted by sand flea - treatment with Abx and supportive care

Question 30

Babesiosis

Answer 30

- intraerythrocytic protozoa - rodents - "Maltese cross" appearance in RBCs with Giemsa-stained peripheral smears

Question 31

What is a normal hemoglobin level in a woman

Answer 31

About 13 or so -remember that if it's like 6 or something, they will be VERY sick... Probably not walking into the clinic, but carried by a stretcher lol

Question 32

If someone has hemolysis, what will the LDH level look like?

Answer 32

- it will be elevated | - it is normally around 200 or so

Question 33

What will the reticulocyte count do when hemolysis is going on?

Answer 33

It will increase - like up to 4% - not 12 or anything like that

Question 34

What do we look for in the indirect coomb's test?

Answer 34

We look for antibodies that could potentially bind RBCs IN THE SERUM -if it's direct, we are looking on the red blood cell

Question 35

What is characteristic of HUS?

Answer 35

- purpura | - acute kidney injury!

Question 36

Immune mediated hemolytic anemia

Answer 36

- cold reacting antibodies... IgM - simple meant may directly lose RBCs or opsonize RBCs for lysis in the spleen - positive Indirect anti globulin test (coomb's test)

Question 37

Tx of warm antibody hemolysis

Answer 37

- most pts are not in imminent danger | - high dose steroids are the mainstay of therapy

Question 38

WHAT can cause cold agglutinin disease?

Answer 38

Mycoplasma | -walking pneumonia

Question 39

Acute intermittent porphyria AIP

Answer 39

- Scandinavian - deficiency of porphobilinogen deaminase - results in excretion of increased ALA and PBG in urine - symptoms rare before puberty - abdominal pain is most common symptom