Anemic/Clotting Disorders

Question 1

How do Thalassemias lead to anemia?

Answer 1

LOW RBC COUNT via inefficient erythropoesis and increased hemolysis of defective RBCs containing excess globins (alpha, beta, gamma tetramers).

Question 2

Where are red blood cells hemolyzed?

Answer 2

Predominately in the spleen (liver and bone marrow also)!

Question 3

Where is the spleen located? What kind of pain can be associated with it? (2)

Answer 3

LUQ, anterior to stomach. Abdominal pain (stomach). Upper shoulder (upon deep breath)

Question 4

What can cause hepatosplenomegaly?

Answer 4

Increased RBC hemolysis. Increased production of RBCs (extramedullary hematopoesis?).

Question 5

When a RBC is lysed, released hemoglobin becomes heme. What does heme break down to? What can that lead to clinically?

Answer 5

Indirect Bilirubin –> Jaundice, scleral icterus, gallstones

Iron –> 2ndary Hemochromatosis

Question 6

What stimulates extramedullary hematopoesis?

Answer 6

Low RBC’s will lead to low O2 delivery. This will make the body think it’s in a hypoxic state, releasing EPO and stimulating extramedullary hematopoesis.

Question 7

How can intravascular hemolysis affect urine?

Answer 7

Hematuria, hemoglobinuria, dark red urine.

Question 8

Between intravascular and extravascular hemolysis, which will affect urine?

Answer 8

Intravascular Hemolysis

Question 9

In what type of vasculature does HgbS commonly cause vaso-occlusion? What could that lead to?

Answer 9

Cerebral –> Stroke

Respiratory –> Acute Chest Syndrom (ACS)

Renal –> hematuria, proteinuria

Penile –> Priapism

Extremities–> Dactylis

Question 10

What risks do chronic blood transfusions carry?

Answer 10

2ndary hemochromatosis or allogeneic immune response

Question 11

What is the purpose of a (Direct) Coomb’s Test?

Answer 11

A test that is used to detect hemolytic antibodies or complement proteins already bound to RBCs.

Question 12

What is an indirect Coomb’s test?

Answer 12

A test for detecting unbound anti-RBC antibodies in the serum.

Question 13

How would haptoglobin levels be affected by intravascular hemolysis? extravascular hemolysis?

Answer 13

intravascular –> low haptoglobin levels (more free Hgb in blood to bind)

extravascular –> somewhat normal haptoglobin levels (less free Hgb in blood to bind)

Question 14

What type of hemolysis (extra, intra?) will Sickle Cell Disease lead to?

Answer 14

Extravascular hemolysis. HbS will be eaten up by spleen.

Question 15

What enzymatic reaction does aminolevulinate dehydratase catalyze?

Answer 15

aminolevulinic acid to porphobilinogen

Question 16

Bloody Diarrhea is indicative of what type of virus?

Answer 16

E.coli, shiga toxin.

Question 17

What is the function of ADAMTS13?

Answer 17

To cleave and inactivate vWF.

Question 18

What is a major difference between HUS and TTP?

Answer 18

TTP can have arterial blockage to the brain as well as the kidneys, causing neurologic symptoms. HUS has arterial blockage primarily in the kidney, no neurologic symptoms.

Question 19

What abnormality will a peripheral smear show you in immune thrombocytopenic purpura (ITP)?

Answer 19

decreased platelet count

Question 20

Patients with a splenectomy or splenic dysfunction are susceptible to infection because…?

Answer 20

Decreased production of IgM and memory B-cells.

parvovirus B19

Question 21

How can sickle cell affect the spleen?

Answer 21

Spleen can become dysfunctional.

Question 22

What long-term prophylactic is usually given after a patient receives Heparin for suspected embolism?

Answer 22

Warfarin

Question 23

What is a big indication in which you should you suspect lead poisoning?

Answer 23

A person’s WORKPLACE/LIVING SITUATION is mentioned. Do they work/live where lead/metal exposure is a risk factor?

Question 24

What are good distinguishers of Paroxysmal Nocturnal Hemoglobinuria? (3)

Answer 24

Anemia, Venous Thrombosis, and PANCYTOPENIA!

Question 25

Stillbirths can be attributed to which clotting disorder?

Answer 25

Antiphospholipid Syndrome.