Heme/Onc Part 2

Question 1

What are some signs that platelet issues are causing the bleeding?

Answer 1

Gingival bleeding
Heavy menses
Petechiae

Question 2

How will vWF disease present?

Answer 2

Platelet-type bleeding with either normal labs or slightly impaired PTT

Question 3

What clotting factor may be low with vWF disease and why?

Answer 3

Factor 8
– vWF carries it in the blood

Question 4

Treatment for vWF disease?

Answer 4

DDAVP

Question 5

Where is the issue with Bernard Soulier?

Answer 5

Platelet antigen GP1B
– cannot bind to vWF

Question 6

How will the platelets look with Bernard Soulier?

Answer 6

LARGE

Question 7

Where is the issue with Glanzmann Thrombasthenia?

Answer 7

Platelet antigen GP2B3A
– cannot form platelet plug and aggregate platelets

Question 8

How will the platelets look with Glanzmann Thrombasthenia?

Answer 8

Normal

Question 9

If the issue is with clotting factors, how will that likely present?

Answer 9

Hemarthroses or large hematomas

Question 10

What study should you get if you suspect a clotting factor issue?

Answer 10

Mixing study

Question 11

Following a mixing study, if the PT/PTT do NOT correct, what is the issue?

Answer 11

Factor inhibitors

Question 12

Following a mixing study, if the PT/PTT DO correct, what is the issue?

Answer 12

Factor deficient

Question 13

What causes Paroxysmal Nocturnal Hemoglobinuria?

Answer 13

Lack of CD55/59 on RBC membrane allows the complement to attack and destroy RBCs

Question 14

How will Paroxysmal Nocturnal Hemoglobinuria present?

Answer 14

Hematuria, often in the morning
Hypercoagulable state – intraabdominal clots

Question 15

What is the diagnostic if you suspect Paroxysmal Nocturnal Hemoglobinuria?

Answer 15

Flow cytometry
= ABSENT CD55/59

Question 16

What is the unique treatment for Paroxysmal Nocturnal Hemoglobinuria?

Answer 16

Eculizumab which inhibits complement activation

Question 17

What is the unique treatment for Paroxysmal Nocturnal Hemoglobinuria?

Answer 17

Eculizumab which inhibits complement activation

Question 18

With G6PD deficiency, hemolytic anemia occurs with oxidative stress. List 3 common drugs that cause this to occur?

Answer 18

TMP-SMX
Dapsone
Nitrofurantoin

Question 19

How will G6PD present?

Answer 19

Fatigue
Pallor
Dark urine
Jaundice

Question 20

What 2 things can be seen on peripheral smear with G6PD defiency?

Answer 20

Bite cells
Heinz bodies

Question 21

If you measure G6PD levels during an acute attack, what will the levels be?

Answer 21

NORMAL

Question 22

What occurs with TTP?

Answer 22

ADAMTS-13 deficiency forms hyaline clots that then shred cells going by!

Question 23

What will be seen on peripheral smear with TTP?

Answer 23

Schistocytes

Question 24

What is the pneumonic for TTP symptoms?

Answer 24

FAT RN

Question 25

TTP symptoms?

Answer 25

FAT RN - Fever - Anemia (MAHA) - Thrombocytopenia - Renal failure - Neuro sx

Question 26

TTP symptoms?

Answer 26

FAT RN - Fever - Anemia (MAHA) - Thrombocytopenia - Renal failure - Neuro sx

Question 27

What is the treatment for TTP?

Answer 27

Exchange transfusion

Question 28

Physiology behind DIC?

Answer 28

Illness causes clots to form everywhere and since using up clotting factors, you also bleed

Question 29

Level of PT/PTT and D-Dimer with DIC?

Answer 29

HIGH

Question 30

Level of Platelets and Fibrinogen with DIC?

Answer 30

LOW

Question 31

PT/PTT, Platelets, Fibrinogen and D-Dimer levels with DIC?

Answer 31

PT/PTT = HIGH Platelets = LOW Fibrinogen = LOW D-Dimer = HIGH

Question 32

Treatment for DIC?

Answer 32

Supportive, treat underlying cause

Question 33

If a patient is on heparin and they develop HIT, what will occur?

Answer 33

Bleeding + CLOT formation while on heparin

Question 34

Signs of HIT?

Answer 34

Recently started on Heparin - Bleeding - CLOT WORSENING

Question 35

What is the treatment for HIT?

Answer 35

Stop heparin -- Start Argatroban and bridge to Warfarin

Question 36

What occurs with ITP?

Answer 36

Autoimmune attack against platelets ONLY

Question 37

What lab change is seen with ITP?

Answer 37

Low platelets only

Question 38

If the platelets are below _____ with ITP, give steroids

Answer 38

< 30K give steroids

Question 39

If the platelets are above 30K, what is the treatment for ITP?

Answer 39

NOTHING just observe

Question 40

Hemolytic anemia lab changes

Answer 40

LOW haptoglobin HIGH bilirubin and LDH