Hematology

Question 1

Pt with mulitple history complaints of non-traumatic swollen/painful joints, not otherwise caused by arthritis is likely to be caused by?

Answer 1

Bleeding disorder - hemarthrosis
Nutritional deficiency - vitamin c or k
Toxins

Question 2

Normal range of von willebrand antigen?

Answer 2

50-200

Question 3

Hemophilia A is a deficiency in factor?

Answer 3

Factor 8

Question 4

The most common Hemophilia in Ashkenazi Jews?

Answer 4

Hemophilia C - Factor 11

Question 5

The most common bleeding disorder in the US?

Answer 5

Von Willebrand disease
*deficiency in ADAMT3 which keeps Willy in check – no clotting happens

Question 6

Prolonged PTT with normal prothrombin time is most likely what bleeding disorder?

Answer 6

Hemophillia A - deficiency in factor VIII (8)

PT measures - 1, 2, 5, 6, 10
PTT measures - 1, 2, 5, 8, 9, 10, 11, 12

**elevated PTT and normal PT = hemophilia
**elevated PT/PTT and normal platelets+ vw antigen = vwd

Question 7

Tx of vwd?

Answer 7

Desmopressin

Question 8

Tx of Hemophilia A?

Answer 8

Factor 8 substitute
Desmopressin or antifibrinolytic only if mild/mod bleeding

antifib: tranexamic acid, aminocaproic acid

Question 9

Most common cause of vitamin K deficiency?

Answer 9

Parenteral nutrition and broad spectrum abx

Question 10

Factor V Leiden is defined as?

Answer 10

Clotting disorder that is genetically inherited – negative feedback is malfunctioning bc of elevated activated protein C which turns off factor V (factor V = negative feedback for stopping clotting)

ALWAYS REMEMBER: Factor V Leiden means chance of DVT!

Question 11

Factor V Leiden is most concerning in what population?

Answer 11

Pregnancy - abruptio placentae, fetal growth disturbances

Question 12

Factor V Leiden tx?

Answer 12

Fresh frozen plasma daily during active bleeding
Oral anticoag long term if thrombosis history
Compression stockings

Question 13

TOC for DIC?

Answer 13

transfusion - plasma, platelets, cryoprecipitate
heparin to prevent clots
fluid replacement

Question 14

Patient presenting with history of venous/arterial thrombi formation, labs showing thrombocytopenia, and recurrent fetal loss that often occurs after week 10 is most likely to have what hematologic disorder?

Answer 14

Clotting disorder - Antiphospholipid antibody syndrome (increased coag activity)

Question 15

Lab values of thrombocytopenia, anti-b2 glycoprotein antibodies, and anticardiolipn antiboides indicate?

Answer 15

Antiphospholipid antibody syndrome - disorder where antiphos antibodies attack phospholipid which then leads to excess clotting

Question 16

TOC for antiphospholid antibody syndrome?

Answer 16

Heparin or warfarin acutely during thrombotic events
Immune suppression for refractory cases