Medicine (Hematologic)

Question 1

What is anemia?

Answer 1

-Decrease in red blood cells leading to less oxygen carrying capacity and delivery to end organs. Can be classified as macrocytic, nomocytic, microcytic

Question 2

What are examples of microcytic, normocytic and macocytic anemias?

Answer 2

-Microcytic: Iron deficiency, thalassemia, lead poisoning

-Normocytic: Acute blood loss, myelodysplastic syndroms, anemia of chronic disease, hemolytic anemias

-Macrocytic: Folate deficiency, vitamin B12 deficiency, alcoholism, drug toxicity

Question 3

What is alpha-thalassemia?

Answer 3

-Autosomal recessive disease: Decrease in alpha-globin chain of Hemoglobin
-Varying degrees based on how many chains are affected
-1-2 deletions mild anemia with no treatment or symptoms
-3 deletions cause severe hemolytic anemia
-4 deletions not compatible with life

Question 4

What is beta thalassemia?

Answer 4

Autosomal recessive disease: Decrease in beta-globin chain of hemaglobin. Common in Greeks
-Mild form: No treatment
-Severe form: Hemolytic anemia. No HbA. Need for transfusion

Question 5

What is pernicious anemia?

Answer 5

-Decrease in intrinsic factor due to autoimmune destruction of gastric parietal cells. Causes vitamin B12 deficiency and macrocytic anemia
-Macrocytic anemia

Question 6

What is sickle cell anemia?

Answer 6

-Autosomal recessive disorder: Intrinsic defect causing extravascular hemolysis of sickled cells. Structural change of hemaglobin due to mutation.

-Severe hemolytic anemia, vaso-occlusive crises (acute chest pain, autosplenectomy, increased risk of infection from encapsulated organisms)

-Treat with hydroxyurea (increases HbF which prevents sickling and bone marrow transplant

Question 7

What is leukemia?

Answer 7

-Disease of the mesenchymal cells of the blood with abnormality in proliferation and increased life span of myeloid or lymphoid cells. Can be classified as acute vs chronic, myeloid vs lymphoid.

Question 8

What are important characteristics of AML?

Answer 8

-Acute myelogenous leukemia
-Most common leukemia in adults
-Gingival infiltrates
-Thrombocytopenia

Question 9

What are important characteristics of CML?

Answer 9

-Chronic myelogenous leukemia
-Less common
-Philadelphia chromosome: Chromosome 22,9 BCR gene
-More indolent

Question 10

What are important characteristics of ALL?

Answer 10

-Acute lymphocytic leukemia
-Most common childhood leukemia
-Cure rate 2/3, even higher in children

Question 11

What are important characteristics of CML?

Answer 11

-Most common overall
-No cure, goal is to prolong life. Stage with Rai system

Question 12

What is hemophilia?

Answer 12

-X-linked recessive bleeding disorder. Type A and B

Question 13

What is hemophilia A?

Answer 13

-Congenital deficiency in clotting factor VIII on X chromosome

Question 14

What is hemophilia B?

Answer 14

-Congenital deficiency in clotting factor IX

Question 15

How is hemophilia A and B classified?

Answer 15

-Severe: Factor levels below 1%, spontaneous bleeds occur
-Moderate: Factor levels between 1-5%. Prolonged bleeding
-Mild: Factor levels above 5%. Prolonged bleeding

Question 16

How is hemophilia A and B managed for outpatient dental procedures?

Answer 16

-Recombinant factor concentrates of VIII or XI. Raise levels to 80-100% and maintain above 50%
-Factors with short half life, should be administered 20 minutes post-op

-TXA (antifibrinolytic agent)
-DDAVP (for Hemophilia A, raises factor VIII and vWF)

Question 17

What is von Willebrand disease?

Answer 17

-Congenital platelet bleeding disorder due to a deficiency or dysfunction of von Willebrand factor. Typically autosomal dominant
-Classified based on quantitative (type I and most common) or qualitative type II

Question 18

How is von willebrand’s disease managed for surgical procedures?

Answer 18

Depends on type:

-Type I: DDAVP (Desmopressin)
-IV: 0.3 micrograms/kg over 20 minutes
-IN: 150 microgram spray
-Need to monitor free water

-Type 2-3: Cryoprecipitate, factor VIII, amicar

Question 19

What is factor V Leiden?

Answer 19

-Autosomal dominant mutation resulting in resistance to activated protein C
-Hypercoagulable state
-Need DVT prophylaxis

Question 20

What is lymphoma?

Answer 20

-Malignancy of lymphocytes that arises in lymph nodes.
-Types are Hogkin and non- hodgkin lymphoma)

Question 21

What is multiple myeloma?

Answer 21

-A malignancy characterized by neoplastic transformation of plasma cells
-Pt may be on anti-resorptive agents

Question 22

How does a transfusion of PRBCs affect CBC labs?

Answer 22

-Each unit increases hemoglobin by 1 g/dL and hematocrit by 3%

Question 23

How does a transfusion of platelets affect labs?

Answer 23

-Increases platelet count by 5-10k (50k minimum for surgery)