Hematology

Question 1

Anemia is defined as __________ or ________

Answer 1

Low hemoglobin

Low hematocrit

Question 2

Symptoms of anemia are a result of ___________

Answer 2

Decreased oxygen delivery to tissues

Question 3

Core symptoms of all types of anemia

Answer 3

Fatigue
Dyspnea on exertion
Palpitations
If severe:
Syncope and chest pain

Question 4

Types of macrocytic anemia (MCV > 100)

Answer 4

B12 Deficiency
Folate Deficiency
Alcoholism
Hypothyroidism
Liver disease

Question 5

Types of microcytic anemia (MCV < 80)

Answer 5

Iron deficiency
Thalassemia
Anemia of chronic dz
Sideroblastic anemia

Question 6

Types of normocytic anemia (MCV 80-100)

Answer 6

Blood loss
Hemolysis
Anemia of chronic dz

Question 7

How does a pt with vitamin B12 deficiency present?

Answer 7

Neurological symptoms

Peripheral neuropathy most common

Question 8

Lab values for vitamin B12 deficiency

Answer 8

Low B12 level
High MMA
May also see: elevated iron, indirect bilirubin and LDH

Question 9

Vitamin B12 deficiency can be secondary to medication such as:

Answer 9

Metformin
PPis
Cimetididne

Question 10

Treatment for vitamin B12 deficiency

Answer 10

Vitamin B12 replacement

IM preferred if severe neurological problems

Question 11

Treatment for folate deficiency

Answer 11

Folic acid

Question 12

A patient with iron deficiency will present with

Answer 12

Blood loss

GI or menses

Question 13

Lab values for iron deficiency

Answer 13

Low serum iron
Low ferritin
High total iron binding capacity
High RDW
Elevated platelet count

Question 14

Treatment for iron deficiency

Answer 14

Always look for a cause!

Ferrous sulfate

Question 15

Complications of ferrous sulfate

Answer 15

Can turn stools black but will have negative guaiac stools

Question 16

Thalassemia is characterized by hemoglobin having reduced

Answer 16

Alpha or beta chains

Question 17

Thalassemia will have _________ or __________ on peripheral smear

Answer 17

Target cells

Tear-drop shaped cells

Question 18

Beta thalassemia is diagnosed with

Answer 18

Electrophoresis

Question 19

Treatment for beta thalassemia

Answer 19

Transfusions

Iron chelation

Question 20

Lab values for anemia of chronic disease

Answer 20

Low serum iron, low iron binding capacity, normal ferritin

Question 21

Most common cause of hemolysis normocytic

Answer 21

Sickle cell disease

Question 22

Ultimate complications of anemia

Answer 22

Arrhythmias
MI
CHF

Question 23

Malignancy of lymphoid stem cells in bone marrow, migrating to the lymph nodes, spleen, liver and other organs

Answer 23

Acute Lymphocytic Leukemia (ALL)

Question 24

Most common childhood malignancy, peaking between ages 3-7 y/o

Answer 24

Acute lymphocytic leukemia (ALL)

Question 25

Children with _____________ have increased rates of ALL

Answer 25

Down syndrome

Question 26

Signs/symptoms of ALL

Answer 26

1. Pancytopenia symptoms: fever, fatigue, lethargy. Bone pain 2. CNS symptoms: HA, neck stiffness, visual changes, vomiting

Question 27

Physical exam with ALL

Answer 27

Pallor, fatigue, petechiae, bruising, hepatosplenomegaly, lymphadenopathy, +/- mediastinal mass

Question 28

Diagnosis of ALL

Answer 28

Bone marrow - hypercellular with >20% blasts, anemia, thrombocytopenia

Question 29

Management of ALL

Answer 29

``` Oral chemo (> 90% remission) Stem cell transplant if relapse ```

Question 30

Most common inherited bleeding disorder (autosomal dominant)

Answer 30

Von Willebrand Disease

Question 31

Characterized as a defect or deficiency in a factor that helps platelets stick to sites of endothelial injury

Answer 31

Von willebrand disease

Question 32

Signs/symptoms of von willebrand diseasee

Answer 32

Bleeding Gingival bleeding, epistaxis, menorrhagia and skin bruising NSAIDs/Aspirin may precipitate an episode

Question 33

Diagnosis of von willebrand disease

Answer 33

Normal platelet count Normal PT Von willebrand factor antigen - decreased Von willebrand factor activity - decreased Factor VIII activity - decreased If all abnormal, perform RIPA

Question 34

First line treatment for von willebrand disease

Answer 34

Desmopressin | Second line: von willebrand factor concentrate

Question 35

Reed sternberg cells pathognomonic - B cell proliferation with bilobed or multilobar nucleus "owl eye"

Answer 35

Hodgkin's Disease Lymphoma

Question 36

Lymph node involvement with Hodgkin's Disease lymphoma

Answer 36

Upper lymph node involvement | Neck, axilla, shoulder, chest (mediastinum)

Question 37

With Hodgkin's disease lymphoma, sometimes you will see pts with painful lymph nodes with

Answer 37

EtOH consumption | Although pts present with painless lymph nodes usually

Question 38

Associated symptoms with Hodgkin's Disease lymphoma

Answer 38

Fever Weight loss Anorexia Night sweats

Question 39

Pel Ebstein Fever

Answer 39

Intermittent cyclical fevers x 1-2 weeks | Hodgkin's lymphoma

Question 40

40% of Hodgkin's Lymphoma is associated with

Answer 40

Epstein-Barr Virus

Question 41

Hodgkin's Lymphoma has an __________ 5 year cure

Answer 41

Excellent

Question 42

Group of inherited (x-linked recessive [male]) bleeding disorders

Answer 42

Hemophilia

Question 43

Factor VIII Deficiency

Answer 43

Hemophilia A

Question 44

Factor IX Deficiency

Answer 44

Hemophilia B or Christmas disease

Question 45

Signs/symptoms of hemophilia

Answer 45

Pt who is bleeding into joints, muscles, or GI tract. First episode can occur anywhere from first few months of life up until age 4

Question 46

Diagnosis of hemophilia

Answer 46

Normal platelet count Normal PT Prolonged aPTT When pts plasma combined with normal plasma, aPTT will correct itself Confirmation done by ordering specific assay

Question 47

Treatment of hemophilia

Answer 47

Hemophilia A - Desmopressin Hemophilia B - specific clotting factor Factor XI Deficiency - no prophylactic therapy needed

Question 48

Lead poisoning causes cell death, shortens the lifespan of RBCs and leads to:

Answer 48

Acquired sideroblastic anemia

Question 49

Signs/symptoms of lead poisoning

Answer 49

``` Abdominal pain with constipation Neurologic symptoms (ataxia, fatigue, learning disabilities, coma, shock) Anemia symptoms, metabolic acidosis ```

Question 50

Diagnosis of lead poisoning

Answer 50

1. Increased serum lead, increased serum Fe, decreased TIBC, increased ferritin 2. Peripheral smear: microcytic, hypochromic anemia with basophilic stippling 3. Ringed sideroblasts in bone marrow 4. XR: lead lines at metaphyseal plates

Question 51

Management of lead poisoning

Answer 51

Remove source of lead. Chelation therapy may be needed if severe (Succimer)