15 - Hematologic

Question 1

At what age does AAP recommend to get an H/H in a baby?

Answer 1

12 mos

Question 2

What does hypochromic, microcytic anemia signify?

Answer 2

Inadequate production of hemoglobin

Question 3

What does normocytic anemia signify?

Answer 3

Systemic illness that impairs adequate marrow synthesis of RBCs

Question 4

What does macrocytic anemia signify?

Answer 4

Vitamin B12 and folic acid deficiency

Question 5

Elevated reticulocyte count suggests:

Answer 5

Appropriate bone marrow response to anemia (hemolysis or blood loss)

Question 6

Common causes of microcytic anemia - FLATS:

Answer 6

Fe (Iron)-deficiency anemia

Lead poisoning

Anemia of chronic disease

Thalassemia

Sideroblastic anemia (rare)

Question 7

What is the MCC of anemia worldwide?

Answer 7

Iron deficiency

Question 8

Txt for IDA:

Answer 8

4-6mg/day elemental iron -> 1% increase in HCT per day

Txt for 3-4 mos

Question 9

Peripheral smear of pt with lead poisoning shows:

Answer 9

Hypochromic, microcytic anemia

Basophilic stippling

Question 10

What serum lead level requires environmental investigation?

Answer 10

20micrograms/dL

Though even 5-10 may cause learning problems

Question 11

Txt for lead poisoning

Answer 11

Chelation therapy

Question 12

MC infectious agent that causes pancytopenia?

Answer 12

Parvovirus B19

Question 13

DDx for macrocytic anemia (FAT rBC)

Answer 13

Folate deficiency

Alcohol

Thyroid (hypo)

B12 deficiency

Cirrhosis / liver disease

Question 14

Normal Hgb (Hgb A) is made of which subunits:

Answer 14

Two alpha, two beta

Question 15

How does alpha thalassemia trait present?

Answer 15

Mild microcytic anemia, decreased MCV, decreased H/H

Question 16

How does alpha thalassemia Hgb H disease present?

Answer 16

3 gene mutations

Microcytic anemia and mild hemolysis

Folic acid supplementation, maybe occasional transfusion during crisis

Question 17

RF for sickle cell crisis?

Answer 17

Hypoxia
Fever
Acidosis
Hypothermia
Dehydration

Question 18

Sickle cell crisis leads to:

Answer 18

Thrombosis
Sequestration
Infarction

Question 19

Why are sickle cell patient’s prone to infection?

Answer 19

Splenic dysfunction

Susceptible to encapsulated bacteria

Question 20

Long-term sickle cell management?

Answer 20

Hydroxyurea

Stem cell transplan (can be curative)

Daily prophylactic oral PCN

Vaccinations

Folate

Question 21

What happens with G6PD deficiency?

Answer 21

Free radicals damage/oxidize Hgb, causing Hgb precipitation (HEINZ BODIES) -> RBC membrane fragility -> hemolysis

Question 22

Triggers of G6PD crisis:

Answer 22

Fava beans

Sulfa drugs

ASA

Primaquine

Nitrofurantoin

Serious infection

Question 23

Bite cells and Heinz Bodies, think:

Answer 23

G6PD

Question 24

Thrombocytopenia =

Answer 24

Platelet count <150K/mm3

**if under 20K, risk for spontaneous bleeding

Question 25

What is the MC childhood bleeding disorder?

Answer 25

Idiopathic thrombocytopenia purpura

Question 26

Clinical presentation of ITP:

Answer 26

Young kids, 1-4 weeks after viral infection Abrupt onset petechiae, purpura, epistaxis, bleeding gums, hematuria

Question 27

Txt for severe ITP?

Answer 27

IVIG Prednisone IV-Anti-D (only in Rh (+) patients) Splenectomy for life-threatening bleeding

Question 28

Hemophilia A

Answer 28

Factor VIII deficient

Question 29

Hemophilia B

Answer 29

Factor IX deficient

Question 30

Hemophilia presentation

Answer 30

Bleeding c minor trauma Hemarthrosis Spontaneous bleeding

Question 31

Dx of hemophilia

Answer 31

Prolonged PTT that corrects on mixing study

Question 32

Txt for hemophilia

Answer 32

Desmopressin (DDAVP) for hemophilia A (no effect on factor IX)

Question 33

What is the MC congenital bleeding disorder?

Answer 33

Von Willebrand Disease vWF bridges platelets and subendothelial collagen

Question 34

Henoch-Schonlein Purpura

Answer 34

HSP Unknown etiology Ages 3-15 50% of cases follow URI’s Small vessel inflammation, leukocytic infiltration, hemorrhage, ischemia, IgA immune complex deposition -> glomerulonephritis

Question 35

Palpable purpura, arthralgia, crampy abdominal pain, think:

Answer 35

HSP

Question 36

Txt for HSP

Answer 36

Supportive Follow renal function NDSAIDs for arthritis Steroids for GI disease and nephritis

Question 37

Acute phase of Kawasaki

Answer 37

``` High fever Conjunctival erythema, cracked lips, strawberry tongue Cervical LAD Scarlatina-like rash Hand-feet swelling ```

Question 38

Subacute phase of Kawasaki

Answer 38

Gradual fever resolution Skin desquamation High platelets CORONARY ARTERY ANEURYSM

Question 39

TXT for Kawasaki

Answer 39

IVIG (goal - coronary aneurysm prevention) ASA (high dose, then reduce dose)(Reyes risk acceptable)

Question 40

What happens if a sickle cell patient get Fifth Disease (Parvovirus B19)?

Answer 40

Aplastic crisis

Question 41

Pyruvate kinase (PK) deficiency

Answer 41

ATP depletion impairs RBC survival Rigid RBCs Hemolysis -> anemia and jaundice Txt - neonates -> exchange transfusion; splenectomy