15 - Hematologic Flashcards

1
Q

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

A

12 mos

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2
Q

What does hypochromic, microcytic anemia signify?

A

Inadequate production of hemoglobin

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3
Q

What does normocytic anemia signify?

A

Systemic illness that impairs adequate marrow synthesis of RBCs

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4
Q

What does macrocytic anemia signify?

A

Vitamin B12 and folic acid deficiency

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5
Q

Elevated reticulocyte count suggests:

A

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

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6
Q

Common causes of microcytic anemia - FLATS:

A

Fe (Iron)-deficiency anemia

Lead poisoning

Anemia of chronic disease

Thalassemia

Sideroblastic anemia (rare)

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7
Q

What is the MCC of anemia worldwide?

A

Iron deficiency

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8
Q

Txt for IDA:

A

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

Txt for 3-4 mos

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9
Q

Peripheral smear of pt with lead poisoning shows:

A

Hypochromic, microcytic anemia

Basophilic stippling

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10
Q

What serum lead level requires environmental investigation?

A

20micrograms/dL

Though even 5-10 may cause learning problems

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11
Q

Txt for lead poisoning

A

Chelation therapy

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12
Q

MC infectious agent that causes pancytopenia?

A

Parvovirus B19

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13
Q

DDx for macrocytic anemia (FAT rBC)

A

Folate deficiency

Alcohol

Thyroid (hypo)

B12 deficiency

Cirrhosis / liver disease

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14
Q

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

A

Two alpha, two beta

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15
Q

How does alpha thalassemia trait present?

A

Mild microcytic anemia, decreased MCV, decreased H/H

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16
Q

How does alpha thalassemia Hgb H disease present?

A

3 gene mutations

Microcytic anemia and mild hemolysis

Folic acid supplementation, maybe occasional transfusion during crisis

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17
Q

RF for sickle cell crisis?

A
Hypoxia
Fever
Acidosis
Hypothermia
Dehydration
18
Q

Sickle cell crisis leads to:

A

Thrombosis
Sequestration
Infarction

19
Q

Why are sickle cell patient’s prone to infection?

A

Splenic dysfunction

Susceptible to encapsulated bacteria

20
Q

Long-term sickle cell management?

A

Hydroxyurea

Stem cell transplan (can be curative)

Daily prophylactic oral PCN

Vaccinations

Folate

21
Q

What happens with G6PD deficiency?

A

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

22
Q

Triggers of G6PD crisis:

A

Fava beans

Sulfa drugs

ASA

Primaquine

Nitrofurantoin

Serious infection

23
Q

Bite cells and Heinz Bodies, think:

A

G6PD

24
Q

Thrombocytopenia =

A

Platelet count <150K/mm3

**if under 20K, risk for spontaneous bleeding

25
Q

What is the MC childhood bleeding disorder?

A

Idiopathic thrombocytopenia purpura

26
Q

Clinical presentation of ITP:

A

Young kids, 1-4 weeks after viral infection

Abrupt onset petechiae, purpura, epistaxis, bleeding gums, hematuria

27
Q

Txt for severe ITP?

A

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

28
Q

Hemophilia A

A

Factor VIII deficient

29
Q

Hemophilia B

A

Factor IX deficient

30
Q

Hemophilia presentation

A

Bleeding c minor trauma
Hemarthrosis
Spontaneous bleeding

31
Q

Dx of hemophilia

A

Prolonged PTT that corrects on mixing study

32
Q

Txt for hemophilia

A

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

33
Q

What is the MC congenital bleeding disorder?

A

Von Willebrand Disease

vWF bridges platelets and subendothelial collagen

34
Q

Henoch-Schonlein Purpura

A

HSP

Unknown etiology

Ages 3-15

50% of cases follow URI’s

Small vessel inflammation, leukocytic infiltration, hemorrhage, ischemia, IgA immune complex deposition -> glomerulonephritis

35
Q

Palpable purpura, arthralgia, crampy abdominal pain, think:

A

HSP

36
Q

Txt for HSP

A

Supportive

Follow renal function

NDSAIDs for arthritis

Steroids for GI disease and nephritis

37
Q

Acute phase of Kawasaki

A
High fever
Conjunctival erythema, cracked lips, strawberry tongue
Cervical LAD
Scarlatina-like rash
Hand-feet swelling
38
Q

Subacute phase of Kawasaki

A

Gradual fever resolution
Skin desquamation
High platelets
CORONARY ARTERY ANEURYSM

39
Q

TXT for Kawasaki

A

IVIG (goal - coronary aneurysm prevention)

ASA (high dose, then reduce dose)(Reyes risk acceptable)

40
Q

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

A

Aplastic crisis

41
Q

Pyruvate kinase (PK) deficiency

A

ATP depletion impairs RBC survival

Rigid RBCs

Hemolysis -> anemia and jaundice

Txt - neonates -> exchange transfusion; splenectomy