Pediatric Anemias (Tucker)

Question 1

Definition of Anemia

Answer 1

Reduction of red cell mass as evidenced by a decrease in hemoglobin or hematocrit

Question 2

Hemoglobin:

Answer 2

the measure of hemoglobin in whole blood, expressed in grams/dl

Question 3

Hematocrit:

Answer 3

fractional volume of red blood cells in whole blood, expressed as a %.
Hematocrit roughly 3 times hemoglobin.

Question 4

Red cell indices of import

Answer 4

RBCs
RDW- differences betw. cell sizes
MCV- avg size
MCHC- important for spherocytosis

Question 5

2 basic ways to classify anemia

Answer 5

1. Physiologic
   - Decreased production with lower than expected retic count and
   - Increased destruction
2. Morphologically
   - Microcytic
   - Normocytic
   - Macrocytic/megaloblastic

Question 6

Some causes of macrocytic anemia

Answer 6

• megaloblastic bone marrow (B12, folate deficiency, medication, hereditary)
• non-megaloblastic (newborn, reticulocytosis, hypothyroidism, liver disease, post-splenectomy, myelodysplastic syndromes, aplastic anemia, dyserythropoietic anemias, down syndrome)

Question 7

Some causes of microcytic anemia

Answer 7

Iron deficiency (blood loss, dietary)
lead
chronic disease
thalassemia
hemobloginopathies
sideroblastic anemias

Question 8

Some causes of normocytic anemias

Answer 8

drugs, renal disease, acute blood loss

infection, medications, splenic pooling

leukemia, aplastic anemia,

hemorrhage, membranopathies, enzymopathies (G6PD, PKD), autoimmune, microangiopathic

Question 9

Evaluation of anemia in children according to mean corpuscular volume

Questions to ask

Answer 9

Important historical questions to ask??
Pallor, lethargy, tachycardia, jaundice, urine color, possible sources of blood loss, stool color, menses, epistaxis. Drug or toxin exposure, possible worm infestation, dietary intake, pica, history of chronic diseases. Prior CBCs other lab work. In infants also note birth history, irritability and poor feeding.

Family history?
Race, ethnicity, anemia, anemia in only males, gallbladder surgery, splenectomy.

Question 10

Evaluation of anemia in children according to mean corpuscular volume- exam and laboratory evaluation

Answer 10

In exam we would look for?
Pallor, icterus, jaundice, organomegaly, Heart murmurs.

Laboratory evaluation?
CBC with smear, retic ount, Coombs test, Total and Direct bilirubin, maybe CMP, lead

Question 11

What is it called when a child eats unusual things?

Answer 11

pika

Question 12

in a black kid you always have to think about

Answer 12

sickle cell

Question 13

houses in philadelphia make you think about

Answer 13

lead poisoning

Question 14

what happens when kids drink too much milk?

Answer 14

not hungry
possible bleeding in the gut
poor intake

–> milk induced iron deficiency

Milk induced iron deficiency anemia. Excess milk intake not only decreases intake of iron rich foods, but may cause microscopic GI bleeding leading to iron deficiency.

Question 15

Iron Deficiency:

what we see in labs

Answer 15

CBC and smear- microcytosis, hypochromia

Iron studies- serum Fe low, ferritin low, TIBC elevated, %saturation low, FEP elevated

Question 16

Chronic Disease:

what we see in labs

Answer 16

CBC and smear- usually normochromic and normocytic, although can have microcytosis
Iron studies- serum Fe low, ferritin elevated, TIBC low, FEP moderately elevated

Question 17

Sideroblastic Anemia:

what we see in labs

Answer 17

CBC and smear- usually microcytosis and hypochromic

Iron studies- serum Fe elevated, FEP normal, TIBC normal to low, ferritin normal to elevated

Question 18

Lead Poisoning

what we see in labs

Answer 18

CBC and smear- basophilic stipling, microcytosis, hypochromia
Iron studies- serum FE is normal to low, TIBC normal, ferritin normal, FEP elevated

Question 19

Thalassemia:

what we see in labs

Answer 19

CBC and smear- microcytosis and hypochromia, target cells
Mentzer index- MCV÷RBC if ≤13 then Thalassemia more likely than Fe deficiency
Iron studies- normal

Question 20

The triad of pallor, jaundice, and splenomegaly raises the suspicion of

Answer 20

a hemolytic process. Other manifestations of hemolysis are dark urine and a change in exercise tolerance. Patients may present with signs of cardiovascular compromise if the hemolytic process is rapid.

Question 21

Autoimmune hemolytic anemic (AHA)

Answer 21

is an acute, self-limited disease in most children.

fever and abdominal pain can be seen i

most common before the fourth birthday and is often associated with an otherwise unremarkable infection

Children older than 10 years and those with an underlying disorder have an increased risk of developing a chronic course.

Question 22

Red blood cell autoantibodies

Answer 22

mediate the hemolytic process. They are classified as either “warm” or “cold,” depending on the temperature at which they are maximally active.

Question 23

dactylitis

Answer 23

most common way that kids with sickle cell present

Question 24

labs you want for sickle dx?

Answer 24

CBC and smear
retic count
sed rate
CRP
blood culture
sickle prep
HB electrophoresis
imaging

Question 25

treatment for sickle cell

Answer 25

hydroxyurea
partial exchange transfusion
bone marrow transplant

Question 26

Complications from sickle cell

Answer 26

vascular- occlusive (triggered by inf, stress, high altitutde; swelling, joint tenderness, elevated WBCs)

Splenic sequestration

aplastic crisis (often due to parvovirus)

Sepsis (often by encapsulated orgs: pneumococcus, meningococcus, H. flu)

acute chest syndrome (infiltrate, fever, tachycardia, chest pain, dyspnea, hypoxia)

Pulmonary hypertension

cardiac- dilated heart, MIs, failure

GI- mesenteric infarcts, liver dysfunction, gallstones

renal- hyposthenuria, enuresis, renal infarcts, priapism (males)

opthalmology: retinopathy

neuro- TIAs, strokes, neurocognitive dysfunction

Question 27

osteomyletis can be caused by what infection?

Answer 27

salmonella

Question 28

Facts about Sickle Cell

Answer 28

RBC lifespan 10-15 days
SS disease most severe, Sickle Beta thalassemia 0 next most severe, Sickle C Disease, Sickle Beta thalassemia +, Sickle E disease less severe
Typical hgb 7-9 with SS, slightly higher with other sickle diseases

Question 29

Thalassemias

Answer 29

Beta Thalassemia HbC, HbD, and HbE all affect beta chains
Beta thalassemia trait, Cooley’s anemia
Alpha Thalassemia affects alpha chains
silent carrier, Thalassemia trait, HbH disease, hydrops fetalis
“The combination of alpha thalassemia with genes for beta chain Hb abnormalities or beta thalassemia results in hematologic diseases that are no more severe than with either trait alone.” – Nelson Textbook of Pediatrics

Question 30

Target cells – what disease?

Answer 30

thalassemia

Question 31

disorder associated with basophilic stippling

Answer 31

lead poisoning

thalassemia can have a little bit