Pediatric Anemias (Tucker) Flashcards
Definition of Anemia
Reduction of red cell mass as evidenced by a decrease in hemoglobin or hematocrit
Hemoglobin:
the measure of hemoglobin in whole blood, expressed in grams/dl
Hematocrit:
fractional volume of red blood cells in whole blood, expressed as a %.
Hematocrit roughly 3 times hemoglobin.
Red cell indices of import
RBCs
RDW- differences betw. cell sizes
MCV- avg size
MCHC- important for spherocytosis
2 basic ways to classify anemia
- Physiologic
- Decreased production with lower than expected retic count and
- Increased destruction - Morphologically
- Microcytic
- Normocytic
- Macrocytic/megaloblastic
Some causes of macrocytic anemia
- 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)
Some causes of microcytic anemia
Iron deficiency (blood loss, dietary) lead chronic disease thalassemia hemobloginopathies sideroblastic anemias
Some causes of normocytic anemias
drugs, renal disease, acute blood loss
infection, medications, splenic pooling
leukemia, aplastic anemia,
hemorrhage, membranopathies, enzymopathies (G6PD, PKD), autoimmune, microangiopathic
Evaluation of anemia in children according to mean corpuscular volume
Questions to ask
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.
Evaluation of anemia in children according to mean corpuscular volume- exam and laboratory evaluation
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
What is it called when a child eats unusual things?
pika
in a black kid you always have to think about
sickle cell
houses in philadelphia make you think about
lead poisoning
what happens when kids drink too much milk?
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.
Iron Deficiency:
what we see in labs
CBC and smear- microcytosis, hypochromia
Iron studies- serum Fe low, ferritin low, TIBC elevated, %saturation low, FEP elevated
Chronic Disease:
what we see in labs
CBC and smear- usually normochromic and normocytic, although can have microcytosis
Iron studies- serum Fe low, ferritin elevated, TIBC low, FEP moderately elevated
Sideroblastic Anemia:
what we see in labs
CBC and smear- usually microcytosis and hypochromic
Iron studies- serum Fe elevated, FEP normal, TIBC normal to low, ferritin normal to elevated
Lead Poisoning
what we see in labs
CBC and smear- basophilic stipling, microcytosis, hypochromia
Iron studies- serum FE is normal to low, TIBC normal, ferritin normal, FEP elevated
Thalassemia:
what we see in labs
CBC and smear- microcytosis and hypochromia, target cells
Mentzer index- MCV÷RBC if ≤13 then Thalassemia more likely than Fe deficiency
Iron studies- normal
The triad of pallor, jaundice, and splenomegaly raises the suspicion of
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.
Autoimmune hemolytic anemic (AHA)
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.
Red blood cell autoantibodies
mediate the hemolytic process. They are classified as either “warm” or “cold,” depending on the temperature at which they are maximally active.
dactylitis
most common way that kids with sickle cell present
labs you want for sickle dx?
CBC and smear retic count sed rate CRP blood culture sickle prep HB electrophoresis imaging
treatment for sickle cell
hydroxyurea
partial exchange transfusion
bone marrow transplant
Complications from sickle cell
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
osteomyletis can be caused by what infection?
salmonella
Facts about Sickle Cell
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
Thalassemias
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
Target cells – what disease?
thalassemia
disorder associated with basophilic stippling
lead poisoning
thalassemia can have a little bit
