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Flashcards in BRS- Heme Deck (114)
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1

At what point in life is the physiologic nadir of hemoglobin concentration?
Time of HbF disappearance?

2-3 months= nadir
6-9 months= HbF disappearance

2

How common is anemia in kids?

20% US kiddos
80% worldwide kiddos

3

Reticulocyte count reflects _____.
Normal percent blood count made up of retics?

number of immature RBCs/ activity of bone marrow
normally 1%.

4

2 Most common types of microcytic, hypochromic anemia in kids? 3 others?

#1: IDA
#2: B thal
(also: lead, sideroblastic, chronic dz)

5

Causes of IDA in kiddos

-lack of dietary iron
-early ingestion of cows milk
-occult blood loss
-menstruation

6

2 weird key symptoms of anemia

spoon shaped nails
diminished attention
also paleness, fatigue etc

7

Labs assc with IDA:

-low ferritin (early)
-increased transferrin
-decreased transferrin saturation
-increased free erythrocyte protoporphyrin

8

3 causes of macrocytic anemia

-B12
-folate
-thiamine
deficiencies

9

Normocytic normochromic anemia with low retic counts

-malignancy
-fanconis
-red cell aplasias

10

Three types of red cell aplasia

TEC
Diamond Blackfan
Parvo B19

11

When should workup to determine cause of anemia begin?

When anemia is not responsive to iron therapy.

12

HbA1 makeup

A2B2

13

PE finding in thalassemias

-increased size of bone in the face (chipmunk facies)
-increased size of bone in the skull (crew cut)

14

What populations are predisposed to a/b thal?

A: Asians
B: Meditteraneans

15

How many types of A thal exist?
B thal?

A thal- 4
B thal- 2
(4 alleles exist for A, 2 for B)

16

What are the two most severe types of a thal?

-HbH disease, some Hb Barts present, which binds O2 very tightly
-Fetal Hydrops, only Hb Barts present, not compatible with life.

(BARTS BABIES BAD!)

17

Labs assc with B Thal

-increased HbF
-low HbA1
-target cells
-high bili/LDH (hemolysis)

18

Complication assc with thalassemia treatment and how to prevent it?

-hemochromatosis due to chronic transfusion
-prevent with deferoxamine

19

Iron level in B thal minor

-normal to high

20

Sideroblastic anemia:
basic pathologic cause

iron in the mitochondria

21

4 causes of of acquired sideroblastic anemia

-lead
-isoniazid
-alcohol
-chloramphenicol

22

B12:
-cogactor for absorption + source
-site of absorption

-intrinsic factor, gastric parietal cells
-terminal ileum

23

2 weird manifestations of B12 def

-beefy red tongue
-neuro findings

24

Three classes of normocytic anemia + how to distinguish them?

-hemolytic (high retics)
-aplasia (low retics, poor bone marrow effort)
-sickle cell (high retics)

25

Three types of aplastic anemia

-malignancy
-red cell aplasia
-drug suppression

26

Hereditary spherocytosis:
-inheritance pattern
-assc protein

-AD
-spectrin

27

3 features assc with hereditary spherocytosis:

-pigmentary gallstones
-aplastic crises
-splenomegaly

28

Test assc with hereditary spherocytosis:

-osmotic fragility studies

29

Condition aside from hereditary spherocytosis that is AD And assc with spectrin?

hereditary elliptocytosis

30

Enzymatic defects of RBCs:
+ which is most common?

-G6PD*
-pyruvate kinase