General

Question 0

what are causes of eosinophilia?C

Answer 0

CHINA
Connective tissue:
-RF
-Churg-strauss vasculitis
Coccidioidomycosis/Chlamydia
Helminth - WORM
-ascariasis
-schistomiasis
Idiopathic hypereosinophilic syndrome
Neoplasm:
-Lymphoma
-HTLV-1
-eosinophilic leukemia
-paraneoplastic
Allergies/Asthma/Atopic dermatitis

(IBD, esonophilic gastro, pneumonia, allergic bronchopulmonary aspergillosis, immune def)

Question 1

Thrombophilic disorders

Answer 1

Factor V Leiden
Prothrombin gene mutation 
Hypergocysteinanemia
Antithrombin III def
Protein C or S deficiency
High lipoprotein A

Question 2

what labs abno do you see in anemia of chronic disease

Answer 2

normocytic
normochromic
Retic - N or low
\+ leucocytosis
Low Fe but N TIBC
May have high ferritin

Question 3

how do you manage anemia of chronic disease?

Answer 3

can use Fe to optimize EPO

may need EPO is very symptomatic

Question 4

Goat milk leads to def in ?

Answer 4

Folate

Question 5

how will giardia infection lead to anemia

Answer 5

Fe malabsorption

Question 6

how does bacterial overgrowth in GI lead to heme abn

Answer 6

affects Vit B12 absorption

Question 7

EBV and CMV affect BM how?

Answer 7

BM suppression and hemophagocytic syndrome

Question 8

how does mycoplasma affect heme

Answer 8

HEMOLYSIS

Question 9

how does parvo virus affect heme

Answer 9

BM suppression

Question 10

what lab values indicate hemolysis

Answer 10

high bili
high LDH
low haptoglobin

Question 11

what are CF specific to Fe def anemia?

Answer 11

apathy
poor concentration
irritability
poor muscle endurance
GI dysfunction

Question 12

during Fe def anemia, at what Hb level might you start seeing RBC changes

Answer 12

when Hb drops below 100, can start seeing microcytic and hypochromia

Question 13

how does Beta thalassemia major present?

Answer 13

severe hypochromic microcytis anemia
sever hemolysis
present early with pallo, jaundice

Question 14

What causes Beta thalassemia Major

Answer 14

both allele have thal mutation

B0/B0

Question 15

what are clinical features associated with Beta Thalassemia MAJOR

Answer 15

high output heart failure
maxilla hyperplasia,
flat nasal bridge, 
frontal bossing
osteopenia/pathologic bone fractures
hepatosplenomegaly
FTT

Question 16

how do you treat beta Thalassemia major

Answer 16

1. regular blood transfusion - CMV neg, matched Kell and Rh
2. chelation - deferoxamine or Deferiprone
3. Aim for bone marrow transplant

Question 17

how do you monitor a child receiving frequent Bls transfusion

Answer 17

Ferritometer on MRI
Serum ferritin helps for trending but does not predict stores
liver Bx is Gold but does not predict Cardiac Fe deposition

Question 18

what are complications of frequent bld transfusions?

Answer 18

liver cirrhosis/fibrosis and liver cancer.
Heart failure,
CAn deposit in endo system - FTT

Question 19

what lab values can help you differentiate Fe def anemia from Alph thal trait

Answer 19

• ## Alpha thal has really small RBC (MCV small) but Hb no super low

Question 20

what can look like Fe def anemia but fails to respond to treatment

Answer 20

1. Beta thalassemia minor
2. Alpha thalassemia Trait
3. Celiac disease

Question 21

how can you differentiate Fe def anemia from beta thal trait

Answer 21

Mentzel index=MCV/RBC

if > 13 = Fe def anemia

Question 22

if you hear basophilic stippling, what should you think of?

Answer 22

lead poisoning

usually also have Fe def anemia - need to also fix

Question 23

What is the most common congenital pure red bld cell aplasia

Answer 23

Diamon-Blackfan syndrome

Question 24

How does Diamond-Black syndrome get Dx on Bld test?

Answer 24

``` macrocytosis anemia (can be normo) Low retic Normal Vit B12 and folate high Fetal Hb High fetal i antigen ```

Question 25

what are CF of Diamon-Blackfan syndrome

Answer 25

``` short stature webbed neck cleft lip hypertelorism TRIPHALANGEAL thumb late onset leukemia ```

Question 26

what is the most common acquired red cell aplasia

Answer 26

``` transient erythroblastopenia of childhood (TEC) ? viral induced 6 mo to 3 years recover within 1-2 months no treatment ```

Question 27

what investigations are useful to Dx transient erythroblastopenia of childhood?

Answer 27

``` Low Ret Normocytic anemia 20% will have neutropenia normal or high plt dues to secondary inc in EPO Normal Hb F normal ADA ```

Question 28

patient with cafe au lait spot, microcephaly, horseshoe kidney and short stature. Seen in heme clinic

Answer 28

Fanconi anemia macrocytic anemia and low Ret progresses to pancytopenia

Question 29

how do you Dx Fanconi anemia

Answer 29

need to show DNA break | fibroblast culture

Question 30

what are causes of microcytic anemia

Answer 30

``` TAILS Thalassemia Anemia of chronic disease Iron def Lead poisoning sideroblastic ```

Question 31

what is the genetics for G6PD?

Answer 31

X linked defect in glutathione and can't protect RBC from oxidation

Question 32

How does G6PD usually present?

Answer 32

1. hemolytic anemia post contact with oxidant

Question 33

if see on lab result that pt has Heinz bodies, what enzymopathy should you think off

Answer 33

G6PD can also get cookie cells - bites taken RBC appears blistered

Question 34

what are oxidants that can cause acute hemolysis in pt with G6PD?

Answer 34

1. sepsis, DKA, hepatitis 2. Antimalaria drugs 3. Abx: sulpha, chloremphenicol, septra 4. ASA, vit K analog... 5. Fava beans

Question 35

how do you Dx G6PD and treat

Answer 35

eletrophoresis | Supportive care and avoid oxidants

Question 36

how do you Dx hereditary spherocytosis?

Answer 36

osmotic fragility test

Question 37

How do you treat hereditary spherocytosis?

Answer 37

depend on symptoms | splenectomy can really help

Question 38

what is the DDX for spherocytosis

Answer 38

hereditary Wilson's disease thermal injury C. diff septicemia

Question 39

where is vitamin B12 absorbed?

Answer 39

terminal ileum

Question 40

what might lead to a low vitamin B12

Answer 40

``` mom is vegan baby has strict vegan/veg diet post resection of terminal ileum post gastric Sx - pernicious anemia - congenital or juvenille ```

Question 41

where is folate absorbed

Answer 41

small intestine

Question 42

Goat's milk is low in...

Answer 42

folate

Question 43

what can lead to folate def?

Answer 43

not taking in enough from diet not absorbing from chronic diarrhea, inflammation, AED unable to metabolise drugs with anti-folic abilities: MTX, trimethoprim

Question 44

how can we prevent vaso-occlusive crises?

Answer 44

good hydration avoid triggers hydroxyurea

Question 45

refractory Fe def causes?

Answer 45

Celiac | H. Pylori gastritis