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EOR 3 - Peds > Heme > Flashcards

Heme Flashcards

1
Q

What kind of anemia is iron deficiency anemia?

A

hypochromic, microcytic anemia
elevated RDW
high total iron binding capacity

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2
Q

When is iron deficiency anemia screened for?

A

universal screening at 1yo

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3
Q

What is the treatment for iron deficiency anemia?

A

ferrous sulfate (3-6mg/kg/day) for 2 months

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4
Q

When is packed blood transfusion the treatment for iron deficiency anemia?

A

if there are signs of heart failure

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5
Q

What is the most common cause of anemia in young infants between 6 and 9 weeks of age?

A

physiologic anemia

decrease in erythropoiesis d/t increase tissue oxygenation

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6
Q

What is fanconi anemia?

A

an autosomal recessive pattern

hematologic abnormalities are noted around 7yo

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7
Q

How do pts with fanconi anemia present?

A

pancytopenia (thrombocytopenia normally comes first) (platelets, WBC, RBC)

cafe-au-lait spots
short stature
upper limb abnormalities
deafness

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8
Q

What is the dx for fanconi anemia?

A

bone marrow - patchy hypoplasia

chromosomal breakage analysis – increased DNA breakage when exposed to mytomycin C and diepoxybutane (DNA crosslinking agents)

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9
Q

What is the tx for fanconi anemia?

A

transfusion and ABX for infections

stem cell transplantation

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10
Q

Dyskeratosis congenita

A

mutations in genes in telomere function

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11
Q

How do pts with dyskeratosis congenita present?

A

Classic mucocutaneous traid:

  • luekoplakia
  • hypo/hyperpigmentation
  • nail dystrophy

early graying hair

lung and liver fibrosis, as well as cancer

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12
Q

How is dyskeratosis congenita dx?

A

telomere length testing - will be very short for age

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13
Q

Diamond -Blackfan anemia

A

autosomal dominant inheritance

mutations in ribosomal subunits

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14
Q

How do pts with diamond - blackfan anemia present?

A 
typically in the first 3 months of life 
macrocytic anemia 
poor feeding 
deafness 
short stature 
craniofacial abnormalities
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15
Q

How is diamond blackfan anemia dx?

A

macrocytic anemia

RBC with markedly increased adenosine deaminase activity

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16
Q

What is the treatment for daimond blackfan anemia

A

corticosteroids

stem cell transplant may be curative

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17
Q

What is spherocytosis?

A

congenital hemolytic anemia (cause of neonatal jaundice in the first 24 hours of life)

autosomal dominant inheritance

mutations that affect RBC membrane (spectrin and ankyrin)

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18
Q

How do pts with spherocytosis present?

A

acute aplastic event (parvovirus B19)
jaundice
splenomegaly
early gallstones

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19
Q

How is spherocytosis dx?

A

osmotic fragility test

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20
Q

What is the tx for spherocytosis?

A

symptomatic
folic acid
splenectomy

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21
Q

What is sickle cell anemia?

A

a autosomal RECESSIVe disorder
single point mutation (Glutamic acid to valine) on the beta chain

upon deoxygenation HbS polyermizes within RBC causing sickle shape

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22
Q

How is sickle cell dz dx?

A

in the US, universal newborn screen

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23
Q

How do pts with sickle cell dz present?

A 
functionally aslpenic (HOWELL JOLLY BODIES) develops as early as 3-6 months 
pneumococcal infections
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24
Q

Osteomyelitis is most commonly cause by what pathogen? How does this differ in pts with sickle cell anemia?

A

normally: staph aureus

SCD: SALMONELLA

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25
Q

What is the treatment for SCD?

A

sepsis prevention: PCN prophylaxis started by 3 months of age
immunizations
Fevers >38.5 = medical emergencies

RBC transfusion for acute splenic sequestration

hydroxyurea - reduces hemolysis and increases levels of fetal Hgb

stem cell transplantation could cure SCD

26
Q

What is the difference between alpha thalassemia trait and disease?

A

Trait is 2 gene deletion
Disease is 3 gene deletion

normal trait = 4 alpha globin genes and 2 beta globin genes

27
Q

What kind of labs will you see with alpha thalassemia?

A

microcytosis
hypochromic anemia
HEINZ bodies
Howell Jolly Bodies

28
Q

What is the difference between beta thalassemia major and trait?

A

point mutations

major: most severe (homozygous)
trait: least severe (heterozygous)

29
Q

How do pts with beta thalassemias present?

A

major:
frontal bossing
hypersplenism

trait:
asymptomatic
mild microcytosis noted on CBC

30
Q

How do you dx beta thalassemias?

A

major: at birth only HbF
later HbF and HbA2
no Bs ever

31
Q

What is the treatment for beta thalassemia major?

A

lifelong blood transfusions –> goal is hypertransfusion to shut off erythropoeisis

32
Q

G6PD deficiency

A

X-linked recessive disorder

incomplete protection against oxidative states

33
Q

What is the MC human genetic mutation?

A

G6PD deficiency

34
Q

How do pts with G6PD deficiency present?

A

acute hemolytic anemia after exposure to oxidative stress –> fava beans, sulfa drugs, infections

acute fatigue, jaundice, pallor, dark urine

35
Q

What kind of labs would you expect to see with someone who has G6PD deficiency?

A

normocytic, normocromic anemia, reticulocytosis
BLISTER CELLS
elevated LDH, bilirubin

36
Q

Basophilic stippling

A

lead poisoning

microcytic, hypochromic anemia

37
Q

What is the treatment for lead poisoning?

A

dimercaprol followed by edetate calcium disodium

oral succimer is an alternative

38
Q

What is the most common hereditary bleeding disorder?

A

von Willebrand disease

autosomal dominant

39
Q

What is von Willebrand disease?

A

a protein deficiency that is needed for platelet adhesion

40
Q

How do pts with von Willebrand disease present?

A

easy bruising

platelet type bleeding: dental and nasal bleeding, menorrhagia

41
Q

How is von Willebrand disease dx?

A

PTT is normal or may be increased

VWF antigen assay - increased bleeding time

42
Q

What is the treatment for von Willebrand disease?

A

desmopressin (causes release of VWF from cells)

43
Q

Idiopathic thrombocytopenia purpura

A

acquired autoimmune disorder that results in platelet destruction –> autoantibodies directed against platelets
often follows viral illness

MC in children than adults

44
Q

How does ITP present?

A

thrombocytopenia in the absence of other bone marrow pathology

petechiae, purpura, gingival bleeding
menorrhagia, GI bleeding, intracranial hemorrhage

45
Q

What is the tx for ITP?

A

corticosteroids and/or IVIG

start tx if platelet count is <10,000
or if it is <20,000 + mucosal bleeding

46
Q

ALL (what is it and who gets it?)

A

acute lymphocytic leukemia

affected cell progeny fail to differentiate but continue to proliferate (definition of leukemia?)

early LYMPHOID precursors overtake the bone marrow

MC form of cancer in childhood (peak 2-5yo)
M>F

47
Q

How do pts with ALL present?

A

hepatosplenomegaly
LAD
bone pain

48
Q

How is ALL dx?

A

Philadelphia chromosome

panyctopenia
hypercellular marrow

49
Q

What is the tx for ALL?

A

chemotherapy induction

bone marrow transplant

50
Q

AML (what is it and who gets it?)

A

Acute myeloid leukemia

affect cell progeny fail to differentiate, but continue to proliferate
early MYELOID precursors overtake the bone marrow

80% of leukemias in adults
M >F

51
Q

How do pts with AML present?

A

splenomegaly

gingival hyperplasia

52
Q

How is AML dx?

A

AUER RODS on peripheral blood smear

pancytopenia
hyerpcellular marrow

53
Q

What is the tx for AML?

A

more intensive and toxic than ALL tx

chemo + BMT after remission

54
Q

What is the difference between hodgkin and non-hodgkin lymphoma?

A

hodgkin: lymphocyte neoplasm - B cell —associated with EBV

non-hodgkin: lymphocyte neoplasm - B, T, follicular

55
Q

Who gets hodgkins lymphoma?

A

bimodal peaks
15-19 and 50yo
M&raquo_space;F

56
Q

Who gets non-hogkin lymphoma?

A

> 50yo

57
Q

How do pts with hodgkins lymphoma present?

A

painless LAD of upper central body

EtOH may induce LN pain

58
Q

How is hodgkins lymphoma dx?

A

Reed-Sternberg cells (Owl’s eyes) on biopsy

CXR - mediastinal mass

59
Q

What is the treatment for hodgkins lymphoma?

A

radiation therapy if local

chemo if advanced

60
Q

How do pts with non-hodgkins lymphoma present?

A

PERIPHERAL painless LAD

Burkitt Lymphoma is a type of non-hodgkins –> starry sky appearance

61
Q

What is the treatment for hemophillias?

A

replacement of the missing factor concentrate
Hemophilia A = 8
Hemophilia B = 9
X-linked recessive (although 30% are new mutations)

DDAVP/stimulate

EOR 3 - Peds (9 decks)

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