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PEDS UWORLD > Heme/ONC > Flashcards

Heme/ONC Flashcards

1
Q

Sickle cell anemia Labs

A

Low hematocrit
Increased: reticulocytes, LDL, unconjugated bilirubin
Smear: howell-jolly bodies, polychromasia

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2
Q

Maintenance of Sickle cell

A

Vaccincations
Penicillin (until age 5)
Folic acid supplementation
Hydroxyurea (for recurrent vasoocclusive events)

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3
Q

Management of acute pain crisis in Sickle cell

A

Hydration
analgesia
+/- transfusion

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4
Q

ALL C/F

A

typically Age 2-10

30-50% present with infection and about half with LAD/splenomegaly

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5
Q

Dx ALL with smear

A

lymphoblast typically
>25% lymphoblasts in bone marrow
Lymphoblasts lack peroxidase positive granules but stain with PAS
TdT positive (pre B and pre T lymphoblast)

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6
Q

Aplastic crisis in SC anemia due to

A

Parvo B19 virus
sudden arrest of erythropoiesis
reiculocytes need be less than 1%

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7
Q

Polycythemia of Newborn

is what and causes (5)

A 
Hct >65%
Causes: Inc Erythropoiesis from Intrauterine hypoxia:
Maternal DM
Maternal HTN
Smoker
IUGR
Delayed cord clamping
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8
Q

CF/ of polycythemia in newborn (6)

A 
Ruddy skin
hypoglycemia/hypocalcemia
Resp distress
cyanosis
apnea, irritability, jitterness
Abd distension
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9
Q

Rx: Neonatal polycthemia

A

Partial exchange transfusion

remove blood, infuse normal Saline

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10
Q

Dehydration in newborn typically occurs when? and why

A

unusual in first 2 days of life as they are born with excess extracellular water

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11
Q

Anemia of prematurity labs (5)

A 
smear: normocytic, normochromic anemia
low reticulocyte count, RBC precursores low in BM
Normal WBC/platelet count
Normal Total bilirubin
Hb: 7-10 g/dL
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12
Q

Rx anemia of prematurity

A

Iron supplementation
periodic Hb check
blood transfucion if needed

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13
Q

Causes of anemia of premi

A

Combo of:
diminished RBC production
Shortened RBC life spain
blood loss

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14
Q

Acquired aplastic anemia labs

A

Pancytopenia: Increased MCV
Increased HbF
Bone marrow hypoplasia

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15
Q

Fanconi Anemia C/F

A

BM: aplastic anemia and progressive BM failure
Apperance: short, microcephaly abnormal thumbs, hypogonadism
Skin: hypo/hyperpigmentation, cafe au lait spots, large freckles
Eye/ears: strabismus, low set ears, middle ear abnormalities: Headaches

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16
Q

Fanconi anemia pathophys

A

spontaneous chromosomal breaks

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17
Q

Fanconi anemia risk of developing

A

AML and cancers

BM failure

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18
Q

Rx fanconi

A

Steroids, androgens

BM transplant definitive

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19
Q

Diamond Blackfan syndrome

A

Congenital hypoplastic anemia:
child w/ macrocytic anemia (no hypersegmented nuclei)
low reticulocyte count
congenital anomalies
usually sporadic although can be AD or AR 15% of times

20
Q

Pathology in DBS?

A

intrinsic defect of erythroid progenitor cells which results in creased apoptosis

21
Q

Congenital anomlies associated with DBS

A

Short stature
craniofacial deformities
defects of upper limbs: triphalangeal thumbs

22
Q

Rx of DBS

A

Corticosteroids
Transfusions and deferoxamine
stem cell transplant

23
Q

in C1 inhibitor def. aka hereditary angioedema what is elevated that causes edema

A

C2b

Bradykinin

24
Q

Immune thrombocytopenia (ITP) C/F (3)

A

mc around 2-5 yrs old
Antecedent viral infection
MC: asymptomatic petechiae and ecchymosis
Mucocutaneous bleeding

25
Q

Labs in ITP

A

Isolated thrombocytopenia

26
Q

Rx in kids for ITP

A

Skin manifestation only: observe

Bleeding: IVIG or Steroids

27
Q

Rx in Adult for ITP

A

Plat: >30,000: observe
Platelet below 30,000 or bleeding:
IVIG or glucocorticoids

28
Q

Sickle Cell disease: aplastic crisis

A

transient arrest of erythropoiesis that results in severe drop in Hb and virtual absence of reticulocyte on peripheral smear (

29
Q

Rx aplastic crisis

A

blood transfusion

30
Q

Sickle cell disease: hyperhemolytic crisis

A

sudden severe anemai w/ appropriate reiculocytosis

31
Q

Sickle cell disease: acute chest syndrome

A

fever, chest pain, and infiltrate on chest xray
Pulmonary infarction or infection
Drop in Hb may be seen

32
Q

Adolescent w/ nasal obstruction, visible nasal mass, frequent nosebleed consider:

A

Juvenile angiofibroma (JNA)

33
Q

JNA is worrisome because

A

can invade bone
highly vascularized: bleeding
rx: surgicaly

34
Q

Sickle cell disese sepsis organisms

A

mc: S. Pneumo

2. HiB

35
Q

Sickle cell disease kids should get routine wat

A

12 conjugate pneumococcus and HiB vaccines

plus 23 Polysaccharide pneumococus and meningococcal conjugate vaccines

36
Q

Sickle cell kids shoudl get what pophylaxis

A

penicillin till age 5

37
Q

Hereditary spherocytosis lab tests

A

Increased MCHC
Negative coombs test
Increased osmotic fragility on acidified glycerol lysis test
Abnormal eosin 5 maleimide binding test

38
Q

Hereditary spherocytosis genetic issue

A

AR mutation in ankyrin on RBC membran resulting in spectrin deficiency

39
Q

Elevated RDW is what and what its useful for

A

RBC variability in size
earliest sign in Iron def anemia
Elevated in nutritional deficiences

40
Q

MCHC is

A

Hb/HCT: measures Hb conc of each RBC
Decreased in: Iron def, thallasemia
Increased: spherocytosis

41
Q

Hyperbilirubinemia in black infant is assoc w/

A

G6PD deficiency

42
Q

Spherocytosis is seen in

A

Hereditary spherocytosis
Hyperthermia
G6PD def
ABO incompatibility

43
Q

in DIC there is a consumption of

A

factors II, V, VIII, and platelets

44
Q

Toxicity of MTx

A

GI mucositis
BMS
skin erythema
hepatic dysfunction

45
Q

Toxicity of Vincristine

A

Peripheral neuropathy
constipation
jaw pain
SIADH

46
Q

Doxorubicin toxicity

A 
alopecia
N/V
stomatitis
tissue necrosis
BMS
Cardiotoxicity
47
Q

G6PD presents in kids how/when

A 
3-4 months of age w/
failure to thrive
hypoglycemia
hepatomegaly
acidosis

PEDS UWORLD (9 decks)

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