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Flashcards in Heme/ONC Deck (47):
1

Sickle cell anemia Labs

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

2

Maintenance of Sickle cell

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

3

Management of acute pain crisis in Sickle cell

Hydration
analgesia
+/- transfusion

4

ALL C/F

typically Age 2-10
30-50% present with infection and about half with LAD/splenomegaly

5

Dx ALL with smear

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

6

Aplastic crisis in SC anemia due to

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

7

Polycythemia of Newborn
is what and causes (5)

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

8

CF/ of polycythemia in newborn (6)

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

9

Rx: Neonatal polycthemia

Partial exchange transfusion
(remove blood, infuse normal Saline)

10

Dehydration in newborn typically occurs when? and why

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

11

Anemia of prematurity labs (5)

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

12

Rx anemia of prematurity

Iron supplementation
periodic Hb check
blood transfucion if needed

13

Causes of anemia of premi

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

14

Acquired aplastic anemia labs

Pancytopenia: Increased MCV
Increased HbF
Bone marrow hypoplasia

15

Fanconi Anemia C/F

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

16

Fanconi anemia pathophys

spontaneous chromosomal breaks

17

Fanconi anemia risk of developing

AML and cancers
BM failure

18

Rx fanconi

Steroids, androgens
BM transplant definitive

19

Diamond Blackfan syndrome

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

Pathology in DBS?

intrinsic defect of erythroid progenitor cells which results in creased apoptosis

21

Congenital anomlies associated with DBS

Short stature
craniofacial deformities
defects of upper limbs: triphalangeal thumbs

22

Rx of DBS

Corticosteroids
Transfusions and deferoxamine
stem cell transplant

23

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

C2b
Bradykinin

24

Immune thrombocytopenia (ITP) C/F (3)

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

25

Labs in ITP

Isolated thrombocytopenia

26

Rx in kids for ITP

Skin manifestation only: observe
Bleeding: IVIG or Steroids

27

Rx in Adult for ITP

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

28

Sickle Cell disease: aplastic crisis

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

29

Rx aplastic crisis

blood transfusion

30

Sickle cell disease: hyperhemolytic crisis

sudden severe anemai w/ appropriate reiculocytosis

31

Sickle cell disease: acute chest syndrome

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

32

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

Juvenile angiofibroma (JNA)

33

JNA is worrisome because

can invade bone
highly vascularized: bleeding
rx: surgicaly

34

Sickle cell disese sepsis organisms

mc: S. Pneumo
2. HiB

35

Sickle cell disease kids should get routine wat

12 conjugate pneumococcus and HiB vaccines
plus 23 Polysaccharide pneumococus and meningococcal conjugate vaccines

36

Sickle cell kids shoudl get what pophylaxis

penicillin till age 5

37

Hereditary spherocytosis lab tests

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

38

Hereditary spherocytosis genetic issue

AR mutation in ankyrin on RBC membran resulting in spectrin deficiency

39

Elevated RDW is what and what its useful for

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

40

MCHC is

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

41

Hyperbilirubinemia in black infant is assoc w/

G6PD deficiency

42

Spherocytosis is seen in

Hereditary spherocytosis
Hyperthermia
G6PD def
ABO incompatibility

43

in DIC there is a consumption of

factors II, V, VIII, and platelets

44

Toxicity of MTx

GI mucositis
BMS
skin erythema
hepatic dysfunction

45

Toxicity of Vincristine

Peripheral neuropathy
constipation
jaw pain
SIADH

46

Doxorubicin toxicity

alopecia
N/V
stomatitis
tissue necrosis
BMS
Cardiotoxicity

47

G6PD presents in kids how/when

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