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pediatrics > hematology > Flashcards

hematology Flashcards

1
Q

infants - iron

A

full term infants are born with adequate iron stores to prevent anemia for the first 4-6 months. Preterm infants are at significantly increased risk for iron-def anemia –> iron supplementation should be started at birth in exclusively breastfed preterm infants and continued until age 1

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2
Q

risk for iron def anemia in infants

A

prematurity
iron def mother
cow’s milk before 1 year
Delay start of solid food

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3
Q

timeline of infant nutrition

A 
exclusive beastfeeding until 6 months
introduction of pureed foods at 6 months
introduction of cow's milk at 1 year
\+ VIT D
\+ IRON if premature
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4
Q

management of sickle cell anemia

A

acute pain: fluids, analgesia, +/- transfusion

maintenance: vaccination, PENICILLIN (until 5), folic acid, hydroxyurea (if reccurent vaso-occlusive crises)

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5
Q

osteonecrosis - ESR, CRP

A

both normal (and no leukocytosis)

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6
Q

MCC of pediatric stroke - how to diagnose

A

SCC

- confirm with electrophoresis

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7
Q

Common etiologies of pediatric stroke

A
  1. SCC (MC)
  2. prethrombotic disroders
  3. Congenital cardiac disease
  4. bact meningitis
  5. vasculitis
  6. Focal cerebral arteriopathy
  7. head + neck trauma
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8
Q

hereditary spherocytosis - how to confirm diagnosis

A

eosin-5-maleimide biding and acidified glycerol test

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9
Q

hereditary spherocytosis - treatment

A

floci acid, transfusion, splenectomy

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10
Q

hereditary spherocytosis - complications

A

pigment gallstones

aplastic crisis from B19

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11
Q

reticulocytes in hemolytic anemia in infancts

A

not very high (babies are not able to produce)

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12
Q

characteristics of splenic sequestration in SC anemia

A
  • rapidly enlarged spleen

- high reticulocytes

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13
Q

suscpicion of iron def anemia in baby

A

iron for 1 month –> recheck –> if Hb rise 1, continue for 2-3 more months
if not –> check Hb, colonoscopy, renal studies

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14
Q

Fanconi’s anemia

A

pancytopenia + characteristic congenital anomalies such as: hyperpigmentation on the trunk, neck and interginous areas, cafe au lait spots, short stature, upper limb abnormalities, hypogonadism, skeletal anomalis, eye or eyelid changes, renal malformations
DIAGNOSIS AT 8 YEARS OLD

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15
Q

osteomyelitis vs avascular necrosis in SCA - main difference

A

osteomyelitis has signs of inflammation

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16
Q

the earliest manifestation of vaso-occlusive disease in SC anemia

A

dactylitis

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17
Q

medulobastoma - another symptom

A 
truncal ataxia (vermis location)
rare --> hemisperes of celebelum --> intenional tremor
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18
Q

tumor near hypophisis with calcifucation

A

craniopharyngeoma

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19
Q

SC disease vs trait regarding Hemoglobins percentages

A

trait: HbA: 50-60 / HbF: less than 2 / HbS: 45-45%
disease: HbS: 85-95 / HbF: 5-15

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20
Q

SC disease with hydroxyurea - HbF %?

A

more than 15%

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21
Q

anemia of prematurity - etiology

A
  • impaired EPO production
  • short red blood cell life span
  • Iatrogenic blood sampling
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22
Q

anemia of prematurity - clinical manifestation and labs

A
  • asymptomatic, tachycardia, apnea, poor weight gain

- low reticulocyte count, normocytic, normochromic

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23
Q

anemia of prematurity - treatment

A

minimize blood draws, iron, transfusion

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24
Q

ALL - how to confirm diagnosis

A

more than 25% of blasts in BM biopsy

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25
Q

Clinical features of pineal gland masses

A

Parinaud syndrome: limited upward gaze, Upper eyelid retraction (Collier sign), pupils not reactive to light, reactive to accommodation
Obstruction hydrocephalus: papilledema, headache, vomiting, ataxia

26
Q

Diamond Blackfan anemia

A

macrocytic pure red aplasia associated with several congenital anomalies such as short stature, webbed neck, cleft lip, shielded chest and triphalangeal thumbs

27
Q

diamond Blackfan vs fanconi anemaia regarding age of diagnosis

A

fanconi: 8
Blackdan: 1

28
Q

ITP - treatment

A

children: skin manifestation only: observe, if bleeding, IVIG or glucocortic
adults: platelets more than 30000 and cutaneous symptoms, without bleeding: observe, less than 30 or bleeding: IVIG or glucocort

29
Q

are platelet affected in SC disease?

A

no

only in sequestration

30
Q

Lymphoblast cytolasma - stain

A

pedioedi acid schiff

31
Q

how to differentiate iron def anemia from thalassemia

A

iron def has increased RDW (more than 20%)

32
Q

iron def in cow milk - mechanism

A

low iron
low bioavailability
GI bleeding

33
Q

SC trait - splenic infractions ?

A

rare (at high altitudes)

34
Q

hemarthroses - next step

A

CBC and coagulation studies

35
Q

histology of cells in ALL

A

lack peroxidase positive granules but contain cytoplasmic aggregates of PAS (+) material
+ TDT

36
Q

Wiskott-Aldrich syndrome - clinical manifestation

A
  1. eczema
  2. reccurent infections
  3. microthrombocytopenia (small and few platelets)
37
Q

Wiskott-Aldrich syndrome - treatment

A

stem cell transplant

38
Q

Wiskott- Aldrich syndrpme - etiology

A

XR defect in WAS protein gene –> impaired cytoskeleton changes in leukocytes + platelets

39
Q

what to see in cells in people with splenectomy

A

Howel jolly bodies

40
Q

neonatal polyceythemia - causes definition

A
  1. increased erythropoiesis from intrauteine hypoxia (Maternal diabetes, hypertenson or smoking)
  2. IUGR
  3. Transfusion (twin-twin, delayed cord clamping)
  4. Geneticmetabolic disease
    definition: more than 65%
41
Q

Neonatal polycythemia - clinical presentation

A
  1. asymptomatic (MC)
  2. Ruddy skin
  3. hypoglycemia, hyperbilirubinemia
  4. resp distress, cyanosis, apnea,
  5. irritability
  6. abd distention
42
Q

neonatal polycythemia - treatment

A
  1. IV fluids
  2. glucose
  3. partial exchange transfusion (if symptomatic)
43
Q

iron def anemia in children blood transfusion

A

rare (even if Hb is 4)

44
Q

Cow milk - when and amount

A

introduction at 1 years old

no more than 24 ounces / d

45
Q

characteristic of avascular necrosis’ pain

A

chronic
no fever
no signs of inflammtion

46
Q

hydroxyyrea is used in patients with SC disease to decrease

A
  1. pain crisis
  2. need for transfusion
  3. episodes of acute chest syndrome
    (increase in fetal Hb, which dilutes the amount of sickle hb)
47
Q

Low folic or B12 in infants

A

no anemia in infancy

48
Q

vit K def - PT, PTT

A

increased PT

normal PTT, (increased if severe def)

49
Q

thalassemia β minor - reticulocytes

A

high

50
Q

Mentzer index

A

MCV/RBC

in thalassemia minor is less than 13 (very low MCV and normal to high RBC)

51
Q

RBC in thalassemia minor

A

normal to high

52
Q

tilateral rb

A

bilateral rb PLUS pineal gland tumor

53
Q

MCC of macrocytic anemia in SC disease

A

follate def

54
Q

Diamond black .. - treatment

A

Blood transfusion

Steroids

55
Q

SC disease - sequestration vs aplastic crisis

A

seq: elevated reticulocytes, rapidly enlarging spleen

aplastic crisis:, low reticulocytes, transient arrest of erythropoiesis, 2ry to infection

56
Q

HUS treatment

A
  1. fluid
  2. blood transfusion
  3. dialysis
57
Q

another symptom of vaso-occlusive crisis in SC anemia

A

low grade fever

58
Q

neuroblastoma - periorbital ecchymoses

A

due to orbital metastasis

59
Q

harlequin sign

A

absent facial flushing (Horner syndrome)

60
Q

SSA - sequestration treatment

A

Fluids
Small volume transfusion
Splenectomy

61
Q

How to prevent pmeumonococcal sepsis in SCA

A

Vaccination and penicillin

pediatrics (16 decks)

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