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Laughing Pedi > Heme Onc > Flashcards

Heme Onc Flashcards

0
Q

Iron def treatment (kids and adols)

A

iron elemental 3-6 mg/kg/day in kids

adolescents 325mg daily / BID

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1
Q

physiologic nadir of HCT/Hb in baby

A

2 mos

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2
Q

How to distinguish Thal trait from Fe def?

A

thal trait: all RBC are very small so very small MCV and RDW is normal. MArked discrepancy between Hgb and MCV.
Proportional MCV/Hgb in iron def

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3
Q

Normal MCV:

A

80

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4
Q

1-4 year old with very low Hgb and no retic, with normal MCV

Dx and Tx?

A

Transient Erythroblastenia of Childhood (TEC)

No treatment needed

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5
Q

progressive, normochromic, macrocytic anemia in infancy or early childhood with some congenital anomaly / growth failure?
Dx
Tx

A

Diamond Blackfan anemia - bone marrow has no erthroid precursors
Tx: steroids, pRBCs, stem cell trp

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6
Q

transmission of hereditary spherocytosis?
Exam finding?
Tx needed

A

autosomal dominant
Splenomegaly, may need splenectomy after 5y
need folate supp

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7
Q

chronic stable hemolysis w/ exacerbations, runs in family, MCHC is HIGH

A

Hereditary Spherocytosis

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8
Q

Periph smear showing RBC agglutination in what type of autoimmune hemolytic anemia?

A

Cold IgM autoimmune HA

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9
Q

Direct Coombs shows what?

A

presence of Ab on RBCs

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10
Q

Warm vs cold autoimmune anemia?

A

Warm: IgG: bad, occurs at body temp, splenomegaly
Cold: IgM: Liver, good.(usu from mycoplasma, HIV, or mono)

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11
Q

hypochromasia and microcytosis in mild anemia, maybe scattered target cells should lead you to consider what?

A

thalassemia (less hemoglobin per RBC because decreased hemoglobin produced)

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12
Q

Thalassemia and iron?

A

expect increased GI absorption of iron, and get iron overload even w/o transfusion in severe thal

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13
Q

Hb A2 made of what and when do you see it?

A

alpha 2 and Delta 2: No B2…

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14
Q

Hb A has what?

A

normal: two alpha and two beta

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15
Q

alpha 2 + gamma 2 is what type of hemoglobin?

A

Fetal.

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16
Q

cure for thal major

A

BMT

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17
Q

four gamma chains called what and what is the disorder? what do they get in older yrs?

A

in nweborn you get 4 gammas because no alpha: alpha thal (Barts), as they get older and beta chains mature, you get B4: Hb H

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18
Q

Hemoglobin H disease?
In who?
What is the defect?

A

This is alpha thal with Asians
variable phenotype with mild/moderate hypochromatic, microcytic anemia
Also liver / spleen issues

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19
Q

Hemoglobin C disease?

A

Sickle cell

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20
Q

Hb SS
Hb SC
Hb S beta thal + dz
Most severe?

A

Hb SS. No Hb A present. A2 and F are normal, no excess alpha chains
Shortest life expectancy: 40s

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21
Q

S beta thal null disease course?

A

severe, No Hb A, incr A2 and F, Looks very similar to SS

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22
Q

WHat is dactylitis and what may it be found in?

A

swelling of hands / feet, may be first manifestation of sickle cell

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23
Q

weird bug in sickle cell osteomyelitis?

A

salmonella

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24
Q

lusaback merritt

A

hemangioma and low plts (consumed by hemangioma)

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25
Q

bernard soulier

A

GP1b def causing congenital qualitative plt defect

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26
Q

Fibrinogen in DIC and tx?

A

low, give cryo

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27
Q

purpose of WinRHo (Anti-D) in ITP?

A

for Rh+ kids w/ ITP, trick spleen into causing mild HA and attacking RBC to leave plts alone

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28
Q

Evans vs ITP

A

Evans has autoimmune attack of MORE than one cell line. ITP ONLY plts

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29
Q

% of ITP w/ chronic disease, and who?

A

20%, usu females, infants, adol

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30
Q

splenectomy % success in chronic ITP

A

only 70%… may be liver hemnolysis

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31
Q

platelet trf in ITP?

A

NO, unless ICH

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32
Q

chemo causing clots?

A

Aspariginase

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33
Q

PT/aPTT/plts in hemophilia and vwfD

A

PT normal in both
PTT long in both
plts normal in both

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34
Q

Diagnosis of vWF?

A

vwF Ag quant: < 40%
ristocetin qualitative test
check FVIII and plt function screen

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35
Q

most common type of VFW?

Tx

A

Type 1, tx w/ DDAVP, OCP, AMicar

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36
Q

dx in life threatening bleeding from umb stump but normal PT and PTT

A

Factor 13 def

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37
Q

incidental long aPTT but no bleeding, may clot.

A

Factor 12 def

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38
Q

tumors w/ dissem LAD?

A

ALL, NHL, Neuroblastoma

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39
Q

what stage IV disease can have an ok long term control

A

Stage IV Hodgkins nodular sclerosing type in teen

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40
Q

most important prognostic factors in Neuroblastoma,

also what?

A

age and stage, also n-myc

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41
Q

labs in tumor lysis syndrome?

Who is most at risk

A

Hi K, Hi Uric Acid, high phosphate, low Calcium (gets bound)

Most in new ALL or Burkitt

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42
Q

aplastic anemia vs ALL

A

if pancytopenic from aplastic anemia - no LAD, no HSM

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43
Q

Two tumors to screen for in Beckwith Weidemann

A

Wilms, Hepatoblastoma

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44
Q

tumor causing proptosis

A

rhabdomyosarcoma (NOT retinoblastoma)

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45
Q

mediastinal mass in teen, most likely?

A

Hodgkins

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46
Q

Abdomen vs chest for ALL B or T cell?

A

abdomen B cell

Thorax/ChesT: T cell

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47
Q

Where doesn’t neuroblastoma met to?

A

lung

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48
Q

peripheral smear in sickle cell?

A

Target, Howell-Jolly *splenic destruction, sickled cells, microcytes

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49
Q

what do you look for if you have a right sided Wilms?

A

US of IVC and heart (can infiltrate up into the heart!)

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50
Q

Cisplatin causes what tox

A

ototoxicity

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51
Q

bleomycin causes what tox?

A

pulm fibrosis

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52
Q

vincristine causes what tox

A

neurotox

53
Q

cyclophos causes what s/e

A

hemorrhagic cystitis

54
Q

aspariginase causes what s/e

A

clots and pancreatitis

55
Q

ALL poor prognostic age, and poor prognostic B/T situation?

A

<2 bad

T cell bad, pre-B-cell good

56
Q

Reed Sternberg cell in what?

A

Hodgkins

57
Q

NHL in kids? Presents where?

A

masses in neck, head, abdomen

58
Q

type of cancer presenting with anterior mediastinal mass / pleural effusions

A

T cell lymphoblastic NHL

59
Q

seborrheic rash, focal bone lesion in skull, ear d/c, DI
Dx
Test

A

Langerhans Histiocytosis X

Skin bx, EM

60
Q

Prognosis in neuroblastoma in infancy , before 12 mos

A

95% survival, may spontaneously regress

61
Q

major prognostic features for neuroblastoma (2)

A

age < 1 awesome

n-myc amplification is VERY bad

62
Q

2 ways neuroblastoma causes htn and most common

A 
renal artery compression (most common)
catecholamine production (VMA/HMA)
63
Q

Link between neuroblastoma and what cancer?

A

osteosarcoma later in life

64
Q

Risk of RB if parent had it in two eyes

A

50%

65
Q

Risk of RB if parent or sibling had unilateral RB

A

5%

66
Q

Tumor lysis syndrome in what cancers
what labs
what tx

A

large tumors, leukemia, Burkitts
Labs: hyperphos, hyperkalemia, hyperuricemia
Tx: hydration, alkalinize, allopurinol

67
Q

4causes of anterior mediastinal mass

A

Ts:

thymoma, teratoma, thyroid, Terrible T cell lymphoma

68
Q

never do what in patient with anterior mediastinal mass

A

anesthesize/intubate: mass is below vocal cords

69
Q

s/e of methotrexate

A

oral ulcers

70
Q

s/e of procarbazine

A

CNS

71
Q

s/e of vincristine

A

periph neuropathy, SIADH

72
Q

s/e of asparaginase

A

pancreatitis

73
Q

TIBC in anemia of chronic disease

A

low

74
Q

Free erythrocyte protoporphyrin in lead, iron, thal?

A

FEP is high in lead poisoning and iron def

FEP is normal thalassemia

75
Q

Fanconi and Blackfan anemia have what MCV?

A

Macrocytic: >100

76
Q

hypersegmented polys are ween in what anemia?

A

B12 def macrocytic anemia

77
Q

goat milk diets lead to what kind of anemia and why

A

macrocytic from folate def

78
Q

triad of weakness, paresthesias, sore tongue?

Treatment?

A

Pernicious anemia from lack of intrinsic factor

Treat with IM Cobalamin

79
Q

best way to check for folate def?

A

erythrocyte folic acid concentration

80
Q

what is the diagnosis in a hemolytic normocytic anemia but with normal retic count (1%)

A

Parvo - aplastic crisis, so no retics

81
Q

serum haptoglabin high or low in hemolyltic anemia

A

low

82
Q

coombs direct looks for what?

A

antibodies on surface of RBC. Direct antigloboulin test

83
Q

A vs B type G6PD def?

Key lab features for dx / timing?

A

A: African AMericans, episodic
B: Mediterranean, chronic
Dx w/ heinz bodies and enzyme level BUT not right after acute hemolysis b/c retics will have large amt of G6PD

84
Q

what has high MCHC?

A

HS (small volume cell but same amt of Hb)

85
Q

etiology of HS?

A

spectrin deficiency/mutation

86
Q

HS treatment? (Hereditary Spherocytosis)

A

folic acid, transfusions,

splenectomy can be curative

87
Q

consider what in something that looks like Hereditary Spherocytosis but there is a normal osmotic fragility test?
Also there is increased exercise tolerance in face of anemia?

A

pyruvate kinase deficiency

88
Q

defect in sickle cell?

A

Substitution for glutamic acid to valine at AA #6

89
Q

PCN at what ages for children with sickle cell disease?

A

at least through age 5

90
Q

Treatment of acute chest syndrome in sickle cell?

A

confirm with ABG (not Sat), and then tx w/ transfusion or exchange transfusion if relatively high HCT to start

91
Q

If sickle cell and stroke are suspected, treatment steps?

A

Transfusion then MRI

92
Q

Kids with fanconi anemia / fancony syndrome are assoc w/ what malignancy

A

AML / myelodysplastic syndrome

93
Q

problem in diamond blackfan anemia

A

arrest of maturation of RBC

94
Q

prob n transient erythroblastopenia of childhood (TEC)

A

suppression of erythroid production

95
Q

how to distinguish the common asx RBC anemias, TEC and Diamond blackfan?

A

AGE: infants. babies 2-3 mos for DBA, and Toddlers about 2 years for TEC

96
Q

Treatment of Diamond blackfan anemia?

A

steroids

97
Q

severe bad skin infections and giant granules in neutrophils, and easy bruising, and oculocutaneous albinism

A

Chediak-Higashi Syndrome

98
Q

Treatment for Chediak-Higashi?

A

BMT

99
Q

Typical bugs causing infx in Chediak Higashi?

A

Staph aureus, Strep pyogenes, pneumococcus

100
Q

Bugs that come and get you in CGD?

A

recurrent staph, gram negatives, Serratia, Acinetobacter

101
Q

severe periodontal dz, umbilical cord stuck, and wounds still open?

A

LAD

102
Q

Define neutropenia in infants and others?

A

<1500 in others

103
Q

mucosal ulcerations are a clue to what blood cell prob?

A

neutropenia

104
Q

Abx causing neutropenia

A

macrolides: mycins

105
Q

congenital neutropenia
1 is severe
2. neutrophils look weird (2)

A
  1. severe: Kostmann

2. weird: Schwachman-Diamond, Chediak Higashi

106
Q

oral lesions that come about every month in child < 10y

genetics?

A

cyclic neutropenia- lasts about a week q month (like menstruation)
Autosomal Dom

107
Q

typical cause of infections in cyclical neutropenia?

A

clostridia perfringes: oral lesions

108
Q

list four things in Schwachman Diamond syndrome?
Risk?
When does it present
what does CBC look like

A
  1. pancytopenia / neutropenia, anemia but NORMAL platelets
  2. short stature
  3. pancreatic exocrine issues: diarrhea/steatorrhea
  4. skeletal: clinodactyly/syndactyly AND Thumb problems
    Risk for leukemic transformation
    Presents in infancy
109
Q

When to transfuse platelets in ITP?

A

10-20K

110
Q

Possible tx for ITP?

A

IVIG
WinRho (antiRh-D if pt is Rh pos)
Splenectomy if bad/chronic in older kids, steroids

111
Q

Anaphylactoid purpura is what?

A

HSP

112
Q

Distinguish platelet size in Wiskott Aldrich vs ITP?

A

ITP has big platelets

WAS has small ones

113
Q

low platelets and immunodef in boys?

A

Wiskott Aldrich - x linked

114
Q

what is kasabach-merritt syndrome?

A

hemangioma traps platelets, normal marrow

115
Q

List issues in Fanconi anemia/syndrome? (5)

Timing

A
  1. upper limb/thumb anomaly
  2. low platelets / cytopenias and anemias
  3. renal abnormalities
  4. short stature
  5. abnormal pigmentation
    Timing is in first rew years, not infancy
116
Q

Problem with platelet aggregation but normal platelet count and PT/PTT

A

Glanzmann throbasthenia

117
Q

deficiency in vitamin K factors results in what PT/PTT issue

A

long PT (Extrinsic pathway: KEPT)

118
Q

hemophilia has what pt/ptt issue

A

prolonged PTT

119
Q

PT/PTT etc issues in von willebrands

A

long PTT, normal PT, long bleeding time

req for Factor VIII function and affects plts

120
Q

nonspecific inhibition of various clotting factors and no correction with 1:1 plasma mixing. (long aptt, clotting)

A

lupus anticoagulant

121
Q

best lab to order in suspected vwD?

A

ristocetin cofactor activity if suspected type 1 (most common) (type 3 has no vWF adn Type 2 has dyfunctional so Ag is helpful there)

122
Q

chromosome for NF1

A

17

123
Q

chromosome for NF2

A

22

124
Q

elevated Hb A2 in what?

A

beta thal

125
Q

if you have Hg A and Hg S, but A>S what do you have?

A

sickle trait

126
Q

inhibitors that do not allow for correction of aPTT in mixing study?

A

heparin and lupus anticoagulant

127
Q

only factor not made in liver? (Check in DIC vs liver prob)

A

Factor VIII (and v)

128
Q

shortest half life in the warfarin / Vitk antagonist? Prob/tx?

A

Protein C (can cause warfarin necrosis / purpura fulminans) - cover w/ heparin 24-48 hrs

129
Q

what does factor v leiden mutation cause?

A

APC resistance

130
Q

recurrent infx, rashes, diarrhea, TCP and small platelets, high igA and low IgM

A

wiskott Aldrich

131
Q

eczema, low platelets, immune dysfunction

A

Wiskott Aldrich

Laughing Pedi (24 decks)

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