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BOARDS > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

physiologic drop in H/H occurs when in infants

A

2nd to 3rd month of life (8-10 wks) in full term, 1 to 2 months (7-8 wks) in preemies

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2
Q

etiology of physiologic anemia in newborns

A

low EPO production

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3
Q

normal MCV calculation

A

70 + 2(age in yrs)

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4
Q

normal MCV in children

A

70-90

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5
Q

microcytic anemia MCV

A

<70

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6
Q

hemoglobin F composition

A

2 alpha and 2 gamma chains

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7
Q

hemoglobin A composition

A

2 alpha and 2 beta chains

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8
Q

hemoglobin A2 composition

A

2 alpha and 2 delta chains

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9
Q

hemoglobin bart

A

on neonatal electrophoresis with alpha thalassemia (gamma chains)

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10
Q

one defective alpha allele

A

asymptomatic alpha thalassemia

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11
Q

2 defective alpha alleles

A

asymptomatic but with mild microcytic hypochromic anemia form of alpha thalassemia

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12
Q

3 defective alpha alleles

A

alpha thalassemia causing hemoglobin H disease leading to hemolysis and hepatomegaly - treated with splenectomy

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13
Q

hemoglobin H disease

A

alpha thalassemia with 3 defective alpha alleles, treatment is splenectomy

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14
Q

4 defective alpha alleles

A

alpha thalassemia that causes hydros fettles and results in stillbirth or death soon after birth

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15
Q

beta thalassemia minor aka

A

beta thalassemia trait

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16
Q

defect in one beta globulin gene allele

A

beta thalassemia minor - asymptomatic with incidental mild microcytic anemia

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17
Q

beta thalassemia minor on electrophoresis

A

elevated A2, normal iron

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18
Q

beta thalassemia intermedia

A

mutation in both beta globulin gene alleles but one mutation with a mild phenotype

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19
Q

beta thalassemia major

A

mutation in both beta globulin gene alleles with severe phenotype

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20
Q

xray with hair on end appearance of bone

A

in beta thalassemia major 2/2 extra medullary hematopoiesis

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21
Q

beta thalassemia major treatment

A

chronic transfusions

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22
Q

2 long term complications of chronic transfusions

A

cholelithiasis and hemosiderosis (iron deposits in liver, heart and pancreas)

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23
Q

sickle cell anemia on electrophoresis

A

hemoglobin F and hemoglobin S

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24
Q

beta thalassemia on electrophoresis

A

low/no A1, elevated Hgb A2 and Hgb F

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25
Q

treat iron deficiency anemia for how long

A

ferrous sulfate for 2 months after Hgb normalizes

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26
Q

RDW in iron deficiency and thalassemia

A

RDW high in iron deficiency anemia and lead toxicity, low/normal in thalassemia

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27
Q

gold standard test for lead toxicity

A

whole blood lead level

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28
Q

smear finding with lead toxicity

A

ringed sideroblast

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29
Q

MCV in microcytic anemias

A

> 100

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30
Q

frequent causes of vitamin B12 deficiency

A

intrinsic factor deficiency (pernicious anemia), bacterial overgrowth, bowel resection, vegetarian diet, HLA-B27/Crohn’s

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31
Q

goats milk is associated with

A

folate deficiency

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32
Q

serum haptoglobin in hemolytic anemia

A

low (used up for transporting released Hgb as cells break open)

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33
Q

reticulocyte count in hemolytic anemia

A

elevated (unless cause is parvo, then would not be elevated)

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34
Q

heinz bodies

A

small purple granules in the red cell due to damage to hemoglobin - seen in G6Pd deficiency

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35
Q

G6PD deficiency inheritance

A

x-linked

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36
Q

G6PD deficiency testing timing

A

must be weeks after an episode otherwise could be falsely negative

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37
Q

most common inherited cause of hemolytic anemia

A

hereditary spherocytosis

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38
Q

MCHC in hereditary spherocytosis

A

increased mean corpuscular hemoglobin concentration (more hemoglobin per volume - cell is smaller)

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39
Q

diagnostic test of choice for hereditary spherocytosis

A

osmotic fragility test (cell breaks open easier in hypotonic solution) or EMA flow cytometry

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40
Q

etiology of hereditary spherocytosis

A

spectrin deficiency or spectrin mutation (less common = ankyrin deficiency)

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41
Q

cure for hereditary spherocytosis

A

splenectomy

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42
Q

most common reason for need of blood transfusion in kids with hereditary spherocytosis

A

parvovirus B19 causing an aplastic crisis

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43
Q

cause of sickle cell anemia

A

valine is substituted for glutamic acid at amino acid 6 of the beta globin chain

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44
Q

when does sickle cell anemia clinically present

A

after 6 months

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45
Q

sequestration crisis

A

pooling of blood in the liver and spleen usually in response to an infection, medical emergency and usually requires transfusion. splenectomy if has several

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46
Q

prophylaxis in sickle cell

A

penicillin through at least 5 years old and pneumococcal vaccine series

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47
Q

smear of sickle cell will show

A

Howell-jolly bodies (spleen is what usually removes them and they are functionally asplenic)

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48
Q

functional asplenia in sickle cell occurs by

A

age 5

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49
Q

children with sickle cell are resistant to

A

malaria

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50
Q

diagnosis of acute chest syndrome

A

chest pain, infiltrate on CXR and hypoxia on ABG

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51
Q

vaccines required before splenectomy

A

H flu, S. pneumonia and N. meningitidis

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52
Q

acute chest treatment

A

transfusion (simple if Hct low, exchange if Hct is high)

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53
Q

medication that can cause an aplastic crisis

A

chloramphenicol

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54
Q

type of anemia in Fanconi’s anemia

A

macrocytic anemia with elevated Hgb F

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55
Q

physical characteristics of Fanconi’s anemia

A

abnormal skin pigmentation, growth retardation, renal abnormalities, absent or hypoplastic thumb

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56
Q

type of anemia in Diamond Blackfan anemia

A

macrocytic anemia

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57
Q

Fanconi anemia inheritance

A

recessive

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58
Q

fanconi anemia is at risk for transformation to what

A

AML or myelodysplastic syndrome

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59
Q

Fanconi anemia cure

A

bone marrow transplant

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60
Q

profound isolated red cell anemia

A

Diamond Blackfan anemia (present at birth/chronic) and transient erythroblastopenia of childhood

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61
Q

etiology of diamon-blackfan anemia

A

arrest in maturation of red cells

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62
Q

etiology of transient erythroblastopenia of childhood

A

suppression of erythroid production

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63
Q

Diamond Blackfan anemia physical features

A

dysmorphic facies, thumb abnormalities, urogenital defects

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64
Q

to decrease risk of febrile non hemolytic transfusion reaction

A

use leukocyte filtered blood

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65
Q

neutropenia definition

A

ANC < 1000 during first year of life, then ANC < 1500

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66
Q

severe neutropenia definition

A

ANC < 500

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67
Q

recurrent mucosal ulcerations think

A

neutropenia

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68
Q

kostmann syndrome aka

A

severe congenital neutropenia

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69
Q

medications that can cause neutropenia

A

macrolides, nafcillin and penicillin

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70
Q

cyclic neutropenia inheritance

A

autosomal dominant

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71
Q

cyclic neutropenia presentation

A

low WBC count for one week every month or so with oral lesions and enlarged lymph nodes

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72
Q

cyclic neutropenia treatment

A

manage infection and start daily rig-CSF (recombinant human granulocyte colony stimulating factor)

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73
Q

chronic benign neutropenia is usually outgrown by

A

age 2

74
Q

kostmann syndrome inheritance

A

aka severe congenital neutropenia - autosomal recessive

75
Q

kotsmann syndrome treatment

A

daily rhG-CSF, if they do not respond then stem cell transplant

76
Q

etiology of kostmann syndrome

A

arrest in the development of neutrophils

77
Q

Scwachman-Diamond syndrome features

A

pancytopenia w/ recurrent infections, pancreatic exocrine insufficiency w/ steatorrhea and short stature, skeletal abnormalities

78
Q

thrombocytopenia definition

A

platelets < 150,000

79
Q

medications that can lower platelet count

A

sulfas, seizure meds and vancomycin

80
Q

medications that cause abnormal platelet function

A

aspirin and ibuprofen

81
Q

neonatal alloimmune thrombocytopenia

A

transient severe thrombocytopenia 2/2 platelet destruction by maternal antibodies (fetal platelets display antigen inherited by father)

82
Q

neonatal alloimmune thrombocytopenia vs autoimmune thrombocytopenia

A

mom is normal in alloimmune, antibodies also attacking mom’s platelets in autoimmune

83
Q

kasabach-merritt syndrome

A

hemangioma causes localized consumptive coagulopathy leading to thrombocytopenia, increased risk for DIC, normal bone marrow

84
Q

TAR syndrome

A

Thrombocytopenia Absent Radius - symptomatic by 4 months

85
Q

WBC in TAR syndrome

A

high

86
Q

vitamin K dependent factors

A

2, 7, 9 and 10

87
Q

vitamin K deficiency effects the

A

extrinsic pathway leading to elevated PT

88
Q

factor 8 deficiency

A

hemophilia A

89
Q

factor 9 deficiency

A

hemophilia B

90
Q

coags in hemophilia

A

prolonged PTT

91
Q

hemophilia inheritance

A

x-linked, boys only

92
Q

prolonged PT think

A

vitamin K deficiency - factors 2, 7, 9 and 10

93
Q

prolonged PTT think

A

hemophilia (factors 8 and 9)

94
Q

normal PT, slightly prolonged or normal PTT think

A

von wile brand disease

95
Q

treatment for major surgery or life threatening bleeds with vWD

A

factor 8 concentrate

96
Q

treatment for smaller bleeds in vWD

A

either nothing or DDAVP (increases plasma vWF and factor 8)

97
Q

aminocaproic acid use

A

helps with mucosal bleeding by inhibiting fibrinolysis

98
Q

DIC labs

A

low platelets, low fibrinogen, elevated d-dimer, prolonged thrombin time

99
Q

FFP to replace

A

clotting factors

100
Q

cryoprecipitate to replace

A

fibrinogen

101
Q

most common primary malignant bone tumor in kids

A

osteosarcoma

102
Q

mets of osteosarcoma

A

lungs

103
Q

xray with sunburst pattern

A

osteosarcoma (calcified blood vessels)

104
Q

xray with onion skinning

A

Ewing sarcoma (periosteal reaction)

105
Q

tibia or femur pain that is worse at night and relieved by ibuprofen

A

osteoid osteoma

106
Q

xray with central radiolucent area surrounded by thick sclerotic bone

A

osteoid osteoma

107
Q

most common childhood malignancy

A

leukemia

108
Q

most common childhood leukemia

A

ALL

109
Q

common sites of relapse with ALL

A

CNS and testes

110
Q

unexplained pruritis think

A

lymphoma

111
Q

nest step with unexplained LAD

A

CXR

112
Q

non-tender enlarged cervical or supraclavicular LAD in teens with weight loss, fever, night sweats

A

hodgkin’s lymphoma

113
Q

lymph node biopsy with Hodgkin’s lymphoma

A

reed Sternberg cells

114
Q

non-hodgkin lymphoma

A

smaller child with more rapid presentation with airway compression or non tender mass in abdomen

115
Q

presentations of langerhans cell histiocytosis

A

persistent scalp or diaper seborrheic rash, chronic ear drainage or chronic mastoiditis, lytic lesions in the skull or vertebra, excessive urination

116
Q

langerhan cell histiocytosis diagnosis

A

skin biopsy and electron microscopy

117
Q

most common solid tumor of infancy

A

neuroblastoma

118
Q

physical findings with neuroblastoma

A

raccoon eyes, opsoclonus myoclonus

119
Q

likely cause of HTN in neuroblastoma

A

renal artery compression

120
Q

neuroblastoma diagnosis

A

tumor biopsy OR elevated urine VMA and HMA with neuroblasts on bone marrow OR MIBG scan

121
Q

neuroblastoma prognosis

A

excellent if <1, poor if >1

122
Q

diagnosis of retinoblastoma

A

US or MRI (avoid CT because increased risk of secondary primary cancers)

123
Q

sporadic vs genetic retinoblastoma

A

sporadic is usually unilateral and genetic is usually bilateral

124
Q

genetic retinoblastoma inheritance

A

autosomal dominant with incomplete penetrance

125
Q

retinoblastoma gene

A

RB1 on the long arm of chromosome 13

126
Q

risk for what other tumor if retinoblastoma is genetic

A

pineal gland tumor

127
Q

most common soft tissue sarcoma in children

A

rhabdomyosarcoma

128
Q

constipation with palpable mass on rectal exam

A

rhabdomyosarcoma

129
Q

grape like mass protruding from vagina

A

rhabdomyosarcoma

130
Q

cancers that get worse after trauma/injury

A

osteosarcoma and rhabdomyosarcoma

131
Q

location of rhabdomyosarcoma

A

usually head/neck in age 2-6 and truncal or extremity in older kids

132
Q

wilms tumor aka

A

nephroblastoma

133
Q

most common pediatric abdominal malignancy

A

wilms tumor

134
Q

physical findings that can be seen with films tumor

A

aniridia and hemihypertrophy

135
Q

most common presentation of wilms tumor

A

asymptomatic abdominal mass (may also have HTN and/or gross hematuria)

136
Q

calcifications on abdominal mass

A

neuroblastoma (wilms does not calcify)

137
Q

labs in tumor lysis syndrome

A

elevated phosphate, potassium and uric acid, low calcium

138
Q

how to prevent tumor lysis syndrome

A

hydration, alkalization and allopurinol

139
Q

patient on chemo exposed to varicella requires

A

varicella zoster immunoglobulin

140
Q

giving systemic steroids to someone with undiagnosed cancer could cause

A

tumor lysis syndrome

141
Q

masses that can cause airway compression

A

Thymoma
Teratoma
Thyroid carcinoma
Terrible lymphoma

142
Q

cause of SVC syndrome

A

compression on SVC by anterior mediastinal mass usually with lymphoma

143
Q

SVC syndrome presentation

A

plethora (red face), facial swelling, upper extremity edema and distended neck veins

144
Q

alkylating agents side effects

A

gonadal dysfunction

145
Q

cyclophosphamide side effect

A

hemorrhagic cystitis

146
Q

cisplatin side effect

A

hearing loss and peripheral neuropathy

147
Q

bleomycin side effect

A

pulmonary fibrosis

148
Q

anthracyclines (doxorubicin, daunomycin) side effect

A

cardiac toxicity

149
Q

vincristine/vinblastine side effect

A

neurotoxicity and SIADH

150
Q

methotrexate side effects

A

oral and GI ulcers, bone loss

151
Q

first site of RBC formation in fetus

A

yolk sac

152
Q

what organ produces most of the fetal RBCs at 3 months

A

liver

153
Q

what organ produced EPO in fetus

A

liver (kidneys take over soon after birth)

154
Q

predominant hemoglobin at birth

A

HbF (90%)

155
Q

lifespan of mature RBC

A

120 days

156
Q

diagnosis in 4 yr old w/ low iron, high TIBC, low transferrin saturation and low ferritin

A

iron deficiency anemia

157
Q

RDW in iron deficiency anemia

A

high

158
Q

cooley anemia aka

A

beta thalassemia major

159
Q

diagnosis of 9 month old with pallor, irritability, growth retardation, HSM, jaundice and Hgb electrophoresis with Hgb F only

A

beta thalassemia major aka Cooley anemia

160
Q

adenine is replaced by thymidine resulting in valine encoded instead of glutamic acid on chromosome 11

A

sickle cell

161
Q

common first symptom in sickle cell

A

dactylitis

162
Q

leading cause of death with sickle cell

A

acute chest syndrome

163
Q

substitution of lysine for glutamic acid in the 6th position of the B-globin chain results in

A

hemoglobin C

164
Q

diagnosis with severe thrombocytopenia, small platelets, eczema and immunodeficiency

A

wiskott-aldrich syndrome

165
Q

macrothrombocytopenia with giant platelets that do not aggregate to ristocetin (they do aggregate to adenosine diphosphate, epinephrine and collagen)

A

Bernard-soulier syndrome

166
Q

how much does a RBC transfusion of 10mL/kg raise Hgb

A

2.5-3 g/dL (1 unit it 250-350 mL)

167
Q

most likely diagnosis with orbital chroma and HSM

A

AML

168
Q

classic histologic feature of Hodgkin lymphoma on lymph node biopsy

A

reed Sternberg cell

169
Q

most common type oh lymphoma in kid

A

non-hodgkin’s

170
Q

most likely diagnosis with mass in ileocecal junction

A

burkitt lymphoma

171
Q

common translocation in ewing sarcoma

A

t (11;22)

172
Q

xray with stalks or broad based projections from the surface of the bone with an associated cartilage cap

A

osteochondroma

173
Q

syndrome with gremlin mutations in p53 gene, CNS tumors including gliomas, ependymomas and choroid plexus tumors, sarcoma in children and maternal breast cancer

A

li-fraumeni syndrome

174
Q

disease associated with hemgioblastomas in the cerebellum, medulla and spinal cord

A

von Hippel-lindau disease

175
Q

syndrome with triad of impaired upward gaze, dilated pupils that accommodate but don’t react to light, retraction nystagmus with lid retraction

A

parinaud syndrome (compression of midbrain tectum with pineal tumors)

176
Q

most common malignant CNS tumor in childhood

A

medullloblastoma

177
Q

most common CNS tumors in childhood

A

gliomas

178
Q

most common posterior fossa tumor of childhood

A

cerebellar astrocytoma

179
Q

most common benign liver tumor of childhood

A

hemangioma

180
Q

histologic feature required for diagnosis of Langerhans cell histiocytosis

A

intra/extracellular staining with CD207 (lantern) or extracellular staining with CD1a

BOARDS (24 decks)

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