Hematology/Oncology Flashcards

1
Q

Age for physiologic nadir of Hgb

A

Term: 8-10 weeks

Preterm: 7-8 weeks

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2
Q

Etiology of physiologic anemia in newborns

A

Low erythropoietin production

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3
Q

Hemoglobin F

A

2 alpha + 2 gamma

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4
Q

Hemoglobin A

A

2 alpha + 2 beta

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5
Q

Hemoglobin A2

A

2 alpha + 2 delta

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6
Q

Diagnosing thalassemia

A

Hemoglobin electrophoresis
Thalassemia panel

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7
Q

One defective alpha allele
Asymptomatic

A

Alpha thal (silent carrier)

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8
Q

2 defective alpha allele
Mild Microcytic hypochromic anemia
Asymptomatic

A

Alpha thal minor/trait

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9
Q

3 defective alpha allele
Hemolysis
Hepatomegaly

A

Alpha thal intermedia
Hemoglobin Barts (at birth)
Hemoglobin H disease

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10
Q

4 defective alpha allele

A
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11
Q

4 defective alpha allele
Hydrops fetalis
Still birth

A

Alpha thal major

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12
Q

1 defective beta allele
Asymptomatic

A

Beta thal minor/trait

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13
Q

Progressive severe microcytic hypochromic anemia
HSM
Extramedullary hematopoesis
F only pattern on newborn screen
Small for age

A

Beta thal major
Copley’s anemia

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14
Q

Treatment for beta thal major

A

Chronic transfusion therapy
Transplant

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15
Q

Long term complications with beta thal major

A

Cholelithiasis
Hemochromatosis

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16
Q

Hemoglobin F
Hemoglobin S

A

Sickle cell anemia

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17
Q

Elevated Hgb A2 and F
Low or now Hgb A1

A

Beta thalassemia

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18
Q

Microcytic anemia
High RDW
High FEP

A

Iron deficiency
Lead toxicity

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19
Q

Microcytic anemia
Normal RDW
Normal FEP

A

Thalassemia

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20
Q

Most common environmental illness in kids

A

Lead poisoning

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21
Q

Gold standard test for lead poisoning

A

Whole blood (NOT fingerstick) lead level

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22
Q

Ringer sideroblast

A

Iron poisoning
Sideroblastic anemia

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23
Q

Elevated Methylmalonate and homocysteine levels

A

B12 deficiency

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24
Q

Anemia
Cows milk

A

Iron deficiency

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25
Q

Anemia
Goat milk

A

Folate deficiency

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26
Q

Only elevated homocysteine level

A

Folate deficiency

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27
Q

Hemolytic anemia
No elevated retic count

A

Parvovirus in sickle cell or hereditary spherocytosis

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28
Q

IgG
SLE
Lymphoma
Ceftriaxone
Penicillin
+ Spherocytes

Treatment

A

Warm autoimmune hemolytic anemia

Tx: steroids, rituximab, splenectomy

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29
Q

IgM
B cell lymphoma
Mono/EBV
Influenza
Mycoplasma
No Spherocytes

Treatment

A

Cold autoimmune hemolytic anemia

Tx: avoid cold, rituximab. No steroids.

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30
Q

Hemolytic anemia
X-linked
Oxidative stress
Heinz bodies, helmet/blister cells

Diagnosis

A

G6PD deficiency

Test several weeks after episode (2-3 mo). Testing near time of diagnosis can lead to false negative results

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31
Q

Cytoskeleton spectrin membrane defect
Negative coombs test (non immune hemolysis)
Osmotic fragility test
EMA flow cytometry

A

Hereditary spherocytosis

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32
Q

Most common cause of aplastic crisis in hereditary spherocytosis

A

Parvovirus B19

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33
Q

Treatment for hereditary spherocytosis

A

Folate
Transfusion
Splenectomy

34
Q

Cause of sickle cell disease

A

Amino acid 6 substitution
Valine fort glutamic acid

35
Q

Sickle cell
Acute pain secondary to ischemia or infarction
Dactylitis

Treatment

A

Vasoocclusive crisis

Rehydration + pain control

36
Q

Sickle cell
Shock
Pooling if blood in liver or spleen

Treatment

A

Sequestration crisis

Medical emergency
Transfusion.
Splenectomy after 2nd or 3rd episode

37
Q

Infectious prophylaxis for sickle cell

A

Penicillin VK
Vaccines

38
Q

Leading cause of mortality in sickle cell patients

Management

A

Acute chest syndrome
Due to fat embolism

Exchange transfusion

39
Q

Sickle cell
Chest pain
Infiltrate on CXR
hypoxia

A

Acute chest syndrome

40
Q

Management for CVA in sickle cell

A

Transfusion then image

41
Q

Macrocytic anemia
Elevated Hgb F
Abnormal skin pigmentation
Growth retardation/developmental delays
Renal abnormalities
Thumb/forearm abnormalities
Eye/ear anomalies
Recessive

A

Fanconi’s anemia

Treatment: BMT

42
Q

Arrest in maturation of red cells
Infant
Chronic
Thumb abnormalities
Urogenital defexts
Craniofacial problems
Snub nose
Webbed neck
Steroids

A

Diamond Blackfan anemia

43
Q

Suppression of erythroud production
Toddler
Transient
No steroids

A

Transient erythroblastopenia of childhood

44
Q

Recurrent mucosal ulceration
Gingivitis
Cellulitis
Abscess
Pneumonia
Septicemia

Staph aureus, strep Epi, gram neg, enterococci

A

Neutropenia

45
Q

Neutropenia
Autosomal dominant
Oral ulcers
< 10 yo
Lasts 1 week per month
Clostridium perfringens, gram neg

Treatment

A

Cyclic neutropenia
(ANC nadir < 200)

Manage infections
Start daily rhG-CSF

46
Q

Most common neutropenia in childhood
Anti-neutrophil antibodies
< 5 yo

A

Chronic benign neutropenia

47
Q

Arrest in development of neutrophils
Autosomal recessive

A

Severe congenital neutropenia
Kostmann syndrome

48
Q

Pancytopenia
Pancreatic exocrine insufficiency
Short stature
Diarrhea/steatorrhea
Recurrent infections
Skeletal abnormalities

A

Shwachman-Diamond syndrome

49
Q

Platelet destruction by maternal antibodies
Mothers platelets are normal

A

Neonatal alloimmune thrombocytopenia

50
Q

Mother and neonate both of low platelets

A

Neonatal autoimmune thrombocytopenia

51
Q

Thrombocytopenia
Localized consumptive coagulopathy
Normal bone marrow

A

Kasabach-Merritt syndrome

52
Q

Thrombocytopenia
Absent radius
Renal agenesis
Tetrology of Fallot
Decrease in megakaryocytes on bone marrow exam

A

TAR syndrome

53
Q

Deficiency in vitamin K
Elevated PT
Born at home
Exclusively breastfed

A

Hemorrhagic disease of newborn

54
Q

Elevated PTT
X-linked recessive
Deep joint bleeds

A

Hemophilia A (F8 deficiency)
Hemophilia B (F9 deficiency/christmas)

55
Q

Most common malignant bone tumor in children and adolescents

A

Osteosarcoma

56
Q

Unilateral limb pain
Elevated LDH, ALP, EsR
Adolescent going through growth spurt
Sunburst pattern

A

Osteosarcoma

57
Q

Onionskinning

A

Ewing sarcoma

58
Q

Central radiolucency surrounded by thick sclerotic bone
Pain worsens at night
Pain relieved by ibuprofen

A

Osteoid osteoma

59
Q

Most common congenital condition associated with leukemia

A

Down syndrome

60
Q

Reed sternberg cells
Non tender enlarged cervical or supraclavicular lymph node
Slow growing
Unexplained pruritus

A

Hodgkin’s Lymphoma

61
Q

Supraclavicular node

Next step

A

Lymphoma

CXR

62
Q

Most common solid tumor of infancy

A

Neuroblastoma

63
Q

Most common childhood malignancy

A

Leukemia

64
Q

Persistent bone or joint pain
Non tender hard smooth abdominal mass
Raccoon eyes and proptosis
Horner syndrome
Opsiclonus-myoclonus
Bluish-purplish subcutaneous nodules

A

Neuroblastoma

65
Q

Diagnosing Neuroblastoma

A

Biopsy

Elevated urine VMA and HMA along with neuroblasts found in bone marrow

66
Q

Painless abdominal mass
Elevated AFP
Higher risk with premature birth

A

Hepatoblastoma

67
Q

Leukocoria
Strabismus
Normal red reflex in one eye

A

Retinoblastoma

68
Q

Diagnosing retinoblastoma

A

MRI or US

Avoid CT - can increase risk of second primary cancers

69
Q

Trilateral retinoblastoma

A

Bilateral retinoblastoma + pineal gland tumor

Risk for osteosarcoma, malignant melanomas

70
Q

Unilateral/unifocal retinoblastoma

Bilateral/multifocal retinoblastoma

A

Sporadic

Genetic

71
Q

Constipation
Visible or palpated rectal mass
Grape-like mass from vagina
Can get worse after trauma injury

A

Rhabdomyosarcoma

72
Q

Panhypopituitarism
Bitemporal hemianopia

A

Craniopharyngioma

73
Q

Tumor lysis syndrome

Treatment

A

High Phos, K, Uric acid
Low Ca

Hydration, allopurinol, alkalinization

74
Q

Adverse effect
Alkylating agents

A

Gonadal dysfunction

75
Q

Adverse effect
Cyclophosphamide

A

Hemorrhagic cystitis

76
Q

adverse effect
Cisplatin

A

Hearing loss
Peripheral neuropathy

77
Q

Adverse effect
Bleomycin

A

Pulmonary fibrosis

78
Q

Adverse effect
Anthracycline (doxorubicin, daunorubicin)

A

Cardiac toxicity

79
Q

Adverse effect
Vincristine/vinblastin

A

Neurotoxicity
SIADH

80
Q

Adverse effect
Methotrexate

A

Oral and GI ulcers
Bone loss

81
Q

Treatment for priapism

A

< 4 hr : intracavernosal phenylephrine

> 4 hr : aspiration with phenylephrine. If fails, surgical fistula between corpus cavernosum and spongiosum. If fails, exchange transfusion

82
Q

Bone-within-bone appearance

A

Sickle cell disease