Hematology/Oncology Flashcards
1
Q
Age for physiologic nadir of Hgb
A
Term: 8-10 weeks
Preterm: 7-8 weeks
2
Q
Etiology of physiologic anemia in newborns
A
Low erythropoietin production
3
Q
Hemoglobin F
A
2 alpha + 2 gamma
4
Q
Hemoglobin A
A
2 alpha + 2 beta
5
Q
Hemoglobin A2
A
2 alpha + 2 delta
6
Q
Diagnosing thalassemia
A
Hemoglobin electrophoresis
Thalassemia panel
7
Q
One defective alpha allele
Asymptomatic
A
Alpha thal (silent carrier)
8
Q
2 defective alpha allele
Mild Microcytic hypochromic anemia
Asymptomatic
A
Alpha thal minor/trait
9
Q
3 defective alpha allele
Hemolysis
Hepatomegaly
A
Alpha thal intermedia
Hemoglobin Barts (at birth)
Hemoglobin H disease
10
Q
4 defective alpha allele
A
11
Q
4 defective alpha allele
Hydrops fetalis
Still birth
A
Alpha thal major
12
Q
1 defective beta allele
Asymptomatic
A
Beta thal minor/trait
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
14
Q
Treatment for beta thal major
A
Chronic transfusion therapy
Transplant
15
Q
Long term complications with beta thal major
A
Cholelithiasis
Hemochromatosis
16
Q
Hemoglobin F
Hemoglobin S
A
Sickle cell anemia
17
Q
Elevated Hgb A2 and F
Low or now Hgb A1
A
Beta thalassemia
18
Q
Microcytic anemia
High RDW
High FEP
A
Iron deficiency
Lead toxicity
19
Q
Microcytic anemia
Normal RDW
Normal FEP
A
Thalassemia
20
Q
Most common environmental illness in kids
A
Lead poisoning
21
Q
Gold standard test for lead poisoning
A
Whole blood (NOT fingerstick) lead level
22
Q
Ringer sideroblast
A
Iron poisoning
Sideroblastic anemia
23
Q
Elevated Methylmalonate and homocysteine levels
A
B12 deficiency
24
Q
Anemia
Cows milk
A
Iron deficiency
25
Anemia
Goat milk
Folate deficiency
26
Only elevated homocysteine level
Folate deficiency
27
Hemolytic anemia
No elevated retic count
Parvovirus in sickle cell or hereditary spherocytosis
28
IgG
SLE
Lymphoma
Ceftriaxone
Penicillin
+ Spherocytes
Treatment
Warm autoimmune hemolytic anemia
Tx: steroids, rituximab, splenectomy
29
IgM
B cell lymphoma
Mono/EBV
Influenza
Mycoplasma
No Spherocytes
Treatment
Cold autoimmune hemolytic anemia
Tx: avoid cold, rituximab. No steroids.
30
Hemolytic anemia
X-linked
Oxidative stress
Heinz bodies, helmet/blister cells
Diagnosis
G6PD deficiency
Test several weeks after episode (2-3 mo). Testing near time of diagnosis can lead to false negative results
31
Cytoskeleton spectrin membrane defect
Negative coombs test (non immune hemolysis)
Osmotic fragility test
EMA flow cytometry
Hereditary spherocytosis
32
Most common cause of aplastic crisis in hereditary spherocytosis
Parvovirus B19
33
Treatment for hereditary spherocytosis
Folate
Transfusion
Splenectomy
34
Cause of sickle cell disease
Amino acid 6 substitution
Valine fort glutamic acid
35
Sickle cell
Acute pain secondary to ischemia or infarction
Dactylitis
Treatment
Vasoocclusive crisis
Rehydration + pain control
36
Sickle cell
Shock
Pooling if blood in liver or spleen
Treatment
Sequestration crisis
Medical emergency
Transfusion.
Splenectomy after 2nd or 3rd episode
37
Infectious prophylaxis for sickle cell
Penicillin VK
Vaccines
38
Leading cause of mortality in sickle cell patients
Management
Acute chest syndrome
Due to fat embolism
Exchange transfusion
39
Sickle cell
Chest pain
Infiltrate on CXR
hypoxia
Acute chest syndrome
40
Management for CVA in sickle cell
Transfusion then image
41
Macrocytic anemia
Elevated Hgb F
Abnormal skin pigmentation
Growth retardation/developmental delays
Renal abnormalities
Thumb/forearm abnormalities
Eye/ear anomalies
Recessive
Fanconi’s anemia
Treatment: BMT
42
Arrest in maturation of red cells
Infant
Chronic
Thumb abnormalities
Urogenital defexts
Craniofacial problems
Snub nose
Webbed neck
Steroids
Diamond Blackfan anemia
43
Suppression of erythroud production
Toddler
Transient
No steroids
Transient erythroblastopenia of childhood
44
Recurrent mucosal ulceration
Gingivitis
Cellulitis
Abscess
Pneumonia
Septicemia
Staph aureus, strep Epi, gram neg, enterococci
Neutropenia
45
Neutropenia
Autosomal dominant
Oral ulcers
< 10 yo
Lasts 1 week per month
Clostridium perfringens, gram neg
Treatment
Cyclic neutropenia
(ANC nadir < 200)
Manage infections
Start daily rhG-CSF
46
Most common neutropenia in childhood
Anti-neutrophil antibodies
< 5 yo
Chronic benign neutropenia
47
Arrest in development of neutrophils
Autosomal recessive
Severe congenital neutropenia
Kostmann syndrome
48
Pancytopenia
Pancreatic exocrine insufficiency
Short stature
Diarrhea/steatorrhea
Recurrent infections
Skeletal abnormalities
Shwachman-Diamond syndrome
49
Platelet destruction by maternal antibodies
Mothers platelets are normal
Neonatal alloimmune thrombocytopenia
50
Mother and neonate both of low platelets
Neonatal autoimmune thrombocytopenia
51
Thrombocytopenia
Localized consumptive coagulopathy
Normal bone marrow
Kasabach-Merritt syndrome
52
Thrombocytopenia
Absent radius
Renal agenesis
Tetrology of Fallot
Decrease in megakaryocytes on bone marrow exam
TAR syndrome
53
Deficiency in vitamin K
Elevated PT
Born at home
Exclusively breastfed
Hemorrhagic disease of newborn
54
Elevated PTT
X-linked recessive
Deep joint bleeds
Hemophilia A (F8 deficiency)
Hemophilia B (F9 deficiency/christmas)
55
Most common malignant bone tumor in children and adolescents
Osteosarcoma
56
Unilateral limb pain
Elevated LDH, ALP, EsR
Adolescent going through growth spurt
Sunburst pattern
Osteosarcoma
57
Onionskinning
Ewing sarcoma
58
Central radiolucency surrounded by thick sclerotic bone
Pain worsens at night
Pain relieved by ibuprofen
Osteoid osteoma
59
Most common congenital condition associated with leukemia
Down syndrome
60
Reed sternberg cells
Non tender enlarged cervical or supraclavicular lymph node
Slow growing
Unexplained pruritus
Hodgkin’s Lymphoma
61
Supraclavicular node
Next step
Lymphoma
CXR
62
Most common solid tumor of infancy
Neuroblastoma
63
Most common childhood malignancy
Leukemia
64
Persistent bone or joint pain
Non tender hard smooth abdominal mass
Raccoon eyes and proptosis
Horner syndrome
Opsiclonus-myoclonus
Bluish-purplish subcutaneous nodules
Neuroblastoma
65
Diagnosing Neuroblastoma
Biopsy
Elevated urine VMA and HMA along with neuroblasts found in bone marrow
66
Painless abdominal mass
Elevated AFP
Higher risk with premature birth
Hepatoblastoma
67
Leukocoria
Strabismus
Normal red reflex in one eye
Retinoblastoma
68
Diagnosing retinoblastoma
MRI or US
Avoid CT - can increase risk of second primary cancers
69
Trilateral retinoblastoma
Bilateral retinoblastoma + pineal gland tumor
Risk for osteosarcoma, malignant melanomas
70
Unilateral/unifocal retinoblastoma
Bilateral/multifocal retinoblastoma
Sporadic
Genetic
71
Constipation
Visible or palpated rectal mass
Grape-like mass from vagina
Can get worse after trauma injury
Rhabdomyosarcoma
72
Panhypopituitarism
Bitemporal hemianopia
Craniopharyngioma
73
Tumor lysis syndrome
Treatment
High Phos, K, Uric acid
Low Ca
Hydration, allopurinol, alkalinization
74
Adverse effect
Alkylating agents
Gonadal dysfunction
75
Adverse effect
Cyclophosphamide
Hemorrhagic cystitis
76
adverse effect
Cisplatin
Hearing loss
Peripheral neuropathy
77
Adverse effect
Bleomycin
Pulmonary fibrosis
78
Adverse effect
Anthracycline (doxorubicin, daunorubicin)
Cardiac toxicity
79
Adverse effect
Vincristine/vinblastin
Neurotoxicity
SIADH
80
Adverse effect
Methotrexate
Oral and GI ulcers
Bone loss
81
Treatment for priapism
< 4 hr : intracavernosal phenylephrine
> 4 hr : aspiration with phenylephrine. If fails, surgical fistula between corpus cavernosum and spongiosum. If fails, exchange transfusion
82
Bone-within-bone appearance
Sickle cell disease
