Hematology/Oncology Flashcards
Age for physiologic nadir of Hgb
Term: 8-10 weeks
Preterm: 7-8 weeks
Etiology of physiologic anemia in newborns
Low erythropoietin production
Hemoglobin F
2 alpha + 2 gamma
Hemoglobin A
2 alpha + 2 beta
Hemoglobin A2
2 alpha + 2 delta
Diagnosing thalassemia
Hemoglobin electrophoresis
Thalassemia panel
One defective alpha allele
Asymptomatic
Alpha thal (silent carrier)
2 defective alpha allele
Mild Microcytic hypochromic anemia
Asymptomatic
Alpha thal minor/trait
3 defective alpha allele
Hemolysis
Hepatomegaly
Alpha thal intermedia
Hemoglobin Barts (at birth)
Hemoglobin H disease
4 defective alpha allele
4 defective alpha allele
Hydrops fetalis
Still birth
Alpha thal major
1 defective beta allele
Asymptomatic
Beta thal minor/trait
Progressive severe microcytic hypochromic anemia
HSM
Extramedullary hematopoesis
F only pattern on newborn screen
Small for age
Beta thal major
Copley’s anemia
Treatment for beta thal major
Chronic transfusion therapy
Transplant
Long term complications with beta thal major
Cholelithiasis
Hemochromatosis
Hemoglobin F
Hemoglobin S
Sickle cell anemia
Elevated Hgb A2 and F
Low or now Hgb A1
Beta thalassemia
Microcytic anemia
High RDW
High FEP
Iron deficiency
Lead toxicity
Microcytic anemia
Normal RDW
Normal FEP
Thalassemia
Most common environmental illness in kids
Lead poisoning
Gold standard test for lead poisoning
Whole blood (NOT fingerstick) lead level
Ringer sideroblast
Iron poisoning
Sideroblastic anemia
Elevated Methylmalonate and homocysteine levels
B12 deficiency
Anemia
Cows milk
Iron deficiency
Anemia
Goat milk
Folate deficiency
Only elevated homocysteine level
Folate deficiency
Hemolytic anemia
No elevated retic count
Parvovirus in sickle cell or hereditary spherocytosis
IgG
SLE
Lymphoma
Ceftriaxone
Penicillin
+ Spherocytes
Treatment
Warm autoimmune hemolytic anemia
Tx: steroids, rituximab, splenectomy
IgM
B cell lymphoma
Mono/EBV
Influenza
Mycoplasma
No Spherocytes
Treatment
Cold autoimmune hemolytic anemia
Tx: avoid cold, rituximab. No steroids.
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
Cytoskeleton spectrin membrane defect
Negative coombs test (non immune hemolysis)
Osmotic fragility test
EMA flow cytometry
Hereditary spherocytosis
Most common cause of aplastic crisis in hereditary spherocytosis
Parvovirus B19
Treatment for hereditary spherocytosis
Folate
Transfusion
Splenectomy
Cause of sickle cell disease
Amino acid 6 substitution
Valine fort glutamic acid
Sickle cell
Acute pain secondary to ischemia or infarction
Dactylitis
Treatment
Vasoocclusive crisis
Rehydration + pain control
Sickle cell
Shock
Pooling if blood in liver or spleen
Treatment
Sequestration crisis
Medical emergency
Transfusion.
Splenectomy after 2nd or 3rd episode
Infectious prophylaxis for sickle cell
Penicillin VK
Vaccines
Leading cause of mortality in sickle cell patients
Management
Acute chest syndrome
Due to fat embolism
Exchange transfusion
Sickle cell
Chest pain
Infiltrate on CXR
hypoxia
Acute chest syndrome
Management for CVA in sickle cell
Transfusion then image
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
Arrest in maturation of red cells
Infant
Chronic
Thumb abnormalities
Urogenital defexts
Craniofacial problems
Snub nose
Webbed neck
Steroids
Diamond Blackfan anemia
Suppression of erythroud production
Toddler
Transient
No steroids
Transient erythroblastopenia of childhood
Recurrent mucosal ulceration
Gingivitis
Cellulitis
Abscess
Pneumonia
Septicemia
Staph aureus, strep Epi, gram neg, enterococci
Neutropenia
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
Most common neutropenia in childhood
Anti-neutrophil antibodies
< 5 yo
Chronic benign neutropenia
Arrest in development of neutrophils
Autosomal recessive
Severe congenital neutropenia
Kostmann syndrome
Pancytopenia
Pancreatic exocrine insufficiency
Short stature
Diarrhea/steatorrhea
Recurrent infections
Skeletal abnormalities
Shwachman-Diamond syndrome
Platelet destruction by maternal antibodies
Mothers platelets are normal
Neonatal alloimmune thrombocytopenia
Mother and neonate both of low platelets
Neonatal autoimmune thrombocytopenia
Thrombocytopenia
Localized consumptive coagulopathy
Normal bone marrow
Kasabach-Merritt syndrome
Thrombocytopenia
Absent radius
Renal agenesis
Tetrology of Fallot
Decrease in megakaryocytes on bone marrow exam
TAR syndrome
Deficiency in vitamin K
Elevated PT
Born at home
Exclusively breastfed
Hemorrhagic disease of newborn
Elevated PTT
X-linked recessive
Deep joint bleeds
Hemophilia A (F8 deficiency)
Hemophilia B (F9 deficiency/christmas)
Most common malignant bone tumor in children and adolescents
Osteosarcoma
Unilateral limb pain
Elevated LDH, ALP, EsR
Adolescent going through growth spurt
Sunburst pattern
Osteosarcoma
Onionskinning
Ewing sarcoma
Central radiolucency surrounded by thick sclerotic bone
Pain worsens at night
Pain relieved by ibuprofen
Osteoid osteoma
Most common congenital condition associated with leukemia
Down syndrome
Reed sternberg cells
Non tender enlarged cervical or supraclavicular lymph node
Slow growing
Unexplained pruritus
Hodgkin’s Lymphoma
Supraclavicular node
Next step
Lymphoma
CXR
Most common solid tumor of infancy
Neuroblastoma
Most common childhood malignancy
Leukemia
Persistent bone or joint pain
Non tender hard smooth abdominal mass
Raccoon eyes and proptosis
Horner syndrome
Opsiclonus-myoclonus
Bluish-purplish subcutaneous nodules
Neuroblastoma
Diagnosing Neuroblastoma
Biopsy
Elevated urine VMA and HMA along with neuroblasts found in bone marrow
Painless abdominal mass
Elevated AFP
Higher risk with premature birth
Hepatoblastoma
Leukocoria
Strabismus
Normal red reflex in one eye
Retinoblastoma
Diagnosing retinoblastoma
MRI or US
Avoid CT - can increase risk of second primary cancers
Trilateral retinoblastoma
Bilateral retinoblastoma + pineal gland tumor
Risk for osteosarcoma, malignant melanomas
Unilateral/unifocal retinoblastoma
Bilateral/multifocal retinoblastoma
Sporadic
Genetic
Constipation
Visible or palpated rectal mass
Grape-like mass from vagina
Can get worse after trauma injury
Rhabdomyosarcoma
Panhypopituitarism
Bitemporal hemianopia
Craniopharyngioma
Tumor lysis syndrome
Treatment
High Phos, K, Uric acid
Low Ca
Hydration, allopurinol, alkalinization
Adverse effect
Alkylating agents
Gonadal dysfunction
Adverse effect
Cyclophosphamide
Hemorrhagic cystitis
adverse effect
Cisplatin
Hearing loss
Peripheral neuropathy
Adverse effect
Bleomycin
Pulmonary fibrosis
Adverse effect
Anthracycline (doxorubicin, daunorubicin)
Cardiac toxicity
Adverse effect
Vincristine/vinblastin
Neurotoxicity
SIADH
Adverse effect
Methotrexate
Oral and GI ulcers
Bone loss
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
Bone-within-bone appearance
Sickle cell disease
