Heme/Onc Flashcards
Diagnosis of von Willebrand
vWF antigen
vWF activity/ristocetin cofactor
- Normal > 50%
- Abnormal < 30%
- 30-50% is borderline
Factor VIII
Prolonged PTT in some with factor 8 problems
von Willebrand Disease (Inheritence and Px)
autosomal dominant
mucosal bleeding
Wiskott Aldrich
Immunodeficiency, Eczema, thrombocytopenia
immunoglobulin problems: Elevated IgA and IgE (WEEskott AAAAldrich) & low IgM and IgG)
Long term chemo complication f/u
Doxo/Radiation - ECHO q2-5 years based on anthracycline dose and radiation dose
Bleomycin - baseline PFTs, then PRN
Prednisone - bone density, annual eye exam
Vincristine - annual exam for peripheral neuropathy
Post radiation screening
PFTs - baseline then prn
ECHO q2-5 years
TSH, T4 annually
Ataxia, nystagmus and muscle jerks
neuroblastoma
Urine HVA/VMA to diagnose
Lymphoma
B symptoms, supraclavicular node, pancytopenia, risk of SVC syndrome from mediastinal mass, do a CXR first to r/o mediastinal mass before CT so they don’t die in the scanner
Hemoglobin electrophoresis
FA = Normal
FS = sickle cell disease
FAS = sickle cell trait
FSA = sickle cell-beta thalassemia
0% HBA = sickle cell disease
50% HBA/HBS = trait
stroke in SCD
Screening
Annual TCD ultrasounds (age 2 to 16) to identify high-risk patients
Acute management
Maintain high index of suspicion, evaluate with CT scan +/- MRI
In children, AIS is more common than hemorrhagic, but either may occur
Exchange transfusion for acute stroke to get HbS < 30%
Prevention
Strong evidence for chronic transfusions for primary and secondary prevention
Mentzner index
Mentzer index = MCV/RBC
<13 = thalassemia (marrow produces normal number of cells (RBC) but MCV small)
(thal-LESS-semia)
> 13 = Iron Def Anemia (marrow can’t produce as many cells (low substrate) so MCV AND RBC low)
treatment of vWD Type 3
treatment with a virally attenuated, VWF-containing concentrate (Humate P)
Cryoprecipitate can be used if not a baby (it has vWF and factor 8 but is not virally attenuated)
leukemia prognosis predictors
Age - <1yr or >10yr = worse outcomes
Concentration of circulating blasts (WBC count >50 = worse outcomes)
Philadelphia chromosome (t9;22)
– occurs in 3-5% of kids with ALL
– translocation site involves abl proto-oncogene with production of an abnormal fusion protein (bcr abl) that has tyrosine kinase activity
– worse response to conventional chemo
– tyrosine kinase inhibitor imatinib – – actively binds to bcr abl fusion protein
JAK kinase
– greater freq of this mutation in high risk ALL
– worse treatment outcomes
An 11 month old presents with a scaly rash all over, especially in the diaper area. He also has exopthalmos and HSM. Xrays show bony lucencies on the scalp. What is the likely diagnosis?
langerhans cell histiocytosis (LCH)
- bone: bony lucencies
- skin (difficult-to-treat scaly, papular, seborrheic dermatitis of the scalp, diaper, axillary, or posterior auricular regions)
- hent: exopthalmos
- endo: DI
- lymphadenopathy, HSM
- pulm infiltrates
diagnosing LCH
Tissue biopsy = diagnostic → easiest to perform on skin or bone lesions
Other studies to be done in all patients: CBC, LFTs, coag, skeletal survey, chest XR, and measurement of urine osmolality
Opsoclonus myoclonus syndrome (OMS)
paraneoplastic syndrome characterized by rapid multidirectional eye movements, involuntary muscle spasms, and irritability. Thought to be secondary to autoimmune reaction
transient myeloproliferative disease
Trisomy 21
high leuks, blasts in the peripheral blood, associated anemia, thrombocytopenia, and hepatomegaly.
don’t need bone marrow - do flow cytometry
kids with T21 should be screened with CBC + diff + smear at birth to evaluate for TMD
No treatment if low risk as most spontaneously resolve.
Indications to treat: hyperleukocytosis (WBC>100), severe liver dysfunction, hydrops fetalis, cardiac effusions, and DIC.
Low dose 7 days cytarabine arabinoside; high risk toxicity
Questions about heparin insensitivity
= antithrombin 3 deficiency
Ewing Sarcoma
XR and BMA findings
Eww, onions, Eww moths (moth eaten lytic appearance), Eww cancer in my axial bones. Bilateral BMA and biopsy shows small round blue cell tumor
Osteosarcoma
XR: Sunburst O like the sun
tumors at the ends of long bones near the physis
Leukemia PEARLS
ulcerative mucositis, pancytopenia, HSM
Vaccines in SCD
13-valent pneumococcal conjugate and polysaccharide vaccines against Streptococcus pneumoniae
Both conjugated quadrivalent meningococcal (A,C,W,Y) and serogroup B vaccines targeting Neisseria meningitidis
Extra booster dose against Haemophilus influenzae type B (Hib), immunization against hepatitis A and B, and annual influenza vaccines are all recommended.
In the context of travel, vaccination against Salmonella typhi and malaria prophylaxis should be offered
tumor lysis syndrome
high potassium
high phosphate
high urate
low calcium
Treatment for TLS:
- hyperhydration
- rasburicase (neutralizes the high urate)
- urine alkalinization
Leukemia Presentation
Cytopenias (Normal or high WBC but neutropenia)
Bulky disease (HSM, LNs, mediastinal mass, bony pain, leukemia cutis)
Sanctuary sites (CNS, Testicular - must look)
Constutional (Wt loss >10%, drenching night sweats, fever)
Leukemia investigations
CBC, smear
tumor lysis labs
coags
MUST GET CXR
LP, Bone marrow aspirate
