hem onc Flashcards
normal MCV
70-90
range of alpha thal
missing one - silent
missing two - asymptomatic with mild microcytic hypochromic anemia
missing three - hgb H disease (hgb Barts as nbn) –> tx with splenectomy
missing four –> hydrops, stillbirth (or intrauterine transfusions –> BMT)
small for age Mediterranean child with anemia, HSM
beta thal major
F only on NBS
thickened bone due to extramedullary hematopoeisis
complications of beta thal major
cholelithiasis
hemochromatosis
how long to tx iron def anemia
until 2 months after hgb normalizes (to replenish stores)
anemias with high RDW
iron deficiency and lead toxicity
anemia with normal/low RDW
thalassemias
ringed sideroblast
lead poisoning vs sideroblastic anemia
free erythrocyte protoporphyrin
elevated in lead poisoning and iron deficiency
normal in thalassemia
why should you correct folate and B12 deficiency together?
if you only correct folate, could get reversible neuro damage
G6PD inheritance pattern
X linked recessive
smear findings in G6PD
heinz bodies, helmet cells, blister cells
inheritance of hereditary spherocytosis
AD
dx of hereditary spherocytosis
osmotic fragility or
EMA flow cytometry
amino acid substitution in SCD
glu –> val
macrocytic anemia, renal/eye/ear anomalies, abnormal skin pigment, growth retardation, forearm anomalies
Fanconi anemia
AR
risk of transformation to AML/MDS
infant with macrocytic anemia, dysmorphic facies
DBA
how to reduce risk of febrile non hemolytic transfusion reaction
leukocyte reduced blood
mucosal ulceration, gingivitis, cellulitis, abscesses, pneumonia
neutropenia
infant with severe recurrent infections, neutropenia
Kostmann syndrome = severe congenital neutropenia
risk of transformation to MDS and AML
tx: gCSF, BMT
cytopenias, pancreatic insufficiency, skeletal anomalies
Schwachmann Diamond
at risk for leukemic transformation
mild neutropenia in healthy infant
isoimmune neonatal neutropenia
monthly recurrent oral ulcers, fever, enlarged lymph nodes
cyclic neutropenia
AD
bugs responsible for death in cyclic neutropenia patients
clostridium perfringens and gram negative organisms
tx cyclic neutropenia
gCSF
incidental neutropenia in child
chronic benign neutropenia
anti-neutrophil antibodies
usually grow out of this by age 5
meds that cause thrombocytopenia
sulfas, seizure meds, vanc
meds that cause platelet dysfunction
aspirin, ibuprofen
anaphylactoid purpura
= HSP
age and prognosis in ITP
worse in older children - more likely to be chronic
other findings in TAR
renal agenesis
lymphocytosis
vit K dependent factors
2, 7, 9, 10
vit K deficiency and coag studies
prolonged PT and PTT
coag studies in hemophilia
prolonged PTT
dx hemophilia
measure factor levels
what to do for asymptomatic hemophilia pt with head trauma
give factors
coag studies in vWD
normal PT, normal or slightly prolonged PTT
vWF activity low
tx vWD
vasopressin for minor bleeding
factor VIII for major bleeding/surgery
amicar
bone tumor that metastasizes to lungs
osteosarcoma
sunbursting
bone tumor with onionskinning
Ewing
leg pain worse at night, relieved by NSAIDs, XR with radiolucent area surrounded by thick bone
osteoid osteoma
benign
sites with highest risk of relapse in ALL
CNS and testes - sanctuary sites
unexplained lymphadenopathy, cough, unexplained pruritus
lymphoma
most common solid tumor of infancy
neuroblastoma
persistent bone/joint pain, B symptoms, UTI, raccoon eyes, horner syndrome, opsoclonus-myoclonus, excess catechols
neuroblastoma
dx of neuroblastoma
biopsy
urine VMA and HMA (NOT urinary catecholamines)
age and neuroblastoma prognosis
< 1 yr: good
> 1 yr: bad
inheritance of RB
bilateral - AD with incomplete penetrance
unilateral - sporadic
RB1 gene on chr13
additional risk with RB
pineal gland tumors
most common soft tissue sarcoma
rhabdomyosarcoma
labs in tumor lysis
elevated uric acid, phosphate, potassium, LDH, renal insufficiency
why not to give steroids if can’t rule out malignancy
risk of TLS
dz that can cause anterior mediastinal masses
thymoma
teratoma
thyroid carcinoma
lymphoma (most likely to cause airway compromise)
cyclophosphamide SEs
hemorrhagic cystitis
cisplatin SEs
hearing loss and peripheral neuropathy
bleomycin SEs
pulmonary fibrosis
doxo/daunorubicin SEs
cardiac toxicity
vincristine/vinblastine SEs
neurotoxicity and SIADH
methotrexate SEs
oral ulcers, GI ulcers, bone loss
