hematology and oncology Flashcards
Anemia /w reticulocytosis (high retics) ddx
- membranopathy: hereditary spherocytosis or eliptocytosis
- sickle cell
- enzyme: PK def, G6PD def
non-immune hemolysis (coombs -ve)
- HUS, TTP, DIC
- burns, wilson’s, vit def (hemolysis)
immune mediated (coombs+)
- auto-immune hemolysis: IgG (warm) or cold agglutinin
- hemolytic dis of newborn, transfusion incompatibility
- drug induced
anemia ./w low retics ddx
- microcrocytic: thalassemia, lead, sideroblastic, chronic disease
- normocytic: anemia chronic disease, renal failure, malignancy / marrow infiltrate, HIV, transient erythroblastopenia of childhood
- megaloblastic: B12 deficiency, folate deficiency, marrow failure (fanconi, myelopdysplastic, aplastic), hypothyroid, T21, liver disease, drugs
B12 deficiency causes
pernicious anemia, ileal resection, vegan, congenital transporter deficiency
w/u for anemia
CBC + diff
retics
peripheral smear
optional based on hx + PE
- hemolysis: LDH, haptoglobulin (low), indirect bili, coombs, G6PD
- spherocytosis: osmotic fragility test
- megaloblastic: RBC folate, serum b12
- thalassemia or SCD: sickle screen, Hb electrophoresis
- stool + urine for bloodloss
- Fe panel
Fe Deficiency anemia before 6mo ddx
blood loss, premies, SGA
otherwise very rare
complications / presentation of Fe def anemia
irritability, poor concentration, GI sx, reduced immunity, poor school performance, pica
w/u for Fe deficiency anemia
CBC, retics, blood smear
Ferritin
transferrin / TIBC (high)
soluble transferrin receptor (high)
serum iron
management of Fe deficiency anemia
elemental iron 4-6mg/kg/d + vit C
limit cow milk to 2 cups
follow up CBC + retics in 2wks, sub optimal response, r/o non-compliance, losses, malabsorption
treat for 3-6mo (replenish stores)
if severe: transfuse
hereditary spherocytosis
- extravascular hemolysis
- splenomegaly
- dx: osmotic fragility testing, flow cytometry
- tx: transfusions, splenectomy
G6PD deficiency
episodic anemia, jaundice, dark urine
triggers: drugs, fava beans
dx: heinz body + G6PD level (false neg in hemolysis)
tx: avoid triggers
Alpha thalassemia
have 4 genes for it
- 1 bad gene = silent
- 2 bad genes = alpha-thal trait, mild microcytosis (at risk for hydrops baby)
- 3 bad = HbH disease / alpha thalassemia disease, microcytosis + mild anemia
- 4 bad = hydrops fetalis, fatal (no alpha globin genes)
Beta Thalassemia
- minor = 1 copy, beta thalassemia trait. Genetic counselling
- intermedia - genetic interactions. mild anemia, childhood dx, occasional transfusions
- major = two bad copies, profound anemia, marrow expansion + bony growth, iron overload (increase absorption)
- needs transfusions + iron chelation
sickle cell disease events / complications list
vaso-occlusive (pain) aplastic crisis (parvovirus) splenic sequestration crisis acute chest syndrome fever / sepsis syndrome stroke priaprism gallstones
sickle cell pain episode sx + treatment
trigger: infection, fever, acidosis, hypoxia, dehydration, heat, cold
abdo, bone, joint pain, dactylitis
treatment:
- hydroxyurea or chronic transfusions
- spleen exam by fam or doc (surveillance)
- outpatient: early analgesia
- in ER: IV fluids, opioids + NSAIDs. CBC, retics, cultures, bili, liver enzyme, Cr, blood gas, CXR/imaging
- in-patient: pain mngmt, hydration, monitor ae, incentive spirometry + mobilization
aplastic crisis presentation + management
fatigue + pallor, parvo = trigger
transfusion RBCs
droplet-contact precautions
splenic sequestration crisis presentation + management
pallor, shock, splenomegaly
IVF +/- transfusion, splenectomy if recurrent or symptomatic + chronic
acute chest syndrome description + management
pulmonary infarction +/- infection
- IV cephalosporin
- PO macrolide (eg azithro)
- O2
- RBC if 10-20 < baseline
- exchange transfusion if rapidly progressing
stroke in SCD managemetn
- CT/MRI head
- if confirmed, exchange transfusion
- prophylactic transfusions or hydroxyurea for secondary prevention
priaprism management in SCD
- hydration, analgesia, uro consult
thrombocytopenia definition
plt < 150
ddx thrombocytopenia
low production
- infiltration
- infection
- injury
- genetic
destruction
- ITP
- SLE
- neonatal
- allo/autoimmune
consumption
- HIT
- DIC
- HUS
- TTP
- thrombosis
sequestration
- (hepato)splenomegaly
ITP presentation
plt < 100
normal Hb + WBC
can be acute or chronic, if chronic consider secondary causes
purpura +/- other bleeding, feels well
often following a viral infection
ITP work-up
CBC retics blood smear direct cooms lytes LDH Cr uric acid LFTs blood type
management of ITP
observe if pt > 10 + no bleeding (avoid high risk activities)
avoid NSAIDs + ASA
if <10 or significant bleeding:
- prednisone x4d or IVIG once
- consider anti-D Ig if RH +ve
- refractory: ritux, splenectomy, high dose steroids, immunosupressants
- target >20 (spont bleed), or >50 surgery, >100 for neurosurg or major bleed
DON’T transfuse Plt (will get eaten up, other txs effective)
