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)
neutropenia definition
ANC < 1.5 (includes bands)
neutropenia work-up
CBC /w diff retics blood smear Viral Ig / PCRs (?viral suppression) LDH, uric acid (malignany markers) consult heme
ddx + management of neutropenia
- febrile + severe: admit for pip-tazo
- septic - treat as sepsis
broad differential
- well /w isolated neutropenia = refer to heme
- leukemia / infilatration
- aplastic anemia
- glycogen storage disease
- immune def
- cyclic neutropenia
- viral suppression
- auto-immune
- drug induced
- hypersplenism
- vit b12/folate def
- BEN (will be >0.8)
types of childhood leukemia
- ALL (most common, peak age 2-5)
- AML
- CML (adults)
- JMML
acute leukemia presentation
non-specific "viral" type symptoms anemia low WBC (fever, sepsis) low plt (bruising, petechiae, etc) bone pain
extramedullary:
liver, spleen, LN infiltration
CNS infiltration (asymptomatic, ICP, seizures, palsies)
testicular enlargement (painless)
work-up for leukemias
CBC + diff (WBC high or low, Hb and plt normal)
smear: blasts (or not – may only be in marrow)
PTT, INR
lytes: high K, uric acid, high P, low Ca - tumor lysis syndrome
blood culture if febrile
bone marrow aspirate
LP for CNS involvement
CXR to r/o mediastinal mass
ALL vs AML
ALL:
3-7 yo
lower risk: B cells, age 1-9, no mets
higher risk: T cells, induction failure, high WBC
AML: 15-40yo low risk: 1-10y, de novo, <4wks to remission higher risk: high WBC, older slightly worse than ALL
treatment for leukemia
- chemo (induction, consolidation, re-intensification, maintenance)
- 2.5 - 3 years for ALL
- 5 - 6 mo for AML
lymphadenopathy is normal if
<10mm (5 for elbow, 15 for inguinal)
any palpable supraclav, popliteal, iliac (above inguinal)
ddx for lymphadenopathy
- viral: EBV, CMV, adeno
- malignancy: lymphoma, leukemia, mets
- bacteria: staph, GAS, TB, cat scratch disease
- rickettsia
- fungal/protozoa
- autoimmune: RA, SLE
- storage disease: neimann-pick, gauchers
- kawasaki
- serum sickness
- sarcoid
SCD general tx
NOTE - in newborn screen
infection: prophylactic pen V from 2m-5y, s pneumo + n menigitides extra vaccines
stroke: annual screening until age 16-18 /w transcranial dopplers for narrowing in arteries, if +ve prophylactic transfusion
hydroxurea: decreases pain crises (increase HbF), but cause marrow suppression. Criteria to use (frequent or severe pain). Monitor, renal, liver, CBC.
transfusions: for Hb < 50-60, surgery, or complications.
folic acid
generalized lymphadenopathy work-up
consider
- CBC, diff
- lytes
- liver, renal
- blood culture, fungal serology
- EBV / CMV / HIV, toxo
- Igs, T and B cell counts
- CRP/ESR, ANA, dsDNA, RF
- Tb test
- imaging - US of LN, CXR (mediastinum, retropharyngeal)
- CT if concern dep space infection
indications for LN biopsy
> 2cm, increasing over 2 wks, no decrease after 4wks
supraclavicular
hard, matted, rubbery
fever, weight loss, abnormal CXR, hepatosplenomegaly
malignant LN staging
chest/abdo/pelvis CT, PET scan, bone marrow bx, LP
regional LN management
- observation at first ok
- can trial oral antibiotics
- bx if: supraclavicular, non-cervical area, consitutional sx, >6wks, rapidly growing and no dx
Hodkins lymphoma characteristics
- single growing node
- older
- painless, rubbery
- pruritis
- pain /w EtoH ingestion
- anorexia, B sx
- reed sternberg cells
tx = chemo +/- rads
non-hodkins lymphoma
multiple sites, peripheral nodes, non-contig spread. mediastinal mass, lymphadenopathies, abdominal or head + neck mass.
younger (except lymphoblastic)
treatment: chemo, CNS prophylaxis
anterior mediastinal mass
lymphoma
thymoma, teratoma, germ cell tumor, ectopic thyroid, thyroid Ca
middle mediastinal mass
lymphoma
neuroblastoma, sarcoma, esophageal cyst
posterior mediastinal mass
neuroblastoma paravertebral soft tissue infection ganglioneuroma lymphoma anatomical variants
symptoms of mediastinal mass
cough, CP, constiutional hormonal / cytokine sx airway issues horners SVC syndrome: cough, orthopnea, dyspnea, head + neck edema RVOT obstruction, cardiac tamponade
medulloblastoma
cerebellar tumor
rapid growth, ICP symptoms, cerebellar ataxia, CN palsies
workup/tx: MRI head + spine, LP, surgical excision
+/- ICP shunt, rads, chemo
