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Paediatrics > hematology and oncology > Flashcards

hematology and oncology Flashcards

1
Q

Anemia /w reticulocytosis (high retics) ddx

A
  • 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
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2
Q

anemia ./w low retics ddx

A
  • 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
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3
Q

B12 deficiency causes

A

pernicious anemia, ileal resection, vegan, congenital transporter deficiency

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4
Q

w/u for anemia

A

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
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5
Q

Fe Deficiency anemia before 6mo ddx

A

blood loss, premies, SGA

otherwise very rare

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6
Q

complications / presentation of Fe def anemia

A

irritability, poor concentration, GI sx, reduced immunity, poor school performance, pica

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7
Q

w/u for Fe deficiency anemia

A

CBC, retics, blood smear

Ferritin
transferrin / TIBC (high)
soluble transferrin receptor (high)
serum iron

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8
Q

management of Fe deficiency anemia

A

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

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9
Q

hereditary spherocytosis

A
  • extravascular hemolysis
  • splenomegaly
  • dx: osmotic fragility testing, flow cytometry
  • tx: transfusions, splenectomy
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10
Q

G6PD deficiency

A

episodic anemia, jaundice, dark urine

triggers: drugs, fava beans
dx: heinz body + G6PD level (false neg in hemolysis)
tx: avoid triggers

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11
Q

Alpha thalassemia

A

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)
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12
Q

Beta Thalassemia

A
  • 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
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13
Q

sickle cell disease events / complications list

A 
vaso-occlusive (pain)
aplastic crisis (parvovirus)
splenic sequestration crisis
acute chest syndrome
fever / sepsis syndrome
stroke
priaprism
gallstones
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14
Q

sickle cell pain episode sx + treatment

A

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

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15
Q

aplastic crisis presentation + management

A

fatigue + pallor, parvo = trigger

transfusion RBCs
droplet-contact precautions

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16
Q

splenic sequestration crisis presentation + management

A

pallor, shock, splenomegaly

IVF +/- transfusion, splenectomy if recurrent or symptomatic + chronic

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17
Q

acute chest syndrome description + management

A

pulmonary infarction +/- infection

  • IV cephalosporin
  • PO macrolide (eg azithro)
  • O2
  • RBC if 10-20 < baseline
  • exchange transfusion if rapidly progressing
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18
Q

stroke in SCD managemetn

A
  • CT/MRI head
  • if confirmed, exchange transfusion
  • prophylactic transfusions or hydroxyurea for secondary prevention
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19
Q

priaprism management in SCD

A
  • hydration, analgesia, uro consult
20
Q

thrombocytopenia definition

A

plt < 150

21
Q

ddx thrombocytopenia

A

low production

  • infiltration
  • infection
  • injury
  • genetic

destruction

  • ITP
  • SLE
  • neonatal
  • allo/autoimmune

consumption

  • HIT
  • DIC
  • HUS
  • TTP
  • thrombosis

sequestration
- (hepato)splenomegaly

22
Q

ITP presentation

A

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

23
Q

ITP work-up

A 
CBC
retics
blood smear
direct cooms
lytes
LDH
Cr
uric acid
LFTs
blood type
24
Q

management of ITP

A

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)

25
Q

neutropenia definition

A

ANC < 1.5 (includes bands)

26
Q

neutropenia work-up

A 
CBC /w diff
retics
blood smear
Viral Ig / PCRs (?viral suppression)
LDH, uric acid (malignany markers)
consult heme
27
Q

ddx + management of neutropenia

A
  • 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)
28
Q

types of childhood leukemia

A
  • ALL (most common, peak age 2-5)
  • AML
  • CML (adults)
  • JMML
29
Q

acute leukemia presentation

A 
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)

30
Q

work-up for leukemias

A

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

31
Q

ALL vs AML

A

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
32
Q

treatment for leukemia

A
  • chemo (induction, consolidation, re-intensification, maintenance)
  • 2.5 - 3 years for ALL
  • 5 - 6 mo for AML
33
Q

lymphadenopathy is normal if

A

<10mm (5 for elbow, 15 for inguinal)

any palpable supraclav, popliteal, iliac (above inguinal)

34
Q

ddx for lymphadenopathy

A
  • 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
35
Q

SCD general tx

A

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

36
Q

generalized lymphadenopathy work-up

A

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
37
Q

indications for LN biopsy

A

> 2cm, increasing over 2 wks, no decrease after 4wks

supraclavicular

hard, matted, rubbery

fever, weight loss, abnormal CXR, hepatosplenomegaly

38
Q

malignant LN staging

A

chest/abdo/pelvis CT, PET scan, bone marrow bx, LP

39
Q

regional LN management

A
  • observation at first ok
  • can trial oral antibiotics
  • bx if: supraclavicular, non-cervical area, consitutional sx, >6wks, rapidly growing and no dx
40
Q

Hodkins lymphoma characteristics

A
  • single growing node
  • older
  • painless, rubbery
  • pruritis
  • pain /w EtoH ingestion
  • anorexia, B sx
  • reed sternberg cells

tx = chemo +/- rads

41
Q

non-hodkins lymphoma

A

multiple sites, peripheral nodes, non-contig spread. mediastinal mass, lymphadenopathies, abdominal or head + neck mass.

younger (except lymphoblastic)

treatment: chemo, CNS prophylaxis

42
Q

anterior mediastinal mass

A

lymphoma

thymoma, teratoma, germ cell tumor, ectopic thyroid, thyroid Ca

43
Q

middle mediastinal mass

A

lymphoma

neuroblastoma, sarcoma, esophageal cyst

44
Q

posterior mediastinal mass

A 
neuroblastoma
paravertebral soft tissue infection
ganglioneuroma
lymphoma
anatomical variants
45
Q

symptoms of mediastinal mass

A 
cough, CP, constiutional
hormonal / cytokine sx
airway issues
horners
SVC syndrome: cough, orthopnea, dyspnea, head + neck edema
RVOT obstruction, cardiac tamponade
46
Q

medulloblastoma

A

cerebellar tumor

rapid growth, ICP symptoms, cerebellar ataxia, CN palsies

workup/tx: MRI head + spine, LP, surgical excision
+/- ICP shunt, rads, chemo

Paediatrics (23 decks)

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