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Pediatrics > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Fanconi anemia

A

Pancytopenia!

  • macrocytic RBC
  • hypocellular bone marrow

Autosomal recessive

Aplastic anemia + bone marrow fail

Sx:
Short
Microcephaly
Abnl thumbs
Hearing loss
Microphthalmia
thrombocytopenia --> neutropenia --> anemia

Dx:
- Chromosomal breaks on genetic analysis in presence of diepoxybutane

Tx:

  • transfusions
  • abx
  • steroids
  • bone marrow transplant

Complication:
AML

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2
Q

1 RBC enzyme deficiency causing anemia

A

G6PD

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3
Q

Iron deficiency anemia

A

Microcytic hypochromic

Usually 6-24 mo old

#1 cause = inadequate intake of iron
Usually on diet of milk
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4
Q

Iron deficiency anemia

  • signs
  • dx
  • tx
A

Pale
Tachy
Koilonychia (spoon nails)

Dx

  • retic count (minimal)
  • CBC
  • LOW serum iron and ferritin
  • TIBC high

Tx

  • ferrous sulfate (6 mg/kg/d)
  • reticulocytosis should be seen within 72 h of start of ferrous sulfate
  • get retic count 5d after starting iron
  • Hg increase in 3-4 weeks
  • continue iron for 4-5 months after Hb level returned to normal
  • check Hct 1 mo after starting therapy

If iron therapy doesn’t work, consider Meckels

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5
Q

Hemolytic anemia

A

Premature destruction (eg spherocytosis) vs. enzymatic (G6PD)

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6
Q

Hereditary spherocytosis labs

A

Spherocytes

Increased

  • retic count
  • indirect bilirubin
  • LDH

Decreased
- haptoglobin

Negative coombs

Osmotic fragility test +

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7
Q

G6PD labs

A

Blister cells

Reticulocytosis

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8
Q

Tx spherocytosis

A

Folate

Splenectomy for severe but only do > 5 yo

Remember to give vaccines for

  • pneumo
  • meningo
  • Hib
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9
Q

Tx G6PD

A

Avoid triggers (sulfas, fava beans, high dose ASA, antimalarials)

Oral folic acid

Splenectomy w/ chronic disease

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10
Q

Sickle Cell disease

A

Valine –> glutamic acid @ 6th position of Beta chain

Sickling when oxygen low

NO features as newborn since HbF still there
Sx 2-4 months

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11
Q

Sickle cell sx

A

Dactylitis (swelling)

Autoinfarct of spleen –> encapsulated organism infection prone

Vasoocclusive episodes

Gallstones 2/2 increased bile production from chronic hemolysis

Prone to salmonella osteo

Aplastic crisis (2/2 parvovirus, etc)

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12
Q

Tx sickle cell

A

Maintenence:

  • Immunize
  • Ppx w/ PCN until 5 yo
  • folic acid suppement
  • hydroxyurea for pts w/ recurrent vascoocclusive events

Acute pain crises

  • hydration
  • analgesia
  • transfusion

Fevers –> IV ceftriaxone

Aplastic crisis –> transfusion

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13
Q

Beta Thalassemia major

A

surplus of alpha globin chains

Sx after 6 months

PE:

  • facial deformities 2/2 expansion of marrow
  • splenomegaly
  • heart failure 2/2 high urine output
  • gallstones

Labs

  • hypochromic microcytic anemia
  • hemosiderosis
  • increased unconj bilirubin
  • dx w/ Hb electrophoresis
  • high levels of HbF

Tx

  • transfusion therapy
  • chelation
  • deferoxamine for hemochromatosis
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14
Q

Diamond Blackfan sydnrome

A

Congenital pure red blood cell anemia

Pale infants in first few days
Anemia 2-6 mo old

Macrocytic anemia
- but no hypersegmented neutrophils

Bone marrow - reduced red cell precursors

Elevated EPO

PE:

  • short stature
  • webbed neck
  • cleft lip
  • shielded chest
  • triphalangeal thumbs

Tx

  • transfusions
  • steroids
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15
Q

Acquired Aplastic Anemia

A

Dec in RBC, WBC, platelet

Causes

  • ionizing radiation
  • chloramphenicol, sulfas, anticonvulsants
  • parvovirus, mono
  • idiopathic

High mortality rate!
Death 2/2 hemorrhage or infection

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16
Q 
Idiopathic Thrombocytopenic Purpura (ITP)
- etiology
- features
- dx
- tx
-
A

Immune mediated, can be preceded by viral infection

Signs:

  • acute onset
  • bruising and petechial rash
  • joint bleeding rare
  • NO splenomegaly

Dx

  • bone marrow - normal or increased megakaryocytes; all other findings and cell lines normal
  • varying degress of thrombocytopenia

Tx

  • excellent even w/p Tx
  • IVIG
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17
Q

Other causes of thrombocytopenia

A

Wiskott Aldrich (X linked)

Kasabach-Merritt

TAR syndrome

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18
Q

Kasabach Merritt

A

Rapidly enlarging cavernous hemangioma
+
Consumption coagulopathy –>
Thrombocytopenia

Usually cutaneous lesion
Low plt
Anemia

Tx:

  • surgery to remove
  • laser therapy
  • high dose steroids
  • interferon
  • aminocaproic acid for coagulopahty
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19
Q

Thrombocytopenia + absent radius

A

TAR syndrome

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20
Q

Hemophilia A

  • definition
  • presentation
  • PE
  • dx
  • tx
A

X linked recessive

Deficiency factor 8

Bleeding seen in neonatal period
Hemarthrosis can happen

Dx:

  • family hx
  • prolonged bleed after circumcision
  • increased PTT
  • decreased factor 8 activity, normal vWF
  • plt normal
  • PT normal

Tx

  • tx bleeds w/ factor 8
  • DDAVP to increase factor 8 levels in mild cases
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21
Q

Hemophilia B

  • definition
  • presentation
  • PE
  • dx
  • tx
A

X linked recessive

Deficiency factor 9
Less common than A

Vit K dependent factor - not distinguishable clinically from A

Dx:
- low factor 9

Tx:
- replace factor 9

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22
Q

von Willebrand disease

  • definition
  • presentation
  • PE
  • dx
  • tx
A

1 inherited bleeding tendency

Autosomal dominant

p/w nosebleeds, gum bleeds; spontaneous hemarthrosis rare

Dx:

  • increased bleeding time
  • mildly increased PTT
  • normal platelets, PT

Tx

  • FFP
  • Cryoprecipitate
  • DDAVP
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23
Q

Function of vWF

A

Adhesion platelets

Platelet to platelet aggregation

Carrier factor 8

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24
Q

Acute lymphocytic leukemia

  • definition
  • risks
  • presentation
A

Lots of lymphoblasts in bone marrow

Peak @ 3-4 yo

Increased risk with:

  • downs
  • ataxia telangiectasia
  • von Recklinhausen
  • sideroblastic anemia

Presentation

  • anorexia
  • pallor
  • bleeding
  • bruising
  • LAD
  • splenomegaly
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25
Q

ALL

  • dx
  • tx
A

Dx:

  • Blasts on periph smeark
  • abnormal CBC
  • maybe mediastinal mass on CXR

Tx:

  • vincristine + prednisone + L asparagine
  • ppx CNX radiation or intrathecal MTX
  • maintenance therapy for 2-3 years

Bone marrow is most common site of relapse

Poor prognosis if < 2 yo, boy, black, CNS mets

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26
Q

Tumor lysis sydnrome

A

Hyperuricemia 2/2 chemo

Tx

  • hydration
  • alkalization of urine
  • allopurinol

Be weary of Hyper K

Hyper PO4 can cause decrease in Ca resulting in tetany

27
Q

Hodgkin disease

  • risk factors
  • what is it?
  • presentation
A

Older kids

Risk factors:

  • EBV???
  • immunodeficiency?

Pathology:

  • Reed sternberg cell
  • 4 major histo types:
  • lymphocyte predominant
  • nodular sclerosing
  • mixed cellularity
  • lymphocytic depleted (worst prognosis)

Presentation

  • localized adenopathy (firm, nontender enlarged cervical or supraclavicular LN)
  • mediastinal mass
  • night sweats
  • fever, wt loss, anorexia
28
Q

When do you do bone marrow bx for pts with HOdgkins?

A

Stage 3 or 4 disease

Pts w/ B symptoms

29
Q

What are B symptoms

How often do they happen?

A

30% kids

Temp > 100.4
Night sweats
Wt loss more than 10% in 6 months

30
Q

Tx Hodgkin’s disease

A

Determined by disease stage, age, presence or absence of B sx

Chemo:

  • Mechlorethamine hydrochloride
  • vincrisine sulfate
  • procarbazine
  • prednisone

OR

Doxyrubicin
Bleomycin
Vinblastine
Dacarbazine

Radiotherapy to LN

Prognosis good

31
Q

Non-hodgkin lymphoma

  • what is it
  • risks
A

Neoplastic proliferation of immature lymphoid cells
vs ALL - accumulate outside of bone marrow

NHL kids younger than HL kids

Usually extranodal in presentation

Neoplasms of mature B cells

  • Burkitts
  • diffuse large B cell
  • mantle cell
  • follicular lymphoma

Neoplasms of mature T cells

  • adult T cell
  • mycosis fungoides/sezary syndrome
32
Q

Burkitt’s lymphoma - genetics

A

t (8,14)

33
Q

Mantle cell lymphoma - genetics

A

t (11,14)

translocation of cyclin D1 (11) and heavy chain Ig (14)

34
Q

Follicular lymphoma - genetics

A

t(14,18)

translocation of heavy chain Ig (14) and bcl-2 (18)

Indolent course! difficult to cure. bcl2 —| apoptosis

35
Q

What is Adult T cell lymphoma usually caused by?

A

HTLV-1

36
Q

Tx nonhodgkins lymphoma

A

Surgical debulking

CHOP

  • cyclophosphamide
  • vincristine
  • MTX
  • prednisone
37
Q

Brain tumors in kids

A

1 solid tumors in childhood

2/3 infratentorial

38
Q

Cerebellar astrocytoma

A

1 tumor of kids and best prognosis

2 histo types of astrocytoma:

1) pilocytic (most common, benign)
2) diffuse or fibrillary

Kids 5-10 yo

MRI to see

Tx:

  • resection
  • chemo depending on type, age, and if tumor is recurrent

5 yr survival = 90%

39
Q

Medulloblastoma

A

2 most common in kids

Primitive neuroectodermal tumor

Sometimes originate from roof of 4th ventricle

Aggressive, can mets extracranially

< 7 yo usually
Boys > girls

Usually in midline of cerebellum
Can cause hydrocephalus

Tx:

  • surgical excision
  • radiation
  • chemo

Poor prognosis if < 4 yo

40
Q

Brain stem fliomas

A

3 common posterior fossa tumor in kids

2 types:

1) anaplastic (poor prognosis)
2) low grade focal

Tx:

  • limited radiotherapy
  • chemo for palliative
  • surgery

Poor prognosis in general

41
Q

Ependymoma

A

Can be infra or supratentorial

4th ventricle is #1 site

Usually present as obstructive hydrocephalus

Tx:

  • surgery usually doesn’t get it all
  • radiotherapy
  • cyclic chemo

Prognosis for 5 yr

  • low grade (70%)
  • high grade (20%)
42
Q

Infratentorial tumors

A

Cerebellar astrocytoma
Medulloblastoma
Brainstem glioma
Ependymoma

43
Q

Supratentorial tumors

A

Craniopharyngioma

Optic gliomas

44
Q

Craniopharyngioma

A

Supratentorial

Cystic + solid areas that tend to calcify

Can present w/

  • short stature
  • periph vision loss
  • hydrocephalus

Can see calcifications on Xray or CT

Tx:

  • resect
  • radiation maybe

Need to f/u closely b/c can get hypothyroidism, DI, adrenal insufficiency

45
Q

Optic gliomas

A

Low grade astrocytomas

Cause decreased VA and pallor of the dics

More common in pts with:

  • neurofibromatosis
  • chiasmal tumors

Can present with asymmetric nystagmus if has chiasmatic tumor

Tx

  • delayed until tumor progresses
  • no tx if optic N tumor but normal vision - reeval q6 mo w/ CT
  • chemo for pts w/ visual changes
  • carboplatin + vincristine
46
Q

Wilms Tumor

  • what is it
  • risk
  • presentation
  • ddx
A

Tumor of neoplastic embryonal renal cells of the metanephros
- blastema + epithelium + stroma

Histo

  • spindle shaped cells
  • anaplasia
  • fibrillar inclusions
  • w/ presence of striated muscle

Most renal tumors = Wilms tumors

Associations:

  • WAGR (Wilms tumor, ANiridia, GU anomalies, MR)
  • Beckwith-Wiedemann
  • Denys Drash syndrome

Usually start ~3 yo

HTN in many presentations

Usually asymptomatic ab mass
- DOES NOT cross midline

Ddx - Neuroblastoma which is tumor of renal Origin

47
Q

Wilms tumor

  • dx
  • tx
  • prognosis
A

Dx

  • UA = microscopic or gross hematuria
  • US = see if intrarenal
  • CT to see how much the tumor covers
  • bx to stage

Tx

  • surgical resection
  • radiotherapy
  • actinomycin D + vincristine

Prognosis - most are good

48
Q

Neuroblastoma

  • what is it
  • risks
  • presentation
A

1 solid malignancy in kids outside of CNS

Malignancy of neural crest cells - usually give rise to chromaffin and adrenal medulla

Usually in preschool kids, mostly < 5 yo
Boys > girls

Assoc w/ Beckwith Wiedemann syndrome

Presentation:

  • ab mass
  • HTN
  • resp distress if in thoracic tumor
  • horner syndrome
  • opsoclonus myoclonus causing “dancing eyes and feet syndrome”

Mets common to bone, liver, skin

CROSSES the midline

49
Q

Neuroblastoma

  • dx
  • tx
  • complications
  • prognosis
A

Dx

  • CT scan
  • VMA and HMA levels for dx and response to therapy
  • bx
  • should do bone marrow bx and bone scan for mets
  • other markers: enolase, ferritin, LDH

Tx

  • Stage I + II = surgery
  • stage III + IV = chemo
  • -vincristine + cyclophosphamide + doxorubicin + cis-platinum + etoposide + danorubicin
  • high rate of spontaneous regression w/o therapy in stage IV-S (small tumors < 1 yo w/ mets but none to cortical bone)

Complications

  • < 1 yo best prognosis
  • > 2 yo worst prognosis
  • N-myc amplification or 1p deletion poorer prognosis
50
Q

Rhabdomyosarcoma

  • what is it
  • presentation
A 
Striated muscle tumor
#1 sarcoma in kids

Presentation

  • # 1 = mass (nasopharynx, face, trunk, GU, etc)
  • vaginal rhabdo –> vaginal grapelike mass (sarcoma botryoides)
  • tumors can mets to lung or bone
51
Q

Rhabdomyosarcoma

  • dx
  • tx
  • complications
A

Dx

  • CT or US
  • bone marrow, bone scan, CXR, and chest CT before surgery

Tx

  • dependent on primary tumor location
  • rarely is it complete resectable
  • will need combo of surgery or radiation or chemo

Primary site, extent of dz, and tx used determines best prognosis

52
Q

Anemia of prematurity tx

A

with iron supp, hg checks, blood transfusion if needed

Iron is given not because iron deficiency but because it will be stored and reutilized during active phase if eruthropoiesis

53
Q

Burr cells

A
  • spiculated RBCs seen in uremia or as artifact of prep
54
Q

Lab findings of sickle cell

A

Dec:
- hematocrit

Increased:

  • retic
  • serum LDL
  • unconj bilirubin

Peripheral smear:

  • sickled cells
  • howell jolly bodies
55
Q

Main dose limiting side effect of hydroxyurea

A

Myelosuppression

56
Q

Wilms tumor vs neuroblastoma

A

Wilms tumor more common than neuroblastoma

WIlms does not cross midline

Neuroblastoma crosses midline

57
Q

How do you dx paroxysmal noctunral hemoglobinuria?

A

sugar water test

58
Q

The presence of Howell-Jolly bodies means…

A

splenectomy

59
Q

1 cancer in kids

A

Acute lymphoblastic leukemia

Ages 2-5 yo

60
Q

Iron deficiency in kids

A

Fe store usually run out by 6 mo so need to supplement then

DO NOT use cows milk –> will cause iron deficiency anemia

+ iron (oral) therapy in those with deficiency

  • recheck Hg in 4 weeks
  • con’t oral therapy until Hg normalizes
61
Q

1 complication in sickle cell TRAIT is

A

painless hematuria

2/2 sickling in renal medulla

62
Q

Kasabach Merritt phenomenon

A

Seen with large vascular anomalies

Platelet and RBC sequestration within vascular tumor causes peripheral thrombocytopenia, coagulopathy, and microangiopathic hemolytic anemia

Tx:

  • steroids
  • a-interferon
  • vincristine
63
Q

DIC lab values

A

Consumption of

  • fibrinogen
  • 2, 5, 8

Prolongation of:

  • PT
  • PTT
  • TT

Decrease in

  • factor 8
  • platelet

Increase in:
- fibrin split production

Pediatrics (17 decks)

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