Leukemia Flashcards

1
Q

how do you calculate the absolute neutrophil count

A
  • ANC= WBC X total neutrophils
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2
Q

what is a normal ANC

A
  • > 1000

- usu 1500- 8000

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3
Q

what is a neutropenic ANC

A
  • 500-1000
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4
Q

what is severe neutrophenia

A
  • chronically < 500
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5
Q

what is the role of neutrophils

A
  • fight bacterial infections
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6
Q

indications for bone marrow bx

A
  • pt specific i.e. unexplained anemia
  • pancytopenia
  • abnormal cells in circulation
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7
Q

contraindcations for bone marrow bx

A
  • hemophilia
  • severe DIC
  • other severe bleeding disorders
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8
Q

things that are NOT contraindications to bone marrow bx

A
  • thrombocytopenia

- therapeutic anticoagulation

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9
Q

sites for bone marrow aspiration

A
  • posterior superior iliac crest and spine*
  • anterior iliac crest
  • greater troch
  • vertebral bodies or ribs
  • sternum- usu C/I
  • tibia in kids < 12-18 mo
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10
Q

what causes leukemia

A
  • too many progenitor cells produced in bone marrow
  • abnormal cells dont die when they should
  • overproduction of bad cells interferes with prod of good cells
  • cells accumulate and occupy space -> leukostasis
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11
Q

how do you categorize leukemias

A
  • lymphoid vs myeloid cells

- acute vs chronic

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12
Q

what is the blast cell count in leukemia

A
  • > 20%
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13
Q

what is the blast cell count in myelodysplastic syndrome

A
  • < 20%
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14
Q

what type of leukemia is most common in kids

A
  • ALL
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15
Q

what type of leukemia is most common in adults

A
  • AML
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16
Q

what are myelodysplastic syndromes

A
  • bone marrow does not make enough good cells
  • make immature cells < 20%
  • may present with initial low grade anemia
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17
Q

types of myelodysplastic syndromes

A
  • refractory anemia
  • refractory cytpopenia with multilineage dysplasia
  • refractory anemia with ringed sideroblasts
  • refractory cytopenia with multilineage dysplasia and ringed sideroblasts
  • refractory anemia with excess blasts
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18
Q

risk factors for myelodysplastic sydromes

A
  • age*- rarely in pts < 60
  • smoking
  • long term exposure to chemicals
  • radiation
  • inherited disorders
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19
Q

inherited disorders that cause myelodysplastic syndromes

A
  • fanconi anemia
  • shwahmann diamond syndromes
  • familial platelet disorder
  • severe congenital neutropenia
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20
Q

acute myelogenous leukemia (AML)

A
  • rapid accum of immature meyloid cells
  • accum in bone marrow -> interfere with other production lines
  • increased incidence with age
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21
Q

risk factors for AML

A
  • genetics- trisomy 21, fanconi
  • chemicals
  • high dose radiation
  • drugs- alkylating agents, topoisomerase II inhibitors, chloramphenicol, phenylbutazone
22
Q

si/sx of AML

A
  • can be gradual or abrupt
  • fatigue
  • anorexia, weight loss
  • fever +/- infx
  • bleeding, easy bruising
  • HA
23
Q

PE findings for AML

A
  • fever
  • splenomegaly
  • LAD
  • sternal tenderness
  • ecchymosis, petechiae
  • papilledema
  • CN abnormalities
  • gum hypertrophy
  • skin infiltrates
  • bone pain
24
Q

work up for AML

A
  • auer rods on smear*
  • bone marrow bx and aspirate*
  • LP if suspect CNS involvement
  • immunophenotyping
  • cytogenetics
25
treatment for AML
- depends on genetics and prognosis* - goal is destruction of all leukemia cells - after remission pts will cont to be treated to prevent relapse - may get HLA matched SCT
26
chronic myeloid leukemia (CML)
- increased proliferation of granulocytes (neutrophils, eos, basos) - BCR-ABL rearrangement -> philadelphia chromosome
27
at what age is CML usu diagnosed?
- in 60s
28
cause of CML
- no known genetic, geographical, or ethnic assoc - no chemical or infx assoc - possible increased risk with ionizing radiation
29
stages of CML
- chronic - accelerated: 15% blasts - blast: 30% or more blasts, extramedullary disease and resembles AML - some progress through stages in order, some dont
30
PE findings for CML
- splenomegaly- in blast crisis - mild hepatomegaly - LAD
31
dx of CML
- bone marrow aspirate and bx* - cytogenetics + Philadelphia chromo - fish/molecular studies + for BCR-ABL gene
32
what determines prognosis of CML
- % blasts - cytogenetics - age of pt - platelets - spleen size
33
what is assoc with a poor prognosis for CML
- older age - severe anemia - neg Ph chromosome - thrombocytopenia - hepato/ splenomegaly
34
treatment for CML
- imatinib | - if acute or blast phase, use tyrosine kinase inhibitor + chemo -> allogenic SCT
35
acute lymphoblastic leukemia (ALL)
- proliferation and accumulation of lymphoid progenitor cells - most common childhood cancer, responds well to tx - bimodal age distribution: kids, 40s
36
etiology of ALL
- largely unknown - possible chromo translocation in utero - high incidence in kleinfelter and down syndrome - possible viral assoc
37
viral assoc with ALL
- HTLV-1 | - varicella
38
risk factors for ALL
- prev CA tx - exposure to radiation - genetic disorder, esp down syndrome - sibling with ALL
39
si/sx of ALL
- signs of bone marrow failure - malaise, fatigue - bleeding, bruising - secondary infection - B sx - CNS involvement: CN palsies, meningeal infiltration
40
what are B sx
- fever - weight loss - night sweats
41
work up for ALL
- bone marrow aspirate and bx - LP if CNS involvement - anemia, thrombocytopenia, lymphoblasts - platelets < 100,000 - immunophenotyping - cytogenetics - molecular studies
42
chronic lymphocytic leukemia (CLL)
- most common leukemia in western hemisphere - expansion of CD5 pos B cells - B cells undergo gene arrangement in marrow -> immuno-incompetent B cells
43
possible causes for CLL
- 15-20% of pts have family member with CLL or lymphoproliferative disorder - single nucleotide polymorphisms have been identified
44
gene alterations assoc with CLL
- most common is 13q deletion - worst prognosis is 17p - 11q assoc with advanced disease
45
si/sx of CLL
- insidious onset - recurring infx - enlarged LN - fatigue - loss of appetite - B sx
46
PE findings for CLL
- LAD- cervical, supraclavicular, axillary | - spleno/ hepatomegaly
47
dx of CLL
- flow cytometry to confirm B cell surface antigens - must distinguish mantle cell and large granular leukemia - AIHA commonly present - smudge cells on smear
48
staging systems used for CLL
- Rai staging: 0-IV | - binet staging: A, B or C
49
treatment for CLL
- early stage- follow pt, no tx - chemo - immunochemo - B cell signaling inhibitors - stem cell transplant - palliative care
50
harry cell leukemia
- rare - B cell lineage - mostly in adult males - presents like CLL but NO LAD