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_MS2 HemeOnc > 8/14- ALL > Flashcards

8/14- ALL Flashcards

1
Q

What is the stem cell problem in the following conditions:

  • Aplastic anemia:
  • Myeloproliferative Syndromes:
  • Myelodysplasia:
  • Acute Leukemia:
A

- Aplastic anemia: stems are gone

- Myeloproliferative Syndromes: SCs lead to progenitros with increased proliferation, normal maturation

- Myelodysplasia: SCs support ineffective hematopoiesis (seems to mature, but ineffective; low counts but cellular marrow)

- Acute Leukemia: SCs proliferate but do not mature

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2
Q

What does this show?

A

Blasts (probably lymphoid)

  • Not as distinct nucleoli as myeloblast
  • Little cytoplasm
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3
Q

Thyroid mass suggests what?

A

Lymphoma

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4
Q

TdT(+) is a marker of what?

A

Very young cells

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5
Q

What is CD34(+) a marker of?

A

Stem cells (very young)

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6
Q

What is t(11;19) associated with?

A
  • Prevalent in childhood and infant ALL
  • Associated with CNS involvement and a poor prognosis
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7
Q

What is the genetic alteration common in childhood/infant ALL and associated with CNS involvement/poor prognosis?

A

t(11;19)

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8
Q

What are some characteristics of ALL?

A

Acute Lymphocytic Leukemia (ALL):

  • Bone marrow > 30% lymphoblasts
  • May present as mediastinal or LN only: lymphoblastic lymphoma
  • Acquired somatic mutations in a single lymphoid progenitor cell
  • The blast shares many features of normal lymphoid progenitors– “arrested development”
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9
Q

Clinical presentations of ALL?

A
  • Fatigue
  • Malaise
  • Arthralgias, arthritis
  • Lymphadenopathy
  • Hepatosplenomegaly
  • Fever, Infection
  • Bleeding
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10
Q

What is this?

A

Acute Lymphoblastic Leukemia

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11
Q

Epidemiology of ALL?

A

Most common type of leukemia in children

  • Children > adults
  • Males > females
  • Whites > blacks

85% B cell; 15% T cell

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12
Q

T/F: Pts with ALL commonly present in a characteristic way? How?

A

False; there is variable presentation

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13
Q

What are the variable presentations of ALL?

A
  • Completely asymptomatic: 1-2% (CBC may be wnl or very closely to nl)
  • Significant HSM in 2/3
  • Clinically significant adenopathy in 1/2
  • Fever in 60% (life threatening sepsis at Dx is rare but not impossible)
  • Bone pain in 1/4
  • Some bleeding manifestation (petechiae, purpura) in 1/2

Variable prodromal period:

  • Vague hx of not feeling well for days-weeks (THIS IS AN ACUTE PROCESS)
  • Occassionally several months (BEWARE PRE-TREATMENT, e.g .steroids)
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14
Q

What causes ALL?

A
  • Don’t know; idiopathic
  • In utero events (1, 2, vs. 10,000 “hits”)
  • Environmental exposure/drug-toxin metabolizing genes
  • Blasts vs. the immune system (stochastic?)
  • “Genetics”
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15
Q

ALL is associated with what other conditions?

A

- Down

- Unstable genes: ataxia telangiectasia, Bloom, Fanconi

- Wiskott Aldrich, congenital Hypogammaglobulinemia

- In utero: high risk for twin if one twin has ALL

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16
Q

DDx of ALL?

A

Nonmalignant conditions:

  • Juvenile rheumatoid arthritis
  • infectious mononucleosis
  • ITP
  • Pertussis and Parapertussis
  • Aplastic anemia
  • Other viral illnesses
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17
Q

When should a pediatrician consider Dx of ALL?

A

Combo of:

  • Unexplained adenopathy or marked HSM
  • Bone pain
  • Bleeding symptoms/pallor

Meets the threshold of obtaining a CBC:

  • 2+ more cell lines “down” or WBC up and one other line down
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18
Q

Incidence of acute lymphoid leukemia by age?

A

More in KIDS

  • Peak at 4 yo
  • 75% of childhood leukemia
  • Most common pediatric cancer
19
Q

What is this?

A

Lymphoblasts in CSF from ALL patient

20
Q

What is a big difference between ALL and AML?

A

ALL commonly includes CNS involvement

  • Like meningitis, with headache and CN palsies
21
Q

What is the typical presentation of T cell ALL?

A

Young boy with a mediastinal mass

  • If there is no bone marrow involvement, we call this “lymphoblastic lymphoma” (can be B or T cell disease)
  • If you wait long enough, you will have bone marrow and peripheral blood involvement
  • Same treatment as ALL, just lymphoblastic lymphoma is the lymph node version
22
Q

Types/characteristics of ALL in terms of cell types involved and presentation?

A

Lymphoblasts in bone marrow and blood

  • FAB L1, L2

Lymphoblastic lymphoma

  • Mediastinal or lymph node T-cell blasts
  • Rare Patients have a B-cell phenotype

FAB L3 leukemia

  • Burkitt’s lymphoma
23
Q

Molecular markers of B cell development

A
24
Q

Molecular makers of T cell development

A
25
Q

Which antigen markers do we care about the most?

  • pre-B:
  • pre-T:
  • Myeloid:
  • “Stem cell”:
A

pre-B:

  • CD10
  • CD19
  • CD20
  • CD22

pre-T:

  • CD3
  • CD5
  • CD7

Myeloid:

  • CD13
  • CD14
  • CD15
  • CD33

“Stem cell”:

  • CD34
  • TDT
26
Q

What are current classification methods of ALL?

A
  • FAB classification on Wright Stain (L1-3)
  • Other morphological and special stain criteria (PAS stain)
  • Immunophenotype
  • Cytogenetics (ploidy/DI;standard G-banding, FISH, RT-PCR)
  • Molecular/Experimental: cDNA microarray
27
Q

What is the breakdown of immunophenotypes in ALL?

A

B-cell precursor ALL: 85%

T-cell precursor ALL :13-15%

B-cell ALL: 1-2%

*Cytogenically, adults are not just big children

28
Q

What are some chromosomal findings with poor prognostic significance in ALL?

A

Poor:

- t(4;11): AFP and MLL/ALL-1/HTRX

- t(1;19): E2Aa and PBX1

- t(9;22): Bcr/ABL (Philadelphia chrom)

  • Euploidy
  • Hypoploidy
  • Extreme hyperdiploidy (i.e. tetraploidy)
29
Q

What are some chromosomal findings with good prognostic significance in ALL?

A

Good:

  • t(12;21): TEL and AML1 (RT-PCR or FISH)
  • Trisomy 4 and 10 (17 but not 5)
  • Hyperdiploidy
30
Q

What are some good clinical prognostic factors in ALL?

A
  • female sex
  • age 3-8
  • low WBC
  • Pre-B phenotype
  • C10 (CALLA +)
  • low LDH
  • hyperdiploid cytogenetics
31
Q

What are some poor clinical prognostic factors in ALL?

A
  • male sex
  • age above 8
  • high WBC
  • L3, T-cell
  • CNS presentation
  • high LDH
  • Ph chromosome
32
Q

What are some accepted principles of therapy in ALL?

A

One drug is NEVER enough

  • Duration of therapy should be > 18 months (130 weeks on POG protocols, COG 2.5 years for girls and 3.5 years for boys)
  • Sanctuary sites need special consideration—primarily CNS
  • Intensity is scaled to risk, but variation in intensity is important
33
Q

Emerging principles of therapy in ALL?

A

Early Response and Degree of that response may be the most important prognostic indicator

  • Minimal Residual Disease (MRD) is probably important
  • Relapse is the worst prognostic sign and salvage rate will depend on timing of the relapse and the intensity of the initial therapy
34
Q

Active Agents to use (chemo) for ALL?

A
  • Vincristine
  • Pred/Dexamethasone
  • L-Asp (Ecoli, Erwinia,Peg)
  • Daunomycin (all anthra)
  • Cytoxan (other alkylators)
  • Ara C
  • 6 mercaptopurine (6MP), MTX
35
Q

Common Approaches to ALL Therapy?

A

(Don’t need to know details)

  • CNS as a sanctuary is a worry
  • Bone marrow transplant used in first remission for high risk disease or in 2nd remission
36
Q

What is BiTE? CAR?

A

Bispecific T cell engager

  • Targets malignant T cells??

Chimeric Antigen Receptor (CAR) involved in harnessing your T cells

  • Anti B19 CAR T used in ALL with 70-90% response rates
  • Often durable but at least allow segue to transplant

Complications:

  • Cytokine release syndrome
  • Encephalopathy
  • B cell depletion
37
Q

What does this show?

A

Burkitt cells in the blood and marrow

  • Characteristic appearance in peripheral blood with medium sized cells with open nuclei and nucleoli visible; cytoplasm is very blue with vacuoles
  • Can be a leukemia– L3 in FAB classification
38
Q

Important genetics in Burkitt lymphoma?

A

t(2;8)

t(8;14)

t(8;22)

39
Q

What is Burkitt lymphoma?

A
  • African variety: jaw tumor, strongly linked to Epstein-Barr Virus infection (50% BL due to EBV in US)
  • May present as abdominal mass
  • Most rapidly growing human tumor
  • Treated with multidrug regimen similar to pediatric leukemia/lymphoma regimens
40
Q

What is shown here?

A

Jaw tumor characteristic in presentation of Burkitt lymphoma (African variety)

41
Q

What is this?

A

Burkitt lymphoma (Starry Night cells)

42
Q

What is Tumor Lysis Syndrome? When does it occur?

A

Occurs in ALL L3 and Burkitt’s Lymphoma

  • High grade lymphomas
  • AML less often

Massive tumor death results in:

  • High LDH
  • Increased Uric Acid
  • Increased Phosphate
  • Hypocalcemia
  • Renal Failure (urate nephropathy, CaPO4 crystals)
  • Hyperkalemia
43
Q

How can Tumor Lysis Syndrome be prevented?

A
  • Hydration
  • Allopurinal
  • Alkalinization
  • Recombinant uricase

_MS2 HemeOnc (33 decks)

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