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Heme > Heme Malignancies 2: Arising in the Bone Marrow > Flashcards

Heme Malignancies 2: Arising in the Bone Marrow Flashcards

1
Q

What is the defining feature of Acute leukemias?

A

Blasts

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2
Q

Immunophenotype of blasts, in general.

A

CD34+

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3
Q

Immunophenotype of MYELOID BLASTS

A

CD34+, CD33+

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4
Q

Immunophenotype of LYMPHOID BLASTS

A

Tdt+, CD10+

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5
Q

Immuniphenotype seen in Lymphomas.

A

Mostly B cells are lymphomas. so CD19+ and CD20+

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6
Q

Can you diagnose a cancer on immunophenotype alone?

A

NO! But it is an essential part of the diagnostic workup.

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7
Q

What are the 4 subtypes of AML?

A
  1. AML with no cytogenetic findings
  2. AML - RUNX1-RUNX1
  3. Acute Premyelocytic Leukemia - PML-RARA
  4. Acute Myelomonocytic Leukemia - CBFC-MYH11
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8
Q

What is the translocation associated with Acute Premyelocytic Leukemia?

A

t(15,17)

PML-RARA

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9
Q

What is the translocation associated with AML (normal with cytogenetics)

A

RUNX1-RUNX1

t(8;21)

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10
Q

What cytogenetic finding is associated with Acute Myelomonocytic Leukemia? (AML subtype)

A

CBFC-MYH11

inv (16,16)

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11
Q

Can you diagnose the 3 subtypes of AML with no cytogenetic findings if the blast count is below 20%?

A

Damn right, and you better catch it quick.

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12
Q

Talk about AML with the PML-RARA TF translocation.

A

t(15;17)
Acute Promyelocytic Leukemia

PML - TF
RARA- TF (retinoid acid receptor alpha)

Inhibits granulocyte differentiation.

Treat with ATRA - All-Trans Retinoic Acid.
Induced differentiation of blasts to granulocytes.

Induces clinical remission.

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13
Q

Immunophenotype of AML with PML-RARA translocation.

A

CD34 (-) ….weird.

CD-13+, CD33+ (immature myelocytes)

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14
Q

What is the translocation number of AML with RUNX1-RUNX1T1 fusion protein?

A

t(8;21)

Rhymes. Run Run 8;21.

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15
Q

What is the immunophenotype of AML with CBFC-MYH11 translocation?

A

CD14+ CD11b+ (MONOCYTES)

CD13+,CD33+ (Granulocytes)

CD34+ (blasts)

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16
Q

Immunophenotype of AML with normal cytogenetics.

A

CD33+, CD34+ (blasts)

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17
Q

What is different about the treatment of AML with normal cytogenetics (compared to the subtypes with cytogeneti anomalies)?

A

How they are treated depends on the result of targeted DNA sequencing studies

18
Q

What is the prognosis for AML with a complex karyotype?

A

Cases with 3 or more cytogenetic findings (translocations, trisomies, monosomies) have a POOR PROGNOSIS.

19
Q

What does ALL stand for?

A

Acute Lymphoblastic Leukemia

20
Q

In what age group do you see the most ALL?

A

75% of ALL cases occur in KIDS UNDER 6 AND over 80% of acute leukemias in kids are ALL.

~80% cure rate in kids, ~50% in adults

See “kids under 6” think ALL.

21
Q

What % of the marrow must be blasts for an ALL diagnosis?

A

25%

22
Q

A child has ALL with a t(9;22) translocation. What is that translocation? What other cancer is this translocation also found in? What’s the difference?

A

ALL t(9;22) is the BCR-ABL fusion protein.

The 9:22 translocation is also in CML, but the fusion protein is DIFFERENT because the breaks occur at different points, creating a variant of BCR-ABL.

23
Q

How can you distinguish a type of AML from ALL if you can only run one test to differentiate?

A

Run a flow cytometric analysis.

The AML markers are CD34+ (blast) and CD33+ (granulocyte).

The ALLs are TdT+, whether they are B or T cell lymphomas.

24
Q

For the following subtypes of B-cell ALL, say whether the prognosis is good or bad.

  1. BCR-ABL
  2. (11;19) MLL rearranged
  3. t(12;21) TEL-AML1(ETV6-RUNX1)
  4. Hyperdiploid (>50 chromosomes)
A
  1. t(9;22) BCR-ABL = bad prognosis
  2. t(11;19) MLL rearranged = bad prognosis
  3. t(12;21) TEL-AML1 (ETV6-RUNX1) = good prognosis
  4. Hyperdiploid (>50 chromocomes) = good prognosis.
25
Q

What is the ALL t(9;22) BCR-ABL immunophenotype?

A

CD10+ (germinal center)
CD19+ (B-cell)
Tdt + (lymphocyte)

26
Q

What’s the ALL t(11;19) MLL rearranged immuniphenotype?

A

CD10 - (how you tell it from bcr-abl ALL)

CD19+ (B-cell)
TdT+

27
Q

What’s the immunophenotype of ALL with t(12;21) ?

Weird one

A

Good prognosis

TdT+
CD34+ (blast)
CD10+
CD20-

28
Q

What’s the immunophentype of T-ALL? (T-cell ALL)

A

TdT+
CD3+
CD5+

29
Q

How do kids with T-ALL present in the clinic?

A

Thymic masses, lymph node masses, splenomegaly.

30
Q

What is the general translocation principle for T-ALL?

A

T-cell Acute Lymphoblastic Leukemia always has an oncogene translocated to an Ig or TCR promotor in Ch 14.

31
Q

And now we move on to MYELOPROLIFERATIVE DISEASES:

A

yes

32
Q

What is a Myeloproliferative disorder?

A

Myeloproliferative disorders are a group of conditions that cause blood cells – platelets, white blood cells, and red blood cells – to grow abnormally in the bone marrow.

33
Q

Name the relevant Myeloproliferative Disorders.

A

CML

Mastocytosis

Primary Myelofibrosis

Polycythemia Vera

Essential Thrombocytopenia

34
Q

How can you tell an infection apart from a myeloproliferative disorder?

A

In infections, bands will outnumber myelocytes and metamyelocytes. The opposite will occur in a myeloproliferative disorder.

Also, toxic granulation in neutrophils will be seen during an INFECTION. Not in a neoplasm.

35
Q

What’s the abnormal chromosome in CML called?

What’s the translocation?

Fusion protein product?

Treatment?

A

Philadelphia chromosome

t(9;22) - constitutive tyrosine kinase activity

BCR-ABL fusion protein

Treat with Imatinib

36
Q

What is the prognosis for CML?

A

GOOD!

90% cure rate

37
Q

You suspect a man has either sepsis or a myeloproliferative disorder. You run a CBC and manual diff, and suspect CML. What test do you do to confirm?

A

RT-PCR of peripheral blood for BCR-ABL fusion protein

38
Q

If untreated, what can CML turn into?

A

Acute leukemia, myeloid OR lymphoid.

39
Q

What myeloproliferative disease results in an increase in red cell count?

A

Polycythemia Vera

40
Q

What is the mutation in 95% of Polycythemia Vera cases?

A

Jak2 mutation. Active Epo receptor even in absence of erythropoietin.

41
Q

What things do you look for to diagnose polycythema vera?

A

CBC: increased RBC ct. and Hct.

Labs: Decreased Epo production (not needed)

Morphology:
Erythroid hyperplasia in the bone marrow and Increased RBCs on smear.

42
Q

Stopped at Essential Thrombocythemia

A

yes

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