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Heme - Strom > Hematologic Malignancies II/III > Flashcards

Hematologic Malignancies II/III Flashcards

1
Q

What 3 malignancies arise from the bone marrow?

A
  1. Acute Leukemias
  2. Myeloproliferative Diseases
  3. Myelodysplasia
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2
Q

What defines an acute leukemia?
• What arbitrary number is set to indicated and acute leukemia?
• Problem with this number?

A

BLASTS (rapidly proliferating clones) in the marrow and often in the bloodstream

Number:
• Marrow Blast Number GREATER than 20%

**Problem is that some people have acute leukemia and their marrow number is not yet greater than 20% blasts

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3
Q

What is the problem with using blast morphology as a diagnostic Characteristic for Acute Leukemias?

A

METHOD OF MORPHOLOGY HAS LITTLE CLINICAL UTILITY

*Not indicative of the clinical course the disease will take

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4
Q

What method would you use to distinguish between an Acute Myeloblastic Leukemia (AML) and Acute Lymphoblastic Leukemia (ALL)?
• How can this method be misleading?
• How do we try to prevent ourselves from being mislead?

A

Immunohistochemical Staining or Flow Cytometry

  • These methods can be misleading because cells can show MIXED phenotypes
  • We try to prevent being mislead by using Mutliple markers.

***Still not a perfect system

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5
Q

Whats the the most useful clinical criterion for determining what type of Acute Leukemia (or cancer in general) your patient has?
• What are the specific advantages over immunohistochemistry or any other current criterion?

A

Genetics

Advantages:
• Increased Prognostic Value
• Predicts Response to Therapy
• Identifies Molecular Targets for Therapy Development

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6
Q

Describe what happens in the t(15:17)(q22;q12) translocation.
• Resultant disease?

A

PML-RARA fusion protein is formed by this translocation in which RAR-alpha (RARA), transcription factor, is linked to PML.

PML is responsible for organizing the nuclear structures

***When PML is fused to RARA, RARA can no longer be activated by Retinoic acid

  • THIS INHIBITS GRANULOCYTE DIFFERENTIATION*
  • **ACUTE MYELOID LYMPHOMA****
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7
Q

How was t(15:17)(q22:q12) translocation AML cured?

• What treatment do we use today to cure it?

A

Original Hypothesis:
• RA-binding domain, and DNA binding domain are still in tact on RARA even when its bound to PML
• RARA is activated by Retinoic acid aka VITAMIN A

Treatment:
• IV vitamin A put the cancer into remission

TODAY:
• VIT A. as well as Arsenic Trioxide is used because ArO3 inhibits K160 on the PML protein

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8
Q

Why did the VIT A put AML caused by PML-RARA into remission?

A

It destabilized the RARA protein (did not activated as was hypothesized), but it still worked

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9
Q

T or F: while indicative of cancer, Auer rods are often seen in reactive conditions.

A

False, they are totally indicative of MYELOID LEUKEMIAS

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10
Q

BE SURE TO LOOK AT THIS MINDMAP

A

BE SURE TO LOOK AT THIS MINDMAP

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11
Q

What is wrong with the following scenario?
A patient is diagnosed with AML and their cytogenetic (chromosomes) findings are normal and the physician begins treatment.

A

**YOU MUST do MOLECULAR studies if the cytogenetics are normal

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12
Q

What is the prognosis of someone who has AML with 3 or more cytogenetic findings?
*What gene can you assume is mutated?

A

BAD prognosis

p53 has probably been mutated leading to the genomic instability seen on the karyotype

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13
Q

Who is most often affected by acute LYMPHOBLASTIC leukemia (ALL)?
• cure rate?

A

75% of cases = KIDS
• in 80% of cases KIDS can be cured

ADULTS
• Cured in less than 50% of cases

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14
Q

What cells do you expect to see MORPHOLOGICALLY in acute Lymphoblastic Leukemia?

A
  • Large Cells
  • Large N/C ratio
  • NO CYTOPLASMIC GRANULES
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15
Q

What transcription factor is mutated in 84% of cases of t(9,22)?
• Disease?

A

Disease:
Acute Lymphoblastic Leukemia via BCR-ABL mutation

Transcription factor:
• IKZF1 - loss causes inhibition of differentiation

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16
Q

What in the manual differential would help you to differentiate between sepsis and a myeloproliferative disease?

A

Sepsis:
Bands > Metamyelocytes > Myelocytes

Myeloproliferative:
“Myloid Bulge” - myelocytes are present greater proportion than bands and metamyelocytes

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17
Q

Why should your threshold for suspecting CML be low?

A

Its Very treatable

18
Q

What are the 3 methods that have been used to measure treatment efficacy in CML patients?

A

Old - Hematologic Response Measured
- look for normalized RBCs

Newer - Cytogenetic Response Measured
- Look for translocation in cells

Newest/BEST - Molecular Response Measured
- Look at genotype

19
Q

What is the risk of missing the diagnosis in a patient with CML?

A

CML can progress to an Acute Leukemia

20
Q

What is the ultimate role of STATS?

A

Upregulated PROLIFERATION AND DIFFERENTIATION

21
Q

How can a Jak 617F mutation result in BOTH Polycythemia Vera and Essential Thrombocythemia?

A

Same Mutation may take place in 2 different lineages that have already committed to a given cell type

22
Q

What is the MOST COMMON reason to see increased platelets in a patient?

A

IRON deficiency, this is way more common than Essential Thrombocythemia

23
Q

What mitogen plays a big role in Mast Cell Growth and Differentiation?

A

SCF

24
Q

How do neoplasms of mast cells usually present in t kids?

A

Benign Cutaneous Lesions

e.g. Urticaria Pigmentosa

25
Q

Where are mastocytosis most commonly localized to?
• Can they spread?
• If so where do they usually go?

A

SKIN, typically they do NOT spread but they definitely CAN.

Most common place to spread:
• BONE MARROW, but may also involve the Lymph Nodes, Spleen, and/or Liver

26
Q

How are myeloproliferative Diseases usually diagnosed?

A
  • Unexplained Cytopenia

* Morphologic Findings in the Bone Marrow

27
Q

What is the general Rule about B cell Malignancies if they are well differentiated?

A

• They can transform into more aggressive forms by acquiring more mutations

***So you can’t just assume you’re safe because its well differentiated

28
Q

What percentage of B cells are lost in Class Switching, Somatic Hypermutation, and Clonal selection (positive selection)?

A

99%

29
Q

What genetic mutation is key to cancer formation in B and T cells?
*why are these cells so susceptible to cancer?

A

**Mutations that TRANSLOCATE an oncogene into the Ig promoter region

**Susceptibility to cancer arises from the fact that SOMATIC RECOMBINATION IS ESSENTIAL TO THEIR Developement

30
Q

What is the location of the B cell promoter regions?

A 
IgH  (14q32)
Ig Lamda (22q11)
Ig Kappa (2p12)
31
Q

What measure do we use to determine the level of clinical involvment of a lymphoma or leukemia?

A

Staging

**Note: there are different Staging Schemes for Different Lymphomas

32
Q

What are the 2 immunophenotypic markers that are bad prognostic indicators in Chronic Lymphocytic Leukemia/Lymphoma?
• Why are they bad?

A

CD38
ZAP-70

**These are bad because they indicate lots of Somatic Hypermutation

33
Q

What disease must you often differentiate CLL from?

* How do you do this?

A

CLL often must be differentiated from Mantle Cell Lymphoma (MCL)

**There are no proliferation centers in the lymph nodes in MCL (NO PSEUDOFOLLICULAR APPEARANCE)

34
Q

T or F: in Hodgkin’s Lymphoma there is no Ig present on the B cell surface.

A

False, this is ONLY true for the Classical Form, Nodular Lymphocyte Predominant Hodgkin lymphoma (NLPHL) DO express Ig receptor

35
Q

Can morphology help differentiate Hodkins Lymphomas?

• Is this sufficient to make a Dx?

A

Yes, morphology is different, in Hodgkin you see R/S cells, in NLPHL you see Popcorn (lympho-histocytic - L&H cells)

36
Q

What is the correlation for morphology and severity of B-cell malignancy?
• Is the same true for T cell malignancies?

A

B-cell malignancy:
Well Differentiated cells result in less severe malignancy

T-cell malignancy:
NO CORRELATION between cell morphology and severity of malignancy

37
Q

T or F: like in B cell lymphomas and Leukemias, T cell lymphomas and Leukemias often involve the translocation of an oncogene into the T-cell receptor promoter.

A

FALSE, This is NOT a recurrent theme in T-cell lymphomas

38
Q

What is the most useful genetic tool in defining a T cell lymphoma?
• What about NK cell lymphoma?

A

Most Useful Tool for T-cell = PCR to determine CLONALITY

DOES NOT APPLY TO NK CELLS

39
Q

How do you reassure yourself that someone has Mycosis Fungoides when you see a tissue biopsy of Lymphocytes infiltrating epidermis?

A
  1. Make sure its T cells that stain for CD3, 4, and 5
  2. MAKE SURE THEY ARE MONOCLONAL

**Reactive or Autoimmune conditions will by polyclonal

40
Q

What is the defining feature of a Myeloproliferative Disease?

A

Chronically Proliferating Clones which differentiate to circulating blood cells

41
Q

What feature of neutrophil granules can help to assure you that you’re looking at a reactive condition and not a malignant one?

A

TOXIC GRANULATION - large basophilic granules in the cytoplasm

42
Q

T or F: if someone has polycythemia Vera they will have elevated EPO.

A

NO, the JAK2V216F is mutated so (renal peritubular) cells will never get signals to synthesize more EPO

Heme - Strom (13 decks)

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