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M2 Heme/Lymph > WBC Disorder Cases - Olteanu & Atallah > Flashcards

WBC Disorder Cases - Olteanu & Atallah Flashcards

1
Q

What is the mechanism of action of imatinib?

Name some other tyrosine kinase inhibitors used clinically to treat CML

A

Tyrosine kinase inhibitor: Binds near ATP binding site of bcr-abl, prevents TK signaling

Dasatinib

Nilotinib

Bosutinib

Ponatinib

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2
Q

If a patient, otherwise in remission, decides to stop taking nilotinib for their CML, what will likely happen?

Is this always true?

A

CML will return

Not necessarily true - the existence of true ‘molecular remission’ is currently under investigation

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3
Q

Combined JAK2 mutation and peptic ulcer disease is likely indicative of what?

A

polycythemia vera

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4
Q

What is the main treatment for AML with t(15;17)?

What is the approximate cure rate with this drug?

Why don’t the others respond to therapy?

What is usually combined with ATRA to enhance its therapeutic effect?

A

ATRA (All Trans Retinoic Acid)

90% cure rate

The remaining 10% usually dies from DIC before ATRA treatment can take effect

arsenic trioxide (more favorable side effect profile, according to wiki)

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5
Q

Describe the general rationale behind hypomethylating agents. Name one.

A

Decitabine (hypomethylating agent)

Rationale: hypermethylation of DNA prevents expression of genes needed to mature developing cells, resulting in pancytopenia (among other things). De-methylating agents allow gene expression and the maturation of chronically immature cell populations

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6
Q

In a patient with WBC: 80K and Blasts: 60K, why would normal fibrinogen be important?

What if flow cytometry and immunohistochemistry reveals the following (picture)?

A

Normal fibrinogen indicates that the patient likely does not have APL -> the differential is AML or ALL…

AML

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7
Q

What is the median age of presentation for AML?

A

~60 years old

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8
Q

What is the most common cytogenetic classification of AML?

What others are there?

A

“Standard” i.e. no specific translocations (51%)

Others include: t(8;21), t(15;17), inv(16), FAB type M3 (28%)

“Poor” - adverse karyotypic abnormalities (i.e. -5, -7, del(5q), qabn(3q)), or resistant disease (20%)

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9
Q

What is 7 + 3 therapy?

What is it used to treat?

Is this an effective treatment?

A

Ara-C (7 days) + Daunorubicin (3 days)

Inductive therapy for treatment of AML

Not really. It has a high mortality rate (~10%) and depends on the hope that if you kill off most of the bone marrow, the stuff that grows back will be ‘good’ marrow.

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10
Q

What is the approximate prevalence of JAK2 mutations in the following myeloproliferative neoplasms (MPNs)?

  • Polycythemia vera
  • Essential thrombocythemia
  • Primary myelofibrosis
A
  • 100%
  • 50%
  • 50%
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11
Q

Teardrop cells, with splenomegaly, fatigue, cachexia, bone pain, and night sweats is indicative of what?

A

myelofibrosis

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12
Q

What is the difference between a lymphoma and leukemia?

A

In leukemia, cancers cells arise from the bone marrow and are in the blood

In lymphoma, cancers cells arise from the lymphatics and are more solid tumors.

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13
Q

What is the difference between a myeloid leukemia and lymphoid leukemia?

A

The derivatives of the cancer cell! Myeloid leukemias come from myeloid derviatives

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14
Q

What are the four major types of B cell cancers?

What are their markers?

A

Mantle Cell Lymphoma

  • CD5+
  • FCM7+

Chronic Lympocytic Leukemia

  • CD5+
  • CD23+

Follicular Lymphoma

  • CD5-
  • CD10+

MALT lymphoma

  • CD5-
  • CD10-
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15
Q

What are some prognostic indicators for Chronic Lymphocytic Lymphoma?

A

Good

  • Low RAI stage
  • Mutated IGHV gene
  • 13q deletion

Bad

  • High RAI stage
  • Unmutated IGHV gene
  • CD38/ZAP70 expression
  • 11q, 17p deletion
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16
Q

What is the RAI system?

What are its stages?

A

Staging system for leukemias/lymphomas

Stage 0: lymphocytosis

Stage 1: stage 0 + adenopathy

Stage 2: stage 1 + organomegaly

Stage 3: stage 2 + anemia

Stage 4: stage 3 + thrombocytopenia

17
Q

What are some indications to treat CLL?

A

Lymphocyte doubling time <6 months

Hemoglobin <10g/dL

Platelets <100k/uL

Autoimmune anemia or thrombocytopenia

Enlarged Lymph nodes

Richter Transformations

Night sweats, weight loss, and fever

18
Q

Hairy cell Leukemia

Who does it normally affect?

What is seen on a blood smear?

A

Normally affects middle-aged men

Lymphocytes with hairy projections that stain positive for TRAP, and a lack of other cell types

19
Q

Hairy Cell Leukemia

What are clinical symptoms?

What is seen on flow?

A

splenomegaly and fibrosis of the marrow

CD22 and CD11c

20
Q

Sezary Syndrome

Describe the cells

What are associated disorders?

A

T cells with a cerebriform nucleus

CD4+

Mycosis fungoides, erythroderma

21
Q

Adult T cell Lymphoma/Leukemia

Describe the cells

What are associated disorders?

A

CD4+ T cell with “flower like” nucleus

HTLV-1 infection, Hypercalcemia

22
Q

Large Granular Lymphocytic Leukemia

What cell is involved?

How does it present?

What is it associated with?

A

Cytotoxic T cells

Splenomegaly, neutropenia, anemia

Autoimmune diseases

23
Q

What is the proliferative disorder called for

Mast cells

RBCs

Platelets

Eosinophils

Neutrophils/Monocytes

A

Systemic Mastocytosis

Polycythaemia vera

Essential Thrombocythaemia

Chronic eosinophilic leukemia

CML or Primary myelofibrosis

24
Q

What characterizes a myeloproliferative disorder?

How is it different from a myelodysplastic disorder?

A

Hypercellular bone marrow with working hematopoiesis leads to cytosis

In MDS, the bone marrow is hypercellular, but hematopoiesis is broken leading to cytopenias

25
Q

CML

What age is most commonly diagnosed?

What are the clinical findings?

A

40-60 years old

Heptosplenomegaly, fatigue, weakness, weight loss, anorexia

26
Q

What causes CML? How is it treated?

What is seen on the blood smear?

A

Expansion of pluripotent stem cells due to BCR-ABL fusion gene. TK inhibitors or stem cell transplant will cure.

Leukocytosis (with immature myeloid) with basophilia

27
Q

What are the three stages of CML?

What happens in the end phase?

A

Chronic phase (3 years), Accelerated Phase (1 year), Blast phase

disease progresses to either AML or ALL

28
Q

What causes polycythaemia vera?

What is seen on the blood smear?

A

expansion of pluripotent stem cells due to JAK2 mutation.

Increased RBCs along with raised numbers of granulocytes and platelets.

29
Q

What are clinical symptoms of polycythaemia vera?

What causes them?

A
  • Splenomegaly- hematopoiesis outside of bone
  • thrombosis- over production of platelets and RBCs
  • Gout- increased uric acid from increased cell death
  • Peptic ulcer, heat urticaria, ruddy face- increased histamine from mast cells
30
Q

What distinguishes polycythaemia vera from non-pathologic RBC expansion?

A

Low EPO

High SaO2

31
Q

How is polycythaemia vera treated?

What can polycythaemia vera evolve to?

A

phelobotomy to remove extra cells

15-20% to “spent phase” similar to PMF

2-3% to AML or MDS

32
Q
A

M2 Heme/Lymph (32 decks)

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