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Heme/Lymph Exam 1 (Erik Hammond) COPY > Lecture 1: The Leukemias > Flashcards

Lecture 1: The Leukemias Flashcards

1
Q

How do the cells of acute vs. chronic leukemias differ?

A
  • Acute = the cells represent immature precursor cells (blast cells)
  • Chronic = the cells are more mature appearing
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2
Q

In older patients with mild pancytopenia and macrocytosis what should also be in the differential diagnosis?

A

Myelodysplasia or early AML

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3
Q

In acute leukemia (especially ALL) patients often present with what complaint?

A

Bone pain

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4
Q

What is Sweet’s Syndrome aka acute febrile dermatosis (AFND); and what is it associated with?

A
  • Cutaneous manifestation of AML w/ biopsy demonstrating myeloblasts in the dermis (histiocytic variant of AFND)
  • Can be confused with pyoderma gangrenosum
  • Tx = management of AML
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5
Q

Auer rods are seen as azurophilic rods in the cytoplasm of cells in pt’s with what disorder?

A

AML

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6
Q

Gingival hypertrophy is most commonly seen in which variant of AML?

A

The M4 and M5 variants

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7
Q

Multiple smudge cells seen on peripheral smear are associated with what condition?

A

CLL

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8
Q

What are some of the known risk factors for developing AML?

A
  • Exposure to radiation and benzene
  • Chemotherapy, especially alkylating agents
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9
Q

Radiation is a risk factor for all leukemias except for?

A

CLL

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10
Q

When the leukocyte count is very high in pt with AML, the leukemic blasts can occlude the microcirculation leading to respiratory failure and cerebral dysfunction, this is known as what?

A

Leukostasis syndrome

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11
Q

How is the diagnosis of AML confirmed?

A

Bone marrow aspiration + biopsy showing >20% blasts

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12
Q

Which antigens expressed by myeloblasts can be used as markers for AML?

A
  • CD34 (stem cell marker)
  • HLA-DR
  • CD33 and CD13
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13
Q

Which translocation is most frequently observed in children with AML?

A

t(8;21)

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14
Q

Most cases of AML with t(8;21) are classied as what type of AML; what is the prognosis in adults and children?

A
  • AML w/ maturation
  • Favorable prognosis in adults
  • Poor prognosis in kids
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15
Q

Which genetic abnormality is seen in most causes of Acute Myelomonocytic Leukemia (AMML)?

A

inv(16); some have t(16;16)

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16
Q

Which tx do pt’s with AMML due to inv(16) or t(16;16) mutation have a good response to?

A

Intensive Chemotherapy

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17
Q

Which secondary mutation seen in AMML due to inv(16) mutations confers a poor prognosis?

A

KIT mutations

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18
Q

Recurring translocations involving 11q23.3 are seen in what type of AML; what is the prognosis?

A

Acute MONOcytic leuekmia; assoc. w/ poor outcome

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19
Q

FLT3-ITD mutations may occur in any subtype of AML, but are most common in which 2; what is the prognosis w/ this mutation?

A

APL and AML with normal karyotype; poor prognosis

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20
Q

Mutations of NPM1 are most often seen in what type of AML; prognosis?

A
  • Most frequent in acute monocytic leukemia
  • In absence of FLT3 mutations is assoc. w/ favorable prognosis
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21
Q

Pt’s with AML require immediate hospitilization for what?

A
  • Placement of durable venous access (Hickman catheter, subcutaneous port)
  • Initiation of chemotherapy
  • Irradiated blood and platelet transfusion support
  • If febrile and leukopenic, Abx for presumed infection
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22
Q

What is the standard chemotherapy induction regimen for AML?

A
  • 3 days of an anthracycline, such as danorubicin or idarubicin
  • 7 days of continous infusion of cytarabine (aka Ara-C)
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23
Q

Which complication may be seen in some patients during the initiation for AML?

A

Tumor lysis syndrome = abrupt necrosis of a large mass of leukemia cells and release of intracellular contents into circulation

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24
Q

After induction therapy for AML, patients with neutropenic fever should receive what therapy; what is done if there is persistent neutropenic fever following therapy?

A
  • Should receive broad-spectrum Abx
  • If there is persistent neutropenic fever despite Abx this warrants empiric ANTI-FUNGAL therapy
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25
Q

Older pt’s with AML that have significant comorbidities and high-risk cytogenic abnormalities have lower remission rates and are best treated how?

A

Palliatively, rather than w/ induction chemo

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26
Q

In addition to advanced age, poor performance status, and certain cytogenetic abnormalities, what are 2 other asssociations with AML that indicate a poor prognosis?

A

AML related to prior cancer chemo or a pre-existing myeloproliferative neoplasm or dysmyelopoietic syndrome

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27
Q

Describe the induction, consolidation, and maintenance phase of tx for AML?

A
  • Induction: 3 + 7 (anthracycline and cytarabine aka Ara-C)
  • Consolidation: high-dose Ara-C (HDAC)
  • Maintenance: acute promyelocytic leukemia only w/ ATRA
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28
Q

Describe the induction, consolidation, and maintenance phase of tx for ALL?

A
  • Induction: vincristine/prednisone plus others
  • Consolidation: multiple agents plus CNS prophylaxis (MTX or Ara-C)
  • Maintenance: 6-MP and MTX
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29
Q

ALL is a malignancy of B or T lymphoblasts and occurs most commonly in whom?

A

Children although may occur in adults, predominantly in 7th decade

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30
Q

Diagnosis of ALL requires what; examination of what is a essential part of the initial diagnostic evaluation?

A
  • Presence of 20% or more lymphoblasts on BM exam
  • CSF examination for evidence of CNS involvement is essential
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31
Q

What are some of the common presenting signs/sx’s and complications which may occur with ALL?

A
  • Fatigue + dyspnea + bleeding + infection-related fever
  • LAD and hepatosplenomegaly are commom, and CNS-involvement may occur
  • Severe cytopenias and metabolic derangements due to tumor lysis syndrome are common
32
Q

What is the most frequent translocation seen with adult ALL?

A

t(9;22)

33
Q

Most cases of adult ALL are of B-lineage and express which markers?

A
  • CD10+, CD19+, TdT+
  • Frequent myeloid-assoc. Ags CD13 and CD33
34
Q

Hyperdiploidy (>50 and usually <66 chromosomes) is common in which pt’s with ALL?

A

Children

35
Q

Hyperdiploidy is common in the leukemia cells of ALL, gain of which chromosome is most common?

A

Chromosome 21

36
Q

Hyperdiploidy gain of which chromsome is associated with a good prognosis in ALL vs. gain of which is associated with poor prognosis?

A
  • Gain of chromosomes 4, 10, and 17 = favorable prognosis
  • Gain of chromosomes 5 and i(17q) = poor prognosis
37
Q

The t(1;19) translocation is seen in what?

A

6% children w/ B-lineage leukemia (ALL)

38
Q

In ALL due to t(1;19) translocation, what markers are seen in the leukemia cells?

A

CD10+, CD19+, CD34-, and CD9+***

39
Q

A t(8;14) translocation is seen in what type of ALL; common presentation?

A
  • Seen in mature B-cell ALL
  • These pt’s have high incidence of CNS and/or abdominal nodal involvement at diagnosis
40
Q

Ph-like ALL is a novel subgroup of high-risk ALL characterized by increased expression of what?

A

HSC genes, and similar gene expression profile to Ph-positive ALL

41
Q

Induction therapy for ALL commonly consists of which 4 drugs?

A

Anthracycline + Vincristine + L-asparaginase + a glucocorticoid

42
Q

Given the risk for CNS involvement in ALL, how should this be treated?

A

Intrathecal chemoprophylaxis (MTX or Ara-C) WITH or WITHOUT crnaial irradiation

43
Q

Since tumor lysis syndrome is common at diagnosis or shortly after institution of chemotherapy for ALL; all patients should receive what?

A

IV hydration therapy and Allopurinol

44
Q

What is the follow-up for pt’s with ALL who achieve complete remission following therapy?

A

Further intensive chemo with multiple agents for several months followed by 2-3 years of maintenance chemo

45
Q

Majority of pt’s with CML present in chronic phase of disease and may do well for yeasrs, but if untreated, will invariably transform into what?

A

Acute leukemia (myeloid in 2/3’s and lymphoid in 1/3)

46
Q

How do pt’s with CML most often present?

A
  • Fatigue + lethargy + low-grade fever and weight loss
  • Splenomegaly may be striking!
  • LAD is NOT common
47
Q

What is seen in the blood and peripheral smear of pt with CML?

A
  • Elevated blood leukocyte count
  • ↑ granulocytic cells in ALL phases of development on smear; myelocytes and metamyelocytes are most often found
48
Q

Which finding on BM exam should make you consider the accelerated or blast phase of CML may be occurring?

A

When blasts represent >10% of leukocytes on BM exam

49
Q

How is the diagnosis of CML confirmed?

A

Cytogenetic studies of the BM aspirate showing t(9;22) or presence of BCR-ABL gene via PCR

50
Q

How should patients with CML presenting with abdominal pain or discomfort be worked up?

A

Undergo ultrasonography or CT to identify splenomegaly or splenic infarction

51
Q

What is the treatment for CML and for how long?

A
  • Imatinib
  • Dasatinib and nilotinib = newer and may be used to initiate therapy or in pt’s who become resistant to imatinib
  • These drugs are given indefinitely
52
Q

When is allogenic BMT an option for CML?

A
  • May be used as initial therapy in very young pt’s w/ CML
  • More typically used in pt’s showing signs of resistance to tyrosine kinase inhibitors (i.e., Imatinib)
53
Q

What is the follow-up schedule and tests done during the initiation phase and once stable in pt with CML?

A
  • Typically every 1-2 weeks during initiation of tx w/ imatinib
  • Once stable blood counts achieved, pt’s are followed every month to monitor blood counts
54
Q

What type of reduction in BCR-ABL transcripts is needed for the best results in tx of CML?

A

4-fold reduction of BCR-ABL transcripts determined by quantitative PCR

55
Q

Which findings during follow-up of pt with CML are signs of accelerate phase or blast crisis?

A

↑ leukocyte counts + basophilia + fever + enlarging spleen

56
Q

Which immune defect is most common in pt’s with CLL?

A
  • Infection w/ encapsulated organisms (i.e., S. pneumoniae) due to inadequate B-cell function or hypogammaglobulinemia
  • Cell-mediated immune defects that predispose to recurrent herpes simplex virus infections also may be seen
57
Q

Pt’s with CLL are at increased risk for which autoimmune disorders?

A

Most commonly, autoimmune thrombocytopenia

58
Q

In CLL the monoclonal proliferation of B-lymphocytes express which markers?

A

CD19 and CD20, along w/ expression of T-lymphocyte Ag CD5

59
Q

An increase in what helps with the diagnosis of CLL?

A

Increase in mature lymphocytes >5000/uL in the absence of an acute viral illness or other trigger of reactive lymphocytosis

60
Q

Which lab tests are required for diagnosis of CLL?

A

Flow cytometry on a peripheral blood sample is adequate

61
Q

Using the Rai Staging System for CLL, what is Stage 0 - Stage IV?

A
  • Stage 0: lymphocytosis only (ALC >10,000/mm3)
  • Stage I: plus Lymphadenopathy
  • Stage II: plus Hepatosplenomegaly
  • Stage III: plus Anemia (Hb <11 gm)
  • Stage IV: plus Thrombocytopenia (<100,000)

*Remember as L-L-H-A-T

62
Q

How is asymptomatic, early-stage CLL treated vs. later-stage disease?

A
  • Early-stage: requires only observation
  • Late-stage: required active tx
63
Q

What is treatment for pt with CLL who develops autoimmune thrombocytopenia or hemolytic anemia?

A

Chemotherapy for CLL, using prednisone or anti-CD20 tx

64
Q

CLL pt’s with recurrent bacterial infections should be treated how and all pt’s should receive what annually?

A
  • Tx with IVIG infusion therapy
  • All pt’s should receive annual influenza vaccine and be vaccinated for pneumococcal
65
Q

What are the chemotherapeutic agents which may be used for induction tx of CLL?

A
  • Purine analogs (i.e., fudarabine and pentostatin)
  • Alkylating agents (i.e., chlorambucil, bendamustine, and cyclophosphamide)
  • Monoclonal Abs against CD20 (i.e., rituximab and ofatumumab)
66
Q

Young patients with CLL and what high-risk genetic factors may be considered for hematopoietic stem cell transplantation?

A

17p deletion or 11q deletion

67
Q

What is the recommended follow-up for CLL pt’s who have not yet required tx or those achieving remission through chemotherapy?

A
  • Routine monitoring of the CBC every 3-6 months is recommended to assess lymphocyte count
  • PE should include full LN exam and careful assessment of liverandspleen size
68
Q

Clinical picture of Hairy Cell Leukemia is dominated by sx’s related to what?

A

Enlarged spleen and pancytopenia

69
Q

Hairy cell leukemia is associated with increased numbers of what in the bone marrow?

A

Reticulin fibers; cause a “dry tap”

70
Q

Pt’s with hairy cell leukemia have a predisposition to infections by what?

A

Atypical mycobacterial infections

71
Q

Hairy cell leukemia tumor cells express which markers?

A

CD22, CD25, and CD103

72
Q

Soluble levels of what antigen from the cells of hair cell leukemia is an excellent tumor marker for disease activity?

A

Soluble CD25

73
Q

Most cases of Hairy Cell Leukemia have what 2 mutations?

A

Activating BRAF mutation V600E

74
Q

Median age for hairy cell leukemia is when and what is the male to female ratio?

A

Median age = 55 y/o w/ M:F of 5:1

75
Q

Which tx for hairy cell leukemia has been associated with prolonged remissions?

A

Splenectomy

76
Q

What are some of the chemotherapeutic options for hairy cell leukmia; what are complications of some of these agents?

A
  • Nucleosides –> Clardibine and Deoxycoformycin = highly active, but assoc. w/ further immunosuppression and can ↑ risk for opportunistic infections
  • IFN-α is also effective, but not as effective as nucleosides
77
Q

Hairy cell leukemia which is chemotherapy-refractory may be treated with what?

A

Vemurafenib, a BRAF inhibitor

Heme/Lymph Exam 1 (Erik Hammond) COPY (22 decks)

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