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PBR: Hematology > Hematologic Malignancies > Flashcards

Hematologic Malignancies Flashcards

1
Q

What is the single most common childhood malignancy?

A

ALL

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2
Q

What are the three main types of leukemia in children?

A

Acute lymphoblastic leukemia, Acute myeloid leukemia, and Chronic myeloid leukemia

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3
Q

***Image 24-21, differentiate between myeloblast and lymphoblast

A

***Image 24-21

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4
Q

From what cell type does acute lymphoblastic leukemia arise?

A

Lymphoid precursor cells

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5
Q

From what cell type does acute myeloid leukemia arise?

A

Myeloid precursor cells

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6
Q

From what cell type does chronic myeloid leukemia arise?

A

WBC stem cells

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7
Q

Which (7) disorders correlate with increased risk of ALL?

A

Prenatal radiation exposure, postnatal exposure to high doses of radiation, Trisomy 21, Ataxia-telangiectasia, Bloom syndrome, Fanconi Anemia, and neurofibromatosis.

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8
Q

What diagnosis should you consider in a child with pallor and a limp?

A

ALL

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9
Q

What are the “four Ps” of ALL?

A

They describe the most common presentation of ALL: Pallor, Pyrexia, Purpura, and Pain.

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10
Q

T/F: Splenomegaly is common in ALL.

A

True. In addition to fever, bleeding, and bone pain, lymphadenopathy is typical. Generalized lymphadenopathy and hepatosplenomegaly are seen in > 50% of patients.

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11
Q

Which (4) cytopenias are often seen in children with ALL?

A

Normocytic normochromic anemia, reticulocytopenia, severe neutropenia (despite high WBC count), and thrombocytopenia.

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12
Q

What test is required to diagnose ALL? What must it show in order to be diagnostic?

A

Bone marrow evaluation is required for diagnosis and must show at least 25% marrow involvement to be diagnostic.

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13
Q

What is the classic bone marrow finding in patients with ALL?

A

The marrow is classically hypercellular and infiltrated with leukemic lymphoblasts. ***Image 24-22

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14
Q

List 5 good prognostic indicators for ALL.

A

Rapid response to treatment, hyperdiploidy (> 50 chromosomes or DNA index > 1.16), Trisomies of chromosomes 4 and 10, t(12;21) translocation (TEL-AML), and female gender.

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15
Q

List 7 poor prognostic indicators for ALL.

A

Age < 1 year or > 10 years at diagnosis, presence of the Philadelphia chromosome t(9;22), abnormalities of the MLL gene (i.e. t(4;11)), WBC count > 50,000 cells/µL on presentation, mature B-cell leukemia, T-cell leukemia, and African American or Hispanic ethnicity.

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16
Q

What are the four general treatment regimen categories for ALL?

A

Induction, consolidation, maintenance, and CNS preventive therapy.

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17
Q

Which four drugs/drug categories are used in the induction phase of ALL treatment?

A

Vincristine, glucocorticoid (dexamethasone or prednisone), asparaginase, and anthracyclines (doxorubicin or daunorubicin - for patients who are higher risk at time of diagnosis).

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18
Q

How common is remission in patients with standard-risk ALL following the induction phase of their treatment regimen?

A

More than 95% of patients with standard-risk ALL enter remission following induction therapy.

19
Q

Which patients with ALL receive CNS preventive therapy?

A

Every patient with ALL receives CNS preventive therapy, regardless of the initial CNS findings.

20
Q

What is the typical length of the treatment course in patients with ALL?

A

Most patients receive 30-36 months of therapy.

21
Q

How long does the induction phase of treatment typically last in patients with ALL?

A

About a month.

22
Q

How long does the consolidation phase of treatment typically last in patients with ALL?

A

About 6-12 months.

23
Q

Which (5) drugs are typically used in the consolidation phase of ALL treatment? What is the goal of treatment in this phase?

A

The goal of treatment is to intensify therapy by introducing different drugs and combinations that have synergistic effects to reduce the chance for drug resistance. Cytarabine, anthracyclines, methotrexate, cyclophosphamide, and etoposide are most commonly used.

24
Q

How long does the maintenance phase of ALL treatment typically last?

A

18-24 months

25
Q

What (4) drugs are typically used in the maintenance phase of treatment for ALL?

A

Most treatment protocols use daily oral 6-mercaptopurine (6-MP) and weekly methotrexate with intermittent pulses of vincristine and glucocorticoids.

26
Q

What is the most important predictive factor for achieving a 2nd remission in ALL?

A

The length of time the first remission lasted (i.e. the longer the first remission, the better the survival rate).

27
Q

Which two agents commonly used in the treatment of ALL have hepatotoxicity as a long-term side effect?

A

Hepatotoxicity occurs with antimetabolite therapy (methotrexate and 6-MP).

28
Q

Which two agents commonly used in the treatment of ALL have cardiomyopathy as a long-term side effect?

A

Cardiomyopathy is seen with the anthracyclines (doxorubacin and daunorubacin.

29
Q

What secondary malignancy is a known long-term side effect of etoposide use?

A

Acute myeloid leukemia

30
Q

What (9) disorders/exposures correlate with an increased risk of AML?

A

Trisomy 21, Diamond-Blackfan anemia, Fanconi anemia, Bloom syndrome, Kostmann syndrome, Paroxysmal nocturnal hemoglobinuria, neurofibromatosis, and previous exposure to Etoposide or ionizing radiation.

31
Q

What is leukemia cutis?

A

Leukemic infiltration of the skin, which can be seen in the M4/M5 subtypes of AML.

32
Q

What is an orbital chloroma, and what might its presence signify?

A

Chloromas are localized masses of leukemic cells and are often present at the time of AML diagnosis (or could be the 1st clue to diagnosis).

33
Q

What procedure is required for the diagnosis of AML?

A

Bone marrow evaluation.

34
Q

What peripheral blood cell finding is pathognomonic for AML?

A

Finding Auer rods inside peripheral blast cells in the blood is pathognomonic for AML. ***Image 24-24

35
Q

Know how to identify an Auer rod associated with AML (image 24-24). ***

A
36
Q

Which karyotype is associated with Acute Promyelocytic Leukemia?

A

t(15;17)

37
Q

What disorder is commonly present in patients presenting with new-onset Acute Promyelocytic Leukemia?

A

DIC

38
Q

Which three drug classes are typically used in the induction phase of AML treatment?

A

Anthracyclines, etoposide, and cytosine arabinoside (Ara-C).

39
Q

Which subset of AML patients tend to have excellent cure rates?

A

Patients with Acute Promyelocytic Leukemia. They receive retinoic acid in addition to chemotherapy and do not require bone marrow transplant.

40
Q

Which leukemia is commonly associated with the Philadelphia chromosome?

A

Philadelphia chromosome-positive CML.

41
Q

What is the Philadelphia chromosome?

A

t(9;22) chromosome translocation, which is associated with CML.

42
Q

What is the typical management for Philadelphia chromosome-positive CML if diagnosed in the chronic phase of disease?

A

Most patients are treated initially with tyrosine kinase inhibitors such as imatinib or dasatinib. These drugs can induce complete remission with a favorable side-effect profile, but must be taken for life as they are not curative.

43
Q

Patients with which disorder have an increased risk for the development of juvenile myelomonocytic leukemia (JMML)?

A

Neurofibromatosis Type 1

44
Q

Does JMML usually present with a small or enlarged spleen?

A

Children present with a markedly enlarged spleen, modest leukocytosis, thrombocytopenia, and elevated fetal hemoglobin.

PBR: Hematology (9 decks)

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