Hematologic Malignancies Flashcards

1
Q

A malignancy of hematopoietic cells, where the chief manifestation is involvement of the blood and marrow

A

Leukemia

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2
Q

A malignancy of hematopoietic cells, derived from lymphocytes or their precursors, which present primarily as a solid mass

A

Lymphoma

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3
Q

A malignancy of hematopoietic cells, derived from myeloid cells or their precursors, which present primarily as a solid mass

A

Extramedullary myeloid tumor (aka granulocytic sarcoma)

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4
Q

Do hematologic malignancies overlap?

A

Yes!…

While most prefer one type of manifestation…some often overlap

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5
Q

What is a common example of hematologic malignancy overlap?

A

CLL/SLL

chronic lymphocytic leukemia
small lymphocytic lymphoma

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6
Q

What is a high grade malignancy?

A

more aggressive / more rapidly growing

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7
Q

when referring to leukemia, the term ________ is usually used for high grade leukemia?

A

acute

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8
Q

when referring to leukemia, the term _______ is usually used for low grade leukemia?

A

chronic

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9
Q

How does a high grade lymphoma present?

A

rapidly enlarging mass

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10
Q

How does an acute leukemia present?

A

very high white blood cell count with near replacement of normal cells in the marrow

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11
Q

How does low grade lymphoma present?

A

mildly enlarged neck lymph node that has been present for years, or as a mild degree of lymphadenopathy noticed incidentally on imaging study

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12
Q

How does a chronic leukemia present?

A

subtle symptoms - often only noticed incidentally on CBC performed for some other reason

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13
Q

How do you know if a hematopoietic neoplasm is benign or malignant?

A

they are all malignant (with some rare exceptions… such as MGUS)

*mgus = monoclonal gammopathy of uncertain significance

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14
Q

Why are chromosomal translocations clinically important? (2 reasons)

A

their persistent presence allows them to be used as diagnostic markers for certain hematologic malignancies

their persistent presence suggests they play a critical role in the development of the hematologic malignancy they are associated wtih

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15
Q

Why are presence of translocations in lymphoma thought to be so common?

A

the natural susceptibility of the genome to translocations during normal periods of genomic instability, namely during the initial immunoglobulin / T-cell receptor rearrangement during the maturation of B cells / T cells and during the class recombination and somatic hypermutation process during the activation of B cells.

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16
Q

3 common viruses associated with the genesis of lymphomas?

A

Epstein-Barr Virus
Human T-cell leukemia virus-1
Kaposi sarcoma herpesvirus / Human herpesvirus-8 (KSV/HHV-8)

17
Q

Epstein Barr virus - associated with?

A

classical Hodgkin lymphoma
Burkitt lymphoma
B cell non-Hodgkin lymphoma

18
Q

Causative factor in adult T cell leukemia / lymphoma?

A

Human T cell leukemia virus (HTLV-1)

19
Q

Causative factor in primary effusion lymphoma?

A

Kaposi sarcoma herpesvirus / Human herpesvirus-8 (KSV/HHV-8)

20
Q

Frequency rank of Non-Hodgkin Lymphoma?

Frequency rank of Leukemia?

A

7th most frequent in adults

10th most frequent in adults

21
Q

Death rate rank of Non-Hodgkin Lymphoma?

Death rate rank of Leukemia

A

7th most deadly in adults

6th most deadly

22
Q

Most common types of childhood cancer? (top 3)

A

leukemia (37%)
cns/brain - 2nd place
lymphoma (24%)

23
Q

How many hematologic malignancies are there?

A

158

24
Q

Basic Scheme of Hematologic Malignancy classification?

A

Myeloid vs. Lymphoid vs Other

25
Q

WHO multiparameter classification scheme? (5)

A
  • microscopic appearance
  • histologic growth pattern
  • specific cytogenetic or molecular findings
  • relative amt of malignant cells present in blood or marrow
  • presence or absence of certain cell surface / cytoplasmic / nuclear markers
26
Q

This type of hematologic malignancy is usually due to the rapid accumulation of (usually) immature cells in the marrow. These immature cells often replace many of the normal marrow cells, resulting in cytopenias… Often, but not always, the immature cell is the generic-appearing blast

A

Acute Leukemias

27
Q

Tools for evaluating acute leukemias? (3)

A
morphology 
immunophenotyping (flow cytometery / immunohistochemistry)
28
Q

immunophenotyping -

A

refers to the use of antibodies to detect whether certain substances are being expressed by cells - allows us to place morphologically non-distinct cells into a definite lineage

29
Q

This group of conditions is where a clonal population derived from a neoplastic hematopoietic stem cell takes over the marrow, and is not capable of making normal blood cells in one or more lineages

A

Myelodysplatic Syndrome (MDS)

30
Q

What characterizes MDS

A

In most cases, falling peripheral blood cell counts.

31
Q

What do many people regard MDS as a precursor to?

A

AML

32
Q

Is MDS a malignancy in its own right?

A

Arguably… many people die of MDS without progressing to acute leukemia, due to the failure of the marrow to make normal blood cells

33
Q

These are neoplastic clonal proliferations of the marrow where the clone makes normal functioning blood cells, usually in multiple lineages, but makes too many of them in one or more lineages

A

Myeloproliferative Neoplasms (MPNs)

34
Q

What do MDNs have a tendency to progress to?

A

Acute leukemia… but tendency is much less than for MDS

35
Q

____ is a very distinct clinical entity, driven by Hodgkin Reed Sternberg cells

A

Classical Hodgkin Lymphoma

36
Q

What cell line does classical Hodgkin Lymphoma derive from… via which cells?

A

Hodgkin Reed Sternberg cells derive from B cells

37
Q

Refers to any malignancy derived from mature B cells (Excluding CHL or plasma cell neoplasms), T cells, or NK cells… the large majority are derived from B cells

A

Non-Hodgkin Lymphoma

38
Q

Plasma cell neoplasms - include?

A

Plasma cell derived neoplasms is self-explanatory and includes MGUS, plasmacytomas, and multiple myeloma

39
Q

MGUS, plasmacytoma, and multiple myeloma are all categorized as?

A

plasma cell neoplasms