Hematologic Malignancies

Question 1

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

Answer 1

Leukemia

Question 2

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

Answer 2

Lymphoma

Question 3

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

Answer 3

Extramedullary myeloid tumor (aka granulocytic sarcoma)

Question 4

Do hematologic malignancies overlap?

Answer 4

Yes!…

While most prefer one type of manifestation…some often overlap

Question 5

What is a common example of hematologic malignancy overlap?

Answer 5

CLL/SLL

chronic lymphocytic leukemia
small lymphocytic lymphoma

Question 6

What is a high grade malignancy?

Answer 6

more aggressive / more rapidly growing

Question 7

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

Answer 7

acute

Question 8

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

Answer 8

chronic

Question 9

How does a high grade lymphoma present?

Answer 9

rapidly enlarging mass

Question 10

How does an acute leukemia present?

Answer 10

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

Question 11

How does low grade lymphoma present?

Answer 11

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

Question 12

How does a chronic leukemia present?

Answer 12

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

Question 13

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

Answer 13

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

*mgus = monoclonal gammopathy of uncertain significance

Question 14

Why are chromosomal translocations clinically important? (2 reasons)

Answer 14

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

Question 15

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

Answer 15

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.

Question 16

3 common viruses associated with the genesis of lymphomas?

Answer 16

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

Question 17

Epstein Barr virus - associated with?

Answer 17

classical Hodgkin lymphoma
Burkitt lymphoma
B cell non-Hodgkin lymphoma

Question 18

Causative factor in adult T cell leukemia / lymphoma?

Answer 18

Human T cell leukemia virus (HTLV-1)

Question 19

Causative factor in primary effusion lymphoma?

Answer 19

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

Question 20

Frequency rank of Non-Hodgkin Lymphoma?

Frequency rank of Leukemia?

Answer 20

7th most frequent in adults

10th most frequent in adults

Question 21

Death rate rank of Non-Hodgkin Lymphoma?

Death rate rank of Leukemia

Answer 21

7th most deadly in adults

6th most deadly

Question 22

Most common types of childhood cancer? (top 3)

Answer 22

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

Question 23

How many hematologic malignancies are there?

Answer 23

158

Question 24

Basic Scheme of Hematologic Malignancy classification?

Answer 24

Myeloid vs. Lymphoid vs Other

Question 25

WHO multiparameter classification scheme? (5)

Answer 25

- 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

Question 26

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

Answer 26

Acute Leukemias

Question 27

Tools for evaluating acute leukemias? (3)

Answer 27

``` morphology immunophenotyping (flow cytometery / immunohistochemistry) ```

Question 28

immunophenotyping -

Answer 28

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

Question 29

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

Answer 29

Myelodysplatic Syndrome (MDS)

Question 30

What characterizes MDS

Answer 30

In most cases, falling peripheral blood cell counts.

Question 31

What do many people regard MDS as a precursor to?

Answer 31

AML

Question 32

Is MDS a malignancy in its own right?

Answer 32

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

Question 33

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

Answer 33

Myeloproliferative Neoplasms (MPNs)

Question 34

What do MDNs have a tendency to progress to?

Answer 34

Acute leukemia... but tendency is much less than for MDS

Question 35

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

Answer 35

Classical Hodgkin Lymphoma

Question 36

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

Answer 36

Hodgkin Reed Sternberg cells derive from B cells

Question 37

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

Answer 37

Non-Hodgkin Lymphoma

Question 38

Plasma cell neoplasms - include?

Answer 38

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

Question 39

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

Answer 39

plasma cell neoplasms