1 BL Intro to Hema. malignancies

Question 1

Describe Hematologic malignancies

Answer 1

Presence of a CLONAL malignant population of cells derived from a TRANSFORMED CELL that came from the MARROW

Question 2

3 main manners of malignant hematologic manifestation:

Answer 2

1. Leukemia
2. Lymphoma
3. Extramedullary myeloid tumor (granulocytic sarcoma)

Question 3

Leukemia

Answer 3

hematopoietic malignancy derived from stem cell or progenity in blood and marrow.

-ie: granulocytic, monocytic, erythroid, megakaryocytic, and mast cell lineages.

Question 4

Lymphoma

Answer 4

hematopoietic malignancy derived from lymphocytes or their precursors

ie: B cell,T cell, and NK cells lineages

Question 5

Extramedullary myeloid tumor

Answer 5

(aka granulocytic sarcoma) -

hematopoietic malignancy derived from myeloid cells or their precursors which presents primarily as a solid mass.

ie (granulocytes, monocytes, myeloblasts etc.),

Question 6

What is the difference between Chronic Lymphocytic Leukemia (CLL) + Small lymphocytic Lymphoma (SLL) ?

Answer 6

They are the same disease, but where the disease is more involved differs

Blood and marrow - CLL
Solid growths → enlarged lymph nodes - SLL

Question 7

Grade

Answer 7

how much has the tumor cells differentiated? - Clinical aggressiveness of a malignancy, growth rate

Question 8

Difference between High vs Low grade lymphoma

Answer 8

High grade Lymphomas:
- Rapidly enlarging mass

Low grade lymphomas:

- Mild degree of lymphadenopathy 
- Present for years
- Incidentally noticed on imaging study

*If you see lymphoma: think hard mass

Question 9

Difference between High vs Low grade Leukemia

Answer 9

High grade “acute” Leukemia:
- Very high WBC w. replacement of most normal cells in marrow

Low grade “chronic” Leukemia:

- Subtle symptoms
- Incidentally noticed on CBC for different ailment

Question 10

Why do Hematologic malignancies love chromosomal translocations?

Answer 10

Due to natural susceptibility of genome to translocate during normal periods of genomic instability

Question 11

Examples of genomic instability leading to lymphomas containing balanced translocations

Answer 11

1. Initial immunoglobulin/T-cell receptor rearrangement during maturation of B cells/T cells
2. Class recombination and somatic hypermutation process during activation of B cells

Question 12

Class recombination

Answer 12

aka isotype switching (ie: IgG to IgM)

• Examples of genomic instability leading to lymphomas containing balanced translocations

Question 13

Somatic hypermutation:

Answer 13

immune sys. adapts to new microbe → diversify B cell receptors → respond to new threats

-Examples of genomic instability leading to lymphomas containing balanced translocations

Question 14

List 3 viruses known to have oncogenic roles in some cases of lymphoma.

Answer 14

1. Epstein-Barr virus (EBV):
2. Human T cell leukemia virus-1 (HTLV-1):
3. Kaposi sarcoma herpesvirus / Human herpesvirus-8 (KSV/HHV-8):

Question 15

Epstein-Barr virus (EBV):

Answer 15

Causes: Some cases of

• classical Hodgkin lymphoma
• Burkitt lymphoma
• B cell non-Hodgkin lymphomas

Question 16

Human T cell leukemia virus-1 (HTLV-1)

Answer 16

Causes adult T cell leukemia/lymphoma (ATLL)

Question 17

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

Answer 17

Causes:
Primary effusion lymphoma
Kaposi sarcoma (cancer common in pts w AIDS)

Question 18

incidences of leukemia and lymphoma in adult populations

Answer 18

Age adjusted incidence rates  for all ages: (# new cases per population in given time)
		○ #7 - Non-hodgkin lymphoma
		○ #10 - Leukemia
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
	Age adjusted cancer death for all races:
		○ #6 - Leukemia
		○ #7 - non-Hodgkin lymphoma
		○ #15 - Myeloma

Question 19

incidences of leukemia and lymphoma in childhood populations

Answer 19

○ #1 - Leukemia
	37% of all childhood cancers
(○ #2 - Brain/CNS tumors)
○ #3 - Lymphoma
	24% of all childhood cancers

Question 20

WHO Multiparameter Classification System of malignancies

Answer 20

1. Microscopic appearance of the malignant cells
2. Histologic growth patterns of the malignant cells in the marrow, lymph node, or other tissue
3. Presence or absence of specific cytogenetic findings or molecular findings
4. Relative amount of malignant cells present in the marrow or blood
5. Presence or absence of certain cell surface markers, cytoplasmic markers, and/or nuclear markers

Question 21

Current Basic Functional Categories for Hematologic Malignancies

Answer 21

• ACUTE LEUKEMIAS
  
  • MYELODYSPLASTIC SYNDROME (MDS)
  • MYELOPROLIFERATIVE NEOPLASMS (MPNs)
  • CLASSICAL HODGKIN LYMPHOMA (CHL)
  • NON-HODGKIN LYMPHOMA (NHL)
  • PLASMA CELL NEOPLASMS

Question 22

General overview of: ACUTE LEUKEMIAS

Answer 22

Due to the rapid accumulation of immature cells in the marrow → Immature cells replace many of the normal marrow cells → cytopenias (of some marrow lineages)

Question 23

General overview of: MYELODYSPLASTIC SYNDROME (MDS)

Answer 23

Neoplastic clonal population→ takes over the marrow →
cant make normal blood cells in one or more myeloid lineages (dysplasia). →
low blood cell counts

Question 24

General overview of: MYELOPROLIFERATIVE NEOPLASMS (MPNs)

Answer 24

Neoplastic clonal population → take over marrow →
makes too many normal functioning myeloid cells (in multiple lineages) →
increased blood counts

Question 25

General overview of: CLASSICAL HODGKIN LYMPHOMA (CHL)

Answer 25

neoplastic cells derived from B cells

Question 26

General overview of: NON-HODGKIN LYMPHOMA (NHL)

Answer 26

malignancy derived from mature lymphocytes (B cells , T cells, or NK cells), excluding CHL or plasma cell neoplasms.

Question 27

General overview of: PLASMA CELL NEOPLASMS

Answer 27

Neoplasms derived from plasma cells, including MGUS, plasmacytoma, and multiple myeloma.