Intro to Hematologic Malignancies (complete)

Question 1

What are the main manners in which hematologic malignancies manifest?

Answer 1

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

Question 2

How do the different manifestations of hematologic malignancies overlap?

Answer 2

Example: Chronic lymphocytic leukemic and small lymphocytic leukemia

Same disease

Difference: where the disease primarily is — blood and BM (CLL) or lymph nodes (SLL)

Question 3

Describe high grade lymphomas

Answer 3

• May present as a rapidly enlarging mass
• High WBC count w/ near replacement of normal cells in BM

Similar concept to acute leukemia

Question 4

Describe low grade lymphomas

Answer 4

• Mild — mildly enlarge lymph nodes
• Subtle symptoms
• Often noticed incidentally on CBC results performed for another reason

Similar concept to chronic leukemia

Question 5

Describe acute leukemias

Answer 5

• More aggressive, rapidly growing
• High WBC count w/ near replacement of normal cells in BM
• Low platelets, neutrophils, and RBCs

Similar concept to high grade lymphoma

Question 6

Describe chronic leukemias

Answer 6

• Subtle symptoms, gradual
• Often noticed incidentally on CBC results performed for another reason

Similar concept to low grade lymphoma

Question 7

Discuss recurrent translocations in certain hematologic malignancies in regard to the clinical care of patients

Answer 7

• Used as diagnostic markers
• Presence suggests they play a critical role in development of the hematologic malignancies with which they are associated

Eg — CML: t(9;22)

Question 8

What are 3 viruses known to have oncogenic roles in some cases of lymphoma?

Answer 8

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 9

What are the incidences of leukemia and lymphoma in adult populations?

Answer 9

LK: 10th most frequent — rate=12.5

LP: 7th most frequent (non-Hodgkin) — rate=19.6

Question 10

What are the incidences of leukemia and lymphoma in childhood populations?

Answer 10

LK: 37% of childhood cancers (most common type)

LP: 24% of childhood cancers (third most common type)

Question 11

What are the currently recommended classifications for hematologic malignancies?

Answer 11

1) Myeloid malignancies
2) Lymphoid malignancies
3) Other

Question 12

What are myeloid malignancies? (Think classification)

Answer 12

Arise from mature/immature members of …

1) granulocytic,
2) monocytic,
3) erythroid,
4) megakaryocytic, and/or
5) mast cell lineages

Question 13

What are lymphoid malignancies? (Think classification)

Answer 13

Arise from mature/immature members of ….

1) B cell,
2) T cell, or
3) NK cell lineages

Question 14

What are ‘other’ malignancies? (Think classification)

Answer 14

Arise from histiocytes or DCs

• Rare
• Will not be discussed further — just know there’s a classification group for this

Question 15

Which parameters are used to help classify hematologic malignancies?

Answer 15

1) Microscopic appearance of malig cells
2) Histologic growth pattern (in BM, lymph, other)
3) Presence/absence of cytogenetic/molecular findings
4) Relative amount of malignant cells in blood/BM
5) Presence/absence of cell surface/cytoplasmic/nuclear markers

Question 16

What are the basic functional categories for hematologic malignancies?

Answer 16

1) Acute leukemias
2) Myelodysplastic syndrome (MDS)
3) Myeloproliferative neoplasms (MPNs)
4) Classical hodgkin lymphoma (CHL)
5) Non-Hodgkin Lymphoma
6) Plasma cell neoplasms

Question 17

What are the basic expected findings in the blood and marrow for acute leukemias?

Answer 17

• Rapid accumulation of immature cells in BM
• Usually -blasts accumulate — myeloid or lymphoid
• Often replace normal BM cells causing cytopenias

Question 18

What are the basic expected findings in the blood and marrow for Myelodysplastic syndrome (MDS)?

Answer 18

• Clonal pop’n derived from neoplastic HSM takes over BM
• No longer capable of making normal blood cells
• Falling peripheral blood cell counts

Question 19

What are the basic expected findings in the blood and marrow for Myeloproliferative neoplasms (MPNs)?

Answer 19

• Neoplastic clonal proliferations of BM
• Where clone makes normal functioning blood cells but makes TOO MANY of them
• Classified based on underlying cytogenetic abnormality, type of blood cell overproduced, and other stufff

Question 20

What are the basic expected findings in the blood and marrow for classical hodgkin lymphoma (CHL)?

Answer 20

• Driven by HRS cells (which are derived from B cells)

- Disease is its own clinical entity

Question 21

What are the basic expected findings in the blood and marrow for Non-Hodgkin Lymphoma?

Answer 21

Refers to any malignancy derived from mature B cells (excluding CHL or plasma cell neoplasms), T cells, or NK cells

Question 22

What are the basic expected findings in the blood and marrow for plasma cell neoplasms?

Answer 22

Includes…

1) MGUS
2) plasmacytoma
3) multiple myeloma

Question 23

What is leukemia?

Answer 23

• Heme malignancies where chief manifestation involves blood and marrow
• Diseases of both lymphoid and myeloid cells — mature and immature

Question 24

What is lymphoma?

Answer 24

• Heme malignancy derived from lymphocytes or precursors

- Manifests as a mass! unlike leukemia

Question 25

What is a extramedullary myeloid tumor/granulocytic sarcoma?

Answer 25

• Heme malignancy composed of myeloid cells or precursors
• Same cells as leukemia but manifests as a mass

Much less common

Question 26

Epstein-Barr virus causes which types of heme malignancies?

Answer 26

• Develops some cases of Hodgkin lymphoma
• Some Burkitt lymphoma and other B-cell non-Hodgkin
• Overall affects B-cells

Question 27

HTLV-1 causes which types of heme malignancies?

Answer 27

Develops adult T cell leukemia/lymphoma

Question 28

KSHV/HHV-8 causes which types of heme malignancies?

Answer 28

Implicated in primary effusion lymphoma