EXAM #2: CANCER & HEMATOLOGICAL MALIGNANCY

Question 1

What is the definition of a Leukemia?

Answer 1

• Unregulated growth of WBCs (leukocytes) in the bone marrow

Note that this can leads to either increases or decreases of WBCs in the peripheral blood AND bone marrow failure i.e. pancytopenia.

Question 2

What is the broad definition of a Lymphoma?

Answer 2

Discrete tumor masses in the lymph nodes

Question 3

What is the difference between a Leukemia and Lymphoma?

Answer 3

Leukemia= neoplasm of the bone marrow

Lymphoma= neoplasm of the lymph nodes

Question 4

What is the general difference between an acute and chronic leukemia?

Answer 4

Acute= will kill within weeks without treatment

Chronic= patient may survive for years without treatment

Question 5

What do the terms myeloid, myelogenous, and myeloproliferative refer to?

Answer 5

Tumors involving the:

• Granulocytes
• RBCs
• Platelets

And their progenitors*

Question 6

What do the terms lymphoid, lymphocytic, lymphoblastic, and lymphoproliferative refer to?

Answer 6

Tumors involving the:

• B-cells
• T-cells
• NK-cells

And their progenitors

Question 7

What are the two types of acute leukemia?

Answer 7

Acute myeloblastic (AML) 
Acute lymphoblastic (ALL)

Question 8

What is the root of symptoms for patients with ALL and AML?

Answer 8

Pancytopenia

Question 9

What is the difference between ALL-B and ALL-T?

Answer 9

Cell of origin is B vs. T cell in “Acute Lymphoblastic Leukemia”

Question 10

What is the general feature of all AMLs?

Answer 10

Undifferentiated myeloblasts accumulating in the bone marrow

Question 11

What is the most common acute leukemia in adults?

Answer 11

AML

Question 12

What is the basis for the FAB categorization of AML?

Answer 12

Appearance of cells under microscope

Question 13

What is the basis of the WHO categorization of AML?

Answer 13

Pathology, cytogenetics, and molecular characteristics

Question 14

What are the two types of ALL? Which is more common?

Answer 14

B-ALL* (B-cell)
T-ALL (T-cell)

This is the most common type of all ALL cases

Question 15

What is the most common acute leukemia in children?

Answer 15

ALL

Question 16

What is the difference between a myeloproliferative neoplasm and AML?

Answer 16

Myloeproliferative neoplasm can lead to AML

Question 17

List the major myeloproliferative neoplasms.

Answer 17

1) Polycythemia vera
2) Essential thrombocytpoenia
3) Primary myelofibrosis

Question 18

What is polycythemia vera?

Answer 18

RBC overproduction i.e. Hct >55%

Question 19

What is the mutation associated with polycythemia vera?

Answer 19

JAK2 mutation

Question 20

What is Essential Thrombocythemia?

Answer 20

Overproduction of megakaryocytes/ abnormal platelets

Question 21

What mutations are associated with Essential Thrombocythemia?

Answer 21

JAK2 or MPL

Question 22

What is Primary Myelofibrosis?

Answer 22

Fibrosis that obliterates the bone marrow and produces atypical megakaryocytes

Question 23

What is the genetic mutation associated with Primary Myelofibrosis?

Answer 23

JAK2 or MPL

Question 24

What is CML?

Answer 24

Overgrowth of granulocytic and megakaryocytic precursors in bone marrow due to BCR-ABL fusion gene

Can convert to AML

Question 25

What is myelodysplastic syndrome?

Answer 25

Class of stem cell disorders that result in ineffective hematopoiesis; causes defects in cell maturation of all non-lymphoid lineages *****Can convert to AML*****

Question 26

What viral infection causes the predominance of B-cell Lymphoid malignancies?

Answer 26

EBV

Question 27

What cells is ALL typically derived from?

Answer 27

Immature B-cells

Question 28

In CLL and lymphoma, what is the malignant cell population?

Answer 28

Mature B-cells

Question 29

What is the difference between the locations of malignant cells in CLL and lymphoma?

Answer 29

CLL= blood Lymphoma= lymph node or other tissues

Question 30

What cells are malignant in myeloma?

Answer 30

Plasma cells

Question 31

What do malignant plasma cells in myeloma produce?

Answer 31

Monoclonal immunoglobulin

Question 32

What is the difference between Hodgkin and Non-Hodgkin Lymphoma?

Answer 32

Presence of absence of Reed-Sternberg cell

Question 33

What are Reed-Sternberg cells, and what are the characteristics of Reed-Sternberg cells?

Answer 33

These are tumor giant cells seen in Hodgkin's Disease. Characteristics include: - Large size - 2+ nuclei (bilobed) that give an "OWL EYE" appearance - Eosinophilic nucleolus - CD15 and CD30+

Question 34

Describe the progression of Hodgkin Lymphoma.

Answer 34

- Hodgkin Lymphoma is localized to a single group of lymph nodes - Spread is contiguous and predictable

Question 35

What is the prognosis for Hodgkin Lymphoma?

Answer 35

60-90% cure rate with radiation and chemotherapy i.e. GOOD PROGNOSIS

Question 36

What are the three categories of Non-Hodgkin Lymphoma?

Answer 36

Indolent Aggressive V. aggressive

Question 37

What are the characteristics of CLL?

Answer 37

- Increased small round lymphocytes - Smudge cells ****Smudge cells are fragile cells that break on PBS--causing a smudge*****

Question 38

What the major plasma cell disorders.

Answer 38

- Multiple myeloma - Plasmacytoma - Primary Amyloidosis - MGUS - POEMS Syndrome - Waldenstrom Macroglobinemia

Question 39

What is Multiple Myeloma?

Answer 39

Mature B-cell (plasma cell) cancer

Question 40

What PBS features is characteristic of Multiple Myeloma?

Answer 40

Rouleaux formation, which is RBCs stacked like poker chips on PBS

Question 41

What lab features are characteristic of Multiple Myeloma?

Answer 41

Increased immunoglobulin (IgG or IgA) in serum or urine

Question 42

What is a Solitary Plasmacytoma? What lab feature is characteristic of this disease?

Answer 42

This is a plasma cell tumor characterized by a single plasma cell tumor in the bone marrow. - NO or low immunoglobulin in the blood

Question 43

What is Primary Amyloidosis?

Answer 43

Plasma cells secrete monoclonal light chains - These are misfolded and form fibril deposits in tissue - Disrupts organ function

Question 44

What is MGUS?

Answer 44

"Monoclonal Gammopathy of Undetermined Significance" - This is the ASYMPTOMATIC PRECURSOR of mutiple myeloma see with production of immunoglobulin detected in serum or urine ****Only 1-2% of patients with MGUS progress to Multiple Myeloma per year; hence the "undetermined significance"

Question 45

What lab value is associated with Waldenstrom Macroglobulinemia?

Answer 45

IgM ****Associated with hyperviscosity syndrome*****

Question 46

How is Leukemia diagnosed?

Answer 46

PBS | Bone marrow biopsy

Question 47

What is lymphoma diagnosed?

Answer 47

Lymph node biopsy

Question 48

How are plasma cell neoplasms diagnosed?

Answer 48

Bone marrow biopsy