Ch 13 Key Concepts

Question 1

most common type of cancer in children, may be derived from either precursor B or T cells

• highly aggressive tumors manifest with signs and sx of bone marrow failure, or as rapidly growing masses
• tumor cells contain genetic lesions that block differentiation, leading to the accumulation of immature, nonfunctional blasts

Answer 1

acute lymphoblastic leukemia/lymphoblastic lymphoma

Question 2

most common leukemia in adults

• tumor of mature B cells that usually manifests with bone marrow and lymph node involvement
• indolent course, commonly associated with immune abnormalities, including an increased susceptibility to infection and autoimmune disorders

Answer 2

small lymphocytic lymphoma/chronic lymphocytic leukemia

Question 3

most common indolent lymphoma in adults
- tumor cells recapitulate the growth pattern of normal germinal center B cells; most cases are associated with a (14:18) translocation that results in the over-expression of BCL2

Answer 3

follicular lymphoma

Question 4

most common lymphoma of adults

• heterogenous group of mature B cell tumors that share a large cell morphology and aggressive clinical behavior
• rearrangements or mutations of BCL6 gene are recognized associations; one third carry a (14:18) translocation involving BCL2 and may arise from follicular lymphomas

Answer 4

diffuse large B cell lymphoma

Question 5

very aggressive tumor of mature B cells that usually arises at extranodal sites

• strongly associated with translocations involving the MYC proto-oncogene
• tumor cells often are latently infected by EBV

Answer 5

burkitt lymphoma

Question 6

plasma cell tumor that manifests with multiple lytic bone lesions associated with pathologic fractures and hypercalcemia
- neoplastic plasma cells suppress normal humoral immunity and secrete partial immunoglobulins that are nephrotoxic

Answer 6

multiple myeloma

Question 7

multiple myoloma is associated with diverse translocations inolving what locus?

Answer 7

IgH

- leads to frequent dysregulation and over-expression of D cyclins

Question 8

plasma cell neoplasm

• common in older adults, progresses to myeloma at a rate of 1% of patients per year
• M spike, but no other plasma cell neoplasm characteristics

Answer 8

monoclonal gammopathy of unknown significance

Question 9

plasma cell neoplasm

- disseminated disease that pursues an usually indolent course

Answer 9

smoldering myeloma

Question 10

plasma cell neoplasm

• solitary bone lesion identical to disseminated myeloma
• most progress to myeloma within 7-10 years

Answer 10

solitary osseous plasmacytoma

Question 11

plasma cell neoplasm

• solitary mass, usually in the upper aerodigestive tract
• rarely progresses to systemic disease

Answer 11

extramedullary plasmacytoma

Question 12

B cell lymphoma that exhibits plasmacytic differentiation

• clinical sx dominated by herviscosity related to high levels of tumor-derived IgM
• highly associated with mutations in the MYD88 gene

Answer 12

lymphoplasmacytic lymphoma

Question 13

tumor of naive B cells that pursues a moderately aggressive course and is highly associated with translocations involving the cyclin D1 gene

Answer 13

mantle cell lymphoma

Question 14

indolent tumors of antigen-primed B cells that arise at sites of chronic immune stimulation and often remain localized for long periods of time

Answer 14

marginal zone lymphoma

Question 15

morphologically distinct, very indolent tumor of mature B cells that is highly associated with mutations in the BRAF serine/threonine kinase

Answer 15

hairy cell leukemia

Question 16

peripheral NK/T cell lymphoma:

- aggressive T cell tumor, associated in a subset with activating mutations in the ALK tyrosine kinase

Answer 16

anaplastic large cell lymphoma

Question 17

peripheral NK/T cell lymphoma:

- aggressive tumor of CD4 T cells that is uniformly associated with HTLV-1 infection

Answer 17

adult T cell leukemia/lymphoma

Question 18

peripheral NK/T cell lymphoma:
- indolent tumor of cytotoxic T cells or NK cells that is associated with mutations in the transcription factor STAT3 and with autoimmune phenomena and cytopenias

Answer 18

large granular lymphocytic leukemia

Question 19

peripheral NK/T cell lymphoma:

- aggressive tumor, usually derived from NK cells, that is strongly associated with EBV infection

Answer 19

extranodal NK/T cell lymphoma

Question 20

unusual tumor consisting mostly of reactive lymphocytes, macrophages, eosinophils, plasma cells and stromal cells mixed with rare tumor giant cells called Reed-Sternberg cells

Answer 20

Hodgkin lymphoma

Question 21

what are the two broad types of Hodgkin lymphoma?

Answer 21

1. classical

2. lymphocyte predominant

Question 22

which form of Hodgkin lymphoma is frequently associated with acquired mutations that activate transcription factor NF-kB and EBV infection?

Answer 22

classical
- Reed-Sternberg cells make multiple cytokines and chemokines that influence the host response, and the host response in turn makes factors that support the growth of tumor cells

Question 23

lymphocyte predominant Hodgkin lymphoma expresses what type of markers?

Answer 23

B cell markers

- NOT associated with EBV

Question 24

aggressive tumors comprised of immature myeloid lineage blasts, which replace the marrow and suppress normal hematopoiesis

• associated with diverse acquired mutations that lead to expression of abnormal transcription factors, which interfere with myeloid differentiation
• often associated with mutations in genes encoding growth factor receptor signaling pathway components or regulators of the epigenome

Answer 24

acute myeloid leukemias (AMLs)

Question 25

tumors in which production of formed myeloid elements is initially increased, leading to high blood counts and extramedullary hematopoiesis
- commonly associated with mutations that lead to constitutive activation of tyrosine kinase, which mimic signals from normal growth factors

Answer 25

myeloproliferative disorders
- all can transform to acute leukemia and to a spent phase of marrow fibrosis associated with anemia, thrombocytopenia, and splenomegaly

Question 26

what are the most common pathogenic kinases of myeloproliferative disorders?

Answer 26

• BCR-ABL (associated with CML)

- mutated JAK2 (associated with polycythemia vera and primary myelofibrosis)

Question 27

poorly understood myeloid tumors characterized by disordered and ineffective hematopoiesis and dysmaturation

• recently shown to frequently harbor mutations in splicing factors and epigenetic regulators
• manifest with one of more cytopenias and progress in 10-40% of cases to AML

Answer 27

myelodisplastic symdromes