Hematological malignancies

Question 1

definition: leukemia

Answer 1

cancer that starts in blood forming tissue such as bone marrow and causes large number of abnormal blood cells to be produced and enter the blood

Question 2

definition: lymphoma and myeloma

Answer 2

cancers that begin in the cells of the immune system

Question 3

myeloid / myelogenous / myeloproliferative cancer lines involve which cell types?

Answer 3

granulocytes, RBCs, platelets, or their progenitors

Question 4

lymphoid, lymphocytic, lymphoblastic, lymphoproliferative cancer lines involve which cell types?

Answer 4

B cells, T cells, NK cells, or their precursors

Question 5

what are the two types of acute leukemia?

Answer 5

1. acute myeloblastic leukemia (AML)

2. acute lymphoblastic leukemia / lymphoma (ALL)

Question 6

AML and ALL symptoms are often caused by what condition in the bone marrow?

Answer 6

pancytopenia - tumor cells displace or suppress normal blood elements in the bone marrow

Question 7

what is the main general feature of AML?

Answer 7

undifferentiated myeloid blast accumulating in bone marrow

Question 8

what are the two types of ALL? what are their frequencies among total ALL cases?

Answer 8

1. B cell type - 85%

2. T cell type - 15%

Question 9

what is a major concern for myeloproliferative neoplasms / disorders?

Answer 9

may transition into an acute leukemia later on

Question 10

what is the main pathology associated with polycythemia vera? what is the cause?

Answer 10

1. high levels of functional RBCs

2. JAK2 mutation

Question 11

what is the main pathology associated with essential thrombocythemia? what is the cause?

Answer 11

1. overproduction of megakaryocytes

2. JAK2 or MPL mutations

Question 12

what is the main pathology associated with primary myelofibrosis? what is the cause?

Answer 12

1. fibrosis and atypical megakaryocytes

2. JAK2 or MPL mutations

Question 13

what is the pathogenesis of CML? how does it present?

Answer 13

1. BCR-ABL fusion protein from Philadelphia chromosome leads to constitutively active kinase signaling mediating hematopoietic growth
2. overgrowth of granulocytic and megakaryocytic precursors in bone marrow

Question 14

what determines the classification of lymphoma as Hodgkin’s or non-Hodgkin’s?

Answer 14

presence of Reed-Sternberg cell

RS cell - Hodgkin’s lymphoma

Question 15

what is a Reed-Sternberg cell?

Answer 15

large cell with two or more nuclei or nuclear lobes, each of which contains a large eosinophilic nucleolus

Question 16

what are the three classifications of non-Hodgkin’s lymphoma?

Answer 16

1. indolent
2. aggressive
3. very aggressive

Question 17

what type of cancer is characterized by the presence of smudge cells?

Answer 17

chronic lymphocytic luekemia

Question 18

multiple myelomas are composed of what cell type?

Answer 18

mature B cells (plasma cells)

Question 19

multiple myeloma comes from what cell origin?

Answer 19

lymphoid origin!!

Question 20

what will multiple myeloma show on PBS?

Answer 20

Rouleaux formation

Question 21

what are the features of solitary plasmacytoma?

Answer 21

1. solitary mass
2. clonal plasma cells
3. low or no Ig in serum

Question 22

what is primary amyloidosis?

Answer 22

clonal expansion of plasma cells in the bone marrow that secrete monoclonal light chains that are misfolded and form fibril deposits in tissue

Question 23

what is the main pathology associated with primary amyloidosis?

Answer 23

organ problems due to amyloid fibril deposits

Question 24

what is monoclonal gammopathy of uncertain significance?

Answer 24

1. detection of monoclonal Ig in serum or urine without evidence for malignancy of the plasma or B cells
2. risk of progression to multiple myeloma

Question 25

what is POEMS syndrome?

Answer 25

plasma cell disorder characterized by

Polyneuropathy 
Organomegaly 
Endocrinopathy 
Monoclonal protein 
Skin changes

Question 26

what is Waldenstrom’s macroglobulinemia / lymphoblastic lymphoma?

Answer 26

1. IgM secreting

2. associated with hyperviscosity syndrome

Question 27

leukemias are diagnosed by which tests?

Answer 27

PBS and/or bone marrow biopsy

Question 28

lymphomas are diagnosed by which tests?

Answer 28

lymph node biopsy

Question 29

plasma cell neoplasms are diagnosed by which tests?

Answer 29

bone marrow biopsy

Question 30

an Auer rod is pathognomonic for for what cancer?

Answer 30

acute myelogenous leukemia (AML)

Question 31

what is the pathognomonic structure seen in AML?

Answer 31

Auer rod

Question 32

what technique is used to detect the Philadelphia chromosome translocation event for CML?

Answer 32

cytogenetics - karyotype or FISH

Question 33

what technique is used to detect the serum IgGk in someone with amyloidosis?

Answer 33

electrophoresis

Question 34

lytic bone lesions are characteristic of what type of cancer?

Answer 34

multiple myeloma

Question 35

what is a kaplan-meier plot?

Answer 35

compares experimental treatment to control with respect to patient survival % during a phase III trial

Question 36

what is the goal of induction therapy?

Answer 36

remission

Question 37

what is the goal of consolidation therapy?

Answer 37

eliminate undetectable cancer cells

Question 38

what is the goal of maintenance therapy?

Answer 38

maintain remission and reduce risk of relapse