WBC Part 3

Question 1

what cancer often arises in tissue involved by chronic inflammatory disorders (H. Pylori in stomach, Hashimotos in thyroid)

Answer 1

marginal zone lymphoma (MZL)

Question 2

what cancer does that tumor cell recapitulate normal “MALT” tissue

Answer 2

marginal zone lymphoma (MZL)

Question 3

Marginal Zone Lymphomas may recess how

Answer 3

if inciting agent (H. pylori for example) is brought under control or irradiated

Question 4

in marginal zone lymphoma (MZL) what does one expect to see morphologically

Answer 4

pleomorphic population of monocytoid B-cells

plasmacytoid cells with destructive infiltration of host tissues

Question 5

the polyclonal to oligoclonal to monoclonal theory is what and applies to what

Answer 5

marginal zone lymphoma (MZL)
reactive polyclonal inflammatory reaction acquires mutations over time that emerges as oligoclonal or monoclonal population
still dependent on reactive T-cells for growth and survival

Question 6

acquisition of t(11;18) or t(1;14) leads to what in marginal zone lymphomas (MZL)

Answer 6

leads to neoplastic population that no longer responds to antibiotics- growth now independent of extrinsic stimuli

Question 7

Lymphoplasmacytic lymphoma (LPL) primarily involves what tissues

Answer 7

bone marrow +/- spleen

nodes

Question 8

Lymphoplasmacytic lymphoma (LPL) secretes what

Answer 8

monoclonal IgM

Question 9

what is Waldenstrom macroglobulinemia

Answer 9

a hyperviscosity syndrome caused by the monoclonal IgM secreted by lymphoplasmacytic lymphoma (LPL)

Question 10

the increased blood viscosity caused by IgM causes what

Answer 10

visual symptoms
neurologic symptoms
bleeding
autoimmune hemolytic anemia
cryoglobulinemia

Question 11

what is cryoglobulinemia and when is it seen

Answer 11

precipitation of IgM at low temps (fingers and toes)

seen in lymphoplasmacytic lymphoma (LPL)

Question 12

how can one alleviate the hyperviscosity symptoms associated with lympoplasmacytic lymphoma (LPL)

Answer 12

plasmapheresis- removes large amounts of IgM

Question 13

Hairy Cell leukemia (HCL) is associated with a mutation in what?

Answer 13

BRAF

Question 14

“hairy” B-cells involving the blood, bone marrow, and splenic red pulp are seen in what

Answer 14

hairy cell leukemia (HCL)

Question 15

if your patient is an elderly male with splenomegaly and pancytopenia, what cancer should you suspect

Answer 15

hairy cell leukemia (HCL)

Question 16

clinical feature of hairy cell leukemia (HCL) are?

Answer 16

symptoms related to cytopenias (infection, fatigue, weakness)
splenomegaly (BM involvement)
monocytopenia

Question 17

diffuse fried egg morphology is characteristic of what cancer

Answer 17

multiple myeloma

hairy cell leukemia

Question 18

what morphologic features would one expect in hairy cell leukemia

Answer 18

diffuse fried egg look

round to reniform nucleus with moderate amount of pale blue cytoplasm which has “hairs”

Question 19

a dry tap is seen in what cancer and why

Answer 19

hairy cell leukemia- cells incite reticulin fibrosis

Question 20

what flow cytometry results would one expect to find in hairy cell leukemia (HCL)

Answer 20

CD19, CD20, surface light chain restriction

CD11c, CD25, CD103

Question 21

hairy cell leukemia neoplastic cells are very sensitive to what type of chemo

Answer 21

purine analogs and thus have long remissions

Question 22

CD19, CD20, surface light chain restriction

CD11c, CD25, CD103

Answer 22

hairy cell leukemia

Question 23

plasma cell neoplasms in general are a group of diseases caused by what

Answer 23

clonal plasma cell proliferation that secretes a single immunoglobulin (heavy chain and/or light chain)

Question 24

what is M component (protein)

Answer 24

a monoclonal protein identified in the blood or urine of plasma cell neoplasms

Question 25

what is Bence Jones proteinuria?

Answer 25

excess free monoclonal light chains that damage renal tubules (directly and by cast formation)

Question 26

what are the subcategories of plasma cell neoplasms

Answer 26

Multiple myeloma Monoclonal Gammopathy of Understated Significance (MGUS) Plasmacytoma

Question 27

multiple myeloma is defined by what

Answer 27

M-protein in the blood or urine, clonal plasma cells, and end organ damage

Question 28

in multiple myeloma the neoplastic cells produce what factors and why

Answer 28

IL-6 for proliferation and survival | MIP1alpha which cause bone destruction

Question 29

what are the clinical signs of multiple myeloma

Answer 29

think CRAB hyperCalcemia (polyuria, constipation, confusion) Renal involvement (Ig light chains- bence jones protein) Anemia (weakness, fatigue) Bone lytic lesions Back pain

Question 30

what abnormal labs are seen in multiple myeloma

Answer 30

CBC- anemia | Metabolic panel- hypercalemia, increased creatinine

Question 31

what lab technique is used to identify and quantitate light chain specificity

Answer 31

serum protein immunofixation

Question 32

what immunoglobulins are the most common the be increased in multiple myeloma

Answer 32

IgG in 55% of cases | IgA in 25% of cases

Question 33

what would one see on an X-Ray of a multiple myeloma patient

Answer 33

punched out lytic lesions

Question 34

what would one see on a blood smear of multiple myeloma

Answer 34

``` Rouleaux formation (RBCs stacked like poker chips) diffuse fried egg appearance ```

Question 35

Serum and Urine Protein Electrophoresis would show what in multiple myeloma

Answer 35

M spike from M component (protein)

Question 36

what is required for diagnosis of multiple myeloma

Answer 36

a Bone Marrow sample- look for plasmacytosis, groups and sheets

Question 37

what is the most common cause of monoclonal gammopathy

Answer 37

monoclonal gammopathy of undetermined significance (MGUS)

Question 38

monoclonal gammopathy of undetermined significance can progress to what

Answer 38

multiple myeloma (happens to 25% in 20 years)

Question 39

how can one check MGUS progression to multiple myeloma

Answer 39

periodic assessment of Bence Jones protein and serum M protein levels

Question 40

what is plasmacytoma

Answer 40

solitary bone or soft tissue clonal plasma cell mass with no evidence of marrow or organ disease

Question 41

what is the most common site in the bone and in the soft tissue to find a plasmacytoma

Answer 41

bone- spine | soft tissue- upper respiratory tract/oropharynx

Question 42

how does one determine MGUS in a patient

Answer 42

exclude all other causes of monoclonal gammopathy (indolent or smoldering multiple myeloma, other lymphomas)

Question 43

what specific test is use for hairy cell leukemia (HCL)

Answer 43

tartarate-resistant acid phosphate (TRAP)

Question 44

a positive TRAP test is significant in what

Answer 44

hairy cell leukemia

Question 45

what cytokine is released by neoplastic cells of Multiple Myeloma

Answer 45

IL-6

Question 46

clock face plasma cells are seen in what?

Answer 46

multiple myeloma