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WBC Disorders > WBC Part 3 > Flashcards

WBC Part 3 Flashcards

1
Q

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

A

marginal zone lymphoma (MZL)

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2
Q

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

A

marginal zone lymphoma (MZL)

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3
Q

Marginal Zone Lymphomas may recess how

A

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

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4
Q

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

A

pleomorphic population of monocytoid B-cells

plasmacytoid cells with destructive infiltration of host tissues

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5
Q

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

A

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

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6
Q

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

A

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

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7
Q

Lymphoplasmacytic lymphoma (LPL) primarily involves what tissues

A

bone marrow +/- spleen

nodes

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8
Q

Lymphoplasmacytic lymphoma (LPL) secretes what

A

monoclonal IgM

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9
Q

what is Waldenstrom macroglobulinemia

A

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

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10
Q

the increased blood viscosity caused by IgM causes what

A 
visual symptoms
neurologic symptoms
bleeding
autoimmune hemolytic anemia
cryoglobulinemia
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11
Q

what is cryoglobulinemia and when is it seen

A

precipitation of IgM at low temps (fingers and toes)

seen in lymphoplasmacytic lymphoma (LPL)

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12
Q

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

A

plasmapheresis- removes large amounts of IgM

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13
Q

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

A

BRAF

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14
Q

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

A

hairy cell leukemia (HCL)

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15
Q

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

A

hairy cell leukemia (HCL)

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16
Q

clinical feature of hairy cell leukemia (HCL) are?

A

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

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17
Q

diffuse fried egg morphology is characteristic of what cancer

A

multiple myeloma

hairy cell leukemia

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18
Q

what morphologic features would one expect in hairy cell leukemia

A

diffuse fried egg look

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

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19
Q

a dry tap is seen in what cancer and why

A

hairy cell leukemia- cells incite reticulin fibrosis

20
Q

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

A

CD19, CD20, surface light chain restriction

CD11c, CD25, CD103

21
Q

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

A

purine analogs and thus have long remissions

22
Q

CD19, CD20, surface light chain restriction

CD11c, CD25, CD103

A

hairy cell leukemia

23
Q

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

A

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

24
Q

what is M component (protein)

A

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

25
Q

what is Bence Jones proteinuria?

A

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

26
Q

what are the subcategories of plasma cell neoplasms

A

Multiple myeloma
Monoclonal Gammopathy of Understated Significance (MGUS)
Plasmacytoma

27
Q

multiple myeloma is defined by what

A

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

28
Q

in multiple myeloma the neoplastic cells produce what factors and why

A

IL-6 for proliferation and survival

MIP1alpha which cause bone destruction

29
Q

what are the clinical signs of multiple myeloma

A

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

30
Q

what abnormal labs are seen in multiple myeloma

A

CBC- anemia

Metabolic panel- hypercalemia, increased creatinine

31
Q

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

A

serum protein immunofixation

32
Q

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

A

IgG in 55% of cases

IgA in 25% of cases

33
Q

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

A

punched out lytic lesions

34
Q

what would one see on a blood smear of multiple myeloma

A 
Rouleaux formation (RBCs stacked like poker chips)
diffuse fried egg appearance
35
Q

Serum and Urine Protein Electrophoresis would show what in multiple myeloma

A

M spike from M component (protein)

36
Q

what is required for diagnosis of multiple myeloma

A

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

37
Q

what is the most common cause of monoclonal gammopathy

A

monoclonal gammopathy of undetermined significance (MGUS)

38
Q

monoclonal gammopathy of undetermined significance can progress to what

A

multiple myeloma (happens to 25% in 20 years)

39
Q

how can one check MGUS progression to multiple myeloma

A

periodic assessment of Bence Jones protein and serum M protein levels

40
Q

what is plasmacytoma

A

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

41
Q

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

A

bone- spine

soft tissue- upper respiratory tract/oropharynx

42
Q

how does one determine MGUS in a patient

A

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

43
Q

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

A

tartarate-resistant acid phosphate (TRAP)

44
Q

a positive TRAP test is significant in what

A

hairy cell leukemia

45
Q

what cytokine is released by neoplastic cells of Multiple Myeloma

A

IL-6

46
Q

clock face plasma cells are seen in what?

A

multiple myeloma

WBC Disorders (8 decks)

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