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Heme > Lec 15 Lymphoproliferative Disorders > Flashcards

Lec 15 Lymphoproliferative Disorders Flashcards

1
Q

What are some markers of the earliest pre-B cells?

A
  • TdT enzyme
  • CALLA surface antigen [CD10]
  • Ig heavy chain gene rearrangment
  • CD19
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2
Q

What are some markers of mature B cells?

A
  • CD20
  • intracytoplasmic Ig
  • light chain rearrangement
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3
Q

What do you call terminally differentiated B cells?

A

plasma cells = b lymphocytes that produce and secrete Ig following antigen exposure

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4
Q

What markers do earliest T cell precursors express?

A
  • enzyme TdT
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5
Q

What are some markers of mature T cells?

A
  • lose TdT
  • T cell receptor on surface
  • initial co-express CD4 and CD8 –> then commit
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6
Q

What is the most common type of leukemia?

A

CLL = chronic lymphocytic leukemia

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7
Q

Who gets CLL?

A

> 60 years old

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8
Q

What are some immunologica phenomena with CLL?

A

autoimmune hemolytic anemia; autoimmune thrombocytopenia; hypoimmunity [hypogammaglobulinemia] –> increased risk of infection

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9
Q

What is etiology of CLL?

A

clonal disease arising from mature B cells

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10
Q

What do you see on CLL labs?

A
  • peripheral blood lymphocytosis –> absolute lymphocytes > 5000
  • high WBC
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11
Q

What do you see on CLL blood smear?

A
  • mature lymphocytes
  • smudge cells
  • autoimmune hemolytic anemia
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12
Q

What do you see on bone marrow in CLL?

A

replacement of normal bone marrow cells by homogenous population of small lymphocytes

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13
Q

What is prognosis of CLL?

A

slowly progressive; survival up to 20 yrs

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14
Q

What is treatment of CLL?

A

if asymptomatic/early stage –> nothing

for symptomatic/later stage –> hchemo or monoclonal antibody rituximab or ibrutinib

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15
Q

What is hairy cell leukemia?

A

mature B cell tumor in the elderly; cells have hair-like cytoplasmic projections

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16
Q

How does hairy cell leukemia present?

A

panctopenia, splenomegaly

marrow/spleen infiltrated wtih typical cells; associated wtih marrow fibrosis

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17
Q

How can you diagnose hairy cell leukemia?

A

stains TRAP [tartrate resistant acid phosphatase]

flow cytometry

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18
Q

What is treatment for hairy cell leukemia?

A

cladribine = adenosine analog that inhibits adenosine deaminase

curative

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19
Q

What is prolymphocytic leukemia?

A

aggressive variant of CLL w/ less mature prolymphocytes

20
Q

What is monoclonal B cell lymphocytosis?

A

presence of absolute number clonal B lymphocytes < 5000

do not have adenopathy, hepatosplenomegaly, cytopenias

can progress to overt CLL

21
Q

What is a feature that is shared by plasma cell disorders?

A

monoclonal Ig protein in serum produced from single clone of plasma cells = M proteins

22
Q

What are some other diseases where you can see monoclonal Ig?

A
  • autoimmune diseases [RA, SLE]
  • liver disease [hepatitis]
  • HIV, bacterial endocarditis, TB
23
Q

What are Bence Jones proteins?

A

isolated light chains present in urine of some patients with M spikes due to excess light chain synthesis

24
Q

What is monoclonal gammopathy of undetermined significance [MGUS]?

A

monoclonal expansion of plasma cells with serum monoclonal protein spike in absence of underlying B cell malignancy

can be a precurser to multiple myeloma

25
What is multiple myeloma?
malignant tumor of plasma cells --> destroys bone tissue causing pain and compromising normal bone marrow function
26
Who gets multiple myeloma?
- more common in african american than cuacasion | - median age 65
27
What is clinical presentation of multiple myeloma?
- hypercalcemia - renal insufficiency - anemia - bone lytic lesions - back pain - monoclonal M protein - increased plasma cells in bone marrow
28
How do you diagnose multiple myeloma?
presence of monoclonal Ig protein in blood and urine; increased number of plasma cells in bone marrow
29
What do you see on peripheral smear in multiple myeloma?
rouleaux fomration = RBCs stacked like poker chips | - plasma cells wtih clock face chromatin and large eccentric nuclei
30
What kind of bone disease do you see in multiple myeloma?
punched out lytic lesions and diffuse bone loss [osteopenia] b/c myeloma cells produce osteoclast activating factors
31
Why do you get hypercalcemia in multiple myeloma?
b/c of bone dissolution
32
What is cause of renal disease in multiple meloma?
- precipitation of bence jone proteins | - amyloid deposition
33
What type of Ig is normally M ptotein in myeloma?
50% IgG; 20% IgA; 20% light chain only
34
What type of amyloid in multiple myeloma?
polymer of light chain fragments = primary amyloidosis [AL]
35
What do you see on CBC in multiple myeloma?
anemia, neutropenia, thrombocytopenia
36
WHat happens to ESR in multiple myeloma?
ESR is elevated
37
What are two conditions that will slow down ESR?
- polycythemia | - sickle cells
38
What do you see on bone marrow aspirate in multiple myeloma?
- hypercellular - infiltration of malignant palsma cells --> perinuclear halo, eccentric nuclei, clock face chromatin, but atypically large and have prominent nucleoli + may have multiple nuclei
39
What is prognosis of multiple myeloma?
uncurable disease w/ median survival 3 years
40
What is treatment for multiple myeloma?
give recombinant EPO, biphosphonates, treat infection
41
What is waldenstrom's macroglobulinemia?
see in older ~ 60 yo proliferation of clonal lymphocytes that secrete IgM lymphocytes have morphologic heterogeneity
42
What are clinical features of waldenstroms macroglobulinemia?
more similar to low grade lymphoma than multiple myeloma present with lymphadenopathy, hepatosplenomegaly, bone marrow infiltration lymphoplasmacytoid appearance of cells no bone lesions
43
What is characteristic clinical finding of waldenstrom's macroglobulinemia?
elevated monoclonal IgM in blood --> hyperviscosity syndrome --> fatigue, headache, dizziness, visual disturbance, tinnitus, heart failure
44
What is treatment for waldenstroms?
no curative therapy; treat hyperviscosity with plasmapheresis to remove IgM protein
45
What is solitary plasmacytoma?
solid tumor of plasma cells in bone or upper airways may secrete monoclonal protein; may progress to multiple myeloma
46
What is prognosis for solitary plasmacytoma?
median survival 10 yrs

Heme (20 decks)

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