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
Q

What is multiple myeloma?

A

malignant tumor of plasma cells –> destroys bone tissue causing pain and compromising normal bone marrow function

26
Q

Who gets multiple myeloma?

A
  • more common in african american than cuacasion

- median age 65

27
Q

What is clinical presentation of multiple myeloma?

A
  • hypercalcemia
  • renal insufficiency
  • anemia
  • bone lytic lesions
  • back pain
  • monoclonal M protein
  • increased plasma cells in bone marrow
28
Q

How do you diagnose multiple myeloma?

A

presence of monoclonal Ig protein in blood and urine; increased number of plasma cells in bone marrow

29
Q

What do you see on peripheral smear in multiple myeloma?

A

rouleaux fomration = RBCs stacked like poker chips

- plasma cells wtih clock face chromatin and large eccentric nuclei

30
Q

What kind of bone disease do you see in multiple myeloma?

A

punched out lytic lesions and diffuse bone loss [osteopenia] b/c myeloma cells produce osteoclast activating factors

31
Q

Why do you get hypercalcemia in multiple myeloma?

A

b/c of bone dissolution

32
Q

What is cause of renal disease in multiple meloma?

A
  • precipitation of bence jone proteins

- amyloid deposition

33
Q

What type of Ig is normally M ptotein in myeloma?

A

50% IgG; 20% IgA; 20% light chain only

34
Q

What type of amyloid in multiple myeloma?

A

polymer of light chain fragments = primary amyloidosis [AL]

35
Q

What do you see on CBC in multiple myeloma?

A

anemia, neutropenia, thrombocytopenia

36
Q

WHat happens to ESR in multiple myeloma?

A

ESR is elevated

37
Q

What are two conditions that will slow down ESR?

A
  • polycythemia

- sickle cells

38
Q

What do you see on bone marrow aspirate in multiple myeloma?

A
  • 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
Q

What is prognosis of multiple myeloma?

A

uncurable disease w/ median survival 3 years

40
Q

What is treatment for multiple myeloma?

A

give recombinant EPO, biphosphonates, treat infection

41
Q

What is waldenstrom’s macroglobulinemia?

A

see in older ~ 60 yo
proliferation of clonal lymphocytes that secrete IgM

lymphocytes have morphologic heterogeneity

42
Q

What are clinical features of waldenstroms macroglobulinemia?

A

more similar to low grade lymphoma than multiple myeloma

present with lymphadenopathy, hepatosplenomegaly, bone marrow infiltration

lymphoplasmacytoid appearance of cells

no bone lesions

43
Q

What is characteristic clinical finding of waldenstrom’s macroglobulinemia?

A

elevated monoclonal IgM in blood –> hyperviscosity syndrome –> fatigue, headache, dizziness, visual disturbance, tinnitus, heart failure

44
Q

What is treatment for waldenstroms?

A

no curative therapy; treat hyperviscosity with plasmapheresis to remove IgM protein

45
Q

What is solitary plasmacytoma?

A

solid tumor of plasma cells in bone or upper airways

may secrete monoclonal protein; may progress to multiple myeloma

46
Q

What is prognosis for solitary plasmacytoma?

A

median survival 10 yrs