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6 White Blood Cell disorders > 6.5,6,7 Lymphadenopathy, Lymphoma > Flashcards

6.5,6,7 Lymphadenopathy, Lymphoma Flashcards

1
Q

Follicular lymphoma

  • feared complication
  • how does it present
A
  • progression to diffuse large B-cell lymphoma
  • presents as an enlarging lymph node (pt already has many enlarged lymph nodes)
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1
Q

How to distinguish Follicular lymphoma from reactive follicular hyperplasia (which can occur from infection)?

(4 methods)

A
  1. Disruption of lymph node architecture (follicles spread throughout node in follicular lymphoma)
  2. No tingible body macrophages–In follicular hyperplasia, you see tingible body macrophages in the follicles–these are macrophages eating apoptosed B cells.
  3. BCL2 expression in follicular lymphoma
  4. monoclonality in follicular lymphoma (increased kappa/lambda light chain ratio)
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2
Q

Follicular lymphoma

-genetic cause, mech

A
  • t(14:18) – 14 is Ig Heavy chain, 18 is BCL2
  • There is overexpression of BCL2, which prevents Cytc C from leaving the mitrochrondria for apoptosis. Therefore, B Cells in the germinal center of follicle that are undergoing somatic hypermutation cannot die when they are supposed to.
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2
Q

Marginal Zone Lymphoma

  • proliferation of what cells
  • what is the marginal zone
A
  • Small cell CD20+ B cells, expand the marginal zone
  • the marginal zone surrounds the mantle zone around a follicle. It normally does not exist until there is activation of B-Cells from the germinal center during inflammation. Therefore, it’s assoc with chronic inflammation states.
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2
Q

Burkitt lymphoma

  • proliferation of what
  • assoc with what infection
A
  • intermediate size B cells, CD20+
  • EBV infection
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2
Q

C-MYC

-what WBC disorder is this assoc with

A
  • Burkitt lymphoma
  • 8:14 translocation
  • C-MYC is a TF for cell growth
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3
Q

Lymph node:

  • 3 regions, and the cells in each
  • What conditions cause hyperplasia of each region?
A
  1. Cortex–B cells–RA, early HIV
  2. Paracortex–T cells–Viral infections (eg EBV from infectious mononucleosis)
  3. Medulla–Sinus histiocytes–drainage of region with cancer
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5
Q

Lymph node draining a region of cancer–what part of lymph node has hyperplasia?

A

-Medulla, where the sinus histiocytes are.

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7
Q

Nodular sclerosis subtype of Hodgkin lymphoma

-appearance on histology

A

“nodular sclerosis”

  • bands of sclerosis in the lymph node divide lymph tissue into nodules
  • RS cels are present in lake-like spaces (‘lacunar cells’)
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8
Q

(HL) Hodgkin Lymphoma

-proliferation of what, with what cell markers?

A
  • proliferation of Reed-Sternberg cells, which are large B cells with multilobed nuclei (‘owl eyes’), CD15 and CD30 positive. (not CD20)
  • RS cells secrete cytokines, which attract other leukocytes which form the bulk of the tumor
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8
Q

Reed-Sternberg cell

A
  • large B-cell, CD15 CD30 positive
  • proliferation in Hodgkin lymphoma
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9
Q

Follicular lymphoma

-Tx

A
  1. Rituximab (anti CD20)–the proliferative small B cells are CD20+
  2. low dose chemo
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10
Q

BCL2

-assoc with what WBC disorder

A
  • follicular lymphoma
  • 14:18 translocation
  • BLC2 overexpression prevents Cytc C from leaking from mitochondria for proper apoptosis of B cells undergoing somatic hypermutation
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12
Q

RA, early HIV–cause hyperplasia of lymph node in what region?

A

Cortex, where B cells live.

-HIV affects this region b/c the follicular dendritic cells are CD4+

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13
Q

MALToma

  • what is it
  • what can cause it
A
  • Marginal zone lymphoma in mucosal sites–MALT (mucosa associated lymphoid tissue)
  • can get MALToma from H Pylori gastritis.
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14
Q

Name assoc disorders:

  1. C-MYC
  2. BCL2
  3. Cyclin D1
A
  1. Burkitt lymphoma (8:14)
  2. Follicular lymphoma (14:18)
  3. Mantle cell lympoma (11:18)
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16
Q

LAD (Lymphadenoapathy)

-divided into 2 forms

A
  1. painful (acute infection)
  2. painless (chonic inflammation, metastatic carcinoma, lymphoma)
17
Q

Name the assoc disorders and genes

  1. t(8:14)
  2. t(11:14)
  3. t(14:18)
A
  1. Burkitt lymphoma, C-MYC (a TF for growth)
  2. Mantle cell lymphoma, Cyclin D1 (promote G1-S)
  3. Follicular lymphoma, BCL2 (prevents apoptosis)
18
Q

(HL) Hodgkin Lymphoma

-clinical presentation (4 typical symptoms)

A

‘B’ symptoms from cytokines:

  1. fever
  2. chills
  3. weight loss
  4. night sweats
19
Q

Hashimoto’s thyroiditis:

-assoc with what WBC disorder?

A

Marginal zone lymphoma

(chronic inflammation)

20
Q

NHL (non Hodgkin lymphoma):

-Divide into small, intermediate, large B cells: list types

A
  1. small B cell: (think well differentiated)
    - follicular, mantle cell, marginal zone, small lymphocytic (CLL)
  2. Intermediate sized B cells
    - Burkitt lymphoma
  3. Large B cells
    - diffuse large B-cell lymphoma
21
Q

Painless lymphadenopathy

-differential?

A
  1. chronic inflammation (eg autoimmune)
  2. metastatic carcinoma
  3. lymphoma
21
Q

Mantle cell lymphoma

-genetics, mech

A
  • t(11:14)
  • 11 has Cyclin D1, which promotes G1/S transition in cell cycle by phosphorylating proteins
22
Q

What subtype of Hodgkin lymphoma is assoc with HIV and the elderly?

A
  • lymphocyte poor Hodgkin lymphoma
  • worst prognosis of all subtypes (most aggressive)
24
Q

Cyclin D1:

-assoc with what WBC disorder

A
  • Mantle zone lymphoma
  • 11:14 translocation– Cyclin D1 allows cell to advance G1 to S
25
Q

Diffuse large B Cell lymphoma

  • how does it present?
  • population
A

-late adulthood, presents as enlarging lymph node, or an extranodal mass

27
Q

Lymphoma

  • definition
  • where can they occur
A
  • neoplastic proliferation of lymphoid cells that forms a mass
  • can be in lymph node, or extranodal tissue
28
Q

Burkitt lymphoma

  • populations
  • classic presentations (2)
A

Extranodal mass in child or young adult.

  1. African form–in the jaw
  2. Sporadic form–in the abdomen
28
Q

Nodular sclerosis subtype of Hodgkin lymphoma

  • classic presentation
  • population
A

-enlarging cervical or mediastinal lymph node in a young female adult

30
Q

Burkitt lymphoma

-appearance in histology

A

-“starry sky” appearance–majority of B cells are dark (sky), with tingible body macrophages eating the rapidly dying ones (stars)

31
Q

(HL) Hodgkin Lymphoma

-Subtypes

  • most common?
  • best prognosis?

-worst prognosis?

A
  1. Nodular sclerosis (70%)
  2. lymphocyte rich (best prognosis)
  3. mixed cellularity (assoc with IL5 eosinophils)
  4. lymphocyte poor (worst prognosis)
32
Q

Diffuse large B Cell lymphoma

-proliferation of what?

A
  • large B-cells, CD20+
  • diffuse growth, high grade, very aggressive
33
Q

Burkitt lymphoma

-genetic mutation, mech

A
  • 8:14 translocation with C-MYC
  • C-MYC is a TF that stimulates cell growth
34
Q

Mantle cell lymphoma

  • where is the mantle zone
  • how does this typically present
A
  • mantle zone surrounds the follicle. The marginal zone surrounds the mantle zone
  • Presents as painless LAD in late adulthood
35
Q

Diffuse large B Cell lymphoma

-2 different ways it forms

A
  1. sporadically
  2. transformation from a low-grade lymphoma (eg follicular lymphoma)
36
Q

(HL) Hodgkin Lymphoma

which subtype is assoc with abundant Eosinophils?

A

-mixed cellularity type, with RS cells producing IL-5

37
Q

Follicular lymphoma

-How does it initially present, in what population?

A

-Typically presents in late adulthood, with painless LAD

38
Q

Marginal Zone Lymphoma

-assoc with what disorders (3)

A
  • assoc with chronic inflammation states b/c marginal zone is formed only during inflammation by activated B cells
    1. Hashimoto’s thyroiditis (years later)
    2. Sjogren syndrome (years later)
    3. H Pylori gastritis–MALToma
39
Q

Viral infections–cause hyperplasia of what lymph node region?

A

-Paracortex–where T cells live.

T cells are defense against viruses

40
Q

Sjogren syndrome

-assoc with what WBC disorder

A

Marginal zone lymphoma

(chronic inflammation)

41
Q

Follicular lymphoma

-proliferation of what cells, with what markers present

A

-Small B cells (CD20+), proliferation forms follicle-like nodules

6 White Blood Cell disorders (6 decks)

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