6.5,6,7 Lymphadenopathy, Lymphoma Flashcards
Follicular lymphoma
- feared complication
- how does it present
- progression to diffuse large B-cell lymphoma
- presents as an enlarging lymph node (pt already has many enlarged lymph nodes)
How to distinguish Follicular lymphoma from reactive follicular hyperplasia (which can occur from infection)?
(4 methods)
- Disruption of lymph node architecture (follicles spread throughout node in follicular lymphoma)
- No tingible body macrophages–In follicular hyperplasia, you see tingible body macrophages in the follicles–these are macrophages eating apoptosed B cells.
- BCL2 expression in follicular lymphoma
- monoclonality in follicular lymphoma (increased kappa/lambda light chain ratio)
Follicular lymphoma
-genetic cause, mech
- 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.
Marginal Zone Lymphoma
- proliferation of what cells
- what is the marginal zone
- 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.
Burkitt lymphoma
- proliferation of what
- assoc with what infection
- intermediate size B cells, CD20+
- EBV infection
C-MYC
-what WBC disorder is this assoc with
- Burkitt lymphoma
- 8:14 translocation
- C-MYC is a TF for cell growth
Lymph node:
- 3 regions, and the cells in each
- What conditions cause hyperplasia of each region?
- Cortex–B cells–RA, early HIV
- Paracortex–T cells–Viral infections (eg EBV from infectious mononucleosis)
- Medulla–Sinus histiocytes–drainage of region with cancer
Lymph node draining a region of cancer–what part of lymph node has hyperplasia?
-Medulla, where the sinus histiocytes are.
Nodular sclerosis subtype of Hodgkin lymphoma
-appearance on histology
“nodular sclerosis”
- bands of sclerosis in the lymph node divide lymph tissue into nodules
- RS cels are present in lake-like spaces (‘lacunar cells’)
(HL) Hodgkin Lymphoma
-proliferation of what, with what cell markers?
- 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
Reed-Sternberg cell
- large B-cell, CD15 CD30 positive
- proliferation in Hodgkin lymphoma
Follicular lymphoma
-Tx
- Rituximab (anti CD20)–the proliferative small B cells are CD20+
- low dose chemo
BCL2
-assoc with what WBC disorder
- follicular lymphoma
- 14:18 translocation
- BLC2 overexpression prevents Cytc C from leaking from mitochondria for proper apoptosis of B cells undergoing somatic hypermutation
RA, early HIV–cause hyperplasia of lymph node in what region?
Cortex, where B cells live.
-HIV affects this region b/c the follicular dendritic cells are CD4+
MALToma
- what is it
- what can cause it
- Marginal zone lymphoma in mucosal sites–MALT (mucosa associated lymphoid tissue)
- can get MALToma from H Pylori gastritis.
Name assoc disorders:
- C-MYC
- BCL2
- Cyclin D1
- Burkitt lymphoma (8:14)
- Follicular lymphoma (14:18)
- Mantle cell lympoma (11:18)
LAD (Lymphadenoapathy)
-divided into 2 forms
- painful (acute infection)
- painless (chonic inflammation, metastatic carcinoma, lymphoma)
Name the assoc disorders and genes
- t(8:14)
- t(11:14)
- t(14:18)
- Burkitt lymphoma, C-MYC (a TF for growth)
- Mantle cell lymphoma, Cyclin D1 (promote G1-S)
- Follicular lymphoma, BCL2 (prevents apoptosis)
(HL) Hodgkin Lymphoma
-clinical presentation (4 typical symptoms)
‘B’ symptoms from cytokines:
- fever
- chills
- weight loss
- night sweats
Hashimoto’s thyroiditis:
-assoc with what WBC disorder?
Marginal zone lymphoma
(chronic inflammation)
NHL (non Hodgkin lymphoma):
-Divide into small, intermediate, large B cells: list types
- small B cell: (think well differentiated)
- follicular, mantle cell, marginal zone, small lymphocytic (CLL) - Intermediate sized B cells
- Burkitt lymphoma - Large B cells
- diffuse large B-cell lymphoma
Painless lymphadenopathy
-differential?
- chronic inflammation (eg autoimmune)
- metastatic carcinoma
- lymphoma
Mantle cell lymphoma
-genetics, mech
- t(11:14)
- 11 has Cyclin D1, which promotes G1/S transition in cell cycle by phosphorylating proteins
What subtype of Hodgkin lymphoma is assoc with HIV and the elderly?
- lymphocyte poor Hodgkin lymphoma
- worst prognosis of all subtypes (most aggressive)
Cyclin D1:
-assoc with what WBC disorder
- Mantle zone lymphoma
- 11:14 translocation– Cyclin D1 allows cell to advance G1 to S
Diffuse large B Cell lymphoma
- how does it present?
- population
-late adulthood, presents as enlarging lymph node, or an extranodal mass
Lymphoma
- definition
- where can they occur
- neoplastic proliferation of lymphoid cells that forms a mass
- can be in lymph node, or extranodal tissue
Burkitt lymphoma
- populations
- classic presentations (2)
Extranodal mass in child or young adult.
- African form–in the jaw
- Sporadic form–in the abdomen
Nodular sclerosis subtype of Hodgkin lymphoma
- classic presentation
- population
-enlarging cervical or mediastinal lymph node in a young female adult
Burkitt lymphoma
-appearance in histology
-“starry sky” appearance–majority of B cells are dark (sky), with tingible body macrophages eating the rapidly dying ones (stars)
(HL) Hodgkin Lymphoma
-Subtypes
- most common?
- best prognosis?
-worst prognosis?
- Nodular sclerosis (70%)
- lymphocyte rich (best prognosis)
- mixed cellularity (assoc with IL5 eosinophils)
- lymphocyte poor (worst prognosis)
Diffuse large B Cell lymphoma
-proliferation of what?
- large B-cells, CD20+
- diffuse growth, high grade, very aggressive
Burkitt lymphoma
-genetic mutation, mech
- 8:14 translocation with C-MYC
- C-MYC is a TF that stimulates cell growth
Mantle cell lymphoma
- where is the mantle zone
- how does this typically present
- mantle zone surrounds the follicle. The marginal zone surrounds the mantle zone
- Presents as painless LAD in late adulthood
Diffuse large B Cell lymphoma
-2 different ways it forms
- sporadically
- transformation from a low-grade lymphoma (eg follicular lymphoma)
(HL) Hodgkin Lymphoma
which subtype is assoc with abundant Eosinophils?
-mixed cellularity type, with RS cells producing IL-5
Follicular lymphoma
-How does it initially present, in what population?
-Typically presents in late adulthood, with painless LAD
Marginal Zone Lymphoma
-assoc with what disorders (3)
- 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
Viral infections–cause hyperplasia of what lymph node region?
-Paracortex–where T cells live.
T cells are defense against viruses
Sjogren syndrome
-assoc with what WBC disorder
Marginal zone lymphoma
(chronic inflammation)
Follicular lymphoma
-proliferation of what cells, with what markers present
-Small B cells (CD20+), proliferation forms follicle-like nodules