non-hodgkins lymphoma pathology

what are the 2 kinds of non-hodgkins lymphomas?
B and T cell
what is the difference between leukemias and lymphomas?
leukemias are typically abnormal WBCs, produced in the bone marrow and circulating in peripheral blood. lymphomas are aggregates of neoplastic lymphocytes that form a mass
what is the precursor for lymphoblastic leukemia?
B cells
what is the precursor for lymphoblastic lymphoma?
T cell lineage
when are mature (peripheral) B cell lymphomas seen?
these are most common in elderly men, and often involve bone marrow and peripheral blood. these are IDed by CD20 which are markers for small B cells
what are some examples of mature B cell neoplasms?
CLL/SLL, prolymphocytic leukemia, hairy cell leukemia, lymphoplasmacytic lymphoma, marginal zone lymphoma, follicular lymphoma and mantle cell lymphoma
what is CLL/SLL? what are some characteristics?
this is a the most common leukemia, (CLL/SLL are essentially the same), it is rare under 40 and generally indolent (depending on stage). it is marked by hypogammaglobulinemia and increased infections.
what is the growth center of leukemia?
where the cells grow out and progress to mature B cells
what is a marginal zone lymphoma? what are the 3 different kinds?
extra nodal MZLs are mature B cell lymphomas that arise in post-germinal center (pale area in lymph node/memory zones) memory B cells that circulate, causing: MALTomas (often in the GI) that may undergo neoplastic change, splenic and nodal marginal zone lymphomas
how can helicobacter pylori cause it a MALToma in the stomach?
this is a extranodal marginal zone lymphoma that may arise from *helicobacter pylori* due to chronic inflammation (lymphocytes might undergo neoplastic change and make further clones). tx of helicobacter in this case will also help in tx of the lymphoma
how can hashimoto’s thyroitis and sjogren’s syndrome cause extra nodal marginal zone lymphomas/MALTomas? who does this more often occur to?
hashimotos’s thyroiditis (autoimmune disease where thyroid is almost completely replaced by lymphocytes, looks like a lymph node), and sjogren’s syndrome (infiltration of lymphocytes in the lacrimal gland, salivary gland – usually presents with dry mouth and dry eyes) can lead to lack of tolerance, chronic inflammation and aggregation of lymphocytes can lead to extra nodal marginal zone lymphomas/MALTomas that may be present in the breast, eye and skin. these autoimmune-associated lymphomas do have a slight female preponderance
what are some characteristics of a splenic marginal zone lymphoma?
the cells may have villous projections that project microscopically, there is usually bone marrow/blood involvement (leukemiaish), it has an indolent course, it is usually seen in older females, and is associated with a *deletion in chr 7q21 which can cause the lymphocyte to constantly divide or protect it from apoptosis (lymphocyte sticks around and the more lymphocytes divide the greater the chance of developing a neoplasm)
what is a nodal marginal zone lymphoma?
these marginal zone lymphomas have a monocytoid B cell component, often have *neutrophil infiltration* (not seen in many lymphomas, may be related to acute inflammation), and are more aggressive than MALTomas
what can cause a follicular lymphoma (a prominent kind of lymphoma)?
follicular lymphomas are associated with t(12:8) translocations and bcl-2 gene (antiapoptotic) mutations. TP53 is inactivated and P38/MAPK is deleted. *C-MYC is also activated (growth factor)
how are follicular lymphomas categorized (graded)?
size and architecture: low grade: follicular small cell and higher grade: diffuse large cell
what characterizes mantle cell lymphomas?
these are often seen in middle aged/elderly men and the cells resemble the normal mantle zone B cells that surround the germinal centers. there is often extranodal involvement (lymphoid aggregates throughout other portions of the body) and it is an aggressive small cell lymphoma with poor response to chemotherapy (large cells are rare and median survival is 3-4 yrs)
what are factors that can lead to and potentiate mantle cell lymphoma?
cyclin D can be overexpressed due to t(11:14), which can lead to Rb/p27 suppressor effects being overcome -> continues to be pushed into the cell cycle
what is one of the most common forms of lymphoma?
large B cell lymphomas, seen most commonly in 60 yr olds (but broad age range), these are rapidly growing/usually symptomatic earlier and mass at a single site (either nodal/extranodal). these are aggressive, but are *potentially curable. they have prominent vesicular nuclei (bubbly) and basophilic cytoplasm
what are morphologic variants of large B cell lymphoma?
large B cell lymphomas can be centroblatic, immunoblastic, T cell/histiocyte rich, anaplastic large B cell, lymphomatoid granulomatosis type and plasmablastic
what does it mean to day that LBCL cells are centroblastic?
these are large, cleaved and non-cleaved cells that mke up the diffuse counterpart of nodular follicular lymphoma, these cells are usually more *aggressive
how do immunoblastic cells appear in LBCL?
immunoblastic cells have large vesicular nuceli, prominent nucleoli, and thick nuclear membranes. a nuclear hoff can usually be seen in plasma cells (pale cytoplasm around the nucleus) and intracytoplasmic Ig may also be visible. these are seen associated with immunodeficiency, immunosuppression, and immune mediated diseases (hashimotos/sjogrens/SLE/AIDS)
what characterizes T cell/histiocyte rich LBCL?
LBCL is still an overall B cell neoplasm, but it can have a T cell population w/in it. there maybe histiocytes (macrophages in tissue) and a *diffuse growth pattern w/fine interstitial fibrosis
how do anaplastic cells present in LBCL?
this rare variant can result in large bizarre tumor cells
what are the clinical presentations of LBCL?
primary mediastinal (thymic) B cell lymphoma can present with respiratory problems and blood flow (superior vena cava syndrome – swelling in head/neck). primary CNS lymphomas are often associated with HIV, seizures and mental status changes. primary effusion lymphomas can erupt in body cavities.
who are primary mediastinal (thymic) LBCLs seen in? what are they characterized by?
primary mediastinal (thymic) LBCLs are commonly seen in women in their 30’s presenting with superior vena cava syndrome, airway obstruction, local invasion and *multilobated cells (bubbly cells (not small dense lymphocytes))
when is primary CNS lymphoma usually seen?
primary CNS lymphomas are often associated with (late) HIV and are frequently associated with EBV
when are primary effusion lymphomas often seen?
primary effusion lymphomas are usualy associated with HIV and are caused by HHV-8 (many oncogenic DNA viruses play a role once your immune system has been decreased due to a virus, such as HIV)
what do mature B cells express in burkitt lymphoma? what virus is it associated with? what presentation of burkitt does the endemic virus cause?
burkitt lymphoma mature B cells express CD10 and BCL6. it results from *c-myc rearrangements due to a *t(8:14). endemic EBV causes jaw/facial bone burkitt cell lymphoma.
how does a burkitt lymphoma appear histologically?
“starry sky” lymphocytes are part of the dark sky and the macrophages (to clean up dead cells that couldn’t keep up with the rapid growth) are the stars. (homogeneous medium sized cells, rounded nuclei, multiple nuclei, basophilic (dark) cytoplasm) all of these cells are marked by rapid turnover, proliferation and apoptosis
how do peripheral T and B cell lymphomas compare?
T cell lymphomas are less common but usually more aggressive (variable prognosis). they have a high incidence of bone marrow involvement and can include small lymphocytic, mixed cell and large cell/immunoblastic types.
what lymphoma is associated with skin lesions and a virus and is more common in japan?
adult T cell lymphoma/leukemia which is also associated with hypercalcemia, lytic bone lesions (osteoclastic activity), is aggressive and **associated with HTLV 1 (a retrovirus)
how does adult T cell lymphoma appear histologically?
the T cells have bubbly/foamy cytoplasm, and have prominent nuclei (perhaps 2)
who is anaplastic large cell lymphoma more common in? what antigen might it express?
anaplastic large cell lymphoma is more common in **young adults, it is involved in the lymph nodes and marrow and is aggressive. anaplastic large cell lymphoma is responstive to chemotherapy and may express **antigen ALK1
how does angioimmunoblastic T cell lymphoma present?
with advanced disease, usually 50-70% bone marrow involvement, systemic symptoms and prominent vascularity. it is composed of a mixture of plasma cells, lymphocytes and eosinophils. it is aggressive.
how does mycosis fungiodes (MF) appear?
this affects adults, and features multiple skin lesions with papillary dermal fibrosis and pautrier’s microabscesses (small area of neutrophil infiltration). the cells have cerebriform nuclei (brain-like appearance). this has an indolent by progressive course and is related to sezary syndrome.
what is sezary syndrome?
like MF but with generalized adenopathy and cerebriform cells in circulation. it has an unfavorable prognosis
what lymphoma is associated with hepatosplenomegaly? is this associated with a chromosomal defect? who does this affect disproportionately? what cell type predominates/can it affect other organs?
hepatosplenic T-cell lymphoma which is associated with a trisomy of chromosome 8 and seen more commonly in young black males. it is usually composed of monotonous small to medium sized cells, is aggressive and can infiltrate the liver, spleen and marrow
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