Lymphoma- Weir Flashcards
Human T-cell leukemia virus-1 (HTLV-1) is associated with what WBC malignancy?
Adult T-cell leukemia/lymphoma
EBV is associate with what WBC malignancies?
Burkitt lymphoma, 30-40% of Hodgkin lymphoma, many B cell lymphomas arising in the setting of T cell immunodeficiency, and rare NK cell lymphomas.
Kaposi sarcoma herpesvirus/human herpesvirus-8 (KSHV/HHV-8) is associated with which WBC malignancy?
An unusual B-cell lymphoma that presents as a malignant effusion, often in the pleural cavity.
What influence does smoking have on the incidence of acute myeloid leukemia?
increased 1.3-2 fold, presumably because of exposure to carcinogens, such as benzene
All lymphomas are broken into one of two categories based on distinctive pathologic features and treatment:
Hodgkin lymphoma (HL) and non-hodgkin lymphomas (NHLs)
Plasma cell neoplasms most often arise in this tissue and only infrequently involve lymph nodes or the peripheral blood:
bone marrow
2/3rds of all NHLs and virtually all HLs present as this, clinically.
enlarged, non-tender lymph nodes (often >2cm)
What is the most common plasma cell neoplasm and how does it most often present?
multiple myeloma; causes bony destruction of the skeleton and often presents with pain due to pathologic fractures
The lymphoid neoplasms can be sorted into these 5 broad categories based on cell of origin:
1) precursor B-cell neoplasms (neoplasms of immature B cells)
2) peripheral B-cell neoplasms (neoplasms of mature B cells)
3) precursor T-cell neoplasms (neoplasms of immature T-cells)
4) peripheral T-cell and NK-cell neoplasms (neoplasms of mature T cells and NK cells)
5) Hodgkin lymphoma (neoplasms of Reed-Sternberg cells and variants)
The vast majority (85-90%) of lymphoid neoplasms are of __-cell origin.
B-cell
Are NK-cell tumors common?
No, very rare
Lymphoma staging provides generally useful prognostic information for all lymphomas, but due to the orderly spread of this particular lymphoma (sometimes restricted to one group of lymph nodes), staging is of most utility in guiding therapy in treating ________ lymphoma.
Hodgkin
What stage is a lymphoma pt with lymph nodes in two locations on one side of the diaphragm?
Stage II
What is the most common form of adult leukemia in the Western world?
CLL
Which of the following is commonly associated with Burkitt's lymphoma? HIV EBV HHV8 HTLV1 H. pylori
EBV
Which is true if a pt has an indolent (does not bother pt) lymphoma?
More likely cured than aggressive lymphoma
Must approach with aggressive therapy
May be watched until it bothers the pt
Usually localized
May be watched until it bothers the pt.
Indolent lymphomas are more likely to be higher stage.
Treat them when they are symptomatic because they are incurable except @ stages I-II.
Survival is independent of early treatment.
What is the most common indolent lymphoma?
Name the translocation associated.
follicular lymphoma
t(14:18) bcl-2 upregulated (anti-apoptosis)
What is going on (chromosomally) in small lymohocytic/CLL?
17p deletion results in p53 loss and thus loss of control of cell cycle progression.
Mycosis fungoides are associated with what tissues?
Gastic, mucosa around eye, mucosa of lung.
It is a T-cell cutaneous lymphoma
In lymphomas, are aggressive (high grade) tumors more often found at lower or higher stages?
Low stages
Are aggressive lymphomas very curable?
Yes. Compared to indolent that are not often curable. With appropriate treatment (complex treatment) they can be cured when attacked early on.
Describe the lymphoma staging system.
Stage 1: single node or lymphoid structure
Stage 2: two or more lymph regions on ONE side of the diaphragm
Stage 3: Both sides of diaphragm involved
Stage IV: Extranodal (cancer has left lymph system)
What is the most common lymphoma in the US?
Diffuse large B cell (37%) followed by follicular (29%)
What are the treatments of indolent lymphomas?
Local irradiation Alkylators and prednisone Anthracyclines Fludarabine Ab therapy Radiation labeled Abs Combination therapy: CHOP ( ); fludarabine + mitoxantrone
What are the treatments of aggressive lymphomas?
Combination chemos: rCHOP (cyclophosphamide, Hydroxydaunorubicin, vincristine, prednisone + rituximab); HyperCVAD Ab therapy plus CHOP Radio-immunotherapy Short course chemo + radiation Bone marrow transplant for relapse
How does rituximab work to fight cancer?
It is a mAb for CD20. Binds to CD20 on B cells and makes them a target for phagocytosis by macrophages or killing my NK cells. Also initiates complement cascade for same effect.
Alemtuzumab is a targeted therapy mAb for:
CD52
brentuximab vedotin is a targeted therapy mAB for:
CD30
ibrutinib is a targeted therapy inhibitor of:
Bruton Tyrosine Kinase
Which of the following is commonly associated with lymphoma after transplants? HIV EBV HHV8 HTLV1 H. pylori
EBV
What infections are associated with lymphoma?
HIV EBV HHV8 HTLV1 H. pylori
What is the most common cutaneous lymphoma?
Mycosis fungoides
You see abnormal cells in the peripheral blood that have characteristic, cerebriform, large, and clefted nuclei with fine chromatin pattern and scanty cytoplasm. What are you tihinking?
Sezary syndrome. A type of lymphoma
Hodgkin disease is derived from a B or T cell?
B cell
If you see smudge cells, what should you be thinking?
CLL
Describe the Rai staging system for CLL.
0= lymphocytosis 1= enlarged lymph nodes 2= hepatosplenomegaly 3= Hb < 10 (non-immune) 4= platelets <100 K/uL (non-immune)
17p deletions and TP53 mutations are associated with which malignancies of the blood?
multiple myeloma and CLL
Unmutated VH (variable heavy chain) genes are a bad prognostic indicator for CLL. Why is that?
It means the cells have not differentiated.
Poor prognostic indicators (prognosis is poor) of CLL are:
High stage Increased rate of lymphocyte doubling time Beta 2 microglobulin Deletion of 17p, TP53 mutations Unmutated VH genes
How do you treat CLL?
Alkylators: chlorambucil, cyclophosphamide, bendamustine
Purine analogues: Fludarabine
Chemotherapy combos: FCR
Immunotherapy: rituximab (CD20- ofatumumab is better), alemtuxumab (CD52)
ibrutinib (Bruton’s tyrosine kinase), Idelalisib (PI3Kinase delta inhibitor)
Radiation
Corticosteroids
When do you start treating hairy cell leukemia (it is an indolent lymphoma)?
When the pt becomes cytopenic
How do you treat hairy cell leukemia?
2-chlorodeoxyadenosine and deoxycoformycin
What are 2 complications of CLL?
Frequent infections.
Progression to Diffuse large B-cell lymphoma
