Flashcards in Lymphomas- Weir Deck (77):
The first 12 slides are from the "I'd like you to know" slide.
What 4 major factors must a doctor consider when he/she decides if cure is possible for a patient with lymphoma?
1. Tumor histology
2. Tumor stage
3. Condition of patient (co-morbidities, etc.)
4. Available therapies
Name the most common indolent lymphoma.
Name the most common aggressive lymphoma.
Diffuse large cell lymphoma
Be able to stage a patient with lymphoma.
1. Stage I: single node or lymphoid structure
2. Stage IE: one site other than a lymph node
3. Stage II: 2+ lymph regions on one side of diaphragm
4. Stage III: both sides of diaphragm
5. Stage IV: extranodal beyond E (sole site of disease)
What are the major treatment decision differences between indolent and aggressive lymphoma?
Indolent: only treat when symptomatic
Aggressive: early therapy required
Understand the mechanism and site of action of Rituximab.
Binds to B cell's CD20 surface antigens.
Understand the mechanism and site of action of Ibrutinib.
Binds to BTK (enzyme essential for B cell development).
What is the name of the most common cutaneous lymphoma and what cell can you find in the blood in some of these patients?
Mycosis fungoides, a T cell lymphoma. If patient has Sezary Syndrome, abnormal cells are found in the peripheral blood with characteristic cerebriform, large, and clefted nuclei with fine chromatin pattern and scant cytoplasm.
What is a common complication of radiation therapy in young Hodgkin's Disease women?
Name 2 complications of CLL.
1. Coomb's + autoimmune hemolytic anemia
2. Infections due to hypogammaglobulinemia
Name 2 poor prognostic chromosome abnormalities in CLL.
1. del(17p): deletion of p53 region or mutation (worst prognostic factor)
2. Unmutated variable heavy chain (VH) genes
When is BM transplantation used in lymphomas?
Aggressive lymphomas that relapse.
End of "I'd like you to know" section.
Masses found in lymphoma can cause what?
2. Ureteral obstruction
3. Spinal cord compression
Replacement of BM in lymphoma causes what?
Reduction in the number of plasma cells in lymphoma causes what?
What 3 tumor products are produced in lymphomas?
1. Uric acid
3. Products of cell lysis
What paraneoplastic syndromes can be associated with lymphomas?
1. Autoimmune hemolytic anemia
2. Idiopathic thrombocytopenia purpura
What are the toxicities of therapy observed in lymphoma patients?
Infections, numbness, and shortness of breath.
What are the 4 WHO classifications of lymphomas?
3. Highly Aggressive
4. B or T Cell
What are the 4 indolent lymphomas?
2. CLL/small lymphocytic
3. MALT lymphomas
4. Mycosis fungoides
What are the 4 aggressive lymphomas?
1. Diffuse (except small lymphocytic)
2. Large cell
3. Mantle cell
4. Peripheral T cell lymphomas (NOS, angioimmunoblastic, anaplastic)
What are the 2 highly aggressive lymphomas?
1. Burkitt's and non-Burkitt's small cleaved
2. T cell lymphoblastic lymphoma
What are the 6 lymphoma staging tests?
1. History and exam
2. CAT scan: chest, abdomen, and pelvis
3. BM aspirate and bx w/ flow cytometry
4. Gallium or PET scan
5. Chemistries, CBC, LDH
6. CSF if high risk disease
If a large node does not light up on PET scan, what does this mean?
The lymph node is not active i.e. dead, necrotic, etc.
Indolent lymphomas are usually found in what stages?
Indolent lymphomas have a high or slow progression?
T or F. Simple therapies work well for indolent lymphomas.
Are indolent lymphomas curable?
Yes, if found in Stage I or II. However, they are not curable in Stages III and IV.
Is it better to treat an indolent lymphoma in the early or late stages?
Doesn't matter. Patient survival is independent of early treatment.
When do you treat indolent lymphomas?
Only when the pt is symptomatic.
How do aggressive lymphomas differ from indolent lymphomas?
Often found in lower stage. Rapid progression. Requires complex therapies. Potentially curable. Early therapy required.
What is the most common lymphoma in the US?
Diffuse large cell lymphoma
What factors in the International Prognostic Index get you a point?
Age >60, Stage III-IV, LDH elevated, Performance status >1, 1+ extranodal sites.
For IPI, what are the 4 categories and their score?
1. Low risk: 0-1
2. Low intermediate risk: 2
3. High intermediate risk: 3
4. High risk: 4
For the Follicular IPI, what factors get you a point?
Age >60, nodal sites >4, LDH elevated, Stage III-IV, Hb <12.
What are the 7 herapies for indolent lymphomas?
1. Local irradiation (indolents very sensitive to radiation)
2. Alkylators and prednisone
5. Ab therapy
6. Radiation-labeled Ab's
7. Combination chemotherapy
What are the 3 alkylators used in indolent lymphoma therapy?
What are the 2 combination chemotherapies used for indolent lymphomas?
1. CHOP: cyclophosphamide, hydroxydaunorubicin, vincristine, prednisone
2. Fludarabine + Mitoxantrone
What are the 5 therapies for aggressive lymphomas?
2. Ab + CHOP (R-CHOP)
4. Short course chemo + radiation
5. BM transplant for relapse
What is R-CHOP?
Rituximab + CHOP
What are the 4 new emerging targeted therapies for lymphomas?
1. Alemtuzumab: anti-CD52
2. Brentuximab vedotin: anti-CD30
3. Ibrutinib: anti-BTK
4. Inotuzumab ozogamicin: anti-CD22
What types of lymphomas does Alemtuzumab treat?
CLL and T cell leukemias resistant to therapy
What types of lymphomas does Brentuximab vedotin treat?
Hodgkin's and CD30+ T cell lymphomas
What has Ibrutinib shown clinically?
Dramatic responses to refractory disease.
What 5 infections are associated with lymphomas?
4. H. Pylori
HIV infection is associated with what?
Primary CNS lymphoma and Kaposi's sarcoma.
HTLV-1 infection is associated with what?
Peripheral T cell lymphoma
EBV infection is associated with what?
Most commonly associated with lymphoma after transplant.
H. Pylori infection is associated with what?
HHV8 is associated with what?
Kaposi's sarcoma and pleural effusion lymphoma
What cell type is seen in Hodgkin's disease?
What are the 4 types of Classical Hodgkin's Disease?
1. Lymphocyte rich
2. Nodular sclerosis
3. Mixed cellularity
4. Lymphocyte depleted
What 3 ways can you treat early stage Hodgkin's?
1. Short course chemo + focal radiation
2. Radiation alone
3. Chemo alone (moving towards this option)
What 2 combination chemotherapies are used to treat late stage Hodgkin's?
1. ABVD (preferred)
What does ABVD consist of?
Adriamycin (doxorubicin/hydroxydaunorubicin), Bleomycin, Vinblastine, Dacarbazine
What does MOPP consist of?
Mustargen, Oncovin (vincristine), Procarbazine, Prednisone
Since Hodgkin's responds so well to therapy, what is the main concern of the disease?
Complications/toxicities from the therapies.
In Hodgkin's, what are the late complications of therapy?
Acute leukemia and myelodysplasia. Solid tumors of breast, lung, stomach, bone, or soft tissues. CAD. Radiation and chemo pneumonitis. Infertility. Hypothyroidism.
Why is ABVD preferred for young women with Hodgkin's?
It only minimally decreases fertility in women.
What type of malignancy is CLL?
Low grade B cell malignancy
What age patients usually have CLL?
Describe symptoms of CLL.
Often asymptomatic. Lymphocytosis, lymphadenopathy, splenomegaly, anemia, autoimmune cytopenias, hypogammaglobulinemia.
Describe the Rai staging of CLL.
1=enlarged lymph nodes
Describe the Binet staging of CLL.
What stages in the Rai and Binet systems of CLL would require early treatment?
What are the 5 poor prognostic indicators for CLL?
1. High stage
2. Increased lymphocyte doubling time
3. B2 microglobulin increased
4. del(17p), TP53 mutations, del(11q)
5. Unmutated VH genes, ZAP-70, CD38
del(17p) results in loss of what?
What are the 5 treatments of CLL?
1. Alkylators: chlorambucil, cyclophosphamide
2. Purine analogues: fludarabine
3. Immunotherapy: rituximab, alemtuzumab
Hairy Cell Leukemia is what type of disorder?
Indolent B cell proliferative disorder
Which gender is more likely to have HCL?
What are the 3 symptoms of HCL?
3. Decreased cell-mediated immunity
What stain is used to show hairy cells in HCL?
TRAP stain (tartrate-resistant acid phosphatase)
HCL shows very high and enduring response rates to which 2 drugs?
What are the 4 types of T cell lymphomas?
1. Mycosis fungoides
4. Adult T cell leukemia/lymphoma