Flashcards in Chapter 17: Non-Hodgkin's lymphoma Deck (53):
What is the primary sign of Non-hodgkin's lymphoma?
superficial, asymmetric, painless lymphadenopathy.
What are the constitutional symptoms of NHL?
(2) night sweats
(3) weight loss
They are commonly associated with disseminated disease.
What are often the presenting features of NHL?
(2) Neutropenia (leading to infections)
(3) Thrombocytopenia (leads to bleeding/purpura)
What are some gross pathological signs of NHL?
(3) Skin involvement (mycoses fungoides, and Sezary syndrome)
Generally speaking how are NHLs identified?
Lymph node biopsy with morphological, immunophenotypic, and genetic investigation.
How does light chain clonality impact the diagnosis of NHL?
Polyclonal light chains are indicative of chronic inflammation. Monoclonal light chains are indicative of malignancy.
What are the hematological findings associated with NHL?
(1) Normochromic, Normocytic anemia
(2) Autoimmune hemolytic anemia
(3) Neutropenia (advanced disease)
What are the biochemical findings associated with NHL?
(1) increased LDH
(2) Increased uric acid.
(3) paraprotein on Ig electrophoresis.
What translocation is associated with Burkitt's lymphoma?
t(8; 14) Burkitt's lymphoma
What translocation is associated with follicular lymphoma?
What translocation is associated with Mantle cell lymphoma?
What translocation is associated with anaplastic large cell lymphoma?
What methods are used to stage NHLs?
(1) imaging (CT, XR, MRI, PET)
(2) Biopsy, BM aspiration, Trephine biopsy.
What are the low grade NHLs?
(1) Follicular lymphoma
(2) Lymphocytic lymphomas
(3) Lymphoplasmacytoid lymphomas
(4) Mantle cell lymphoma
(5) Marginal zone lymphoma
What is the most common form of NHL?
What is the pathogenesis of follicular lymphoma?
B cells undergo malignant transformation by the t(14; 18) translocation, which causes the constitutive activation of the Bcl-2 gene.
What is the general age range for follicular lymphoma?
Middle aged to elderly
What is the median survival from diagnosis for follicular lymphoma?
What is the presentation of follicular lymphoma?
Painless lymphadenopathy with most patients presenting in stage III or IV.
What is the most effective chemotherapy against follicular lymphoma?
CVP (chlorambucil/cyclophosphamide, vincristine, and prednisolone.) achieves a response in up to 90% of patients.
If chemotherapy is not effective enough what alternative therapies can be considered for follicular lymphoma?
(2) Autologous stem cell transplant
(3) Allogenic stem cell transplant ( in younger patients)
What are lymphocytic lymphomas?
Lymphocytic lymphomas are considered a tissue phase of CLL and are treated as such.
What are lymphoplasmacytoid lymphomas?
Lymphomas associated with the production of monoclonal immunoglobulin M. Also called Waldenstroms macroglobulinemia.
What are the complications associated with Lymphoplasmacytoid lymphomas?
Anemia and hyperviscosity.
How is Lymphoplasmacytoid lymphoma treated?
Oral chlorambucil, fludarabine, or monoclonal antibodies. Plasma exchange can reduce viscosity.
What is mantle cell lymphoma?
A malignancy derived from pre-germinal B cells located in primary follicles or in the mantles of secondary follicles.
What is the characteristic phenotype of mantle cell lymphoma?
What is the pathogenesis of mantle cell lymphoma?
t(11; 14) translocation that merges Bcl-1 with an Ig heavy chain gene promoter.
What is the clinical presentation of Mantle cell lymphoma?
(2) bone marrow infiltration
(3) tumor cells in the peripheral blood.
What histological findings are associated with mantle cell lymphoma?
Cells with characteristically angular nuclei.
What is the prognosis for mantle cell lymphoma?
Even though it is considered low grade the prognosis for mantle cell lymphoma is poor. The median survival is 3 years post diagnosis.
How is mantle cell lymphoma treated?
CVP or CHOP or fludarabine with rituximab.
What are marginal zone lymphomas?
Lymphomas that are typically extranodal and localized.
What is the pathogenesis of marginal zone lymphomas?
chronic inflammation of the tissue involved.
What is the most common mantle cell lymphoma?
Gastric MALT lymphoma caused by H. pylori infection.
How are marginal zone lymphomas treated?
Most can be treated with chemotherapy and rituximab. Splenectomy can be used for splenic marginal zone lymphoma.
What are the high grade NHLs?
(1) Diffuse large B-cell lymphoma
(2) Burkitt's lymphoma
(3) Lymphoblastic lymphomas.
What are diffuse large b cell lymphomas?
Malignant disorders characterized by rapidly progressive lymphadenopathy due to fast cellular proliferation.
What features of diffuse b cell NHL are associated with a poor prognosis?
(1) High age
(2) More extranodal sites
(3) higher serum LDH
(4) Bulky disease
What features of diffuse B cell NHL are associated with a good prognosis?
(1) germinal center origin
(2) t(3; 27)
How is diffuse large B cell NHL treated?
R-CHOP is the first line therapy. Prophylactic therapy for CNS disease with MTX may also be considered.
What is the prognosis for diffuse large b cell lymphoma?
Long term survival is approximately 65%.
What is Burkitt's lymphoma?
The lymphomatous correlate of L3 ALL.
Where is Burkitt's lymphoma endemic?
Africa and the caribbean where there is chronic malaria exposure. It is associated with EBV infection.
What is the pathogenesis of Burkitt's lymphoma?
t(8; 14) moves the C-MYC oncogene under the control of an Ig heavy chain promoter thus over expressing the C-MYC oncogene.
What chemotherapy regimen is highly effective against Burkitt's lymphoma?
High dose methotrexate and cyclophosphamide.
What is peripheral t cell NHL?
Malignancy derived from post thymic T cells. Treated with CHOP. The prognosis is poor.
What is angioimmunoblastic lymphadenopathy?
It is a T cell malignancy of the elderly associated with
(3) Skin rashes
(4) Polyclonal IgG increase
What is mycosis fungoides?
Mycosis funcoides is a chronic cutaneous T cell lymphoma that presents with
(1) Severe pruritis
(2) psoriasis like lesions
What is Sezary syndrome?
A T cell lymphoma with circulating T-lymphoma cells that presents with
(2) generalized lymphadenopathy
(3) CD4+ cells with folded nuclear chromatin.
What is Adult T cell lymphoma?
A malignancy of T cells that is caused by the HTLV-1. It presents with
(2) cutaneous infiltrations
What are angiocentric lymphomas?
Typically involve the sinuses or gluten induced enteropathy