Heme - Non-Hodgkin Lymphoma (NHL) - Rad Onc Clinical Review Questions with Correct Answers Latest Update

Heme - Non-Hodgkin Lymphoma (NHL) - Rad Onc Clinical Review Questions with Correct Answers Latest Update What is the pathologic definition of non-Hodgkin lymphoma (NHL)? - Answers NHL is a monoclonal expansion of malignant B or T cells that lacks the pathologic characteristics of Hodgkin disease (HD) (no RS cells) and is typically characterized by nodal/focal involvement vs. the more disseminated presentation of leukemias. How does the clinical presentation of NHL differ from that of HD? - Answers NHL typically involves more nodes at presentation, is more likely to be extranodal, is more likely to spread in a noncontiguous fashion, and has a prognosis that is more strongly affected by histologic subtype than HD. What are the most common presenting signs or Sx of NHL? - Answers Painless adenopathy (axillary, inguinal, femoral) is the most common presenting sign of NHL. ∼30% of pts have B Sx. Waxing and waning adenopathy suggests an indolent form of NHL. Tumor bulk may cause intestinal obstruction, urinary tract obstruction, or nerve compression. What are B Sx? - Answers B Sx include fever >380C, >10% body weight loss in 6 mos, or drenching night sweats. What is the NCI working formulation for NHL? - Answers The working formulation groups NHL by clinical aggressiveness or grade with subgroups based on cell type or presentation; i.e. Low-, Intermediate- and High-grade NHL. Which histologies constitute low-grade NHL? - Answers Low-grade NHL: follicular (grades 1-2), chronic lymphocytic leukemia (CLL), MALT, mycosis fungoides Which histologies constitute intermediate-grade NHL? - Answers Intermediate-grade NHL: follicular (grade 3), mantle cell, diffuse large B-cell lymphoma (DLBCL), T/natural killer (NK) cell, peripheral T cell, anaplastic large cell Which histologies constitute high-grade NHL? - Answers High-grade NHL: Burkitt, lymphoblastic What is the WHO classification of NHL? - Answers The WHO classification divides NHL into B- and T-cell/NK cell neoplasms. The indolent, aggressive, and highly aggressive subgroups roughly correlate to the NCI working formulation groups. Is there a relationship between clinical aggressiveness and curability of NHL? - Answers Advanced-stage indolent NHL is rarely curable. Intermediate-grade NHL may be curable even in advanced stages. Without Tx, what is the life expectancy for pts with NHL of varying aggressiveness? - Answers Pts with indolent NHL have survival measured in yrs. Pts with aggressive NHL have survival measured in mos, and those pts with highly aggressive Dz have an expected survival of wks. What % of NHL is indolent, and what are the most prevalent subtypes? - Answers ∼35% of NHL is indolent by the WHO classification. 95% of indolent NHL are follicular lymphoma (FL) (grades 1-2; 65%), small lymphocytic lymphoma/chronic lymphocytic lymphoma (SLL/CLL) (18%), and marginal zone B-cell lymphoma or MALT lymphoma (12%). Which common cytogenetic abnormalities are associated with Follicular Lymphomas? - Answers t(14;18) is seen in 90% of FLs. This results in overexpression of antiapoptotic Bcl-2. Which common cytogenetic abnormalities are associated with SLL and CLL? - Answers Trisomy 12, Chromosome 13 deletion, and t(14;19) are associated with SLL and CLL. (Think 12, 13, 14, 19) Which common cytogenetic abnormalities are associated with MALT lymphoma? - Answers Trisomy 3 (60%) and t(11:18) (25%-40%) are associated with MALT lymphoma. How is FL graded? - Answers FL demonstrates a mix of centrocytes (small, cleaved cells) and centroblasts (large, noncleaved cells). Grade correlates to the density of centroblasts (e.g., 0-5 centroblasts/high-power field (hpf), grade 1; >15 centroblasts/hpf, grade 3a). What is SLL? - Answers Small lymphocytic lymphoma (SLL) is the same Dz entity as CLL but with a predominant manifestation in the spleen, liver, or nodes as opposed to peripheral blood or BM. What is Richter syndrome? What is its rate of occurrence? - Answers Richter syndrome is the transformation of SLL or CLL into DLBCL. It occurs in roughly 5% of cases. How is bulky mediastinal Dz commonly defined? - Answers Bulky mediastinal Dz is commonly defined as a mass greater than one third of the intrathoracic diameter at T5-6 on upright PA film. How is bulky Dz defined outside of the mediastinum? - Answers Outside of the mediastinum, bulky Dz is variably defined in clinical trials but most often is either any mass >5 cm or any mass >10 cm What are the pertinent focused aspects of the physical exam in a person with suspected NHL? - Answers The physical exam should include complete nodal assessment including epitrochlear and popliteal groups. Cervical adenopathy palpable above the hyoid bone should prompt an ENT exam. (The Waldeyer ring is more frequently involved in NHL than in HD.) Exam of extranodal at-risk sites including the liver, spleen, testicles, bones, abdomen, and flanks is appropriate. What lab studies should be performed? - Answers Laboratory studies should include: CBC with differential, CMP, LDH, β2-microglobulin, serum protein electrophoresis, HIV, hepatitis B virus (essential as it may reactivate with rituximab Tx), and hepatitis C virus. BM Bx should be performed for all lymphomas. LP should be performed for CNS Sx, testicular or paranasal sinus involvement, or immunodeficiency. What imaging studies should be performed? - Answers The imaging workup should include CT C/A/P. PET is appropriate in most cases. MRI brain should be performed for CNS Sx, testicular or paranasal sinus involvement, or immunodeficiency. How is NHL staged? - Answers NHL is staged similar to HD using the Ann Arbor (AA) system: Note: Pts without B Sx are designated with an A, otherwise with a B. Pts with splenic involvement are designated with an S. Describe Stage I NHL - Answers Stage 1: involvement of 1 LN region or localized involvement of