Hodgkin and Non-Hodgkin Lymphoma Flashcards
Lymphoma and corresponding developmental stage.
Acute lymphoblastic leukemia – immature B-cell Mantle cell lymphoma – mantle cell
Follicular lymphoma – germinal center B-cell Hodgkin lymphoma – germinal center B-cell Burkitt lymphoma – germinal center B-cell CLL/SLL – mature B-cells of pre- or post-GC stage Plasma cell myeloma – plasma cell
Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) Definitions
CLL = lymphocytosis of ≥5 × 109/L for at least 3 months, Monoclonal B cell with mature immunophenotype positive for CD5, weak CD20, weak surface immunoglobulin, CD23, weak CD22, and weak CD11c Typically negative for CD10, FMC7, and CD79b
SLL = Extramedullary sites of disease predominate,
Diffuse infiltrate of small lymphocytes,
and Monoclonal B cell with mature phenotype similar to CLL
CLL/SLL Clinicla features
CLL is the most common leukemia in the Western world and accounts for ~ 30% of all leukemia
SLL accounts for ~7% of Non-Hodgkin lymphoma (NHL)
Median 65 years, male predominance, M:F=2:1
Most elderly patients with CLL are either asymptomatic (70% of cases) or only
mildly symptomatic, whereas some patients may present with fatigue, infection, autoimmune hemolytic anemia, hepatosplenomegaly, lymphadenopathy, or extranodal involvement.
CLL/SLL Morphologic features
SEE HANDOUT.
Lymphocytosis!!!
CLL/SLL immunophenotype
Positive: CD5, CD23, CD19
Weak: CD20, surface immunoglobulin, CD22, CD11c
Negative: CD10, FMC7
CLL/SLL common genetic findings
Deletion of 13q14 (50% of cases and favorable diagnosis)
Trisomy 12 in <20% of cases.
Follicular Lymphoma (FL) Definitions
A lymphoma of germinal center B cells (centrocytes and centroblasts) with typically at least a partially follicular pattern
FL clinical features
requency: 40% of adult lymphomas in US, 20% worldwide
Age: mostly adults, median 60 years
Gender: male = female
Locations: mostly lymph nodes, also spleen, bone marrow, Waldeyer’s ring, GI tract,
skin and soft tissue
Clinical presentations: more than 80% cases have widespread stage III or IV disease
at diagnosis, 40% have bone marrow involvement; however, patients often
asymptomatic except for lymphadenopathy
FL morphologic features
Closely packed follices, extranodal or intramedullary involvement, Follicals with scant or absence mantle of zone.
Centrocytes and Centroblasts are the tumor cells.
FL immunophenotype
B-cell markets positive: CD_19, CD20+, BCL2+ psitive, Germinal center B-cell marker positive CD10 and BCL6.
FL cytogenetic findings
75-90% of cases have translocations of t(14:18)(q32:q21)
Mantel Cell Lymphoma Definition
A B-cell neoplasm composed of monomorphic small to medium-sized lymphocytes with irregular nuclei that morphologically resemble centrocytes but often have slightly less irregular nuclear contours. The tumor cells typically express B-cell markers (CD19 and CD20) and CD5, and do not express CD23. This neoplasm is genetically characterized by BCL1 gene rearrangement at 11q13, leading to a constant overexpression of cyclin D1, which plays an important pathogenetic role in tumor development.
MCL clinical features
MCL comprise approximately 3-10% of NHL
Age: middle to older age, median age 60 years; M:F=2:1
Location: mostly lymph nodes, also spleen and bone marrow; most common
extranodal sites, gastrointestinal tract and Waldeyer’s ring
Clinical presentations: most patients present with stage III or IV with
lymphadenopathy, hepatosplenomegaly; >50% of cases with massive splenomegaly and marrow involvement; >25% cases with peripheral blood involvement that can mimic prolymphocytic leukemia
MCL Immunophenotype
B-cell markers positive: CD19+, CD20+
MCL Cytopgenetic finding
t(11:14)(q13;q32) involving BCL1 gene
MCL clinical behavior
in contrast to other small b-cell lymphoma that are mostly indolent, MCL is usually moderatly aggressive.
Burkitts Lymphoma (BL) definition
A highly aggressive B-cell lymphoma, often presenting at extranodal sites or as a leukemic form. The tumor is typically composed of monomorphic, medium-sized B cells with basophilic cytoplasm and a high mitotic rate. Translocations involving the MYC oncogene on chromosome 8 at band 8q24 are a constant feature
BL clinical features
Epidemiology:
o Endemic BL: typically in the malaria belt of equatorial Africa, as the most
common childhood malignancy in this region, peak 4-7 years of age, involves
jaw or abdomen, 95% EBV positive.
o Sporadic BL: mostly in children or young adults. 30% of childhood
lymphomas
o Immunodeficiency-associated BL: primarily in HIV patients
Locations:
o Endemic: jaw and other facial bones in 50% cases, also distal ileum, cecum
and omentum
o Sporadic: mostly in ileocecal area o Immunodeficiency-associated: HIV
May present in blood and diffuse marrow involvement at diagnosis
BL cytogenetic findinds
Most cases have a characteristic translocation, t(8;14)(q24;q32), which juxtaposes the MYC gene at 8q24 next to IGH@ at 14q32
BL clinical behavior
Highly aggressive but potentially curable. Curable with aggressive therapy in ~60% cases.
Plasma Cell Neoplasms Definition
A clonal proliferation of immunoglobulin-producing plasma cells that ecrete a single class of immunoglobulin or a polypeptide subunit of a single immunoglobulin, which is usually detectable as a monoclonal protein (M protein) on serum or urine protein electrophoresis. Most plasma cell neoplasms originate as bone marrow tumors, but they occasionally present in extramedullary sites.
Plasma Cell myeloma
Definition: a bone marrow–based, multifocal plasma cell neoplasm associated with an M protein in serum or urine. PCM originates from the marrow with disseminated marrow involvement in most cases. Other organs may be secondarily involved. The diagnosis of myeloma is made by a combination of clinical, morphologic, immunologic, and radiographic information. The disease spans a clinical spectrum from asymptomatic to highly aggressive disease.
Hodgkin Lymphoma (HL)
Classification: the WHO classification has recognized two major types of Hodgkin lymphoma, the nodular lymphocyte-predominant subtype of Hodgkin’s lymphoma (NLPHL) and the classical Hodgkin’s lymphomas (CHLs), which is further classified into four subtypes.
Nodular lymphocyte-predominant Hodgkin’s lymphoma Classical Hodgkin’s lymphoma
o Nodular sclerosis classical Hodgkin’s lymphoma
o Lymphocyte-rich classical Hodgkin’s lymphoma
o Mixed cellularity classical Hodgkin’s lymphoma
o Lymphocyte-depleted classical Hodgkin’s lymphoma
