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Overview
Concepts, classification, biology Epidemiology Clinical presentation Diagnosis Staging Three important types of lymphoma
Conceptualizing lymphoma
neoplasms of lymphoid origin, typically causing lymphadenopathy leukemia vs lymphoma lymphomas as clonal expansions of cells at certain developmental stages
ALL
CLL
nave
Lymphomas
MM
Lymphoid progenitor
AML
Hematopoietic stem cell Myeloid progenitor
Myeloproliferative disorders
Neutrophils Eosinophils Basophils Monocytes Platelets
Red cells
B-cell development
stem cell lymphoid progenitor
progenitor-B
CLL
memory B-cell
MM DLBCL, FL, HL
ALL
pre-B immature B-cell plasma cell
Classification
Biologically rational classification
Diseases that have distinct morphology immunophenotype genetic features clinical features
Lymphoma classification
(2001 WHO)
B-cell neoplasms
precursor mature
NonHodgkin Lymphomas
Hodgkin lymphoma
NonHodgkin lymphoma
Indolent
Aggressive
Months
Curable in some
Curable in some Curable in most
Treat
Weeks
Treat
Treat
Mechanisms of lymphomagenesis
Genetic alterations Infection Antigen stimulation Immunosuppression
Epidemiology of lymphomas
5th most frequently diagnosed cancer in both sexes males > females incidence
NHL increasing Hodgkin lymphoma stable
2000
Incidence/100,000/annum
100 20 40 60 80 0
Age (years)
0-1 1-4 5-9 10-14 15-19 20-24 25-29 30-34 35-39 40-44 45-49 50-54 55-59 60-64 65-69 70-74 75-79 80-84 85+
incidence/100,000/annum
0 1 2 3 4 5 6
Age (years)
0-1 1-4 5-9 10-14 15-19 20-24 25-29 30-34 35-39 40-44 45-49 50-54 55-59 60-64 65-69 70-74 75-79 80-84 85+
Clinical manifestations
Variable
severity: asymptomatic to extremely ill time course: evolution over weeks, months, or years
Systemic manifestations
fever, night sweats, weight loss, anorexia, pruritis
Local manifestations
lymphadenopathy, splenomegaly most common any tissue potentially can be infiltrated
Staging of lymphoma
Stage I Stage II Stage III Stage IV
Follicular lymphoma
most common type of indolent lymphoma usually widespread at presentation often asymptomatic not curable (some exceptions) associated with BCL-2 gene rearrangement [t(14;18)] cell of origin: germinal center B-cell
defer treatment if asymptomatic (watch-and-wait) several chemotherapy options if symptomatic median survival: years despite indolent label, morbidity and mortality can be considerable transformation to aggressive lymphoma can occur
Hodgkin lymphoma
Hodgkin lymphoma
cell of origin: germinal centre B-cell Reed-Sternberg cells (or RS variants) in the affected tissues most cells in affected lymph node are polyclonal reactive lymphoid cells, not neoplastic cells
Reed-Sternberg cell
classic RS cell
(mixed cellularity)
lacunar cell
(nodular sclerosis)
popcorn cell
(lymphocyte predominance)
cytokines
Epidemiology
less frequent than non-Hodgkin lymphoma overall M>F peak incidence in 3rd decade
Clinical manifestations:
lymphadenopathy contiguous spread extranodal sites relatively uncommon except in advanced disease B symptoms
I,II
III,IV
60-70%
70-80%
secondary malignancy
skin, AML, lung, MDS, NHL, thyroid, breast...
cardiac disease
Overview
Concepts, classification, biology Epidemiology Clinical presentation Diagnosis Staging Three important types of lymphoma