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Path - Overview of Benign + Malignant Lymphoid Proliferations (i) (mantle…
Path - Overview of Benign + Malignant Lymphoid Proliferations (i)
intro
lymphoma = malignancy of lymphocytes (B cells most common)
currently lots of trials for targeted tx (small molecule inhibitors)
presentation
persistent lymphadenopathy + its effects
e.g. SVC obstruction - oedema in head
extra-nodal swelling (e.g. hepatosplenomegaly)
B symptoms
worsen prognosis :cry:
indicate more aggressive tumour
PUO
anaemia
weightloss
night sweats
systemic
must be Dx + graded by histopathologist, staged via imaging (radiologist), clinical exam (clinician) + lab tests (BM trephine)
discussed @ MDM
tx usually chemo (carcinogenic) +/- radio (increases mediastinum carcinoma future risk) +/- targeted tx
never confined to 1 location, always has access to bloodstream
Classification
aims to provide Dx criteria, allow correct Tx, provide prognostic data, allow comparison between tx trials
internationally reproducible
nodal
extra-nodal
e.g. lymphoid polyps in GIT
Hodgkin's
20%
60-70% in 20-30 y/os, 2nd spike in 60-70 y/os (with a worse prognosis)
enlarged firm (usually cervical) node
1/3 have B symptoms
rare symptoms = pruriris + node pain after drinking alcohol
diagnosed by specific malignant REED-STERNBERG cell (essential for Dx)
binucleated, large vesicular (open) nuclei
prominent eosinophilic (pink) nucleoli
v few in sample (often < 1% of cell pop, rely on clinical symptoms + take more tissue if needed)
also a variety of benign reactive cells
RS cell secretes CKs to attract them
lymphocytes, plasma cells, eosinophils
classification based on the reactive cell mixture
classical
more common
better survival rate
80% cured with chemo
4 subtypes
nodular sclerosing
fibrous bands
mixed cellularity
eosinophils
lymphocyte rich
lymphocyte depleted
nodular lymphocyte predominant type
markers
CD15+30 stain RS cells
CD3 stains the reactive cells but not the RS cell itself
high LDH
non-Hodgkin's
80%
B cell
T cell
marker = CD3
low grade (small cells)
high grade (large cells)
Hodgkin's lookalikes
microscopically
ki1 (anaplastic large cell) lymphoma
T cell lymphoma
T cell rich B cell lymphoma
use immunomarkers to distinguish
Reactive lymphoid follicle
follicle/germinal centre
B cells (marker = CD20)
centrocytes (B cell with a cleaved nucleus)
formed following the cessation of centroblast prolif (activated B cell that is enlarged + proliferating)
histiocytes
in centre: lymphocytes + plasma cells
mantle
marginal zone
Normal Lymphoid cells
lymphoblasts, then lymphocytes
follicular centre cells (FCC)
mantle cells
marginal cells
plasma cells
immunoblasts (lymphocytes activate by antigen, no longer naive, will undergo clonal expansion)
mantle cell lymphoma
diffuse replacement of node by malignant mantle cells
usually @ 60-70 y/o
often high stage @ presentation
cyclin D1 +ve
poor prognosis as doesn't respond to tx
20-40% 5yr survival
can't be eradicated