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Diseases of Lymph Nodes WBCs (Lymphoid Neoplasms Lymphomas …
Diseases of Lymph Nodes
WBCs
Lymphadenopathy
Causes:
Reactive Proliferations
BENIGN
synonym =
Lymphadenitis
Enlarge in response to:
Infections
Cell debris
Foreign material
Antigen
Acute Nonspecific Lymphadenitis
Kids = generalized (bacterial, viral)
Adults = localized (focal infection of eye, teeth, etc.)
Nodes TENDER
:!?:What is the difference between the necrotizing and suppurative? are they the same?
Follicular Hyperplasia w/ Necrosis & PMNs
Pyogenic Infections
Bubonic Plague
Yersinia Infection from Fleas
Follicular Hyperplasia w/
Suppurative Graulomas
Process:
Follicular hyperplasia
Neutrophils
Activated macrophages = suppurative granuloma
Cat-Scratch Disease
Bartonella
Excoriation from cat scratch
Drains to local lymph nodes
Enlarged for
months
Does
not
grow on agar
Need to
silver stain
w/ Warthin-Starry Staining
Serologic
tests for Bartonella henselae confirms Dx
Mesenteric Lymphadenitis
Assoc. w/ enterocolitis c/b
Yersinia enterocolitica
or
Campylobacter jejuni
Numerous intraabdominal LNs drain intestines = ENLARGED (can see on CT)
Mimics acute appendicitis (used to find upon appendectomy w/o CT)
Self-limited
:!: Link to bacterial GI infections later on
Lymphogranuloma Venerum
Chronic Nonspecific Lymphadenitis
:star2: :star2: :star2: :star2: :star2:
Follicular Hyperplasia
Reactive B Cells
Large Germinal Centers
HIV
RA
Paracortical - Intrafollicular Hyperplasia
T-Cell Immunoblasts w/ Macrophages & Eosinophils
Viral Infections
EBV
CMV
Vaccinations
SLE
Sinus Histocytosis
Increased macrophages in sinuses
Draining cancer
Inflammation
"Mixed Pattern"
Toxoplasmosis
Specific Inflammatory Patterns
Granulomatous Lymphadenitis
Types:
:!?: Is this different from Non-specific?
Where do we find the necrotizing supperative one?
Caseating Granulomas
TB, Fungal (cocci)
Noncaseating Graulomas
Sarcoidosis, early TB, Crohn's
Necrotizing Supperative
later in lecture :!:
Metastatic Malignancy
Lymphoma
Lymph Node Structure
Follicles
listed from innermost to outermost
Becomes hyperplastic w/in days of antigen exposure
Germinal Center
Apoptotic Tingible Bodies
=
Reactive Benign Process
Mantle Zone
Naive B Cells
Marginal Zone
Memory B Cells
Paracortex
Subcapular Sinus
Early site of cancer filtration
Lymphoid Neoplasms
Lymphomas
Lymphocytic Leukemias
1/3 have leukemic presentations
80-85% are B cell origin
T cell origin more agressive
Nodal
Extranodal
Brain
Skin
Intestines
Other organs
All Lymphomas are Malignant
TYPES of Lymphoma:
Indolent
Intermediate
Aggressive
Resemble some stage of B or T cell differentiation
Dx:
Take LN out
Biopsy w/histological examination required for Dx
1/3 Culture - for infection
Immunophonotype:
1/3 Stained Histology
1/3 Flow Cytometry
Testing for
Clonality
:
Disproportion B or T cells
Light chain "restriction"
(k or lamda - normal is 1:1)
Gene rearrnagenent (difficult dx) - specific
translocations
define some lymphomas
SEE TABLE IN NOTES:
Non-
Hodkin's Lymphoma (NHL)
B & T Cell Types can be:
Precursor neoplasm (leukemia)
Peripheral neoplasms (mature; multiple subtypes)
Hodgkin's Disease
Neoplastic proliferation from ONE node or CHAIN of nodes
Predictable spread to contiguous nodes
Reed-Sternberg cells*
= Malignant B cell
Bimodal Distribution
Teens to 40s
ave. age 32
Elderly
Classic Hodgkin Reed-Sternberg Cells
CD15, CD30 :heavy_plus_sign:
Binucleated / Bilobed giant cells
Mirror image nuclei (owl's eyes)
Prominent eosinophilic nucleoli
:pill:
Treatment:
Chemo
(combined)
Anti-CD30
(if chemo not working)
Old Tx = Radiation (lots of complications; no longer used)
CLINICAL FINDINGS
LYMPH NODES:
Enlarged, painless
Cervical :star: most common
Horse Collar
configuration (symmetric, bilateral)
Axillary
Inguinal
Mediastinal :keycap_star: common in Nodular Sclerosing type w/
cough & dyspnea
LAD exclusively below diaphragm is rare (would be in combo w/ cervical)
SKIN:
Generalized puritis
BONE PAIN
indicated bone marrow involved
:warning:poor prognosis
Depressed T Cell Function
"cutaneous anergy"
Classic "B" Symptoms
Fever
-
Pel-Epstein pattern
cyclic fevers 1-2 wk intervals
Night sweats
Weight loss
(10% or more in 6 months)
Know Ann Arbor Staging
Classify as:
STAGE:
Stage 1:
single LN region or extralymphatic site
Stage 2:
> / = 2 LN regions
Same side of diaphragm
May include localized extralymphatic site on same side of diaphragm
Stage 3:
LN regions on
both sides of diaphragm
May include spleen or localized extranodal disease
Stage 4:
Diffuse extralymphatic
disease
EX: bone marrow, liver, lung, skin
rare
"A" or "B" Symptoms
SUBTYPES:
CD15
:heavy_plus_sign:
CD 30
:heavy_plus_sign:
Mixed Cellularity
POOR
prognosis
:male_sign: > F
BIMODAL
: Young Adults & Elderly (>55)
EBV
:heavy_plus_sign:
"B"
symptoms
**Abundant RS cells:
Lymphocytes
Histocytes
Eospinophils**
Nodular Sclerosing
:star:
Most common type
GOOD
prognosis
:female_sign: = :male_sign:
Adolescents / Young Adults
EBV
:heavy_minus_sign:
LN:
mediastinum
, lower cervical, supraclavicular
CD20 :heavy_plus_sign:
BCL6
:heavy_plus_sign:
Lymphocyte Predominance
EXCELLENT prognosis; tends to recur
:male_sign: > F
YOUNG (<35 yo)
EBV
:heavy_minus_sign:
LN: mediastinal, cervical, axillary
L&H variant RS cells ("popcorn" cells)
Lymphocyte Rich
not covered
Lymphocyte Depleted
not covered
EBV Virus
TNF-a Induced Protein
Produce
cytokines
Reactive Eosinphils (infiltrates)
&
Fibrosis
Nonclassic, Mutlinucleated L&H
("
popcorn
cell")
CD20 :heavy_plus_sign:
(CD15 & CD30 negative)
BOARDS ONLY - NOT ON DEVINE'S EXAM
Myeloproliferative Disorders:
Neoplastic proliferations of MATURE myeloid cells
= HIGH WBC
copied over from last exam map
BRAF
mutation in
Langerhans cells (dendritic cells in SKIN)
Most are monoclonal/neoplastic
CD1a
, HLA-DR, S-100, CD207 expression
Langerhans Cell Histiocytosis
Acute Disseminated LCH
Common (as common as HL)
Multisystem, multifocal infiltrates = most severe
Seborrheic eruption
Hepatosplenomegaly
Pulmonary lesions
Bone marrow: PANCYTOPENIA (infection risk)
Osteolytic bone lesions
YOUNG children; <2 yo
Letterer-Siwe Disease
Treatment:
Chemo (50%
BRAF Inhibitor
rapidly fatal w/o therapy
sequelae = neurodegeneration
Eosinophilic granuloma
Unisystem = more common, less aggressive
Low risk
Viscera & Bone
OLDER children >5 & Young adults (up to 30)
Unifocal
One axillary node
Multifocal, Unisystem
Skull lesions
Labs:
Vesicular nuclei with linear grooves
Abundant vacuolated cytoplasm (
be able to ID
)
BIRBECK GRANULES
🏸
S-100, CD207 (Lengerin)
Mutated BRAF
Eosinophils
Pulmonary
ADULT smokers 🚬
Hemophagocytic Lymphohistiocytosis (HLH)
Synonym = Macrophage Activation Syndrome
Systemic activation: macrophages & CD8+ >
Phagocytize blood cell progenitors in BM
Poor
Prognosis
Assoc. Chediak-Higashi Syndrome
EBV
triggers (75%)
Treatment:
immunosuppresion
&
cyototoxic
drugs
Familial Form
Sporadic form
Labs:
Cytopenias of 2 or more cell lines
Hypertriglyceridemia and/or hypfibronogenemia
Increased serum ferritin (out of macrophages)
Increased CD25
Tumor Lysis Syndrome
Complication of hematopoietic malignancy
Common, serious
Tumor cells release contents to blood
Electrolyte & metabolic disturbances -->
Renal Failure
Cardiac arrhythmia
Seizures
Death by multi organ failure
Labs: Related to Renal Failure
Hyperuricemia
Hyperkalemia
Hyperphosphatemia
Hypocalcemia
Elevated LDH