Transcript (PPTX, 69KB)

HIVor AIDS –ASSOCIATED
LYMPHOMAS
H.A. MWAKYOMA, MD
CURRENT FACTS
• HIV patients have increased incidence of
certain tumours
– Kaposis sarcoma
– Non-hodgkin lymphoma
– Cervical cancer
– Scc conjuctiva
– Ano rectal carcinoma
– Leiomyosarcoma in children
AIDS Defining Malignancies (ADMs
 KS
 Lymphoma:
PCNSL,
Immunoblastic,
 Burkitt’s,
Primary Effusion
 Cervical carcinoma
Non-Hodgkin lymphoma
• Recognised as part of AIDS in 1982
Characteristically aggressive and often involve
extra nodal sites
• Some HIV individuals are more prone to develop
lymphoma than others
Aetiology and pathogenesis
• Lymphomas develop against a background of
chronic antigenic stimulation and most are of
B-cell origin
• Cytokenes stimulate expansion once malignant
transformaton has occurred(IL-6, TNF-beta and
IL-10)
• Chemokines produced by HIV infected
macrophages and monocytes produce
autocrine stimulation of the abnormal clone
Clinico-pathological categories of HIV
related lymphomas
• Diffuse large cell lymphoma(DLCL)
– Large non cleaved (LNCCL) ebv 40%
– Immunoblastic plasmacytoid (IBPL) 90%
• Burkitt’s lymphoma (BL) ebv 30%
• Primary lymphomas of the central nervous
system(PCNSL) ebv 100%
• Primary effusion lymphomas (PEL) ebv 90%,
HHV-8 100%
Clinico-pathological categories of HIV
related lymphomas
 Degree and duration of HIV affects type of
lymphoma that developes:
• Primary CNS lymphomas are associated with
profound immunosuppression and occur late
in the course of HIV
• The other types may occur early
• Extranodal lymphomas more common in AIDS
patients
NHL
 70-90% High grade B cell lymphomas (large
B cell, immunoblastic, Burktt’s—c-myc
translocation)
 PCNSL—15% (Primary Central Nervous
System Lymphoma)
 Primary Effusion Lymphoma (“Body Cavity
Lymphoma”)—rare
NHL
 Present at more advanced stage,
 extranodal disease (GI tract common), bone marrow,
liver and lung, CNS,
 80% Stage 4 disease at presentation
• More often with “B” sx—night sweats, fever, weight
loss
 Incidence inversely related to CD4 count but can occur
at any CD4
 Diagnosis same as in non-HIV pt but higher rate of
asymptomatic CNS involvement
• FNA usually not adequate, need excisional BX
AETIOLOGY -NHL
•
Immunodeficiency



•
congenital
Acquired
Autoimmune
Infectious agents(other than HIV)




H.pylori
EBV
HTLV-1
HHV8-kaposi sarcoma related
BURKITT`S LYMPHOMA
BURKITT`S LYMPHOMA
• Highly aggressive type of NHL -B cell type
• Accounts for approx. 40% of childhood lymphomas
• Two types
– Endemic - associated with EBV(95%)
• Frequent involvement of jaw and other facial
bones
– Sporadic
• Extensive intra abdominal and bone marrow
involvement common
• Histologically - starry sky appearance
BL- CLINICAL PRESENTATION
• Abdomen is the most common presenting site
in sporadic cases .
• Typically seen in boys of 5 – 10 years age group
• Exploratory laparotomy is indicated for
diagnosis
• Head and neck region is common site in
endemic cases
• Less common sites-epidural mass, skin
nodules,bone and bone marrow
Primary Effusion Lymphoma
 Rare
 HHV-8
 Serous effusions (pleural, peritoneal,
pericardial, joint effusions) with malignant
lymphocytes
 No mass lesions
 Very poor prognosis
PEL
• Primary effussion lymphomas (body cavity
lymphomas)
– Pleural effusion or ascitis without evidence of bulk
disease
– Thickening of pleural or peritoneal membranes
with no evidence of tumour masses
• Symptoms are from accumulation of fluid
– Dyspnoea, chest or abdominal discomfort
PCNSL
• Primary CNS
– 75% develop in known AIDS patients
– 50% have CD4 of less than 50/dl
– Symptoms similar to SOL (headache, change in
consciousness, focal neurological symptoms,
visual disturbances)
– Rapid onset and therefore difficult to differentiate
from infection
PRIMARY CENTRAL NERVOUS
SYSTEM LYMPHOMA
NHL arising in and confined to the
CNS
Incidence: 0.5-1%of all intracerebral
neoplasms
1.9-6% HIV pt
Immunodeficient state,
renal transplantation
Common in 6th to 7th decade
PCNSL




EBV
100-1000x higher than general population
CD4<100, usually <50
Dx: LP +EBV, MRI with homogeneous,
sometimes ring enhancing lesions, often periventricular, often +mass effect,
 Prognosis: poor in pre-HAART era, overall still
very poor
PCNSL-Clinical Presentation
Disseminated lesion , Brain (Common)
Eye (20%) , Leptomeninges (7%)
Spinal Cord
• Clinical Stage – Stage I E
• Pathology – Intermediate Malignant type
• Investigations – CSF study
Ophthalmic exam with slit
lamp
CXR, CT- Abd, Cranial/spinal
MRI with Gadolinium
Stereotactic biopsy
•
Hodgkin’s Lymphoma and HIV
 Usually advanced stage at time of diagnosis
(stage 3,4)
 More extra-nodal involvement—bone marrow,
liver
 Worse prognostic cell type—mixed cellularity
histologic subtype (nodular sclerosis most
common in non-HIV)
 Worse overall prognosis
 Better outcomes in era of HAART