1. HIVor AIDS –ASSOCIATED LYMPHOMAS
H.A. MWAKYOMA, MD

2. 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

3. AIDS Defining Malignancies (ADMsKS
Lymphoma:
PCNSL,
Immunoblastic,
Burkitt’s,
Primary Effusion
Cervical carcinoma

4. 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

5. 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

6. 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%

7. 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

8. 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

9. 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

10. AETIOLOGY -NHL Immunodeficiency Infectious agents(other than HIV)
congenital
Acquired
Autoimmune
Infectious agents(other than HIV)
H.pylori
EBV
HTLV-1
HHV8-kaposi sarcoma related

11. BURKITT`S LYMPHOMA

12. 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

13. 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

14. Primary Effusion Lymphoma
Rare
HHV-8
Serous effusions (pleural, peritoneal, pericardial, joint effusions) with malignant lymphocytes
No mass lesions
Very poor prognosis

15. 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

16. 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

17. 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

18. PCNSL EBV 100-1000x higher than general population
CD4<100, usually <50
Dx: LP +EBV, MRI with homogeneous, sometimes ring enhancing lesions, often peri-ventricular, often +mass effect,
Prognosis: poor in pre-HAART era, overall still very poor

19. 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

20. 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