Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
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