T H E B A R E E S S E N T I A L S

LYMPHOMAS

BASICS

• Malignancies of lymphocytes• Lymphoid tissue-

• Lymph node• Spleen• Thymus• Waldeyer’s ring• Peyer’s patches• Appendix

• Over/under/abnormal expression of oncogenes• Complex genetics

MANIFESTATIONS

• Varied and endless• Major-

• Lymphadenopathy, Spleno/Hepato-megaly• PUO• Weight loss• Night sweats• Obstruction – SVC, urethra, intestine• GI bleed, intestinal perforation• Effusion – pleural, pericardial• Cytopenia – BM involved, autoimmune

• Indolent types TO Very aggressive• Suspect in various scenarios

DIAGNOSIS

• Definitive – histopath of biopsy of tumour (Sx best)

• Workup and staging-• CXR, USG, CT, PET• CBC, ESR• RFT, LFT, LDH, β2 macroglobulins• Serum protein electrophoresis

HODGKIN’S I

• Reed-Sternberg cells-• Large• B cell origin• Malignant lymphoid cells

HODGKIN’S II

• 4/100,000/year• M:F = 1.5:1• Age – bimodal, 20-35 AND 50-70

• Types (WHO)-• Nodular lymphocyte predominant (slow, localized, rarely fatal)• Classical-• Nodular sclerosing (young, women)• Mixed cellularity (elderly)• Lymphocyte rich (men)• Lymphocyte depleted (very rare)

HODGKIN’S III

HODGKIN’S IV

• Treatment-• Upto IIA – RadioRx alone/ Chemo+adjuvant RadioRx• >IIA – Chemo+adjuvant RadioRx• ABVD-

• Doxorubicin (cardiotox)• Bleomycin (pulmonary fibrosis)• Vinblastine• Dacarbazine

• Resistant – HSCT

• Followup-• Lab investigations• Imaging modalities

NON HODGKIN’S I

• Origin – B / T cell

• 12/100,000/year

• Males slightly more

NON HODGKIN’S II

• Aetiology unclear-• Various factors• Immune deficiencies:

• Organ transplantation • Inherited immune deficiencies • AIDS

• Agricultural chemicals • Autoimmune disorders:

• Rheumatoid arthritis • Lupus erythematosus

• Treated Hodgkin’s disease • Infectious agents:

• Viruses: EBV, HTLV-1, HIV, HHV-8, HCV • Bacteria: Helicobacter pylori, Chlamydia psittaci, Borelia borgdorferi,

Campylobacter jejuni

NON HODGKIN’S III◆ Precursor lymphoid neoplasms

• B-lymphoblastic leukaemia/lymphoma • T-lymphoblastic leukaemia/lymphoma

◆ Mature B-cell neoplasms • Chronic lymphocytic leukaemia/small lymphocytic lymphoma • Splenic marginal zone lymphoma • Lymphoplasmacytic lymphoma • Extranodal marginal zone lymphoma of MALT (lymphoma) • Nodal marginal zone lymphoma • Follicular lymphoma • Primary cutaneous follicle centre lymphoma • Mantle cell lymphoma • Diffuse large B-cell lymphoma (DLBCL), NOS

° T-cell rich DLBCL ° Primary cutaneous DLBCL leg type ° Intravascular DLBCL ° Plasmablastic lymphoma ° Primary effusion lymphoma

• Primary mediastinal DLBCL • Burkitt lymphoma

◆ Mature T-cell neoplasms • Adult T-cell leukaemia/lymphoma • Extranodal NK/T cell lymphoma, nasal type • Enteropathy associated T-cell lymphoma • Hepatosplenic T-cell lymphoma • Subcutaneous panniculitis-like T-cell lymphoma • Mycosis fungoides • Sezary syndrome • Primary cutaneous CD30 positive T-cell lymphoproliferative disorders • Peripheral T-cell lymphoma, NOS • Angioimmunoblastic T-cell lymphoma • Anaplastic large cell lymphoma, ALK positive • Anaplastic large cell lymphoma, ALK negative

WHO 2008

NON HODGKIN’S IV

• Clinical-• More disseminated at presentation• More extra-nodal involvement• More compression syndromes

• Investigations-• BM aspiration & trephine• Immunophenotyping cell surface antigen (B/T origin)• Cytogenetic study• Immunoglobulin estimation• Uric acid (high turnover)• HIV testing

NON HODGKIN’S V

• Staging – Ann-Arbor

• Grading = Treatment• High grade- (diffuse large B-cell)• Rapid prolif• Earlier symptoms• Fatal if untreated• Potentially curable

• Low grade- (nodular)• Slow prolif• Late symptoms, indolent course• Unresponsive to conventional Rx

NON HODGKIN’S VI

• Low grade-• Radiotherapy – localized stage 1• Chemotherapy –• Chlorambucil• Rituximab (CD-20) + Cyclophos+Vincrist+Predni

• HSCT after high dose chemo

• High grade-• Radiotherapy – localized stage 1- decrease compression• Chemotherapy –• Cyclophos+Doxorub+Vincrist+Predini (CHOP)• WITH Rituximab (R-CHOP)

• HSCT – for relapse after successful chemo