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Non Hodgkin’s lymphoma. Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India. Staging. Stage I : Involvement of single LN region (I) or extra lymphatic site (IA E )
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Non Hodgkin’s lymphoma Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India
Staging • Stage I : Involvement of single LN region (I) or extra lymphatic site (IAE ) • Stage II : Two or more LN regions involved (II) or an extra lymphatic site and lymph node regions on the same side of diaphragm • Stage III : Involvement of lymph node regions on both sides of diaphragm, with (IIIE) or without (III) localized extra lymphatic involvement or involvement of the spleen (IIS) or both (IISE) • Stage IV : Involvement outside LN areas (Liver, bone marrow) A : Absence of ‘B’ symptoms B : B symptoms present
Non Hodgkin’s lymphoma • Incidence is increasing • NHL>HD • Median age of presentation is 65-70 yrs • M>F • More often clinically disseminated at diagnosis • B-cell-70% ; T-cell-30%
Clinical features • Widely disseminated at presentation • Nodal involvement: Painless lymphadenopathy, often cervical region is the most common presentation • Hepatospleenomegaly • Extranodal : Intestinal lymphoma ( abdominal pain, anemia, dysphagia); CNS( headache, cranial nerve palsies, spinal cord compression) ; Skin, Testis; Thyroid; LungBone marrow (low grade): Pancytopenia
Clinical features contd • Systemic symptoms • Sweating, weight loss, itching • Metabolic complications: hyperuricemia, hypercalcemia, renal failure • Compression syndrome: • Gut obstruction • Ascites • SVC obstruction • S/C Compression
Classification • REAL • Clinical / Working Formulation • Low grade • Intermediate grade • High grade
Low grade Proliferation: Low Course: Indolent Symptoms: -ve Treatment: Not curable High grade High Rapid, fatal(un-Rx) +ve Potentially Curable Classification
Etiology • Cannot be attributed a single cause • Chromosomal translocations: t (14, 18) • Infection: • Virus:EBV, HTLV,HHV-8, HIV • Bacteria: H.Pylori - Gastric lymphoma • Immunology: • Congenital immunodeficiency, • Immunocompromised patients - HIV, organ transplantation
Management Low grade: • Asymptomatic : No treatment ; • Radiotherapy for localised disease (Stage 1); • Chemotherapy: mainstay is Chlorambucil; Initial response good , but repeated relapses, median survival 6-10 yrs; • Newer: Fludarabine, 2-CdA (Chlorodeoxyadenosine) • Monoclonal antibody: Rituximab • SCT/BMT
Aggressive( high / intermediate grade): Chemotherapy: mainstay CHOP -every 3 weeks, at least 6 cycles Cyclophosphamide, Doxorubicin Hydrochloride, Vincristine, Prednisolone
High risk cases with poor prognostic factors or relapse : High dose chemotherapy combined with autologous BMT / SCT Monoclonal antibody With CNS involvement / leukemic relapse : Similar to ALL
Prognosis • Low grade : Median survival –10 yrs • High Grade: • Increasing age, advanced stage, concomitant disease, raised LDH,T- cell phenotype : Poor prognosis