Non Hodgkin’s lymphomaNon Hodgkin’s lymphoma

Rakesh Biswas

MD, Professor, Department of Medicine, People's College of Medical Sciences,

Bhanpur, Bhopal, India

StagingStaging Stage IStage I : Involvement of single LN region (I) or extra : Involvement of single LN region (I) or extra

lymphatic site (IAlymphatic site (IAEE ) ) Stage IIStage II : : Two or more LN regions involved (II) or an Two or more LN regions involved (II) or an

extra lymphatic site and lymph node regions on the extra lymphatic site and lymph node regions on the same side of diaphragmsame side of diaphragm

Stage IIIStage III : : Involvement of lymph node regions on both Involvement of lymph node regions on both sides of diaphragm, with (IIIsides of diaphragm, with (IIIEE) or without (III) localized ) or without (III) localized extra lymphatic involvement or involvement of the extra lymphatic involvement or involvement of the spleen (IIspleen (IISS) or both (IIS) or both (IISEE) )

Stage IVStage IV : : Involvement outside LN areas (Liver, bone Involvement outside LN areas (Liver, bone marrow)marrow)

AA : Absence of ‘B’ symptoms : Absence of ‘B’ symptoms BB : B symptoms present : B symptoms present

Non Hodgkin’s lymphomaNon Hodgkin’s lymphoma

Incidence is increasingIncidence is increasingNHL>HDNHL>HDMedian age of presentation is Median age of presentation is 65-70 yrs65-70 yrsM>FM>FMore often clinically disseminated at More often clinically disseminated at

diagnosisdiagnosisB-cell-70% ; T-cell-30%B-cell-70% ; T-cell-30%

Clinical featuresClinical features Widely disseminated at presentation Widely disseminated at presentation Nodal involvementNodal involvement: :

Painless lymphadenopathyPainless lymphadenopathy, often cervical , often cervical region is the most common presentationregion is the most common presentation

HepatospleenomegalyHepatospleenomegaly ExtranodalExtranodal : :

Intestinal lymphomaIntestinal lymphoma ( abdominal pain, anemia, ( abdominal pain, anemia, dysphagia); dysphagia); CNSCNS ( headache, cranial nerve palsies, spinal ( headache, cranial nerve palsies, spinal cord compression) ;cord compression) ;

Skin, TestisSkin, Testis; Thyroid; Lung; Thyroid; Lung Bone marrowBone marrow (low grade): (low grade): PancytopeniaPancytopenia

Clinical features contdClinical features contd

Systemic symptomsSystemic symptoms Sweating, weight loss, itchingSweating, weight loss, itching Metabolic complications:Metabolic complications:

hyperuricemia, hyperuricemia, hypercalcemia, hypercalcemia, renal failurerenal failure

Compression syndrome:Compression syndrome: Gut obstructionGut obstruction AscitesAscites SVC obstructionSVC obstruction S/C CompressionS/C Compression

ClassificationClassification

REALREALClinical / Working FormulationClinical / Working Formulation

Low gradeLow grade Intermediate gradeIntermediate grade High gradeHigh grade

ClassificationClassification

Low gradeLow grade

Proliferation: LowProliferation: Low

Course:Course: Indolent Indolent

Symptoms: -veSymptoms: -ve

Treatment: Not curable Treatment: Not curable

High gradeHigh grade

HighHigh

Rapid, fatal(un-Rx)Rapid, fatal(un-Rx)

+ve+ve

Potentially CurablePotentially Curable

Etiology Etiology

Cannot be attributed a single causeCannot be attributed a single cause Chromosomal translocationsChromosomal translocations: t (14, : t (14,

18)18)

Infection:Infection: Virus:Virus:EBV, HTLV,HHV-8, HIVEBV, HTLV,HHV-8, HIV Bacteria: H.Pylori - Gastric lymphomaBacteria: H.Pylori - Gastric lymphoma

Immunology: Immunology: Congenital immunodeficiency,Congenital immunodeficiency, Immunocompromised patients - Immunocompromised patients - HIV, organ transplantationHIV, organ transplantation

ManagementManagement

Low gradeLow grade:: Asymptomatic : No treatment ; Asymptomatic : No treatment ;

RadiotherapyRadiotherapy for localised disease (Stage 1); for localised disease (Stage 1); Chemotherapy: mainstay is Chemotherapy: mainstay is

ChlorambucilChlorambucil; Initial response good , but ; Initial response good , but repeated relapses, median survival 6-10 yrs; repeated relapses, median survival 6-10 yrs; Newer: Fludarabine, 2-CdA (Chlorodeoxyadenosine)Newer: Fludarabine, 2-CdA (Chlorodeoxyadenosine)

Monoclonal antibody: RituximabMonoclonal antibody: Rituximab SCT/BMTSCT/BMT

AggressiveAggressive ( high / intermediate grade):( high / intermediate grade):

ChemotherapyChemotherapy: mainstay : mainstay CHOPCHOP -every 3 weeks, at least -every 3 weeks, at least 6 cycles 6 cycles CCyclophosphamide, yclophosphamide, DDoxorubicin oxorubicin HHydrochloride, ydrochloride, VVincristine, incristine, PPrednisolonerednisolone

High risk cases with poor prognostic High risk cases with poor prognostic factors or relapse : factors or relapse : High dose chemotherapy High dose chemotherapy combined with autologous BMT / SCTcombined with autologous BMT / SCT

Monoclonal antibodyMonoclonal antibody

With CNS involvement / leukemic relapse : With CNS involvement / leukemic relapse : Similar to ALLSimilar to ALL

PrognosisPrognosis

Low grade : Median survival –10 yrsLow grade : Median survival –10 yrsHigh Grade:High Grade:

Increasing age, advanced stage, concomitant Increasing age, advanced stage, concomitant disease, raised LDHdisease, raised LDH,,T- cell phenotypeT- cell phenotype : Poor : Poor prognosisprognosis