Professor Sherry Bowen
WHO Lymphoid NeoplasmsB cell neoplasms T/NK cell neoplasms Hodgkin lymphoma (disease)LymphomaClonal malignant disorders that are derived from lymphoid cells: either precursor or mature T-cell or B-cell Majority are of B- cell originDivided into 2 main types : 1. Hodgkin’s lymphoma 2. Non - Hodgkin’s lymphomaHodgkin’s DiseaseHistologically & clinically a distinct malignant diseasePredominantly, B-cell diseaseCourse of the disease is variable, but the prognosis has improved with modern treatmentClinical featuresBimodal age distribution :young adults ( 20-30 yrs) & elderly (> 50yrs) May occur at any ageM > FLymphadenopathy:most often cervical region asymmetrical, discretepainless, non-tenderelastic character on palpation ( rubbery)not adherent to skin Constitutional symptoms ( B symptoms )Night sweats, sustained fever > 38 degree celsius,loss of weight >10% of body weight in 6 mo Fever sometimes cyclical (‘Pel-Ebstein fever’)Pain at the site of disease after drinking alcoholPallorPruritis Symptoms of Bulky (>10 cm) diseaseLymph node biopsy : Malignant Reed-Sternberg ( RS) Cell: Bi-nucleate cell with a prominent nucleolus. Derived from B cell, at an early stage of differentiation Reactive background of eosinophils, lymphocytes, plasma cells Fibrous tissueNon Hodgkin’s lymphomaIncidence is increasingNHL>HDMedian age of presentation is 65-70 yrsM>FMore often clinically disseminated at diagnosisB-cell-70% ; T-cell-30%StagingStage 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 diaphragmStage 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)Clinical featuresWidely disseminated at presentation Nodal involvement: Painless lymphadenopathy, often cervical region is the most common presentationHepatosplenomegaly Extranodal : Intestinal lymphoma ( abdominal pain, anemia, dysphagia); CNS ( headache, cranial nerve palsies, spinal cord compression) ; Skin, Testis; Thyroid; Lung Bone marrow (low grade): Pancytopenia Systemic symptoms Sweating, weight loss, itchingMetabolic complications: hyperuricemia, hypercalcemia, renal failure Compression syndrome:Gut obstructionAscites Diagnosis and stagingSimilar to HD plus,Bone marrow aspirate & trephineImmunophenotyping : Monoclonal antibodies directed against specific lymphocyte associated antigens B cell antigens ( CD 19, 20, 22); T cell antigens ( CD 2, 3, 5 & 7)Immunoglobulin determination: IgG / IgM praprotein markerHIVClassificationREALClinical / Working FormulationLow gradeInermediate grade High grade EtiologyCannot be attributed a single causeChromosomal translocations: t (14, 18)Infection:Virus:EBV, HTLV,HHV-8, HIVBacteria: H.Pylori - Gastric lymphomaImmunology: Congenital immunodeficiency, Immunocompromised patients - HIV, organ transplantation ................
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