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Lymphomas. MALIGNANT LYMPHOMA. GROUP OF NEOPLASM'S OF VARYING DEGREES OF MALIGNANCY DERIVED FROM BASIC CELLS OF LYMPHOID TISSUE LYMPHOCYTES IN ANY OF THEIR DEVELOPMENT STAGES. NON-HODGKIN’S LYMPHOMA.
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MALIGNANT LYMPHOMA • GROUP OF NEOPLASM'S OF VARYING DEGREES OF MALIGNANCY DERIVED FROM BASIC CELLS OF LYMPHOID TISSUE • LYMPHOCYTES IN ANY OF THEIR DEVELOPMENT STAGES
NON-HODGKIN’S LYMPHOMA • HETEROGENOUS GROUP OF LYMPHOPROLIFERATIVE MALIGNANCIES WHICH CAN INVOLVE BOTH LYMPH NODES & LYMPHOID ORGANS AS WELL AS EXTRANODAL ORGANS & TISSUES
CLASSIFICATION WORKING FORMULATION CLASSIFICATION • LOW GRADE • INTERMEDIATE • HIGH GRADE
RAPPAPORT CLASSIFICATION • NODULAR • DIFFUSE • LYMPHOCYTIC • HISTIOCYTIC
REAL [ REVISED EUROPEAN – AMERICAN LYMPHOMA] • B- CELL NEOPLASMS • T- CELL – NK CELL NEOPLASMS • HODGKIN LYMPHOMA
Etiology: • 1) genetic abnormality • 2) environmental factors like chemicals- pesticides, herbicides, wood preservatives, organic chemicals. • 3) viruses: EBV , HHV 8, • 4) immunodeficiency state: congenital immuodeficiency state like ataxia telengiectasia & • Aquired immunodeficiency state like HIV
Clinical features • High grade and small cell lymphomas are seen in children. • Where as NHL is older than 50 years. • NHL common in males. • Lymphadenopathy followed by systemic symptoms like fever, night sweats, weight loss, fatigue, pruritus noticed.
Oral manifestation • Swelling which may grow rapidly and ulcerate • Pain • Underlying bone is involved • Tooth mobility.
Histopathology • Nodular • Diffuse • IN nodular type there is neoplastic cells tend to aggregate in large cluster. • IN diffuse type monotonous distribution of cells with no evidence of nodularity or germinal center. • Nodular type is seen in adults with better prognosis than the diffuse type.
Follicular /Nodular lymphoma • 22% of non hodgkin’s lymphomas • Predominant growth pattern is seen in lymph node. • 2 principle cell types are observed. • Centrocytes: small cell with irregular or cleaved nuclear contour with scanty cytoplasm. • Centroblast: larger cells with open nuclear chromatin, several nucleoli with moderate amount of cytoplasm.
Diffuse large B cell lymphoma • 31 % of all non Hodgkin’s lymphoma. • Large cell size with diffuse growth pattern. • Tumor cells have a round or oval nucleus, which is vesicular because of margination of chromatin at the nuclear membrane.
BURKITT’S LYMPHOMA • TUMOUR OF CHILDREN OF TROPICAL AFRICA • DENIS PARSONS BURKIT • ENDEMIC & NON ENDEMIC FORM • FASTEST GROWING MALIGNANCY IN HUMANS
C/F • MAX OR MAND • SPORADIC FORM – ABDOMINAL ORGANS
ETIOLOGY • EBV • MALARIA – IMMUNOSUPRESSION – INADEQUATE T CELL RESPONSE AGAINST B CELLS INFECTED LATENTLY WITH EBV.
AFRICAN FORM • SWELLING OF AFFECTED JAW OR OTHER FACIAL BONES • LOOSENING OF TEETH • SWELLING OF LYMPH NODE • NON TENDER & RAPIDLY GROWING IN NECK OR BELOW JAW • ABDOMINAL PRESENTATION – LESS COMMON
SPORADIC FORM • ABDOMINAL TUMOURS • SWELLING & PAIN • BOWEL OBSTRUCTION • METABOLIC DERANGEMENT & RENAL FUNCTION IMPAIRMENT
MAJOR SIGNS • INVOLVEMENT OF JAWS OR FACIAL BONES • ENLARGED CERVICAL LYMPH NODES • ABDOMINAL MASSES • ASCITIS
‘STARRY SKY APPEARANCE’ • SEEN IN AFRICAN JAW LYMPHOMA • SCATTERED MACROPHAGES WITH CLEAR CYTOPLASM OFTEN PHAGOCYTIC CELLULAR DEBRIS
TREATMENT • COMBINATION CHEMOTHERAPY & CNS PROPHYLAXIS
HODGKIN’S DISEASE • MAIN TYPES OF MALIGNANT LYMPHOMA • THOMAS HODGKIN • POTENTIALLY CURABLE MALIGNANT LYMPHOMA
ETIOLOGY • EBV • ASSOCIATED WITH AIDS • GENETIC PREDISPOSITION
C/F • BIMODAL DISTRIBUTION [ 15-34 - >55 YRS] • MALES • COMMON IN WHITES • PAINLESS ENLARGEMENT OF ONE OR MORE CERVICAL L.N • FIRM, RUBBERY IN CONSISTENCY • OVERLYING SKIN NORMAL
UNEXPLAINED WT.LOSS • FEVER • NIGHT SWEATS • CHEST PAIN • SHORTNESS OF BREADTH • PRURITUS • BACK, ABDOMEN, BONE PAIN
O.M • SECONDARY INVOLVEMENT OF MAND & ALVEOLAR MUCOSA
H/P HISTOPATHOLOGICAL VARIANTS: • NODULAR SCLEROSIS • MIXED CELLULARITY • LYMPHOCYTE DEPLETED • NODULAR LYMPHOCYTE – PREDOMINANT H.D
H/P • NODULAR SCLEROSIS : 60 – 80% • NODULAR PATTERN • FIBROSIS DIVIDING IT INTO NODULES • THE CELL TYPE IS LACUNAR TYPE RS CELL. • LACUNAR TYPE: Mono lobulated or multi lobulated nucleus, abundant & pale cytoplasm, small nucleolous • ADOLESCENTS & YOUNG ADULTS • IT INVOLVES MEDIASTINUM & SUPRADIAPHRAGMATIC SITE.
Mixed cellularity: Comprises of 15-30 % of cases. RS are of classic type(large with bilobate double or multiple nuclei, eosinophilihc inclusion like nucleus ). • It commonly affects abdominal lymph nodes and spleen.
Lymphocyte depleted: less than 1%.hypocellular, with large number of RS cells. • It is associated with older age group & HIV patients.
Lymphocyte rich classic hodgkin’s disease: 5 % of the cases. RS cells of classic or lacunar type with a background of lymphocytes.
Nodular lymphocyte predominant HD: 5 % of the cases. Popcorn cells a variant of RS cell is seen within the background of inflammatory cells.
TREATMENT • RADIATION THERAPY & COMBINATION CHEMOTHERAPY
MULTIPLE MYELOMA • MALIGNANCY OF PLASMA CELLS • SUBSET OF B CELLS
PATHOGENESIS • MUTATION OF TERMINALLY DIFFERENTIATED B CELLS • ETIOLOGY -RADIATION -CHEMICAL -OCCUPATION