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Hodgkins lymphoma
It is a unique form of lymphoid malignancy characterised by pleomorphic lymphocyte infiltrate interspersed by malignant multinuclear giant cell(RS cell). Thomas hodgkin in his historic paper entitled On Some Morbid Appearances of the Absorbent Glands and Spleen presented to the Medical Society London on jan 10, 1832 described this disease for the first time. It is said that the hodgkins reed-sternberg cell are clonal lymphoma cells derived from B-cells, hence the term hodgkins lymphoma is preferred over the hodgkins disease.
Barr virus in RS-cells. Chronic stimulation due to EBV infection is postulated to results in pleomorphic infiltration, some of the cell undergo malignant transformation subsequently.
Infection by HIV is also a risk factor for developing hodgkins
lymphoma.
Rarely hodgkins lymphoma can be familial.
are uncommon.
Classification
Diagnosis of hodgkins lymphoma requires demonstration of Reed Sternberg
cells in the biopsy. These RS cells are large multinucleated giant cells with a clear halo around prominent nucleoli(caterpillar or owl eye appearance), which are
-nodular sclerosis
-mixed cellularity
-lymphocyte rich -lyphocyte depleted
Staging
Ann Arbor staging system
I Involvement of a single lymph node region or a single extralymphatic
organ or site(IE)
II- Involvement of two or more lymph node region on the same side of
organs with or without associated lymph node involvement. Bone marrow n liver involvement are always stageIV.
doubtful nodes Complete blood count and biochemistry including LDH Chest x-ray CT scan of thorax, abdomen and pelvis Tissue excision biopsy and aspiration Optional procedures Post nasal space x-ray if cervical nodes are involved Liver biopsy Radioisotope bone scan if stage IV Pleural cytology if there is pleural effusion PET scan Staging laparotomy,lymphangiography and gallium scan
Differential Diagnosis
Non hodgkins lymphoma
Infectious mononucleosis Tubercular lymphadenitis
Bronchogenic carcinoma
Head and neck squamous cell carcinoma Sarcoidosis
Prognostic Factors
Adverse prognostic factors apart from advanced stage
in hodgkins lymphoma include Age- >50 yr ESR- >50 Large mediastinal mass and four or more involved regions Depending on these, three prognostic groups have been identified Early stage favorable group include stage I, II with none of the above adverse prognostic factor Early stage unfavorable group include stage I, II with one or more of the adverse risk factors Advanced stage include stage III, IV, serum albumin <4 gm%, presence of B symptoms.
Treatment strategies in patients with hodgkins lymphoma based on stage of disease and patients age
Young patients Early stage favorable RT alone- extended field(30-36Gy) Elderly patient
Advanced stages
Treatment
Radiotherapy- It offers effective regional control of disease in
patients with hodgkins lymphoma. The mantle field comprises of submandibular, cervical, supraclavicular, infraclavicular, axillary, mediastinal, subcarinal and hilar lymph node and this is used for supra-diaphragmatic disease. Para-aortic, splenic and pelvic field radiations are used for infra-diaphragmatic disease. Involved field radiation is commonly used in the present era of combined modality treatment, the field comprises the entire involved lymph node regions.
nitrogen mustard in 1940, a number of different drugs were developed and showed efficacy in hodgkins lymphoma. The first breakthrough four drug combination was developed in 1967 i.e MOPP(mechlorethamine, vincristin,
used in patients with relapsed and refractory hodgkins lymphoma. BEAM(BCNU, etoposide, cytocine arabinoside, melphalan) is one of the commnly used high dose conditioning chemotherapy regimen. Combination chemotherapy regimen used as salvage therapy in relapsed or residual disease include MINE, DHAP, ESHAP, ICE, mini BEAM, dexa BEAM. With the
radioimmunoconjugates, cellular therapy, newer chemotherapeutics like gemcitabin, vinorelbine are also used.
-Acute myeloid leukaemia -Diffuse high grade NHL -Solid tumors(mostly lung and breast tumor) -Overwhelming bacterial sepsis following splenectomy or splenic irradiation Potentially serious -Myocardial damage from radiation and anthracyclines -Lung fibrosis -Sterility -growth abnormalities in children -opportunistic disease and psychological problems Usually minor -Chemical or clinical hypothyroidism -long term alteration of lymphocyte function
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