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Review Article Volume 2 Issue 1
Recapitulating classic hodgkin’s: primary mediastinal B cell lymphoma
Anubha Bajaj
Received: January 18, 2019 | Published: February 12, 2019
Citation: Bajaj A. Recapitulating classic hodgkin’s: primary mediastinal b cell lymphoma. MOJ Tumor Res. 2019;2(1):6-11. DOI: 10.15406/mojtr.2019.02.00044
Abstract
The world health organization (WHO) designates the primary mediastinal B cell lymphoma as a unique entity with distinct clinical, morphological and biological attributes which comprises of an estimated 2%-4% of the non Hodgkin’s lymphoma. The median age of emergence is at 35 years and a female to male ratio of (F:M) 1.7-2.0:1 is elucidated. The lymphoma appears as a bulky mediastinal mass situated in the anterior or upper mediastinum with invasion of abutting anatomical thoracic structures such as the chest wall, pleura, lungs, pericardium and heart besides a pleural or pericardial effusion in an estimated 30%-50% instances. The lymphoma simulates the histology of a nodular sclerosis Hodgkin’s lymphoma (NSHL) and depicts a post germinal centre phenotype (CD10-, BCL6+/-, MUM1+). Gene expression profiling (GEP) depicts a reoccurring amplification of JAK2 situated on chromosome band 9p24.1 in approximately 50%-70% instances. The lymphoma exhibits a deterioration of the NF?B and JAK/STAT signalling networks which inhibits apoptosis, besides a specific copy number chromosomal gains of REL and JAK2 oncogene with a decline of tumour necrosis factor alpha induced protein 3 (TNF AIP3) or the A20 gene. JAK/STAT signalling cascade is activated chiefly by interleukin 4 and 13 and augments tumour proliferation. Elevated serum lactate dehydrogenase (LDH) is a singular laboratory abnormality 70%-80% instances. Prognostic factors cogitating a declining survival comprise of age greater than 40 years, clinical stage III and IV, bulky disease, male sex, poor performance status(PS) and serum lactate dehydrogenase ( LDH) greater than twice the upper normal limit ( > 2 UNL). Therapeutic regimen administered in PMBCL is a combination of rituximab with cyclophosphamide, doxorubicin, vincristine and prednisone (R CHOP). Dose adjusted etoposide, prednisolone, vincristine, cyclophosphamide and doxorubicin with rituximab (DA EPOCH R) can efficaciously treat the de novo lymphoma.
Keywords: cyclophosphamide, doxorubicin, vincristine, prednisone, etoposide, prednisolone, PMBCL, kidney, adrenal, liver, central nervous system, JAK/STAT
©2019 Bajaj. This is an open access article distributed under the terms of the, which permits unrestricted use, distribution, and build upon your work non-commercially.