Nodal, Haematological, Bicameral: Lymphoblastic lymphoma

An exceptional and aggressive neoplasm with a 2% estimated prevalence of adult Non Hodgkin’s lymphoma is the precursor T cell lymphoblastic lymphoma (T cell LBL). It frequently presents as a cervical, supraclavicular or an axillary lymph node enlargement with a mediastinal tumour in young adults. The Ï«á´½ T cell Acute Lymphoblastic Leukaemia (ALL) roughly exhibits 9%-12% of acute T cell leukaemia in children and adults. The monomorphic dissemination and proliferation of malignant lymphoblast depict a cytology of a medium sized cell with minimal cytoplasma delicate, fine, well dispersed chromatin, a convoluted nuclear membrane and miniature, distinct nucleoli. Lymphoblastic lymphoma is characteristically restricted to the thymic dependent para-cortical zones of the lymph nodes The lymphoma demonstrates pan T cell antigens CD1a, CD2+,CD5+,CD7+, cytoplasmic CD3+ (cyCD3) , CD43+(1,3) and CD71+(transferring receptor antigen) on immune- histochemistry. Chromosomal translocations recognized in T cell lymphoblastic lymphoma are within the alpha and delta T Cell Receptor (TCR) loci situated at chromosome 14q11.2, the beta locus located at chromosome 7q35 and the gamma locus visualized at chromosome 7p14-15 along with partner genes MYC, TAL1, RBTN1,RBTN2, HOX, TAL1, LMO2, FBXW7, NOTCH1, CDKN2A, IL7R, PHF6 TLX1, WT1, MYB & PTEN genes. A whole body Computerized Tomography (CT) scan of head & neck, thorax, abdomen and pelvis or a positron emission tomography (PET-CT) scan is mandated for disease staging and assessment of therapeutic response. An unfavourable outcome in adults is seen with a) age >30 years. b) locally advanced, progressive disease stage III or IV. c) elevated values of serum Lactate Dehydrogensae (LDH) beyond 1.5 times the upper normal limit. d) a central nervous system incrimination. e) malignant cells infiltrating the bone marrow or mediastinum. Distinct therapeutic phases elucidated are the Induction, Consolidation and Maintenance phase.