All submissions of the EM system will be redirected to Online Manuscript Submission System. Authors are requested to submit articles directly to Online Manuscript Submission System of respective journal.
Journal of Blood Disorders and Treatment

Sign up for email alert when new content gets added: Sign up

Nodal, Haematological, Bicameral: Lymphoblastic lymphoma

Author(s): Anubha Bajaj, MD*

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.

Full-Text | PDF

+12 184512974

Recommended Conferences