Blastoid variant mantle cell lymphoma
Part 5 -role of targeted therapies in management
MCL accounts for 3% to 10% of all lymphomas (1) and is more common in elderly men (male-to-female ratio of 2:1) with a median age of 60 years (range, 29 to 85 years) (14). In MCL, the function of genetic susceptibility is not well understood (15). According to epidemiological research, first-degree relatives of MCL patients have a two-fold increased risk of hematological neoplasms (16). Some patients have germline mutations in ATM and CHK2, however these genes are not involved in the few families with lymphoid neoplasms and MCL that have been studied (17).
Figure 1: A proposed molecular pathogenesis model for the development and progression of the major MCL subtypes. In mantle cell lymphoma, the function of SOX11 and genetic events cooperating with cyclin D1. Springer Nature has granted us permission to reprint this post (19). SOX11 stands for sex determining area Y-box 11. MCL stands for mantle cell lymphoma.
The histological features of MCL include a wide range of architectural and cytological variants that can lead to a misdiagnosis with other diseases and are linked to specific clinical and biological characteristics.
Mantle cell lymphoma | aggressive b-cell non-hodgkin’s
Lymphomas of the head and neck can cause a variety of symptoms. It can be difficult to make a reliable and timely diagnosis. Mantle cell lymphoma (MCL) blastoid variant accounts for less than one-third of all MCL cases. Isolated primary appearance on the palatine tonsils is unusual, and the prognosis and results tend to be grim. An 81-year-old man presented with odynophagia, dysphagia, and purulent exudate from obstructive hypertrophic palatine tonsils. Antibiotic treatment failed to cure the symptoms, so the tonsils were biopsied. The blastoid strain of MCL was diagnosed based on cellular morphology, immunophenotype, and genotype. Following staging, the patient received Rituximab-Bendamustine chemotherapy (R-Benda). For more than two years of treatment, the patient is in clinical remission. We present an exceptionally rare case of blastoid MCL that can be mistaken for tonsillitis. We go through the testing and treatment options for this rare cancer.
A class on non hodgkin’s lymphoma by dr vivek veeram
The NIDA/FDA research grant to JJP (P50 DA036107), the AA & MDSIF research grant to JJP (146818), the American Cancer Society research grant to JJP (124171-IRG-13-043-02), Paige’s Cancer Researcher Fund to JJP (Pu33860), and a SUNY Upstate Medical University research grant to JJP are all funding this report.
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Blastoid variant mantle cell lymphoma 2021
Mantle cell lymphoma (MCL) is a rare form of non-Hodgkin lymphoma that mostly affects men in their forties and fifties. MCL is responsible for around 1–9% of cases of lymphoma affecting the orbital and ocular adnexal areas. We identify the case of a 65-year-old man who presented with acute-onset binocular diplopia and proptosis of the left eye after a 24-year history of MCL with initial remission accompanied by numerous relapses. Following that, imaging showed a new left superior orbital mass. Biopsy of the mass revealed two clonally related but distinct cellular components: large cells that appeared to have transformed to the blastoid variant of MCL and clonally related but distinct cellular components showing the classical mantle cell morphology. It is unusual for classical MCL to turn into the blastoid variant of MCL, with just a few reports in the literature. MCL’s blastoid form is known for being violent and having a bad prognosis. The case we’re addressing is probably the first time MCL transformation has been observed in space.