Challenges and Opportunities in Non-Hodgkin Lymphoma
SEEMA DEVI
Abstract
Patients presented with soft tissue lesions without any β symptoms can be a case of Primary extranodal Non- non-Hodgkins lymphoma. According to the World Health Organization classification system, there are two types of Lymphoma with various sub-types of Hodgkins Lymphoma and Non-Hodgkin Lymphoma are classified to assess the disease burden Progression and metastasis Imaging modalities are helpful. Commonest site involvement in Non-Hodgkin Lymphoma in the gastrointestinal tract (44%) followed by Head and Neck and may Involve bone (8%) and Central Nervous System 5%. Diffuse large β-Cell Lymphoma is the most common subtype representing 30%-35% of all Non-Hodgkin Lymphoma Cases. Oral and Para oral sites were involved in 2.5% of the cases. A 27-year-old Male noticed a swelling in the flank which was progressively Increasing in size one year back later it was associated with pain which reduced mobility and pain was increased during walking. Due to the advancement of diagnostic techniques, it is easy to get the radiological picture and correlation with clinical presentation and pathological reports, but these facilities are not available at various centers. Usually, patients are present in very advanced stages because of a lack of awareness about the disease, and how to proceed for diagnosis and treatment resulting in poor outcomes for these patients. This presentation and clinical funding are rare in the case of Lymphoma. This case report allows us to think of another diagnosis rather than usual.
Mounia Bendari
Hematology department, Cheikh Khalifa International University Hospital, Casablanca; Faculty of Medicine, Mohammed VI University of Sciences and Health, Casablanca, Morocco. Research Entity, Mohammed VI Center for Research & Innovation, Rabat, Morocco.
Abderrahmane Elbouzidi
Hematology department, Cheikh Khalifa International University Hospital, Casablanca; Faculty of Medicine, Mohammed VI University of Sciences and Health, Casablanca, Morocco. Research Entity, Mohammed VI Center for Research & Innovation, Rabat, Morocco.
Mariam Ahnach
Hematology department, Cheikh Khalifa International University Hospital, Casablanca; Faculty of Medicine, Mohammed VI University of Sciences and Health, Casablanca, Morocco. Research Entity, Mohammed VI Center for Research & Innovation, Rabat, Morocco.
Said Benchekroun
Pathology department, Cheikh Khalifa International University Hospital, Casablanca; Faculty of Medicine, Mohammed VI University of Sciences and Health, Casablanca, Morocco. Research Entity, Mohammed VI Center for Research & Innovation, Rabat, Morocco.
Abstract
Background: Mantle cell lymphoma (MCL) is a distinct subtype of Non-Hodgkin Lymphoma (NHL), it affects 5 to 8% of NHL, it is a clinically heterogeneous disease occurring within a heterogeneous patient population. The median age at time of diagnosis is over 65 years old. The incidence increased with age, and also affects man more than women with ratio of 3 to 1. Overall survivor is different according to subtype of lymphoma. Most patients present at advanced stage, and 30% present in leukemic phase.
Case report: We report a case of 60-year-old women with relapsed mantle cell lymphoma. The patient had no medical past history. The diagnosis of mantle cell lymphoma was made in 2019 revealed by anemic syndrome, the morphological and histological study of lymph node and bone marrow biopsy confirmed the mantle cell lymphoma with expression of CD19+, CD20+, CD5+, CD23-, Cyclin D1+, and KI 67 at 60%. The PET Scan showed FDG-avid disease, the laboratory studies remarques for high level of LDH, elevated B2 microglobulin. The patient underwent R-DHAP regimen (Rituximab, Aracytine, Cisplatin, prednisone) and achieved complete remission after 2 curses, the PET Scan was negative, and the bone marrow biopsy was normal, the collect of hematopoietic stem cell was performed, and the patients received 2 other courses of RDHAP regimen. She does not benefit from autologous hematopoietic stem cell transplant (HSCT) because of severe infection of COVID19. In reality, patient had a server pneumoniae with chronic fever, she was admitted for intravenous antibiotics, all biological exam showed a high level of C- reactive protein, and high level of d-dimers, but no sign of relapse was found. The patient still febrile during 6 months. Autologous hematopoietic stem cell transplant was not realized, but she received maintenance treatment with Rituximab. One year later, the patient developed a mass under the knee with deep deterioration of general condition, relapse was suspected, the surgical biopsy was performed and histological study confirmed the relapse of mantle cell lymphoma. The aim of this case report is to describe the difficulties to manage mantle cell lymphoma associated to covid 19 infection and report the consequences of therapeutic decision.
Conclusion: Not all mantle cell lymphoma is the same, it is crucial to identify patients appropriate for aggressive treatment, autologous hematopoietic stem cell transplant improves event free survival with unclear benefit in all patients, maintenance Rituximab improves overall survivor. Minimal residual disease may guide future therapeutic decisions and help physicians manage these patients. Our patient does not receive autologous hematopoietic stem cell transplant and suffers from chronic Covid19 infection. This association is complicated for the physician and the difficulties of access to targeted therapy in our country limits the therapeutic proposals for patients in relapse.
A Retrospective Study of 341 Cases of Primary Extranodal Non-Hodgkin Lymphoma of the Head and Neck
Fatima Ezzahra Rizkou
ENT-HNS Department, Mohammed VI University Hospital Center, Marrakech, Morocco.
Yassine Jaouhari, Youssef Lakhdar, Mohammed Chehbouni, Omar Oulghoul, Youssef Rochdi & Abdelaziz Raji
ENT and Neck and Head surgery Department; University medical center Mohammed VI. Marrakech, Morocco.
Othmane Benhoummad
ENT and Neck and Head surgery Department, University hospital of Agadir, Morocco.
Mohamed Ilias Tazi
Hematology department; University medical center Mohammed VI. Marrakech, Morocco.
Ali Bouddounit & Hanane Rais
Anatomical Pathology department, University medical center Mohammed VI. Marrakech, Morocco.
Abstract
Aims: Our goal was to obtain comprehensive and accurate information about primary extranodal non-Hodgkin lymphomas of the head and neck region to contribute to the advancement of medical knowledge in this field.
Materiels and methods: We conducted a retrospective study of 341 patients with primary extra-nodal, non-Hodgkin lymphomas of the head and neck, over a period of 7 years from January 2016 to December 2022, in the departments of ENT and Head and Neck Surgery and Hematology, with the help of the department of anatomical pathology of the university hospital Mohammed VI of Marrakech.
Results: 341 patients, with primary extra-nodal, non-Hodgkin lymphomas of the head and neck, was included, with an average age of 57 years, and a sex ratio male/female of 2.21 . in more than half of the cases; Waldeyer’s ring was concerned; especially palatine tonsils. Type B NHL was the most frequent and involved 308 patients. Diffuse large B-cell lymphoma was the commonly observed histological type, found in 214 patients; followed by a Follicular Lymphoma and Extra-Nodal NK/T‑Cell Lymphoma, Nasal Type. Following the extension workup; patients were all staged according to the ANN ARBOR classification; with a 77.8% of them were localized stages I and II, and of the therapeutically evaluable cases, complete remission was achieved in 112 patients.
Conclusion: Our study focused on all extra-nodal localizations of NHL in the head and neck region. Due to the very heterogeneous nature of these tumors, most of the current studies are limited to a specific site of involvement. Nevertheless, our study provides an overall picture; which those specific studies can be based. The management of NHL has evolved considerably in recent years, insisting on the multidisciplinary character to a better management of these patients.
María Alejandra Deu, María Ceciclia Bertone, Luisina Belén Peruzzo, Carla Luciana Pennella, Cristian Germán Sanchez La Rosa, Myriam Ruth Guitter, Elizabeth Melania Alfaro, Pedro Zubizarreta & María Sara Felice
Pediatric Hematology Oncology Department, Hospital de Pediatría “Prof. Dr. Juan P. Garrahan”, Buenos Aires C1245, Argentina.
Abstract
Spinal cord compression (SCC) is an unusual manifestation of leukemias and lymphomas in children and defines an oncological emergency frequently unsuspected, being a cause of severe sequelae. Our aim was to analyze the characteristics of patients who presented signs or symptoms of spinal cord compression in early phases of malignant hematopoietic diseases. From November-1988 to July-2022, 3878 patients with leukemia and lymphoma were diagnosed. Of them, 36 children (0.92%) presented spinal cord compression signs/symptoms in early phases of their diagnosis: Acute Lymphoblastic Leukemia (n=18), Acute Myeloblastic Leukemia and Myeloid Sarcoma (n=7), Non-Hodgkin Lymphomas (n=9) and Hodgkin Lymphoma (n=2). Clinical characteristics, images and hematological findings, treatment strategies, results and sequelae were analyzed. Sex distribution was 3.5/1 (M/F) and the media age at diagnosis was 10 (range: 4.9-16.9) years. The most common symptoms were back pain (34/36), functional impotence (27/36) and sphincter compromise (10/36). The media time from symptom onset to diagnosis was 47,5 (range: 0-300) days. Magnetic resonance imaging was performed on 33 (92%) patients and showed epidural mass (n=16) or vertebral collapse (n=17) in all of them. Two patients received initial radiotherapy and 11 decompressive surgeries for the management of the urgency spinal cord compression. Bone marrow aspiration was the diagnostic procedure in 69% of cases. All patients received chemotherapy and 94% achieved complete remission. Severe sequelae were observed in 10 patients (paraplegia with neurogenic bladder and kyphoscoliosis). Leukemia and lymphoma should be considered as a differential diagnosis when spinal cord compression is suspected, and magnetic resonance imaging is the mandatory study to confirm this diagnosis as a matter of urgency. Bone marrow involvement was evident due to hematological alterations in 95% of cases allowing to guide the diagnosis and initiate treatment early to reduce sequelae.
Joshua ML Casan & John F Seymour
The Department of Clinical Haematology, The Royal Melbourne Hospital and Peter MacCallum Cancer Centre, Australia; Sir Peter MacCallum Department of Oncology, The University of Melbourne, Australia
Mary Ann Anderson
The Department of Clinical Haematology, The Royal Melbourne Hospital and Peter MacCallum Cancer Centre, Australia; The Division of Blood Cells and Blood Cancer, The Walter and Eliza Hall Institute, Australia
Abstract
Mantle cell lymphoma (MCL) is a rare B-cell non-Hodgkin lymphoma, and remains a clinically challenging disease entity, particularly in the relapsed setting where outcomes are poor. However, recent innovations in targeted therapeutics have expanded treatment options and demonstrate significant efficacy even in relapsed disease. MCL frequently harbours aberrations of apoptosis pathways including over-expression of the anti-apoptotic protein BCL2. Such aberrancy promotes and sustains lymphomagenesis, thus rendering MCL an attractive target for venetoclax, the highly specific, orally bioavailable inhibitor of BCL2. Pre-clinical and early clinical data of venetoclax monotherapy demonstrated high response rates in relapsed/refractory MCL, though the durability of response in high-risk patients appears modest. More recently, clinical trials deploying combination strategies that pair venetoclax with other novel agents have been undertaken, with some promising early data reported. In this article, we review the biological rationale for deploying venetoclax in MCL, as well as the emerging data from clinical trials of venetoclax monotherapy and novel combinations.
David Tucker
Royal Cornwall Hospital NHS Trust
Cristina M. Thiebaud
Abstract
The management of non-Hodgkin lymphoma (NHL), including refractory and relapsed high grade and low-grade NHL has been significantly improved in recent years with the development of cellular therapies which harness the powerful anti-cancer effects of the immune system. These include the ground-breaking and now established technology of chimeric antigen receptor cell therapy as well as the promising new range of bispecific monoclonal antibody therapies. This article will give a summary of the currently available cellular and bi-specific antibody therapies for the treatment of NHL in licenced use and clinical trials, including an overview of their proven efficacy and characteristic side-effect profiles which distinguish them from conventional immunochemotherapy. The relative strengths and weaknesses of these comparable therapies will also be discussed together with consideration of where they may fit into the treatment sequence of NHL in the future. The article will also address the challenges of delivering these innovative technologies in different healthcare settings and how they may alter the future of therapy for patients with this form of cancer.
