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      Acute Laryngeal Dyspnea as the First Presentation of Mantle Cell Lymphoma: A Case Report and Review of the Literature

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          Abstract

          Introduction

          Acute laryngeal dyspnea is a life-threatening emergency, and the causes in adults are most often laryngeal tumors or inflammatory edema. Lymphoma of the larynx and especially the mantle cell type is extremely rare. Case Presentation. We report a case of a 43-year-old woman with no particular pathological history. She presented with progressive dyspnea which has evolved towards an inspiratory bradypnea that worsened until she ultimately required an emergency tracheotomy. Biopsies revealed mantle cell lymphoma. The patient has been staged IVB MIPI 6, and she was treated by immunochemotherapy followed by ASCT. The therapeutic evaluation shows a complete remission, 18 months after, and the patient was always disease free.

          Conclusion

          The laryngeal localization of the mantle cell lymphoma is extremely rare; it may present catastrophically with acute airway obstruction. The diagnosis is mostly histological, hence the interest of deep biopsy. Given its rarity, the therapeutic strategy must be discussed case by case in a multidisciplinary consultation meeting.

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          Mantle cell lymphoma: a retrospective study of 121 cases.

          Fadhela F Bouafia-Sauvy, C Thieblemont, I. Moullet … (1998)
          Mantle cell lymphoma (MCL) patients represent a difficult problem, sometimes to establish the diagnosis but mostly because of their refractoriness to standard lymphoma treatments. Which treatments to apply and to whom is not yet defined. In this study, we attempted to analyze the clinical features, to identify the major prognostic factors, and to evaluate the outcome of 121 MCL patients treated in our institution between 1979 and 1997. Clinical data, treatment modalities, and International Prognostic Index (IPI) score were evaluated. Median age was 63 years. Patients usually presented with advanced stage disease (87%), disseminated lymph nodes (57%), bone marrow involvement (79%), but with a good performance status (PS) (81%). Lymphocytosis >4000/microl and/or peripheral blood involvement was present in 36% of cases, and gastrointestinal disease in 18%. The t(11;14)(q13;q32) and/or bcl-1 rearrangement was detected in 47/57 studied cases. Median overall survival (OS) was 3.12 years and a longer survival was significantly associated with younger age (<70 years), good PS (<2), localized disease (stage I-II), fewer than two extra-nodal sites, absence of spleen or peripheral blood involvement, normal serum LDH and beta2-microglobulin levels, and hemoglobin level greater than 12 g/dl. However, the IPI failed to identify patients with longer OS and in a multiparametric analysis, only older age, hemoglobin less than 12 g/dl, poor PS, and blood involvement were associated with a poorer outcome. Treatment modalities had no impact on survival with 75% of patients relapsing or progressing. Our data showed that the poor outcome of MCL patients is mainly related to adverse patient characteristics, a highly disseminated tumor, and some unknown parameters associated with the refractoriness to standard therapy.
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            Primary laryngeal lymphoma: report of 3 cases and review of the literature.

            Konstantinos Markou, J Constantinidis, Ioannis Kostopoulos … (2010)
            Extranodal lymphomas limited to the larynx are rare, accounting for less than 1% of all laryngeal neoplasms. The aim of this study was to report the experience of our department in the management of these aggressive lesions, as they require special diagnostic and therapeutic attention. The case records of 3 patients with the diagnosis of lymphoma involving the larynx were retrospectively reviewed. The histopathological diagnosis revealed 1 case of marginal zone lymphoma mucosa-associated lymphoid tissue type, 1 case of T-lymphoblastic lymphoma, and 1 case of a rare coexistence of in situ squamous cell carcinoma with an isolated intravascular (angioimmunoblastic) lymphoma of peripheral T-cell origin. Details of the presentation, diagnostic procedures, treatment, and outcome of these patients were presented. Primary laryngeal lymphoma is a rare entity. Early symptoms are subtle and nonspecific, and confirmation of the diagnosis is often difficult. Because of the rarity of this tumor type, the optimal management remains controversial and it seems that should be managed not as a distinct disease entity but as an unusual presentation of non-Hodgkin lymphoma, according to the recent treatment trends.
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              Involvement of the larynx by hemopoietic neoplasms. An investigation of autopsy cases and review of the literature.

              H-P Horny, E Kaiserling (1995)
              Involvement of the larynx by hemopoietic tumors is generally considered a rare event and little is known about the associated clinicopathologic features. Laryngeal tissue removed at autopsy from 14 patients with known disseminated hematologic malignancies and at operation from one patient with multicentric malignant lymphoma of low-grade malignancy (MALToma) of the head and neck region was investigated. A systematic survey of the main clinicopathologic features of the published cases of hemopoietic tumors with laryngeal involvement was also performed. Primary involvement of the larynx by hemopoietic neoplasms must be clearly distinguished from secondary involvement by disseminated or leukemic tumors. Most of the primary tumors are localized lesions that may involve the regional lymph nodes (stages IE or IIE). Radiotherapy is the treatment of choice, and the prognosis is generally favorable. However, secondary involvement by disseminated or leukemic disease carries a very poor prognosis in most cases. Extramedullary plasmacytoma and non-Hodgkin's lymphoma (NHL), particularly B-cell lymphoma of high-grade malignancy, appear to be the most common hemopoietic tumors with primary laryngeal involvement, while primary tumors of myelogenous origin (granulocytic sarcoma and mast cell sarcoma) are extremely rare. Extramedullary plasmacytoma and NHL occur mainly in older persons and in men, are generally associated with a relatively short history of hoarseness and dysphagia, and exhibit preferential involvement of the supraglottic parts of the larynx, in particular the epiglottis and aryepiglottic folds. They are generally polypoid, non-ulcerated lesions.(ABSTRACT TRUNCATED AT 250 WORDS)
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                Author and article information

                Contributors
                Mounir Ababou:
                ORCID: https://orcid.org/0000-0001-5789-6088
                Journal
                Journal ID (nlm-ta): Case Rep Hematol
                Journal ID (iso-abbrev): Case Rep Hematol
                Journal ID (publisher-id): CRIHEM
                Title: Case Reports in Hematology
                Publisher: Hindawi
                ISSN (Print): 2090-6560
                ISSN (Electronic): 2090-6579
                Publication date Collection: 2020
                Publication date (Electronic): 9 September 2020
                Volume: 2020
                Electronic Location Identifier: 8818270
                Affiliations
                1Department of Clinical Hematology, Military Hospital Mohammed V, Mohammed V University, Rabat, Morocco
                2Faculty of Medicine and Pharmacy of Rabat, Mohammed V University, Rabat, Morocco
                Author notes

                Academic Editor: Kostas Konstantopoulos

                Author information
                https://orcid.org/0000-0001-5789-6088
                Article
                DOI: 10.1155/2020/8818270
                PMC ID: 7499331
                PubMed ID: 32963850
                SO-VID: 020f8e81-8d8c-470f-b7b1-e8961f71e922
                Copyright © Copyright © 2020 Mounir Ababou et al.
                License:

                This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 3 July 2020
                Date revision received : 23 August 2020
                Date accepted : 2 September 2020
                Categories
                Subject: Case Report

                Data availability:

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