Introduction: Primary mediastinal large B cell lymphoma is a rare highly aggressive form of non-Hodgkin lymphoma and occurs in 5-7% of all diffuse large B lymphoma and 2% of all non-Hodgkin lymphomas. Originates from thymic medullary cells and has its own histogenesis. Typically affects young women in the third and fourth decade of life. Superior vena cava syndrome is present in more than 50% of cases, with swelling of face, hands, deep venous thrombosis, dyspnea, dysphagia, chest pain, headache and cough. Diagnosis is made by histopathological analysis and immunohistochemical surgical biopsy specimens obtained mediastinoscopy. Timely treatment with cycles of monoclonal antibody and chemotherapy is requirement to relapse and stable state.
Case outline: Patient, 37 years old, came for the first time for review at General practice because she has been repeatedly visited emergency service due to coughing, feeling that something is strangling, the lack of air pressure and pain in the head and swelling of face and neck. Started detailed diagnostics. Radiologically determined mediastinal tumor, computed tomography confirmed it and sent to the referral Institute where video-assisted thoracic surgery and patho histology diagnosed PMBCL CSII AM +. Administered six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone therapy protocols submitted by the expected side effects. Control positron emission tomography found that the tumor mass completely withdrawn. In remission for two years, have a good feeling and occurs at regular checkups.
Conclusion: General practice has a big responsibility in recognizing early symptoms of malignant disease that accelerates the diagnosis and timely start of treatment.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues.
2.
Bhatt VR, Mourya R, Shrestha R, Armitage JO. Primary mediastinal large B-cell lymphoma. Vol. 41, Cancer Treat Rev. p. 476–85.
3.
Traverse-Glehen A, Verney A, Gazzo S. Splenic diffuse red pulp lymphoma has a distinct pattern of somatic mutations amongst B-cell malignancies. Vol. 58, Leuk Lymphoma. p. 666–75.
4.
Isaacson PG, Norton AJ, Addis BJ. The human thymus contains a novel population of B lymphocytes. Vol. 2, Lancet. p. 1488–91.
5.
Zinzani PL, Martelli M, Poletti V. Practice guidelines for the management of extranodal non-Hodgkin’s lymphomas of adult non-immunodeficient patients. Part I: primary lung and mediastinal lymphomas. In: A project of the Italian Society of Hematology, the Italian Society of Experimental Hematology, and the Italian Group for Bone Marrow Transplantation Haematologica. p. 1364–71.
6.
Pileri SA, Gaidano G, Zinzani PL. Primary mediastinal B-cell lymphoma: high frequency of BCL-6 mutations and consistent expression of the transcription factors OCT-2, BOB.1, and PU.1 in the absence of immunoglobulins. Vol. 162, Am J Pathol. p. 243–53.
7.
The International Non-Hodgkin’s Lymphoma Prognostic Factors Project. A predictive model for aggressive non-Hodgkin’s lymphoma. Vol. 329, N Engl J Med. p. 987–94.
8.
Sehn LH, Berry B, Chhanabhai M. The revised International Prognostic Index (RIPI) is a better predictor of outcome than the standard IPI for patients with diffuse large B-cell lymphoma treated with R-CHOP. Vol. 109, Blood. p. 1857–61.
9.
Hamlin PA, Zelenetz AD, Kewalramani T. Age-adjusted International Prognostic Index predicts autologous stem cell transplantation outcome for patients with relapsed or primary refractory diffuse large B-cell lymphoma. Vol. 102, Blood. p. 1989–96.
10.
Shipp MA. Prognostic factors in aggressive non-Hodgkin’s lymphoma: who has “high-risk” disease? Vol. 83, Blood. p. 1165–71.
11.
Hans CP, Weisenburger DD, Greiner TC. Confirmation of the molecular classification of diffuse large B-cell lymphoma by immunohistochemistry using a tissue microarray. Vol. 103, Blood. p. 275–82.
12.
Green MR, Monti S, Rodig SJ. Integrative analysis reveals selective 9p24.1 amplification, increased PD-1 ligand expression, and further induction via JAK2 in nodular sclerosing Hodgkin lymphoma and primary mediastinal large B-cell lymphoma. Vol. 116, Blood. p. 3268–77.
13.
Weniger MA, Gesk S, Ehrlich S. Gains of REL in primary mediastinal B-cell lymphoma coincide with nuclear accumulation of REL protein. Vol. 46, Genes Chromosomes Cancer. p. 406–15.
14.
Twa DD, Chan FC, Ben-Neriah S. Genomic rearrangements involving programmed death ligands are recurrent in primary mediastinal large B-cell lymphoma. Vol. 123, Blood. p. 2062–5.
15.
Sehn LH, Gascoyne RD. Diffuse large B-cell lymphoma: optimizing outcome in the context of clinical and biologic heterogeneity. Vol. 125, Blood. p. 22–32.
16.
Dunleavy K, Pittaluga S, Maeda LS. Dose-adjusted EPOCH-rituximab therapy in primary mediastinal B-cell lymphoma. Vol. 368, N Engl J Med. p. 1408–16.
17.
Minard-Collin V, Brugleres L, Reiter A. Non-Hodgkin Lymphoma in Children and Adolescents: Progress Through Effective Collaboration, Current Knowledge, and Challenges Ahead. Vol. 33, J Clin Oncol. p. 2963–74.
18.
Woessmann W, Lisfeld J, Burkhardt B, Group NFILBFMS. Therapy in primary mediastinal B-cell lymphoma. Vol. 369, N Engl J Med. p. 282–3.
19.
Aoki T, Izutsu K, Suzuki R. Prognostic significance of pleural or pericardial effusion and the implication of optimal treatment in primary mediastinal large B-cell lymphoma: a multicenter retrospective study in Japan. Vol. 99, Haematologica. p. 1817–25.
20.
Ceriani L, Martelli M, Zinzani PL. Utility of baseline 18FDG-PET/CT functional parameters in defining prognosis of primary mediastinal (thymic) large B-cell lymphoma. Vol. 126, Blood. p. 950–6.
21.
Zinzani PL, Martelli M, Magagnoli M. Treatment and clinical management of primary mediastinal large B-cell lymphoma with sclerosis: MACOP-B regimen and mediastinal radiotherapy monitored by Gallium scan in 50 patients. Vol. 94, Blood. p. 3289–93.
22.
Savage KJ, Al-Rajhi N, Voss N. Favorable outcome of primary mediastinal large B-cell lymphoma in a single institution: the British Columbia experience. Vol. 17, Ann Oncol. p. 123–30.
The statements, opinions and data contained in the journal are solely those of the individual authors and contributors and not of the publisher and the editor(s). We stay neutral with regard to jurisdictional claims in published maps and institutional affiliations.