Mediastinal large B-cell lymphoma

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The Mediastinal large B-cell lymphoma (or primary mediastinal B-cell lymphoma , English Primary mediastinal B-cell lymphoma , short PMBCl) is a locally invasive tumor of the anterior mediastinum , which consists of a B-cell of the thymus marks apparent. Histologically, the cells are centroblast-like cells with a tendency to sclerosis. Clinically and biologically, mediastinal large B-cell lymphoma is similar to diffuse large B-cell lymphoma and nodular sclerosing Hodgkin lymphoma .

Epidemiology

Mediastinal large B-cell lymphoma is a very rare tumor with an estimated incidence of 0.4 / 1 million population. Women are four times more likely to be affected than men. The average age of onset is 30–40 years.

Symptoms

The symptoms of mediastinal large B-cell lymphoma arise from the displacement of organs in the chest cavity. These include shortness of breath and irritable cough when the lungs are displaced, swallowing disorders when pressure is applied to the esophagus and upper congestion when pressure is applied to the superior vena cava , which makes it difficult for blood to flow back to the heart from the head and neck area and arms.

therapy

Mediastinal large B-cell lymphoma is treated with a combination of rituximab (an anti-CD20 antibody), chemotherapeutic agents ( vincristine , cyclophosphamide , doxorubicin and possibly etoposide ) and prednisone , which may be followed by radiation therapy .

forecast

The 2-year survival rate after therapy is approximately 90%.

Individual evidence

  1. ↑ Maurizio Martelli, Andrés Ferreri, Alice Di Rocco, Michela Ansuinelli, Peter WM Johnson: Primary mediastinal large B-cell lymphoma . In: Critical Reviews in Oncology / Hematology . tape 113 , p. 318–327 , doi : 10.1016 / j.critrevonc.2017.01.009 ( elsevier.com [accessed March 22, 2018]).
  2. ↑ Kieron Dunleavy: Primary mediastinal B-cell lymphoma: biology and evolving therapeutic strategies . In: Hematology. American Society of Hematology. Education Program . tape 2017 , no. 1 , December 8, 2017, ISSN  1520-4383 , p. 298–303 , doi : 10.1182 / asheducation-2017.1.298 , PMID 29222270 .
  3. ↑ a b Herold, Gerd .: Internal Medicine: a lecture-oriented presentation: taking into account the subject catalog for the medical examination: with ICD 10 key in the text and index . 2016th edition. Herold, Cologne 2016, ISBN 978-3-9814660-5-8 .
  4. ↑ Nirav N. Shah, Timothy S. Fenske, Mehdi Hamadani, Jakub Svoboda: What is the standard of care for primary mediastinal B cell lymphoma; R-CHOP or DA-EPOCH-R? - Response to Melani et al . In: British Journal of Hematology . March 12, 2018, ISSN  1365-2141 , doi : 10.1111 / bjh.15183 , PMID 29528099 .
  5. ↑ Nirav N. Shah, Aniko Szabo, Scott F. Huntington, Narendranath Epperla, Nishitha Reddy: R-CHOP versus dose-adjusted R-EPOCH in front line management of primary mediastinal B-cell lymphoma: a multi-center analysis . In: British Journal of Hematology . tape 180 , no. 4 , February 2018, ISSN  1365-2141 , p. 534-544 , doi : 10.1111 / bjh.15051 , PMID 29265182 .