Primary mediastinal (thymic) large B cell lymphoma
August 26, 2023
Primary mediastinal (thymic) large B-cell lymphoma is a distinct type of diffuse large B-celllymphoma involving the mediastinum, recognized in the WHO 2008 classification.[1][2]: 370–374
Primary mediastinal (thymic) large B cell lymphoma
Other names
Mediastinal large B cell lymphoma, primary mediastinal large B-cell lymphoma (PMLBCL), mediastinal large B-cell lymphoma
PMLBCL arises from a putative thymic peripheral B cell.[3][4] It has several distinctive biological features.[3] Molecular analysis shows that PMLBCL is distinct from other types of diffuse large B-cell lymphomas (DLBCL).[4]MAL gene expression is seen in 70%, unlike other diffuse large B-cell lymphomas.[2]: 370 Gene expression profiling shows considerable variance from other DLBCLs and similarity to Hodgkin disease.[5]: 290–293
PMLBCL is CD20 positive, expresses pan-B markers including CD79a, and has clonal immunoglobulin gene rearrangements and mRNA but paradoxically does not express cytoplasmic or cell surface immunoglobulin.[2]: 370
Clinically, PMLBCL is unusual in several respects. Despite 80% PMLBCL being stage I or II, the presenting anterior mediastinal mass is often over 10 cm and is locally invasive of lung, chest wall, pleura, and pericardium.[3] At initial presentation, PMLBCL is usually confined to mediastinum, but its bulk, rather than additional adenopathy, can sometimes be palpated at the low neck.[3] Increased LDH is seen in approximately 75%,[2]: 370 [3] but unlike other large cell lymphomas, no increase in beta-2 microglobulin is seen even when bulky[2]: 370 which may relate to defective major histocompatibility complex expression.[2]: 370
TreatmentEdit
Multiagent chemotherapy is recommended, but the preferred regimen is controversial, as is consolidative radiotherapy.[3][6][7][8]
EpidemiologyEdit
It affects primarily young adults; the median age is 37 years.[2]: 370 It is more common in females.[3]
Grey zone lymphomaEdit
This section needs expansion. You can help by adding to it. (March 2021)
^Swerdlow, Steven H.; International Agency for Research on Cancer; World Health Organization (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. World Health Organization classification of tumours. Vol. 2 (4th ed.). International Agency for Research on Cancer. ISBN9789283224310.
^ abcdefgJaffe, ES; Harris NL; Vardiman JW; Campo E; Arber DA (2011). Hematopathology (1st ed.). Elsevier Saunders. ISBN9780721600406.
^ abMartelli M, Di Rocco A, Russo E, et al. (2015). "Primary mediastinal lymphoma: diagnosis and treatment options". Expert Rev Hematol. 8 (2): 173–86. doi:10.1586/17474086.2015.994604. hdl:11573/780924. PMID 25537750. S2CID 23040185.
^Sweetenham, J. Lymphomas (Emerging Cancer Therapeutics V3 I2). Demos. November 2, 2012. ISBN9781936287789
^Faris JE, LaCasce AS (2009). "Primary mediastinal large B-cell lymphoma". Clin Adv Hematol Oncol. 7 (2): 125–33. PMID 19367254.
^Dabrowska-Iwanicka A, Walewski JA (2014). "Primary mediastinal large B-cell lymphoma". Curr Hematol Malig Rep. 9 (3): 273–83. doi:10.1007/s11899-014-0219-0. PMC4180024. PMID 24952250.
^Hutchinson CB, Wang E (2011). "Primary mediastinal (thymic) large B-cell lymphoma: a short review with brief discussion of mediastinal gray zone lymphoma". Arch. Pathol. Lab. Med. 135 (3): 394–8. doi:10.5858/2009-0463-RSR.1. PMID 21366467.
^Grant C, Dunleavy K, Eberle FC, et al. (2011). "Primary mediastinal large B-cell lymphoma, classic Hodgkin lymphoma presenting in the mediastinum, and mediastinal gray zone lymphoma: what is the oncologist to do?". Curr Hematol Malig Rep. 6 (3): 157–63. doi:10.1007/s11899-011-0090-1. PMC6324553. PMID 21590365.
External linksEdit
August 26, 2023
primary, mediastinal, thymic, large, cell, lymphoma, primary, mediastinal, thymic, large, cell, lymphoma, distinct, type, diffuse, large, cell, lymphoma, involving, mediastinum, recognized, 2008, classification, other, namesmediastinal, large, cell, lymphoma, . Primary mediastinal thymic large B cell lymphoma is a distinct type of diffuse large B cell lymphoma involving the mediastinum recognized in the WHO 2008 classification 1 2 370 374 Primary mediastinal thymic large B cell lymphomaOther namesMediastinal large B cell lymphoma primary mediastinal large B cell lymphoma PMLBCL mediastinal large B cell lymphomaSpecialtyHematology oncology Contents 1 Signs and symptoms 2 Pathophysiology 3 Treatment 4 Epidemiology 5 Grey zone lymphoma 6 See also 7 References 8 External linksSigns and symptoms EditSuperior vena cava syndrome occurs in 30 50 and pleural or pericardial effusions occur in about one third 3 Pathophysiology EditPMLBCL arises from a putative thymic peripheral B cell 3 4 It has several distinctive biological features 3 Molecular analysis shows that PMLBCL is distinct from other types of diffuse large B cell lymphomas DLBCL 4 MAL gene expression is seen in 70 unlike other diffuse large B cell lymphomas 2 370 Gene expression profiling shows considerable variance from other DLBCLs and similarity to Hodgkin disease 5 290 293 PMLBCL is CD20 positive expresses pan B markers including CD79a and has clonal immunoglobulin gene rearrangements and mRNA but paradoxically does not express cytoplasmic or cell surface immunoglobulin 2 370 Clinically PMLBCL is unusual in several respects Despite 80 PMLBCL being stage I or II the presenting anterior mediastinal mass is often over 10 cm and is locally invasive of lung chest wall pleura and pericardium 3 At initial presentation PMLBCL is usually confined to mediastinum but its bulk rather than additional adenopathy can sometimes be palpated at the low neck 3 Increased LDH is seen in approximately 75 2 370 3 but unlike other large cell lymphomas no increase in beta 2 microglobulin is seen even when bulky 2 370 which may relate to defective major histocompatibility complex expression 2 370 Treatment EditMultiagent chemotherapy is recommended but the preferred regimen is controversial as is consolidative radiotherapy 3 6 7 8 Epidemiology EditIt affects primarily young adults the median age is 37 years 2 370 It is more common in females 3 Grey zone lymphoma EditThis section needs expansion You can help by adding to it March 2021 In between CHD and NHL 3 6 9 10 See also EditLymphoma Diffuse large B cell lymphomaReferences Edit Swerdlow Steven H International Agency for Research on Cancer World Health Organization 2008 WHO classification of tumours of haematopoietic and lymphoid tissues World Health Organization classification of tumours Vol 2 4th ed International Agency for Research on Cancer ISBN 9789283224310 a b c d e f g Jaffe ES Harris NL Vardiman JW Campo E Arber DA 2011 Hematopathology 1st ed Elsevier Saunders ISBN 9780721600406 a b c d e f g h i Armitage JO Mauch PM Harris NL et al 2010 Chapter 21 Non Hodgkin Lymphomas 2nd ed Lippincott Williams amp Wilkins ISBN 9780781791168 a b Martelli M Di Rocco A Russo E et al 2015 Primary mediastinal lymphoma diagnosis and treatment options Expert Rev Hematol 8 2 173 86 doi 10 1586 17474086 2015 994604 hdl 11573 780924 PMID 25537750 S2CID 23040185 Sweetenham J Lymphomas Emerging Cancer Therapeutics V3 I2 Demos November 2 2012 ISBN 9781936287789 a b http www nccn org professionals physician gls pdf nhl pdf bare URL PDF Faris JE LaCasce AS 2009 Primary mediastinal large B cell lymphoma Clin Adv Hematol Oncol 7 2 125 33 PMID 19367254 Dabrowska Iwanicka A Walewski JA 2014 Primary mediastinal large B cell lymphoma Curr Hematol Malig Rep 9 3 273 83 doi 10 1007 s11899 014 0219 0 PMC 4180024 PMID 24952250 Hutchinson CB Wang E 2011 Primary mediastinal thymic large B cell lymphoma a short review with brief discussion of mediastinal gray zone lymphoma Arch Pathol Lab Med 135 3 394 8 doi 10 5858 2009 0463 RSR 1 PMID 21366467 Grant C Dunleavy K Eberle FC et al 2011 Primary mediastinal large B cell lymphoma classic Hodgkin lymphoma presenting in the mediastinum and mediastinal gray zone lymphoma what is the oncologist to do Curr Hematol Malig Rep 6 3 157 63 doi 10 1007 s11899 011 0090 1 PMC 6324553 PMID 21590365 External links Edit Retrieved from https en wikipedia org w index php title Primary mediastinal thymic large B cell lymphoma amp oldid 1077174816, wikipedia, wiki, book, books, library,
