Primary mediastinal (thymic) large B cell lymphoma

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Mediastinal large B cell lymphoma
Classification and external resources
Specialty Hematology and oncology
ICD-O 9679/3
Patient UK Primary mediastinal (thymic) large B cell lymphoma
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Primary mediastinal (thymic) large B-cell lymphoma, also called primary mediastinal large B-cell lymphoma (PMLBCL) and mediastinal 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

Biology

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. It affects primarily young adults; the median age is 37 years.[2]:370 It is more common in females.[3] 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] Superior vena cava syndrome occurs in 30-50%, and pleural or pericardial effusions occur in about one-third.[3] At initial presentation, PMLBCL is usually confined to mediastinum, but its bulk, rather than additional adenpathy, can sometimes may 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

Multiagent chemotherapy is recommended, but the preferred regimen is controversial, as is consolidative radiotherapy.[3][6][7][8]

Grey zone lymphoma

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"In-between CHD and NHL"[3][9][10][11]

See also

References

  1. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Lua error in package.lua at line 80: module 'strict' not found.
  3. ↑ 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 3.8 Lua error in package.lua at line 80: module 'strict' not found.
  4. ↑ 4.0 4.1 Lua error in package.lua at line 80: module 'strict' not found.
  5. ↑ Sweetenham, J. Lymphomas (Emerging Cancer Therapeutics V3 I2). Demos. November 2, 2012. ISBN 9781936287789
  6. ↑ http://www.nccn.org/professionals/physician_gls/pdf/nhl.pdf
  7. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  8. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  9. ↑ http://www.nccn.org/professionals/physician_gls/pdf/nhl.pdf
  10. ↑ Lua error in package.lua at line 80: module 'strict' not found.
  11. ↑ Lua error in package.lua at line 80: module 'strict' not found.
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