Global InformationPlasmablastic lymphoma information
Plasmablastic lymphoma (PBL) is a type of large B-cell lymphoma recognized by the World Health Organization (WHO) in 2017 as belonging to a subgroup of lymphomas termed lymphoid neoplasms with plasmablastic differentiation. The other lymphoid neoplasms within this subgroup are: plasmablastic plasma cell lymphoma (or the plasmacytoma variant of this disease); primary effusion lymphoma that is Kaposi's sarcoma-associated herpesvirus positive or Kaposi's sarcoma-associated Herpesvirus negative; anaplastic lymphoma kinase-positive large B-cell lymphoma; and human herpesvirus 8-positive diffuse large B-cell lymphoma, not otherwise specified. All of these lymphomas are malignancies of plasmablasts, i.e. B-cells that have differentiated into plasmablasts but because of their malignant nature: fail to differentiate further into mature plasma cells; proliferate excessively; and accumulate in and injure various tissues and organs.[1]
The lymphomas in the lymphoid neoplasms with plasmablastic differentiation sub-group that are not PBL have sometimes been incorrectly considered to be variants of PBL. Each of the lymphomas in this subgroup of malignancies have distinctive clinical, morphological, and abnormal gene features. However, key features of these lymphomas sometime overlap with other lymphomas including those that are in this sub-group. In consequence, correctly diagnosing these lymphomas has been challenging.[2] Nonetheless, it is particularly important to diagnose them correctly because they can have very different prognoses and treatments than the lymphomas which they resemble.[1]
Plasmablastic lymphomas are aggressive and rare malignancies that usually respond poorly to chemotherapy and carry a very poor prognosis. They occur predominantly in males who have HIV/AIDS, had a solid organ transplant, or are immunosuppressed in other ways; ~5% of all individuals with PBL appear to be immunocompetent, i.e. to have no apparent defect in their immune system.[2] The malignant plasmablasts in more than half the cases of PBL are infected with a potentially cancer-causing virus, Epstein–Barr virus (EBV), and rare cases of PBL appear due to the plasmablastic transformation of a preexisting low-grade B-cell lymphoma.[3] One variant of PBL, sometimes termed plasmablastic lymphoma of the elderly, has a significantly better prognosis than most other cases of PBL.[4] The development of this variant appears due, at least in part, to immunosenescence, i.e. the immunodeficiency occurring in old age.[3]
- ^ a b Chen BJ, Chuang SS (March 2020). "Lymphoid Neoplasms With Plasmablastic Differentiation: A Comprehensive Review and Diagnostic Approaches". Advances in Anatomic Pathology. 27 (2): 61–74. doi:10.1097/PAP.0000000000000253. PMID 31725418.
- ^ a b Lopez A, Abrisqueta P (2018). "Plasmablastic lymphoma: current perspectives". Blood and Lymphatic Cancer: Targets and Therapy. 8: 63–70. doi:10.2147/BLCTT.S142814. PMC 6467349. PMID 31360094.
- ^ a b Korkolopoulou P, Vassilakopoulos T, Milionis V, Ioannou M (July 2016). "Recent Advances in Aggressive Large B-cell Lymphomas: A Comprehensive Review". Advances in Anatomic Pathology. 23 (4): 202–43. doi:10.1097/PAP.0000000000000117. PMID 27271843. S2CID 205915174.
- ^ Liu F, Asano N, Tatematsu A, Oyama T, Kitamura K, Suzuki K, Yamamoto K, Sakamoto N, Taniwaki M, Kinoshita T, Nakamura S (December 2012). "Plasmablastic lymphoma of the elderly: a clinicopathological comparison with age-related Epstein–Barr virus-associated B cell lymphoproliferative disorder". Histopathology. 61 (6): 1183–97. doi:10.1111/j.1365-2559.2012.04339.x. PMID 22958176. S2CID 205303461.