Clinical characteristics of Plasmablastic Lymphoma
Plasmablastic Lymphoma (PBL) is an aggressive B- cell lymphoma, usually associated with immunocompromised conditions.
Materials and Methods/Case Presentation/Objective:
A retrospective study
Results/Description/Main Outcome Measure(s):
Out of 11 patients, male and female were 7(63.6%) and 4 (36.3%) respectively with median age 40 years (IQR 20-48). Retrovirus positive cases were 3/11 (27.2%) patients, one of them being hepatitis B carrier also. Mass in the head and neck region was the most common presentation 6/11 (54.5%) patients followed by abdominal lump 4/11 (36.3%) cases. One patient presented with pain and swelling in left arm. B symptoms were seen in 4/11 (36.3%) patients. At presentation, hematological parameters were within normal range. Biochemical parameters like liver and kidney function tests were normal except serum LDH 355 IU/L (IQR 271-566, Ref range 105-333 IU/L). Bone marrow (BM) infiltration by plasma cells was seen in 4/11 (36.3%) of cases and all of them had mass in head and neck region at presentation. Histopathology of tumor tissue biopsy from all the cases showed infiltration by large atypical lymphoid cells with plasmacytoid morphology, positive for CD138, MUM1 and negative for CD20 (except in one case). The most common therapeutic regimen was VPD and Bortezomib. Based on poor clinical condition of the patients, 3/11 (27.2%) patients were given dose adjusted EPOCH, 2/11(18.18%) BFM 90 and 2/11(18.1%) R- CHOP protocol. Single patient was lost to follow up before starting treatment. Following VPD & Bortezomib, 2 patients relapsed and they were given ICE regimen. Only 1 patient had died in the duration of follow up, the median follow up being 5 months.
Conclusion(s).:
PBL is not uncommon in immunocompetent patients, that most commonly presents with oral cavity, GIT or skin involvement. Generalized lymphadenopathy is rare. There is no standardized treatment protocol. In HIV positive patients DA-EPOCH is the first line therapy. The median overall survival of PBL patients is 6-19 months.
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