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Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 303-304
Pediatric plasmablastic lymphoma: Diagnostic and therapeutic dilemma

1 Laboratory Oncology Unit, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India
2 Department of Medical Oncology, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication19-Jun-2017

How to cite this article:
Misra A, Bakhshi S, Kumar R, Chopra A. Pediatric plasmablastic lymphoma: Diagnostic and therapeutic dilemma. Indian J Pathol Microbiol 2017;60:303-4

How to cite this URL:
Misra A, Bakhshi S, Kumar R, Chopra A. Pediatric plasmablastic lymphoma: Diagnostic and therapeutic dilemma. Indian J Pathol Microbiol [serial online] 2017 [cited 2022 Aug 9];60:303-4. Available from: https://www.ijpmonline.org/text.asp?2017/60/2/303/208388


Plasmablastic lymphoma (PBL) is an aggressive subtype of diffuse large B-cell lymphoma, characterized by proliferation of large neoplastic cells with immunoblastic/plasmacytic morphology.[1] Most patients present in advanced stage with frequent bone marrow (BM) and extranodal involvement, commonly, oral cavity/jaw.[2] The disease is more common in adults [1],[3] with only about 21 children reported till date.[4] BM involvement has been reported in about one-third of cases [4] but is difficult to recognize in children because the tumor is morphologically and immunologically indistinguishable from multiple myeloma and other lymphomas with plasmacytoid differentiation,[1] conditions equally unexpected in the diagnostic setting in this age group. Here, we present the clinical and hematological features of two children with PBL.

The first patient, an 8-year-old child, presented with mass in the left maxillary area with proptosis, high-grade fever, weight loss, and rapid diminution of vision [Figure 1]. Positron emission tomography (PET) showed diffuse uptake in all bones of the body and computed tomography scan showed a maxillary mass extending into the floor of orbit and roof of the mouth. Orbital biopsy was reported elsewhere as high-grade lymphoma with plasmacytic differentiation, based on the presence of sheets of large cells with eccentric nuclei, prominent nucleoli, frequent mitoses, and a few binucleate and multinucleated cells. Peripheral smear showed neutrophilia without rouleaux formation or plasma cells. BM smears showed sheets of plasma cells with eccentric nuclei and hof. Flow cytometry showed these cells to be immunophenotypically similar to plasma cells/myeloma cells - CD38+, CD138+, CD56+, CD19−, CD20−, and CD45dim. The patient was seropositive for HIV. A final diagnosis of PBL was made. The patient was put on NHL-BFM 90 standard protocol of treatment.[5] The patient was improving until 10 weeks into treatment and died as a result of progression.
Figure 1: Clusters of atypical plasmablastic lymphoid cells

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The second patient, a 12-year-old boy, presented with a 4-month history of pain in shoulder and hip and fever for 1 month. PET scan showed multiple lytic lesions all over the body. Blood chemistry revealed hypercalcemia. Blood counts and hormonal assays for parathormone and thyroid function tests were normal. A very faint band was seen on high-resolution electrophoresis of serum in the fast gamma region, but immunofixation electrophoresis was normal. BM smears showed infiltration by approximately 20% abnormal lymphoid cells which were plasmacytoid. Biopsy from soft tissue mass in the upper arm showed sheets of plasmacytic cells that were LCA+, CD138+, CD56+, CD10+, CD1a−, CD3−, MPO−, TdT−, and CD20−. Viral screen was negative. A final diagnosis for PBL was made. The patient was put on NHL-BFM 90 protocol and has been off therapy for 4 months.

PBL occurs more commonly in immunocompromised patients in the setting of transplant or HIV positivity.[3] PBL may occasionally be the initial presentation of HIV as was seen in our first patient. Diagnosis in both our patients required exclusion of plasmablastic variant of multiple myeloma.[1] Features that favor PBL over multiple myeloma include association with HIV infection and Epstein–Barr virus encoded RNA (EBER) positivity in neoplastic cells. EBER analysis could not be done of our cases. Features favoring myeloma include the presence of monoclonal paraproteinemia, hypercalcemia, renal dysfunction and lytic bone lesions.[3] However, lytic bony lesions and hypercalcemia may be seen in PBL also, as seen in the second patient. The faint band seen on serum electrophoresis could not be confirmed to be monoclonal; in addition, it did not correlate with extent of skeletal involvement. Various differentials to be considered on the biopsy of patients with PBL include rhabdomyosarcomas and malignant rhabdoid tumors. These can be excluded easily by immunohistochemistry.[1]

PBL is associated with high relapse rates and poor prognosis [3] with slightly improved outcome in HIV negative patients.[4] One study group recommends intensive therapy combined with highly active anti-retroviral therapy (HAART)[4] and the other group recommends chemotherapy without HAART indicating no additional survival benefit.[4] We put our patients on NHL-BFM 90 regime [5] with good results. PBL is hard to suspect clinically, difficult to diagnose and treat, especially in pediatric patients. These are the very reasons for interest in the disease in the clinical as well as the laboratory setting, particularly when the patient is a child and BM is involved.

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Confl icts of interest

There are no conflicts of interest.

   References Top

Stein H, Harris N, Campo E. Plasmablastic lymphoma. In: Swerdlow S, Campo E, Harris N, Jaffe E, Pileri S, Stein H, et al., editors. WHO Classification of Tumours of the Haematopoietic and Lymphoid Tissues. Lyon: IARC; 2008. p. 256-7.  Back to cited text no. 1
Castillo JJ, Bibas M, Miranda RN. The biology and treatment of plasmablastic lymphoma. Blood 2015;125:2323-30.  Back to cited text no. 2
Delecluse HJ, Anagnostopoulos I, Dallenbach F, Hummel M, Marafioti T, Schneider U, et al. Plasmablastic lymphomas of the oral cavity: A new entity associated with the human immunodeficiency virus infection. Blood 1997;89:1413-20.  Back to cited text no. 3
Morscio J, Dierickx D, Nijs J, Verhoef G, Bittoun E, Vanoeteren X, et al. Clinicopathologic comparison of plasmablastic lymphoma in HIV-positive, immunocompetent, and posttransplant patients: Single-center series of 25 cases and meta-analysis of 277 reported cases. Am J Surg Pathol 2014;38:875-86.  Back to cited text no. 4
Reiter A, Schrappe M, Tiemann M, Ludwig WD, Yakisan E, Zimmermann M, et al. Improved treatment results in childhood B-cell neoplasms with tailored intensification of therapy: A report of the Berlin-Frankfurt-Münster Group Trial NHL-BFM 90. Blood 1999;94:3294-306.  Back to cited text no. 5

Correspondence Address:
Anita Chopra
Room No. 423, 4th Floor, Laboratory Oncology Unit, Dr. B.R.A. Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_375_16

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