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Year : 2011  |  Volume : 7  |  Issue : 2  |  Page : 214-216

High-grade plasmablastic neoplasm of humerus in an HIV-negative patient, which was indeterminate between plasmablastic lymphoma and plasmablastic myeloma

Department of Pathology, Medical College, 88 College Street, Kolkata, West Bengal, India

Date of Web Publication12-Jul-2011

Correspondence Address:
Santosh Kumar Mondal
Teenkanya Complex, Flat 1B, Block B, 204 R. N. Guha Road, Dumdum, Kolkata-28, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.82943

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 > Abstract 

Plasmablastic lymphoma (PBL) of bone is a rare neoplasm that shares many confusing cytomorphological and immunohistochemical features with plasmablastic plasma cell myeloma (PBPCM). A 47-year-old female patient presented with a bony swelling and bone pain in the left humerus for the last 6 months. On radiological examination (x-ray and computed tomography) it appeared to be a lytic lesion, and a pathological fracture was detected. The patient was HIV-negative. Fine needle aspiration (FNA) was done from the lesion, which was inconclusive. Subsequently, incisional biopsy was taken. Histopathological examination and immunohistochemistry confirmed a high-grade plasmablastic neoplasm, favoring a diagnosis of PBL. Most of the reported cases of PBL have occurred in HIV-positive patients, and the bone is a very rare site. PBL can be confused with PBPCM. A final diagnosis should be rendered only after thorough histopathological and immunohistochemical examination.

Keywords: HIV-negative, plasmablastic type, primary bone neoplasm

How to cite this article:
Mondal SK, Bera H, Biswas PK, Mallick MG. High-grade plasmablastic neoplasm of humerus in an HIV-negative patient, which was indeterminate between plasmablastic lymphoma and plasmablastic myeloma. J Can Res Ther 2011;7:214-6

How to cite this URL:
Mondal SK, Bera H, Biswas PK, Mallick MG. High-grade plasmablastic neoplasm of humerus in an HIV-negative patient, which was indeterminate between plasmablastic lymphoma and plasmablastic myeloma. J Can Res Ther [serial online] 2011 [cited 2022 May 27];7:214-6. Available from: https://www.cancerjournal.net/text.asp?2011/7/2/214/82943

 > Introduction Top

In 1997, Delecluse et al. were the first to describe a B-cell lineage neoplasm with plasmablastic differentiation. [1] They called it plasmablastic lymphoma (PBL). They thought that it could be a new sub-group of diffuse large B-cell lymphoma (DLBCL). Initially, it was thought to occur in HIV-positive individuals only, with the most common site of involvement being the oral cavity. Due to scarcity of reported cases of PBL many aspects regarding its manifestation and diagnosis still remain obscure. Often, the correct diagnosis is delayed due to this tumor's close resemblance to plasmablastic plasma cell myeloma (PBPCM). Bone is an unusual site for PBL. In this case report we present a case of high-grade plasmablastic neoplasm of the humerus, with features favoring a diagnosis of PBL, in an HIV-negative woman.

 > Case Report Top

A 47-year-old woman came to the orthopedic outpatient department complaining of a bony swelling and dull bone pain in upper part of the left humerus for last 6 months. Clinically, the lesion was tender and was fixed to the humerus. The overlying skin was free and unremarkable.

Plain radiograph of the humerus showed a radiolucent lesion at the upper part of the shaft. Overall, the appearance was suggestive of a bony tumor. A pathological fracture was also identified on the same x-ray [Figure 1]a. Subsequent computed tomography (CT) scan revealed a well-defined lytic bone lesion at the same site. No periosteal reaction or matrix calcification was seen. The rest of the bony and cartilaginous structures around the shoulder joint and soft tissue surrounding the tumor were unremarkable.
Figure 1: (A) X-ray shows the bony lytic lesion and a pathological fracture in the left humerus; (B) CT-guided FNA

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The patient was referred to the cytopathology division of the pathology department for fine needle aspiration cytology (FNAC).

Cytologic findings

CT-guided FNA was done using a 20-gauge needle [Figure 1]b. Blood-mixed material was aspirated from the tumor. Smears showed very scanty cellularity, with occasional atypical large cells in a hemorrhagic background. Cytodiagnosis was inconclusive and incisional biopsy was suggested.

Histologic findings

Grossly, multiple tiny tissue pieces were received. Microscopically, the tumor was composed of large cells with eccentric nuclei and prominent nucleoli in diffuse sheets [Figure 2]. Mature plasma cells were sparse. The mitotic count was high. On immunohistochemistry, the tumor cells expressed CD138 (strong membrane positivity), CD10, kappa light chain, and MUM-1. They were immunonegative for lambda light chains, CD45, CD20, and CD3 [Figure 3]. More than 90% of the tumor cells showed nuclear positivity for Ki-67 (proliferative index).
Figure 2: Photomicrograph shows that the tumor is composed of diffuse sheets of large cells with eccentric nuclei and prominent nucleoli (H and E; ×400)

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Figure 3: (A) Tumor cells showing strong membrane positivity for CD138; (B) cells are negative for the B-cell marker CD20

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Enzyme-linked immunosorbent assay (ELISA) and Western blot test confirmed the HIV-negative status of the patient. Serum electrophoresis did not reveal the presence of any abnormal protein. Bone marrow examination (both aspiration and biopsy) was done. It showed mildly hypercellular marrow, but plasma cells were unremarkable and the plasma cell count was normal. In the present case, Epstein-Barr virus-encoded RNA (EBER) detection was not possible as the patient refused the test. However, based on the high proliferative index, CD10 positivity, and normal bone marrow and serum electrophoresis we made a final diagnosis of high-grade plasmablastic neoplasm, most likely PBL.

Treatment was given assuming a diagnosis of PBL. She was treated with six cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) at intervals of 3 weeks. The patient has now been on follow-up for the 1 year; the past year has been uneventful and she is maintaining her normal life and daily activities.

 > Discussion Top

PBL is an aggressive lymphoma. Ten out of twelve cases studied first by Delecluse et al. died within 12 months of diagnosis. Colomo et al., Flaitz et al., and Nasta et al. have all reported very short survival. [1],[2],[3],[4] The diagnosis of PBL is often complicated due to its close morphologic similarities to PBPCM, with which it also shares many immunohistochemical features.

Differentiating PBL from PBPCM is critical, as the treatment is different. Morphologic criteria alone are not enough to differentiate them accurately; rather, a combination of clinical findings plus the results of diagnostic workup are essential for making a definitive diagnosis. As reported by Colomo et al., an increased plasmacytic cell population is seen in PBPCM, whereas PBLs are characterized by extreme paucity or total absence of plasmacytic cells and instead have a monotonous population of plasmablasts. [2] In practice, these morphologic criteria are quite inadequate for distinction between these two entities. According to the studies by Vega et al., both of these conditions are strongly positive (100% for each marker) for CD38, CD138, and MUM-1 and immunonegative for CD20. [5] CD10 is positive for PBL in 67% cases and for PBPCM in only 29% cases. EBER can play a pivotal role in differentiating between PBL and PBPCM. EBER is almost always positive in cases of PBL and negative in PBPCM. [5]

Earlier PBL was thought to occur only in HIV-positive cases, particularly in the oral cavity. But, over time, some cases of PBL were also reported in HIV-negative patients. To the best of our knowledge, it was Scheper et al. who reported the first case of plasmablastic lymphoma in an HIV-negative patient; [6] the site of involvement was the oral cavity. Kim et al. reported six cases of PBL from South Korea, with all six patients being HIV-negative; [7] the sites of involvement were the tonsils, nasal cavity, meninges, oral cavity, stomach, and terminal ileum. The authors suggested that perhaps the occurrence of PBL in Koreans is not so intimately associated with HIV status.

Primary bone lymphoma itself is a rare neoplasm, constituting only 7% of all malignant bone tumors. [8] Here we report a case of primary bone neoplasm with plasmablastic morphology that was suggestive of PBL. PBLoccurs more commonly in HIV-positive patients and shows a predilection for the oral cavity. Our case was unusual in that the patient was HIV-negative and, besides, an uncommon site was involved, i.e., the humerus.

 > References Top

1.Delecluse HJ, Anagnostopoulos I, Dallenbach F, Hummel M, Marafioti T, Schneider U, et al. Plasmablastic lymphomas in the oral cavity: A new entity associated with the human immunodeficiency virus infection. Blood 1997;89:1413-20.  Back to cited text no. 1
2.Colomo L, Loong F, Rives S, Pittaluga S, Martinez A, Lopez-Guillermo A, et al. Diffuse large B cell lymphomas with plasmablastic differentiation represent a heterogenous group of disease entities. Am J Surg Pathol 2004;28:736-47.  Back to cited text no. 2
3.Flaitz CM, Nichols CM, Walling DM, Hicks MJ. Plasmablastic lymphoma: An HIV associated entity with primary oral manifestations. Oral Oncol 2002;38:96-102.  Back to cited text no. 3
4.Nasta SD, Carrum GM, Shahab I, Hanania NA, Udden MM. Regression of plasmablastic in a patient with HIV highly active antiretroviral therapy. Leuk Lymphoma 2002;43:423-6.  Back to cited text no. 4
5.Vega F, Chang CC, Medeiros LJ, Udden MM, Cho-Vega JH, Lau C, et al. Plasmablastic lymphomas and plasmablastic plasma cell myelomas have nearly identical immunophenotypic profiles. Mod Pathol 2005;18:806-15.   Back to cited text no. 5
6.Scheper MA, Nikitakis NG, Fernandes R, Gocke CD, Ord RA, Sauk JJ. Oral plasmablastic lymphoma in an HIV-negative patient: A case report and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:198-206.  Back to cited text no. 6
7.Kim JE, Kim YA, Kim WY, Kim CW, Ko YH, Lee GK, et al. Human immunodeficiency virus-negative plasmablastic lymphoma in Koreans. Leuk Lymphoma 2009;50:582-7.  Back to cited text no. 7
8.Maruyama D, Watanabe T, Beppu Y, Kobayashi Y, Kim S, Tanimoto K, et al. Primary Bone Lymphoma: A New and Detailed Characterisation of 28 Patients in a Single-Institution Study. Jpn J Clin Oncol 2007;37:216-23.  Back to cited text no. 8


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