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Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 304-306
Poly immunophenotypic expression in a case of diffuse large B-cell lymphoma

Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India

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Date of Web Publication19-Jun-2017

How to cite this article:
Singh N, Ketkar A, Gujral S. Poly immunophenotypic expression in a case of diffuse large B-cell lymphoma. Indian J Pathol Microbiol 2017;60:304-6

How to cite this URL:
Singh N, Ketkar A, Gujral S. Poly immunophenotypic expression in a case of diffuse large B-cell lymphoma. Indian J Pathol Microbiol [serial online] 2017 [cited 2022 May 25];60:304-6. Available from: https://www.ijpmonline.org/text.asp?2017/60/2/304/208399


A 53-year-old male presented with weakness, constipation, and weight loss of 6 kg in 1 month. Peripheral blood counts were within normal limits. Liver function tests and biochemical parameters were normal. Magnetic resonance imaging showed enlarged infradiaphragmatic retroperitoneal lymph nodes in the left para-aortic, aortocaval, and retrocaval regions.

Core biopsy was performed from the retroperitoneal lymph node and showed a diffuse proliferation of large neoplastic cells with centrally or eccentrically placed nucleus, a single central nucleolus in most cells and moderate amphophilic cytoplasm. Mitotic activity was frequent [Figure 1]a and [Figure 1]b.
Figure 1: (a) Diff use proliferation of large lymphoid cells (H and E, ×10), (b) large neoplastic lymphoid cells with centrally or eccentrically placed nucleus, a single central nucleolus, and moderate amphophilic cytoplasm (H and E, ×40), (c) CD30 positivity in cells (×40), (d) MUM1 positivity

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Immunohistochemical examination was done. Tumor cells expressed leukocyte common antigen, CD20, CD79 alpha, CD30, CD138, epithelial membrane antigen (EMA), MUM1, and ALK1 [Figure 1]c,[Figure 1]d and [Figure 2]a,[Figure 2]b,[Figure 2]c. In addition, tumor cells also revealed perinuclear dot-like positivity for AE1/AE3. Ki-67 labeling index was 50%–60% [Figure 2]d. CD3 and bcl 6 were negative.
Figure 2: (a) Granular cytoplasmic positivity for ALK (×40), (b) cytokeratin (AE1/AE3) showed a dot-like positivity (×40), (c) epithelial membrane antigen positivity (×40), (d) Ki-67 labeling index 50%–60% (×40)

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Based on the morphology and immunohistochemical profile, a final diagnosis of ALK-positive diffuse large B-cell lymphoma (DLBCL) was made with expression of AE1/AE3.

Bone marrow revealed reactive marrow and the patient received six cycles of chemotherapy.

ALK-positive large B-cell lymphoma has been described in adult as well as pediatric population, at nodal and extranodal sites. The median age is between 43 and 51 years, with a significant male preponderance and a male:female ratio of about 5:1.[1],[2] They have an aggressive clinical course and do not respond well to standard chemotherapy regimens (such as the CHOP regimen) used for other DLBCLs.[3] Six cycles of CHOP were also advised to this patient.

The molecular/cytogenetic profile of these lymphomas is heterogeneous. However, clathrin-ALK rearrangement is one of the most common molecular alterations identified in patients with ALK-positive DLBCL.[1]

These lymphomas usually exhibit a plasmablastic morphology and show positive immunostaining for ALK, EMA, and cytoplasmic expression of immunoglobulins, whereas they are often negative for other B-cell and T-cell markers.[2] Interestingly, a dot-like positivity for cytokeratin (AE1/AE3) was seen in our case. It is extremely rare to see cytokeratin (AE1/AE3) positivity in ALK-positive DLBCL.[1]

Identification of these lymphomas can be challenging, especially distinguishing among plasmablastic lymphoma, ALCL of T-null cell lineage, and poorly differentiated/anaplastic carcinoma. These patients are candidates for targeted therapy and clinical trials evaluating the effectiveness of ALK inhibitors are being undertaken. For these reasons, correct diagnosis is vital in these lymphomas which are probably an under-recognized entity. An extensive IHC workup is needed to pick up such rare subtype of lymphoma.

We are presenting this case in view of its rarity as well as AE1/AE3 positivity in this case of ALK-positive DLBCL.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Laurent C, Do C, Gascoyne RD, Lamant L, Ysebaert L, Laurent G, et al. Anaplastic lymphoma kinase-positive diffuse large B-cell lymphoma: A rare clinicopathologic entity with poor prognosis. J Clin Oncol 2009;27:4211-6.  Back to cited text no. 1
Delsol G, Lamant L, Mariamé B, Pulford K, Dastugue N, Brousset P, et al. A new subtype of large B-cell lymphoma expressing the ALK kinase and lacking the 2; 5 translocation. Blood 1997;89:1483-90.  Back to cited text no. 2
Morgan EA, Nascimento AF. Anaplastic lymphoma kinase-positive large B-cell lymphoma: An underrecognized aggressive lymphoma. Adv Hematol 2012;2012:529572.  Back to cited text no. 3

Correspondence Address:
Neha Singh
C/O H. No. 24, Rohtak Road, Bhiwani - 127 021, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.208399

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