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Year : 2017  |  Volume : 60  |  Issue : 2  |  Page : 206-208
Profiling of peripheral T-cell lymphomas in Kerala, South India: A 5-year study

Division of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

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Date of Web Publication19-Jun-2017


Background: Peripheral T-cell lymphomas (PTCLs) are non-Hodgkin's lymphomas (NHLs) with considerable variation in incidence across the world. They show a wide variety of clinicopathological features and generally associated with poor clinical outcome. Lymphoma data from different geographic regions will definitely aid in routine clinical practice and research work. PTCLs are reported with a higher frequency in Asia as compared to Western countries. Objective: The objective of this study was to analyze the frequency and distribution of PTCLs diagnosed in a tertiary care cancer center in Kerala. Materials and Methods: This was a retrospective study carried out in the Division of Pathology, Regional Cancer Centre, Thiruvananthapuram, for 5 years from January 1, 2011, to December 31, 2015. All PTCLs diagnosed during this period were reviewed and then classified according to the 2016 revision of the World Health Organization classification of lymphoid neoplasms. Statistical significance of the results was evaluated using Chi-square test. Results: Among the total 3108 cases of lymphomas diagnosed at our center, 2404 cases were NHLs (77.35%). PTCLs (n = 333) contributed 13.85% of all NHLs. Among these, PTCL, not otherwise specified, constituted the most common subtype (92 cases, 27.63%), followed by angioimmunoblastic T-cell lymphoma (79 cases, 23.72%), anaplastic large cell lymphoma (75 cases, 22.52%), mycosis fungoides (28 cases, 8.40%), and adult T-cell leukemia/lymphoma (ATLL) (28 cases, 8.40%). Conclusion: This is the largest study on PTCLs reported from Kerala. We document that the frequency of PTCLs is higher than that reported from Western studies. The frequency of ATLL reported from Kerala is much higher than that reported from other states.

Keywords: Distribution, frequency, Kerala, peripheral T-cell lymphomas, South India

How to cite this article:
Nair RA, Vasudevan JA, Jacob PM, Sukumaran R. Profiling of peripheral T-cell lymphomas in Kerala, South India: A 5-year study. Indian J Pathol Microbiol 2017;60:206-8

How to cite this URL:
Nair RA, Vasudevan JA, Jacob PM, Sukumaran R. Profiling of peripheral T-cell lymphomas in Kerala, South India: A 5-year study. Indian J Pathol Microbiol [serial online] 2017 [cited 2021 Dec 6];60:206-8. Available from: https://www.ijpmonline.org/text.asp?2017/60/2/206/208411

   Introduction Top

Peripheral T-cell lymphomas (PTCLs) are a diverse group of non-Hodgkin lymphomas (NHLs) generally associated with poor prognosis. It accounts for around 10%–18% in previous Indian studies,[1],[2],[3] 10%–45% in Far Eastern studies,[4],[5],[6],[7] and around 4%–12% in Western studies.[8],[9],[10],[11] Thus, there exists considerable difference in the frequency and distribution across the world. Increased frequency of T-cell lymphomas in Asian countries may be due to a true increased occurrence, relative reduction in the frequency of many B-cell lymphomas, human T-cell leukemia virus-1 (HTLV-1) infection, or racial predisposition, leading to increased incidence of Epstein–Barr virus-associated lymphomas. Our study was done to ascertain the frequency and distribution of PTCLs in Kerala as there are no studies documented from this region in South India.

   Materials and Methods Top

This study was done in the Division of Pathology in Regional Cancer Centre in Kerala for 5 years from January 2011 to December 2015. The study included both in-house cases and the referred cases from other centers. All the fresh specimens were fixed in 10% neutral-buffered formalin. Sections were cut at 4 μm thickness, and morphological evaluation was done in hematoxylin and eosin-stained sections. Suitable panel of antibodies was decided after the morphological evaluation, and the cases were classified according to the 2016 revision of the World Health Organization (WHO) classification of lymphoid neoplasms.[12] Statistical significance of the difference in frequency and distribution of the PTCLs and the various subtypes between our study and various other studies was analyzed using Chi-square test.

   Results Top

A total of 3108 lymphomas were diagnosed during the 5-year period. PTCLs accounted for 13.85% of NHLs. PTCL, not otherwise specified (PTCL, NOS) (92 cases, 27.63%), was most common, followed by angioimmunoblastic T-cell lymphoma (AITL) (79 cases, 23.72%), anaplastic large cell lymphoma (ALCL) (75 cases, 22.52%), mycosis fungoides (MF) (28 cases, 8.40%), adult T-cell leukemia/lymphoma (ATLL) (28 cases, 8.40%), primary cutaneous CD 30-positive T-cell lymphoproliferative disorder (cutaneous ALCL [C-ALCL]/lymphomatoid papulosis [LyP]) (12 cases, 3.60%), extranodal NK/T-cell lymphoma, nasal-type (ENNKT) (9 cases, 2.70%), subcutaneous panniculitis-like T-cell lymphoma (6 cases, 1.80%), hepatosplenic T-cell lymphoma (HSTCL) (1 case, 0.30%), primary cutaneous gamma delta T-cell lymphoma (1 case, 0.30%), enteropathy-associated T-cell lymphoma (EATL) (1 case. 0.30%), and aggressive NK-cell leukemia (1 case, 0.30%) [Figure 1]. Lymph node was the most common primary site, followed by the skin, lungs, nasal cavity, ovary, bone, liver, and soft tissue [Figure 2].
Figure 1: Frequency of subtypes of peripheral T-cell lymphoma in our study

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Figure 2: Sites of peripheral T-cell lymphomas

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   Discussion Top

Our diagnostic approach in a case of PTCL includes detailed clinical history and clinical examination with special emphasis on skin lesions, evaluation of laboratory findings, peripheral blood and bone marrow, apart from detailed histopathology examination of lymph node with immunohistochemistry. PTCLs accounted for 13.85% of NHLs in our study, which is similar to previous two Indian studies [1],[2] but is lower than a similar study from South India (P = 0.008)[3] and Far Eastern studies (P < 0.0001)[4],[5] and higher than Western studies (P < 0.0001) [Figure 3].[8],[9] Frequencies of subtypes of PTCLs as compared to other studies are shown in [Figure 4]. PTCLs which did not correspond to any of the other specific entities in the WHO classification were classified as PTCL, NOS.[13] PTCL, NOS was the most common subtype constituting around 27.6%, followed by AITL which is similar to a Western study.[9] Other studies from India and North America have shown a higher frequency of ALCL as compared to AITL.[3],[8] AITL typically present with advanced stage disease, frequent pruritic skin rashes, polymorphous infiltrate in lymph nodes with prominent proliferation of arborizing high endothelial venules (HEV) and follicular dendritic cell (FDC) proliferation entrapping the HEVs.[13] FDC meshwork in all cases was highlighted by one of the FDC markers. AITL accounted for 23.72% of NHL, which is significantly higher than similar Indian (P = 0.00001),[3] Western,[8],[9] and Far East studies (P < 0.001).[4],[5]
Figure 3: Frequency of peripheral T-cell lymphomas in various studies

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Figure 4: Comparison of frequencies of peripheral T-cell lymphoma subtypes with other studies

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ALCL is a PTCL characterized histologically by large pleomorphic cells with abundant cytoplasm, usually with horseshoe-shaped nuclei, ALK gene rearrangements, and expression of ALK protein and CD 30. ALCL, ALK-negative is morphologically indistinguishable from ALCL, ALK-positive but lacks the ALK protein.[13] The frequency of ALCL is similar to the previous Indian study [3] and some Western studies (16%–24%)[8] but is significantly higher than Japan and China (P < 0.001)[4],[5] and British population (P = 0.013).[9] ALK-positive ALCL (43 cases) constituted the majority of ALCLs.

ATLL is a highly pleomorphic T-cell lymphoma caused by HTLV-1.[13] Cases typical of ATLL by morphology and immunohistochemistry were advised serum HTLV-1 assay. HTLV-1-positive ATLL constituted 8.40% of PTCLs, which is not reported in studies from other Indian states [1],[2],[3] and in Western studies [8],[9] but is significantly lower than Japan where it constitutes the most common lymphoma (P < 0.001).[5],[7] The alarming incidence of ATLL in Kerala was highlighted by Nair et al. where 15 cases of HTLV-1 positive ATLL were reported which is the largest case series from India and is the third largest documented series from a geographic region in Asia after Japan and Taiwan.[14],[15] MF is a cutaneous T-cell neoplasm characterized by patches, plaques, and tumors and histologically characterized by epidermotropic infiltrates of atypical small- to medium-sized T-lymphoid cells with cerebriform nuclei.[13] MF accounted for around 8.40% which is almost similar to previous Indian studies,[3] higher than Far East studies (P = 0.0003),[4],[5] but lower than Western studies.[8],[9] The frequency of ENNKT is significantly lower than previous Indian studies (P = 0.03),[3] North American studies (P = 0.002),[8] and China (P < 0.0001).[4] HSTCL accounted for 0.3% which is lower than other studies (around 1%–6%).[3],[4] SCPTCL accounted for 1.8% of cases which is similar to Western and Far East studies but lower than a previous Indian study (P = 0.0004).[3] The frequency of C-ALCL/LyP is almost similar to other studies ranging from 1% to 6% but is significantly lower than studies from British population (P = 0.00001).[9] The frequency of EATL (around 0.3%) is similar to Far East studies [4] but lower than similar studies from other parts of the world.[3],[8],[9] There was association with enteropathy in our single case of EATL.[16]

   Conclusion Top

The frequency of PTCLs is almost similar to other Indian studies but is significantly lower than similar study from South India and Far Eastern studies. The frequency of ENNKT and EATL is lower than other studies. The frequency of ATLL is significantly high as compared to previous Indian studies. Future studies including strict evaluation of HTLV-1 status is needed to confirm the endemic nature of HTLV-1 in Kerala, which is of utmost public health importance.

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Conflicts of interest

There are no conflicts of interest.

   References Top

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Burad DK, Therese MM, Nair S. Peripheral T-cell lymphoma: Frequency and distribution in a tertiary referral center in South India. Indian J Pathol Microbiol 2012;55:429-32.  Back to cited text no. 3
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Anderson JR, Armitage JO, Weisenburger DD. Epidemiology of the non-Hodgkin's lymphomas: Distributions of the major subtypes differ by geographic locations. Non-Hodgkin's Lymphoma Classification Project. Ann Oncol 1998;9:717-20.  Back to cited text no. 10
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Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pilleri SA, Stein H, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon, France: IARC; 2008.  Back to cited text no. 13
Nair RA, Jacob PM, Nair S, Prem S, Jayasudha AV, Sindhu NP, et al. Adult T cell leukemia/lymphoma (ATLL) in Kerala, South India – Are we staring at the tip of the iceberg? J Hematopathol 2013;6:135-44.  Back to cited text no. 14
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Correspondence Address:
Rekha A Nair
Division of Pathology, Regional Cancer Centre, Thiruvananthapuram - 695 011, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_762_16

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