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T-Cell Lymphoma: Clinical Presentation and Therapeutic Outcome from a Tertiary Care Hospital in Eastern India

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Abstract

T-cell non-Hodgkin’s lymphoma (T-NHL) is rare and heterogeneous disease. There is a marked difference in biology, clinical presentation and therapeutic outcomes worldwide. This is a retrospective study from May 2014 to May 2018. Patients with established T-NHL after diagnosis were included, and their clinical presentation and therapeutic outcomes were analysed in detail. In the above-mentioned period, a total of 248 NHL patients were evaluated out of which 32(12.9%) had T-NHL. The median age of presentation was 37 years (range 7–69 years), with a male predominance 26 (81.2%). Most common presentation was painless lymphadenopathy 18 (56.2%). B symptoms were found in 8 (25%). In the Ann Arbor staging system, most T-NHL presented with either Stage III or IV with 12 (37.5%) patients respectively. Among the T-NHL subtypes, peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS), 20 (62.5%); anaplastic large-cell lymphoma (ALCL), 7 (21.9%); angioimmunoblastic T-cell lymphoma (AITL), 3 (9.4%); cutaneous T-cell lymphoma (CTCL), 2 (6.2%); and prolymphocytic leukaemia (PLL), 1 (3.1%) were diagnosed. Therapeutic options for T-NHL used were cyclophosphamide, doxorubicin, vincristine, prednisolone and etoposide (CHOEP) based. Response assessment after 6 cycles showed complete response (CR) and partial response (PR), 13 (40.6%) and 16 (50%), respectively. Three patients died during therapy. The progression-free survival (PFS) at 18 months was 57.34%. In our study, 12.9% patients had T- NHL among all patients of lymphoma. Lymphadenopathy and advanced stage disease were most common presentations. Chemotherapy outcomes showed CR in 40.6% of patients and PFS at 18 months was 57.34%.

Introduction

T-cell non-Hodgkin’s lymphomas (T-NHL) constitute approximately 10% of all NHL in the western countries and 10–15% of all NHLs in India [1, 2].The survival rate of T-NHL patients at 3 years are approximately 45% with a median follow-up of 28 months [3]. Peripheral T-cell lymphomas (PTCL) comprise two-thirds of all T-NHL, and the remaining one-third is made up of cutaneous T-cell lymphoma (CTCL) [1].T-NHLs are a group of rare and heterogeneous diseases across the world. Here we have evaluated the clinical presentations and therapeutic outcome of patients with T-NHL from a tertiary care teaching hospital in eastern India.

Materials and Methods

This is a retrospective study from May 2014 to April 2018. About 248 patients with non-Hodgkin’s lymphoma (NHL) attended the haematology outpatient department (OPD) of Nil Ratan Sircar (NRS) Medical College and Hospital, Kolkata, and among them 32 patients who were diagnosed with T-NHL were included in our study. Their clinical presentations and therapeutic outcomes after chemotherapeutic intervention were analysed in detail. All patients were subjected to a detailed history taking and clinical examination.

All patients of lymphoma in our institute undergo complete blood count with peripheral smear evaluation, biochemical parameters like urea, creatinine, uric acid, liver function test, lactate dehydrogenase (LDH), serum sodium and potassium. Viral serology for human immunodeficiency virus (HIV 1 & 2), anti-hepatitis C and hepatitis B Surface antigen were done. Lymph node excision biopsy was planned and conducted in our surgery department, and the histopathological examination (HPE) and immunohistochemical (IHC) assessment were done [4,5,6]. Patients with skin lesions were subjected to skin biopsy in the department of dermatology, followed by histopathological and immunohistochemical (IHC) assessment [7]. Subsequently, we did staging investigations by contrast enhanced computed tomography (CECT) of the neck, thorax, abdomen and pelvis in all patients. Bone marrow aspiration and biopsy were done from the iliac crest and posterior superior iliac spine. The bone marrow biopsy was assessed for lymphoma infiltration.

Lymph node HPE and IHC: The formalin-fixed lymph node biopsy samples were stained with the haematoxylin and eosin stain and analysed by microscopy [4,5,6]. The overall architecture of the lymph node was first analysed under the 10× and 20× magnifications. The lymph nodes were studied histologically by their rounded contour, presence/absence of a capsule, subcapsular sinus and proliferation or effacement of the lymphoid follicles. The individual cellular characteristics were further studied under the 40× magnification of the microscope. On the basis of the lymph node histology and size and nuclear characteristics of the lymphocytes, further immunohistochemistry panels were applied. The diagnosis of T-NHL was confirmed by using IHC markers such as CD45, CD5, CD7, CD3, CD2 and CD1a [7].

Therapeutic interventions and follow-up: Chemotherapeutic options such as CHOP (cyclophosphamide, doxorubicin, vincristine and prednisolone) and CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide and prednisolone) were administered with all precautions, and cardiac echocardiography was done in patients aged more than 45 years. All patients were treated with six cycles of chemotherapy, and response assessment was done after the fourth and sixth cycles with clinical examination and CECT respectively. Subsequently patients were followed up at our OPD at three monthly intervals with serum LDH estimation and clinical examinations. There was a single patient with T-prolymphocytic leukaemia (T-PLL) who was treated with bendamustine-rituximab (BR) based chemotherapy, and subsequent response assessment was done by a bone marrow examination with or without immunophenotyping.

Results

Study Population

There were 32 patients of T-NHL patients in our study, and among them the T-NHL subtypes distributions were PTCL, not otherwise specified (PTCL,NOS), 19 (59.3%); anaplastic large-cell lymphoma (ALCL), 7 (21.9%); angioimmunoblastic T-cell lymphoma (AITL), 3 (9.4%); cutaneous T-cell lymphoma (CTCL),2 (6.2%); and T-prolymphocytic leukaemia (T-PLL), 1 (3%). The median age of presentation was 37 years (range 7–69 years), with a male predominance: 26 (81%). The most common clinical presentation was lymphadenopathy found in 18 (56.7%) patients and B symptoms were found in 8 (25%) patients. Other clinical manifestations included superior vena cava (SVC) like presentation, pleural effusion, skin involvement and periampullary involvement. According to the Ann Arbor staging, in our study, patients mostly presented with either stage III or IV with 12 (37.5%) and 12 (37.5%), respectively. International prognostic index (IPI) scores were low, 2 (6.2%); low intermediate, 8 (25%); high intermediate, 17 (53%); and high, 5 (15.6%), among patients with T-NHL. The chemotherapeutic protocol we administered in our patients were CHOEP13 (40.6%) and CHOP 16 (50.0%). There was a single patient with T-PLL who received BR protocol as per departmental decision. Another patient having ALCL with periampullary involvement initially underwent the Whipple procedure as surgical intervention but succumbed before any chemotherapeutic interventions could be undertaken (Table 1).

Table 1 Type of T-NHL

Distribution of T-Cell Lymphomas

There were 32 patients of T-NHL patients in our study, and among them the T-NHL subtypes distributions were PTCL, not otherwise specified (PTCL,NOS), 19 (59.3%); anaplastic large-cell lymphoma (ALCL), 7 (21.9%); angioimmunoblastic T-cell lymphoma (AITL), 3 (9.4%); cutaneous T-cell lymphoma (CTCL), 2 (6.2%); and T-prolymphocytic leukaemia (T-PLL), 1 (3%) (Fig. 1).

Fig. 1
figure1

Distribution of T-cell lymphomas

Response Assessment After Six Cycles of Chemotherapy and Progression-Free Survival (PFS)

Response assessment was done after six cycles of chemotherapy. The complete response (CR), partial response (PR) and death out of 19 patients were 8 (42.1%), 10 (52.6%) and 1 (5.2%), respectively, among patients of PTCL. In the ALCL subtype out of seven patients, one (14.2%), four (57.1%) and two (28.5%) showed CR, PR and death, respectively. In the AITL subtype, out of three patients, two (66.6%) and one (33.3%) showed CR and PR, respectively. In the CTCL subtype, out of two patients, one of each was in CR and PR. The overall CR rate in our study population was 40.6% and progression-free survival (PFS) at 18 months was 57.34% (Figs. 2 and 3).

Fig. 2
figure2

Response assessment after 6 cycles of chemotherapy

Fig. 2
figure3

Progression free survival (PFS) at 18 months

Discussion

In our analysis, T-NHL comprised of 12.9% of NHL patients. The established prevalence of T-NHL varies from 10 to 15% across studies [1, 2]. A study from southern India also found incidence of T-NHL to be 13.85% (8). In another study by Naresh et al., conducted in Western India, T-NHL comprised 16.2% of all NHL [8].

PTCL, NOS, T-lymphoblastic lymphoma (T-LBL) and ALCL are the most common subtypes of T-NHL seen in India [2, 9, 10]. This was also true in our analysis, with PTCL and NOS being the commonest (59.3%), followed by ALCL (21.9%). In contrast to the data from western countries, CTCL was not found to be as common [3]. Mycosis fungoides is the most common subtype of CTCL but it was seen in only one patient in our study [3].AITL and T-PLL were the other rarer subtypes seen in our study population. In one study, T-LBL was included in the group of T-NHL; however, we have excluded T-LBL from our study analysis in view of current WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 2016 classification [11].

T-NHL is having higher prevalence in males and 26 (81%) patients belonging to the male gender in our study [2]. The median age of presentation was approximately 54 years in a study from Asia [2]. In our patients with T-NHL, the median age of presentation at 37 years (range 7–69 years). Also, a study from China showed similar median age of presentation [12]. This suggests that T-NHL occurs at an earlier age from eastern part of India; however, a long-term study is needed to accurately ascertain it.

The most common presenting symptom was painless lymphadenopathy seen in 56.7% patients; however, there was no classical pattern of lymph node enlargement; B symptoms (25%) were also not uncommon in our patients. Some anecdotal presentations among our patients were SVC syndrome, pleural effusion and periampullary involvement. In some other studies, the most common presentations were painless lymphadenopathy with some extra nodal involvement also seen [3, 13]. According to Ann Arbor staging, patients had advanced stages mostly stage III and IV of 12 (37.5%) and 12 (37.5%), respectively. Using the international prognostic index (IPI), T-NHL patients in our study mostly had high intermediate 17 (53%) and high 5 (15.6%) IPI, respectively. Gisselbrecht et al. have also found high IPI scores in T-NHL patients compared to B-NHL [14]. This advanced stage in our patients could be due to delayed presentation by the patients as they are mostly asymptomatic in their initial stages and also due to long waiting times for patients.

Standard therapy for PTCL, NOS and ALCL is a conventional-dose systemic anthracycline-containing chemotherapy [3, 15]. CHOEP or CHOP chemotherapy was administered to our patients of T-NHL, and necessary follow-up was done. Response assessment after six cycles in our study analysis showed CR and PR 13 (40.6%) and 16 (50%), respectively. Three patients had died in our study. Regarding the deaths, one patient had periampullary involvement with ALCL, and he succumbed to acute pancreatitis post Whipple surgery; the second death was due to febrile neutropenia and sepsis after three cycles of chemotherapy in a PTCL and NOS patient, and the third patient of ALCL was lost after relapsing within 1 month following completion of chemotherapy, and she succumbed to the disease process.

Huang et al. in his study showed that 3-year survival rate of T-NHL is approximately 45% with a median follow-up of 28 months [12]. In our study, the CR after six cycles of chemotherapy was 40.6%, and PFS at 18 months was 57.34%.

With the exception of a few relatively indolent entities, T-NHL is typically aggressive, treatment-resistant and associated with poor prognosis [16]. The rarity of T-NHL patients makes it difficult for well-designed clinical trials with immunotherapeutic agents. The limitation of our study was duration because the disease entity is rare and, hence, longer study duration is necessary for more enrolment of patients and needs longer observation of patients.

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Correspondence to Shuvra Neel Baul.

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Patients were not required to give informed consent to the study because the analysis used clinical data from our departmental lymphoma registry that were obtained after each patient agreed to treatment by written consent.

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Sen, A., De, R., Mandal, P.K. et al. T-Cell Lymphoma: Clinical Presentation and Therapeutic Outcome from a Tertiary Care Hospital in Eastern India. SN Compr. Clin. Med. 2, 186–191 (2020). https://doi.org/10.1007/s42399-019-00215-w

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Keywords

  • Lymphadenopathy
  • T- cell Non-Hodgkin’s lymphomas
  • Clinical presentation
  • Chemotherapy
  • Therapeutic outcomes