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Year : 2022  |  Volume : 18  |  Issue : 3  |  Page : 843-845

Primary pulmonary T-cell lymphoma

1 Department of Pulmonary Medicine, JIPMER, Puducherry, India
2 Department of Pathology, JIPMER, Puducherry, India

Date of Submission06-Feb-2021
Date of Decision22-Feb-2021
Date of Acceptance30-Apr-2021
Date of Web Publication25-Apr-2022

Correspondence Address:
Manju Rajaram
Department of Pulmonary Medicine, JIPMER, Gorimedu, Puducherry - 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.jcrt_227_21

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 > Abstract 

Primary pulmonary lymphoma (PPL) is a rare clonal proliferation of lymphoid tissue involving one or both lungs. It is of two types, B-cell and T-cell lymphomas among which T-cell lymphoma is a rare entity and it is sparsely considered as a differential diagnosis in neoplastic lesions of the lung. Here, we are reporting a case of primary pulmonary T-cell lymphoma. PPL is a rare disease and can present with nonspecific symptoms. Radiologically, it can easily be confused with more common malignancies such as bronchogenic carcinoma with or without metastases. PPL carries different therapeutic and prognostic implications. Therefore, physicians should make every effort to achieve histopathological diagnosis before prognosticating a patient presenting with lung cancer.

Keywords: Immunohistochemistry, pleural effusion, primary pulmonary lymphoma

How to cite this article:
Vemuri MB, Rajaram M, Malik A, Toi PC. Primary pulmonary T-cell lymphoma. J Can Res Ther 2022;18:843-5

How to cite this URL:
Vemuri MB, Rajaram M, Malik A, Toi PC. Primary pulmonary T-cell lymphoma. J Can Res Ther [serial online] 2022 [cited 2022 Oct 1];18:843-5. Available from: https://www.cancerjournal.net/text.asp?2022/18/3/843/343916

 > Introduction Top

Lymphomatous lesions in the lung are rare to find and it can occur in three ways: (1) hematogenous dissemination, (2) contagious invasion from nodal lymphoma, and (3) primary pulmonary involvement.[1] Of these, primary pulmonary lymphoma (PPL) is sparsely reported and is of two types: B-cell lymphoma and T-cell lymphoma. B-cell lymphoma constitutes about 3%–4% and it usually arises from the bronchial-associated lymphoid tissue whereas primary pulmonary T-cell lymphoma is an infrequent and aggressive tumor with nonspecific clinical and radiological findings.[2] A case of primary pulmonary T-cell lymphoma presenting as consolidation along with effusion and nodules is being elicited here.

 > Case Report Top

A 62-year-old male presented with dyspnea on exertion and dry cough for 2 months. Dyspnea progressed gradually from Modified Medical Research Council Grade I to Grade III and was not associated with chest pain, orthopnea, and paroxysmal nocturnal dyspnea. Dry cough was present for 2 months which had no diurnal variation. It was not associated with sore throat, headache, cold, or sinus discharge. He was a nonsmoker and nonalcoholic. On examination, the patient was conscious, oriented, and afebrile. No pallor, icterus, cyanosis, clubbing, lymphadenopathy, or pedal edema was observed. On chest auscultation, air entry was decreased in the right mammary, axillary, infra-axillary, and infrascapular areas. Bronchial breath sounds were heard in the right infraclavicular area. The abdomen examination revealed no ascites or hepatosplenomegaly, and no obvious testicular swelling was observed.

On evaluation, chest X-ray showed bilateral pleural effusion with right upper zone and mid-zone ill-defined heterogeneous opacity and bilateral multiple nodules [Figure 1]. Sputum cytology and acid-fast bacilli were negative. Renal function test, serum electrolytes, and liver function test were within normal limits. Peripheral blood smear showed normocytic normochromic red blood cells along with leukocytosis, and atypical lymphoid cells were seen. 2D ECHO was normal. HIV and HbsAg were negative. Antinuclear antibody and antineutrophil cytoplasmic antibody were negative.
Figure 1: Chest X-ray showing bilateral pleural effusion with right upper zone and middle zone ill-defined heterogeneous opacity and bilateral multiple nodules

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On further evaluation, computed tomography thorax showed right upper lobe and middle lobe consolidation along with bilateral pleural effusion and bilateral multiple pulmonary nodules [Figure 2].
Figure 2: Computed tomography of the thorax showing right upper lobe and middle lobe consolidation and right-sided pleural effusion. Bilateral multiple pulmonary nodules

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Ultrasonography (USG) abdomen showed no hepatosplenomegaly, and bilateral medicorenal disease was present.

USG guided fine needle aspiration cytology of the lung lesion aspirate was diluted with peripheral blood and it revealed many atypical lymphoid cells. No evidence of malignancy was seen in the smear examined.

Pleural fluid cytology showed a fairly monotonous population of small atypical lymphoid cells admixed with many smudge cells and macrophages in a blood-mixed proteinaceous background.

Pleural biopsy was reported as a tiny linear fragment of fibrocollagenous tissue showing diffuse infiltration by atypical cells which were predominantly CD3 positive and negative for terminal deoxynucleotidyl transferase (Tdt) and CD10. There were few reactive cells which were positive for CD20, Ki-67, and B-cell lymphoma (BCL)-2 [Figure 3].
Figure 3: Histopathological examination(HPE) of pleural biopsy with tiny linear fragment of fibrocollagenous tissue showing diffuse infiltration by atypical cells which are predominantly CD3 positive. They are negative for Tdt and CD10. Few reactive cells are positive for CD20, Ki-67, and BCL-2

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From the above biopsy findings, final diagnosis of pulmonary T-cell lymphoma was made based on clinical, radiological, pathological, and immunohistochemical features. Further evaluation of immunohistochemical markers for subtyping of T-cell lymphoma was not done as the patient was not willing for further procedure and due to poor performance status. Hence, the patient was referred to the medical oncology department and was treated with chemotherapy (500 mg cyclophosphamide, 30 mg adriamycin, 2 mg vincristine for the 1st day, and 40 mg/d prednisone for 5 days) for four cycles. Later, the patient deteriorated and expired due to pulmonary infection.

 > Discussion Top

PPL is defined as a clonal, lymphoid proliferation affecting single or both lungs and that has not spread outside the lungs on diagnosis or in the following 3 months.[3] PPL is of either B-cell or T-cell type. B-cell lymphoma arises from the bronchial-associated lymphoid tissue and it constitutes about 3%–4% of non–Hodgkin's lymphoma.[2] Malignant lymphoma of T-cell origin in the lung is very rare and aggressive, therefore, early diagnosis can be achieved using pathology and immunohistochemistry findings. The histomorphology of pulmonary T-cell lymphoma is very broad which includes a mixture of large and small tumor cells, clear cytoplasm, and a common appearance of vessels and eosinophils in stroma.[4]

Peripheral T-cell lymphoma (PTCL) comprises a group of rare, aggressive cancers that develop from T-cells that are at different stages of maturity. The World Health Organization has divided the various types of PTCL into two main categories: (1) precursor T-cell neoplasms, which include precursor T-lymphoblastic lymphoma/leukemia, and (2) peripheral T-cell neoplasms, which are subcategorized as predominantly leukemic disease, predominantly disseminated disease, predominantly extranodal disease, and predominantly nodal disease. The cases that do not match one of the defined entities of PTCL are best categorized as “not otherwise specified.”[5]

Respiratory symptoms, fever, and weight loss are usual presenting symptoms of PPL. The median age of presentation of PPL is about 60 years, except in the case of patients with HIV infection who may present earlier.[6],[7] The chest X-ray of PPL can show pulmonary mass, nodules, consolidation, atelectasis, or pleural effusion. In patients with HIV infection, PPL can present as multiple nodules with cavitation.[8] For histological diagnosis, bronchial or transbronchial or transthoracic fine-needle aspiration or biopsy is required. Histological features include the presence of monomorphic atypical lymphoid cells with high mitotic activity, affecting bronchial, vascular, or pleural structures. Coagulative necrosis may be evident in some cases because of the angiocentricity.[9] Immunohistochemical staining is required to rule out the diagnosis of carcinoma, melanoma, or sarcoma. CD3 was the most specific marker for T-lymphocyte-associated antigens. Other markers which were expressed with T-lymphocytes are CD2, CD5, CD43, and CD45RO. B-lymphocyte-associated antigens are CD20, CD19, and CD79a. In addition, the Tdt (for lymphoblastic lymphoma), CD56 (for natural killer/T-cell lymphoma), and anaplastic lymphoma kinase (for anaplastic large cell lymphoma) were also useful for further characterization of the subtype of lymphoma.[4] In the present case, atypical lymphocytes are CD3 positive, which is a specific marker for T-cell lymphoma. Other markers such as Tdt and CD10 were negative. Further characterization of immunohistochemical markers was needed for further subtyping of the T-cell lymphoma. However, the patient was not willing for the further procedure due to the poor performance status. In our case, the presence of CD3 positivity in biopsy report suggested that our patient is a case of pulmonary T-cell lymphoma.

There were only a few case reports recently on primary pulmonary T-cell lymphoma. A study published by Qiu et al. in 2020 from China reported a case series of 24 patients in which they showed different radiological manifestations of pulmonary lymphoma such as lung infiltration, nodules or masses, and pleural effusion. 16.7% in their case series had single lesion, 83.5% had multiple lesions, and 29.2% had pleural effusion. The present case presented with consolidation and pleural effusion.[10]

A case was reported by Siddique et al. in which the patient initially presented with features of eosinophilic pneumonia on cryobiopsy and later it was diagnosed as pulmonary T-cell lymphoma with postmortem lung biopsy. Hence, it may present with different spectra of radiological and histopathological features and immunophenotyping is vital in diagnosing primary pulmonary T-cell lymphoma.[11]

There is no consensus on treatment for T-cell lymphoma. Current treatment options are surgery (for localized lesions), chemotherapy (for bilateral or extrapulmonary involvement, relapse, or progression), and radiotherapy (rarely). Most of the subtypes of PTCL, the treatment regimen is typically cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) based chemotherapy or etoposide added to CHOP in the frontline setting.[12]

The present case presented with lung consolidation which is a common radiological finding among elderly. Similarly, bronchogenic carcinoma with metastases can also present with lung consolidation with multiple nodules in the same or other lungs in this age group. As PPL and bronchogenic carcinoma have a similar radiological presentation, but different treatment and prognosis, histopathology, and immunohistochemical markers play an important role in early diagnosis of PPL.

 > Conclusion Top

PPL is a rare disease and can present with nonspecific symptoms. Radiologically, it can easily be confused with commoner malignancies such as bronchogenic carcinoma with or without metastases. PPL carries different therapeutic and prognostic implications. Therefore, physicians should make every effort to achieve histopathological and immunohistochemical diagnosis before prognosticating a patient who is presenting with lung consolidation and bilateral pulmonary nodules along with effusion.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Kligerman SJ, Franks TJ, Galvin JR. Primary extranodal lymphoma of the thorax. Radiol Clin North Am 2016;54:673-87.  Back to cited text no. 1
Zhang XY, Gu DM, Guo JJ, Su QQ, Chen YB. Primary pulmonary lymphoma: A retrospective analysis of 27 cases in a single tertiary hospital. Am J Med Sci 2019;357:316-22.  Back to cited text no. 2
Cherian SV, Thampy E, Mauzo SH. Primary pulmonary lymphoma: A case of 'unresolving pneumonia'. Am J Med 2014;127:e3-4.  Back to cited text no. 3
Zhang S, Liang B, Jiang S. Primary pulmonary peripheral T-cell lymphoma: A case report and review of the literature. Thorac Cancer 2014;5:104-7.  Back to cited text no. 4
Harris NL, Jaffe ES, Diebold J, Flandrin G, Muller-Hermelink HK, Vardiman J. Lymphoma classification – From controversy to consensus: The R.E.A.L. and WHO Classification of lymphoid neoplasms. Ann Oncol 2000;11 Suppl 1:3-10.  Back to cited text no. 5
Lee HJ, Im JG, Goo JM, Kim KW, Choi BI, Chang KH, et al. Peripheral T-cell lymphoma: Spectrum of imaging findings with clinical and pathologic features. Radiographics 2003;23:7-26.  Back to cited text no. 6
Cardenas-Garcia J, Talwar A, Shah R, Fein A. Update in primary pulmonary lymphomas. Curr Opin Pulm Med 2015;21:333-7.  Back to cited text no. 7
Ray P, Antoine M, Mary-Krause M, Lebrette MG, Wislez M, Duvivier C, et al. AIDS-related primary pulmonary lymphoma. Am J Respir Crit Care Med 1998;158:1221-9.  Back to cited text no. 8
Borie R, Wislez M, Antoine M, Cadranel J. Lymphoproliferative disorders of the lung. Respiration 2017;94:157-75.  Back to cited text no. 9
Qiu YJ, Zhang MZ. Clinical analyses of 24 patients with primary pulmonary NK/T-cell lymphoma. Zhonghua Xue Ye Xue Za Zhi 2020;41:40-6.  Back to cited text no. 10
Siddiqui N, Charles K, Ferraro AE, Holladay RC, Johnson JC, Kalanjeri S. Pulmonary T-cell lymphoma masquerading as eosinophilic pneumonitis. Lung India 2019;36:543-5.  Back to cited text no. 11
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Avilés A, Castañeda C, Neri N, Cleto S, Talavera A, González M, et al. Results of a phase III clinical trial: CHOP versus CMED in peripheral T-cell lymphoma unspecified. Med Oncol 2008;25:360-4.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3]


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