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  Table of Contents  
Year : 2021  |  Volume : 58  |  Issue : 2  |  Page : 302-306

An atypical case of primary pulmonary peripheral T-cell lymphoma misdiagnosed as a pulmonary infectious disease: A case report and literature review

Department of Pulmonary and Critical Care Medicine, The Second Affiliated Hospital of Fujian Medical University, Quanzhou, China

Date of Submission13-Nov-2019
Date of Decision14-Nov-2019
Date of Acceptance19-Jul-2020
Date of Web Publication27-Jan-2021

Correspondence Address:
Xiaoyang Chen
Department of Pulmonary and Critical Care Medicine, The Second Affiliated Hospital of Fujian Medical University, Quanzhou
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijc.IJC_987_19

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How to cite this article:
Shi L, Zeng Y, Lin H, Chen X. An atypical case of primary pulmonary peripheral T-cell lymphoma misdiagnosed as a pulmonary infectious disease: A case report and literature review. Indian J Cancer 2021;58:302-6

How to cite this URL:
Shi L, Zeng Y, Lin H, Chen X. An atypical case of primary pulmonary peripheral T-cell lymphoma misdiagnosed as a pulmonary infectious disease: A case report and literature review. Indian J Cancer [serial online] 2021 [cited 2022 Dec 4];58:302-6. Available from:

  Introduction Top

Primary pulmonary lymphoma (PPL) is a rare disease, accounting for approximately 0.36–1.2% of all malignant lymphomas and 3–4% of extranodal of non-Hodgkin's lymphomas (NHL).[1] The incidence of PPL has shown no significant difference between males and females. The majority of PPLs are tumors of B-cell origin; primary pulmonary peripheral T-cell lymphoma (PTCL) is rarely reported.[2] These patients are not able to further be classified into distinct disease entities and are diagnosed as cases of PTCL-not otherwise specified (PTCL-NOS). As the most prevalent subtype, PTCL-NOS accounts for 30–35% of PTCL cases in Europe and North America, and for approximately 22% of PTCL cases in Asia.[3] PTCL-NOS is a disease that predominantly presents with nodal lymphomas, though extranodal involvement at the initial or relapse stages is not uncommon. The most common sites of PTCL-NOS progression are the skin and gastrointestinal tract, though the bone marrow, the lungs, and peripheral blood can also be involved. The 5-year survival rate of PTCL-NOS is approximately 32% and the 5-year failure-free survival rate is estimated to be 20%.[4] Furthermore, the lack of specificity in clinical and imaging manifestations may lead to the misidentification of PTCL, which is compounded by the presence of common symptoms such as dyspnea, fever, and multiple nodules. Initially, this condition is often misdiagnosed as pneumonia, tuberculosis, or lung cancer. Considering that the majority of patients are at an advanced stage when diagnosed due to the time of disease progression and the overall aggressiveness of the tumor, early diagnosis and prompt treatments are required for management of this disease. The present study reports a case of PTCL presenting as pneumonia and tuberculosis. However, the progression of the condition revealed no response to treatment for pulmonary infection. PPL has similar manifestations and radiographic features with pulmonary infectious diseases, which may prevent diagnosis. Ultimately, a definitive diagnosis is dependent on biopsy results and the pathological evaluation.

  Case Report Top

A 48-year-old man presented with symptoms of cough, expectoration, and occasional release of blood-streaked sputum for 1 month. A week following his presentation (day 1), he was commenced on a 2-week regimen of moxifloxacin (400 mg intravenous QD), and antituberculosis drugs (isoniazid, rifampicin, pyrazinamide, and ethambutol) (days 7–21). The patient was then transferred to our hospital (day 30) when the treatment regimen was ineffective. There was no significant past medical history, and no history of smoking.

Following an examination of the respiratory system, diminished right lung sounds were noted. An enlarged, painless right-sided supraclavicular lymph node was present. This lymph node was palpable, exhibited dimensions of approximately 15 mm × 15 mm and comprised a rubbery and hard substance.

A chest computed tomography (CT) scan indicated a large mass with air bronchogram in the right lung, patchy shadows in both lungs and pleural effusion in the right side [Figure 1]a and [Figure 1]b. Laboratory investigations revealed a normal white blood cell count of 9400/mm3 and a normal neutrophil count (69%; reference range, 50–70%). Assessment of tumor markers and bone marrow smear was unable to detect any abnormalities. The assessment of the number of antinuclear antibodies (ANA) and of anti-neutrophil cytoplasmic antibodies (ANCA), T-cell spot test, plasma 1-3-β-d glucan test, and plasma galactomannan test was nominal. Furthermore, cerebral, abdominal, and pelvic X-ray examinations detected no abnormalities.
Figure 1: (a and b) Chest computed tomography obtained on first admission (day 30), indicating a large mass in the right lung with air bronchograms, patchy shadows in both lungs and pleural effusion in the right side. (c and d) The infiltrations distributing in the left lung were increased in size and number, with large areas of consolidation in right lung. Images c and d were captured on day 42. (e and f) The computed tomography scan was obtained on day 97 indicating a reduction in size of the lesions following chemotherapy treatment. a, c, and e were lung window scans, while b, d, and f were mediastinal window scans. (g) Radial EBUS showed hypoechoic focus all around the left lingual lobe, from which the lesions were considered for biopsy

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Following a provisional diagnosis of tuberculous pneumonia, an alternate antibiotic regimen was commenced (4.5 piperacillin/tazobactam intravenous TID) and antitubercular agents continued for 2 more weeks. Tissues and bronchoalveolar lavage fluid (BALF) specimens obtained via CT-guided percutaneous needle biopsy (CT-PNB) and bronchoalveolar lavage showed lymphocyte, indicating inflammation. There was no evidence of carcinoma. Cultures of the BALF specimens, including acid-fast bacteria and tumor markers, were negative. However, a repeated CT scan indicated continued growth of the lesions. During the course of diagnostic investigation, the patient underwent a right lung biopsy by CT-PNB on day 33. Biopsy on the right supraclavicular lymph node was performed on day 35 and indicated interstitial fibrosis with moderate lymphocyte infiltration and focal coagulative necrosis. Subsequently, the patient underwent a chest CT scan on day 42, and CT images indicated continuous progressive pulmonary lesions [Figure 1]c and [Figure 1d]. Radial endobronchial ultrasound-guided transbronchial lung biopsy (TBLB) was performed on day 43 [Figure 1]g. The biopsy revealed atypical lymphocytes that were characteristic of malignant T-cell lymphoma [Figure 2]a. These cells expressed specific T-cell makers including cluster of differentiation (CD) 2 and 3, whereas the expression of the B-cell makers, such as CD20, was negative. In-situ hybridization of Epstein–Barr virus-encoded early RNA further demonstrated negative results [Figure 2]b,[Figure 2]c,[Figure 2]d,[Figure 2]e. All these biomarkers resulted in a diagnosis of stage IVB PTCL-NOS. In addition, Ki-67 staining was positive (70%) [Figure 2f]. Due to the increased cost of treatment, the patient selected administration of oral chemotherapy (azathioprine 50 mg per oral BID) compared with the first-line option that included cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). His condition improved considerably following oral chemotherapy administration, and the follow-up chest CT scan obtained 2 months after initial visit demonstrated reduced size of the lesion [Figure 1e and f]. His clinical course supports our final diagnosis.
Figure 2: Pathological examination of lung specimens as determined by radial endobronchial ultrasound-guided transbronchial lung biopsy (a). Pathological features of T-cell lymphoma. (a) was stained with hematoxylin and eosin. Immunostaining was positive for CD2 (b), CD3 (c) and negative for CD20 (d). In-situ hybridization of Epstein–Barr virus encoded early RNA (e). Ki-67 staining was positive (70%) (f). Magnification, 200×

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  Discussion Top

PTCL, a subgroup of NHL, is a malignant lymphoproliferative disease originating from peripheral T-cells, which is difficult to diagnose and can be readily misdiagnosed as pneumonia, tuberculosis, and lung tumor. The patient was initially diagnosed with pneumonia. Antimicrobial agents can be effective against pneumonia pathogens, such as mycobacterium tuberculosis, and therefore were selected to treat the patient. However, this type of treatment was ineffective, and the disease progressed. Negative results were produced by ANA and ANCA assays, making connective tissue disease unlikely. Therefore, the presence of malignancy had to be considered, although the tumor makers were all normal and the only evidence supporting this claim was the lung biopsy.

The following criteria are used to diagnose PPL: (1) involvement of lung and bronchial tissues without evidence of mediastinal adenopathy or a mass on the chest radiographs, (2) absence of previous history of extrathoracic lymphoma, and (3) absence of extrathoracic lymphoma or lymphatic leukemia during diagnosis of primary lymphoma of the lung. Furthermore, for making a diagnosis of PPL, the disease must not present outside of the thorax for more than 3 months after the initial diagnosis. According to the World Health Organization classification of tumors of hematopoietic and lymphoid tissues, PTCLs were divided into three categories: nodal, extranodal, and leukemic types, each with multiple disease entities. PTCLs that are not further classifiable into any of the more distinct disease entities are called PTCL-NOS.[5] In the present study, the patient received chemotherapy following a definitive diagnosis and his condition improved considerably. The CT scan indicated gradual disappearance of lesion. Therefore, according to the criteria and pathological evaluation, the patient was diagnosed with PTCL-NOS.

The following keywords were used to search the PubMed database for PTCL-NOS ( “T-cell lymphoma,” “not otherwise specified,” “primary,” “pulmonary,” and “lung.” The review of literature indicated that a total of 12 cases of PTCL were previously reported between 1999 and 2019 with the exception of the case we report in the present study.[6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16] The clinical features of these cases are summarized in [Table 1]. The patients ranged from 49 to 81 years and exhibited a female-to-male ratio of 1:2. The patients presented with fever, loss of weight, cough, dyspnea, and other symptoms and were nonresponsive to antibiotics. The common radiographical findings included infiltration, consolidation, nodules, masses, and opacities.
Table 1: Primary pulmonary T-cell lymphoma reported since 1999

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CT findings of PTCL usually vary and are nonspecific. The 12 cases reported exhibited various types of disease manifestation as follows: Multiple nodules (5/12), masses (3/12), patchy infiltrations (1/12), ground-glass opacities (3/12), and pyothorax (1/12). In the current study, the patient presented with a large mass with air bronchograms, bilateral patchy infiltrates exhibiting pneumonia-like features. These features were detected by chest CT scan. The patient of the present study exhibited nonspecific manifestation and chest imaging features characteristic of multiple large, high-density shadowy patches in both lungs. Air bronchograms are more common in pneumonia, tuberculosis, and lung cancer and are rarely in pulmonary lymphoma. We hypothesize that this sign may be caused by the lung parenchyma which is invaded by lymphoma cells leading to complete or incomplete obstruction of the proximal bronchus.

Since primary pulmonary T-cell lymphoma is difficult to diagnose, leading to a high degree of initial misdiagnosis, approximately 52.9% of the patients are diagnosed by surgical intervention (including open lung biopsy or video-assisted thoracoscopic surgery) and 47.1% by nonsurgical procedures, such as CT-PNB and TBLB.[8],[9],[10],[12],[14],[15] In one case, the patients' course was complicated by squamous-cell carcinoma.[6] PPL often masquerades as other pulmonary conditions, only to be discovered through ill-response to treatment.[14],[15] Following referral of the patient to our hospital, we carried out lung biopsy at multiple sites via both CT-PNB and TBLB. Finally, we were able to make a definitive diagnosis based on histopathology and immunohistochemistry. Therefore, it is crucial to obtain pathological specimens as early as possible. A definitive diagnosis was not achieved until a third lung biopsy was performed. Therefore, repeated lung biopsy may be required in such cases.

The prognosis of primary pulmonary T-cell lymphoma is poor; the longest survival time has been estimated at 2 years.[4] Accurate diagnosis and early treatment are considered vital for prognosis. According to the Ann Arbor Staging System for lymphoma,[17] the stage was classified as Stage IV. Prognostic models have been previously published for use in patients with PTCL-NOS, such as the International Prognostic Index (IPI) and the Prognostic Index for T-cell lymphoma. These models are used for the estimation of the overall survival of the patients with varying combinations of clinical data including age, lactate dehydrogenase (LDH), performance status, stage of disease, presence of more than one extranodal site, and tumor Ki-67 index.[18] In the present case, the patient was 48 years old (<60 years old); the level of LDH was normal and Ki-67 index was 70% (less than 80%). The patient exhibited an IPI score of 1, which indicated low-risk; after receiving prompt treatment, his condition markedly improved.

To date, no guidelines for PPL treatment have been established. Different methods have been reported in published articles, including chemotherapy, radiotherapy, and surgery. Current studies have shown that 4–6 cycles of CHOP are the most common cycle number for first-line treatment of PTCL-NOS and have been widely adopted as a standard type of therapy.[19] Although overall response rates are estimated to approximately 50%, the relapse rates are high, and the long-term outcomes remain poor.[20] Delay in diagnosis and rapid progress of disease are the main causes of the poor patient outcome. In the present study, the definitive diagnosis was made by lung biopsy. Due to the increased cost of treatment, the patient selected azathioprine oral therapy over CHOP. His condition improved considerably and was followed up in respiratory and hematology departments.

In summary, presentation of the current case and literature review of previous studies indicate that the diagnosis and treatment of primary pulmonary T-cell lymphoma may be a challenge to clinicians. The diagnosis depends on histopathology and immunohistochemistry, often necessitating repeated biopsies to reach a definitive diagnosis. The prognosis of PTCL is poor, even with appropriate treatment. The data presented in this case may be summarized as follows: (1) for pulmonary lesions which cannot be treated effectively with antimicrobial therapy, considerable attention should be paid to the possibility of peripheral pulmonary lymphoma if a large number of lymphocytes or atypical lymphocytes are found in the pathological biopsy; (2) multiple biopsies can improve the diagnostic rate if the condition persists; (3) frequent communication with the pathology department is required to ensure successful diagnosis; and (4) the evidence provided by the clinical data is very helpful for the selection of relevant immunohistochemical examinations and the conclusion of the definitive diagnosis. Early diagnosis and prompt treatment can result in a better prognosis.

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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


Dr. Suqin Cai at the Department of Pathology contributed to detailed pathological analyses.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

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  [Figure 1], [Figure 2]

  [Table 1]


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