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. 2014 Jan 2;5(1):104–107. doi: 10.1111/1759-7714.12042

Primary pulmonary peripheral T-cell lymphoma: A case report and review of the literature

Song Zhang 1, Bin Liang 1, Shujuan Jiang 1,✉
PMCID: PMC4704282  PMID: 26766984

Abstract

Primary pulmonary T-cell lymphoma is a rare condition characterized by fever, cough, dyspnea, and bilateral pulmonary nodules, usually diagnosed by transbronchial biopsy or computed tomography (CT) guided needle biopsy and pathology. In view of its poor prognosis, it is critical to distinguish and diagnose this disease as early as possible. We report here a case of a 39-year-old man who presented with fever, cough, expectoration with multiple nodules, and patch shadow in both lungs by chest CT. Tissue samples were taken by CT guided needle biopsy. The histological sample and immunohistochemistry showed peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS). After the patient was treated by chemotherapy for two courses, his condition deteriorated rapidly and he died 2.3 months after the onset of the disease.

Keywords: Case report, primary pulmonary lymphoma, T cell lymphoma

Introduction

Primary pulmonary lymphoma (PPL) is uncommon and accounts for only 0.5–1% of all primary pulmonary malignancies, less than 1% of all cases of non-Hodgkin's lymphoma (NHL).1 Seventy to 80% of PPL cases are B cell origin and commonly arise from bronchial mucosa associated lymphoid tissue (MALT).2 Very few cases of pulmonary T-cell lymphoma have been reported and the imaging features of this rare cancer have not been well defined. We report here a new case of primary pulmonary peripheral T-cell lymphoma diagnosed by computed tomography (CT) guided needle biopsy, followed by histology and immunohistochemistry, in a 39-year-old male who presented with fever, cough, and expectoration. After he received two courses of chemotherapy, his condition deteriorated rapidly and he died about 70 days after the onset of the disease.

Case report

A 39-year-old man who had a fever, cough, and expectoration, for a period of two weeks, and who demonstrated no response to antibiotics, was referred to our department. The physical examination did not provide significant information. No enlargement in the superficial lymph nodes was found. The main blood test results were: white blood cell (WBC) 2.48 × 109/L; hemoglobin 124 g/L; and blood platelet 90 × 109/L β2-Microglobulin 6.45 mg/L. Antineutrophil cytoplasmic antibody (ANCA) and blood sputum and urine cultures were negative. Electrolyte, renal function, tumor markers and urine analysis were normal. Moreover, no abnormal blood cells were detected in the bone marrow. Chest CT revealed multiple bilateral pulmonary nodules and patch shadow, with no mediastinal adenopathies (Fig. 1). Cerebral and abdominopelvic CT, and the ultrasonic inspection of abdominal organs, thyroid and the prostate gland, were all normal. Therefore, the patient initially received symptomatic treatment. The body temperature fluctuated from 36.2°C to 39.0°C. CT guided transthoracic needle biopsy of the nodule lesion was conducted for further diagnosis. Cytological examination revealed cancer cells. The pathological examination revealed a proliferation of small round cells in the alveolar wall (Fig. 2A) To determine the cell type, a series of lymphocyte-associated antigens were detected by immunohistochemistry. Immunohistochemical analysis revealed positive cell proliferation in LCA, CD3 and CD45RO (Fig. 2B,C), and negative in CD20, CD30, ALK, CD79α, TdT, CD56, P63, CEA, CK5/6 and TTF-1. The patient was diagnosed with primary pulmonary peripheral T-cell lymphoma-not otherwise specified (PTCL-NOS). Because of the double lung involvement and obvious fever, the diagnosis was PTCL-NOS, staging IVB. The patient was treated with chemotherapy (500 mg cyclophosphamide, 30 mg adriamycin, 2 mg vincristine for the first day, and 40 mg/d prednisone for five days) for two courses. However, his condition deteriorated rapidly Approximately two months after the attack of the disease, he died of pulmonary infection as a result of myelosuppression.

Figure 1.

Figure 1

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Chest computed tomography (CT) revealed multiple bilateral pulmonary nodules and patch shadow.

Figure 2.

Figure 2

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(A) Small round cell proliferation in alveolar wall (hematoxylin and eosin [HE], 400×). (B) Positive expression of leukocyte common antigen (LCA) marker by immunohistochemistry (×400). (C) Positive expression of CD3 T-cell marker by immunohistochemistry (×400).

Discussion

PPLs are defined as lymphomas affecting one or both lungs (parenchyma and/or bronchi and/or trachea) with no evidence of extrapulmonary extension in the three months following their appearance.1 Maehara et al. and Asano et al. reported the first cases of T-cell non-Hodgkin's lymphoma (NHL) in the lung in 1991.3,4 Until 2009, only 14 T-cell PPLs cases had been reported. Bernabeu Mora et al.5 reviewed 12 T-cell PPLs3,4,6–11 from 1991 to 2009; most of the patients were Japanese. The age of onset ranged from 29–75, and seven cases were over 65 years old. The female/male ratio was 1:2. The majority had symptoms of fever, cough, and dyspnea. There was another review of four T-cell PPLs from 2008 to 2012.5,12–14 The age of onset in this review ranged from 33–70 years old (Table 1). Three of the four patients were Asian and most of them were over 50 years of age. The pathological histology showed a large number of abnormal lymphocytes in the lung tissue. Immunohistochemistry showed expression of T lymphocyte-associated antigens. Among them, positive expression of CD3 was the most specific one for T lymphocyte. Expression of CD2, CD5, CD43, and CD45RO were positive. Expressions of B lymphocyte-associated antigens were negative, including CD20, CD19, and CD79a. In addition, the TdT (except for lymphoblastic lymphoma), CD56 (except for NK/T cell lymphoma), and ALK (except for anaplastic large cell lymphoma), were also negative. Although treated with effective chemotherapies, all four of the patients died shortly afterward. PTCL-NOS commonly occurred in lymph nodes and/or extra-nodal sites, including the spleen and liver,15 with no specific clinical manifestation.16

Table 1.

Summary of primary pulmonary T-cell lymphoma cases reported since 2008

Year Reference Age Sex Clinical symptoms Radiological findings Diagnostic tool Treatment and response
2009 Bernabeu Mora et al.5 70 F Fever, chest pain Multiple pulmonary nodules and pleural effusion Transbronchial biopsy Chemotherapy, death
2010 Minomo et al.12 68 M Cough, dyspnea Reticular shadow and pleural effusion Transbronchial biopsy Chemotherapy
2010 Liang et al.13 33 M Fever, cough, chest pain, weight loss Multiple pulmonary nodules and air bronchogram CT guided needle biopsy Chemotherapy, died in three months
2010 Shin et al.14 52 M Fever, cough, sweating Masses with portion of central necrosis CT guided needle biopsy Chemotherapy, death
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CT, computed tomography.

Malignant lymphoma of T-cell origin in the lung is very rare, therefore, diagnosis depends on pathology and immunohistochemistry. The histomorphology is very broad, including a mixture of large and small tumor cells, clear cytoplasm, and a common appearance of vessels and eosinophils in stroma. Most PTCL-NOS immunophenotypes were not disease-specific,17 and commonly presented as a loss of one or more broad-spectrum antibodies against the T cell. In our report, the patient was definitively diagnosed as peripheral T-cell lymphoma based on the immunohistochemistry, which consisted of positive expressions of T cell-associated antigen, including LCA, CD3 and CD45RO, and negative expressions of CD56, CD20, CD79a, ALK and l TdT, together with negative expressions of P63, CEA, CK5/6 and TTF-1.

For most of the subtypes of T-cell PPL, first line therapy is typically cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisone (CHOP)-based chemotherapy. Adult T-cell lymphoma has a poor prognosis as a result of the life-threatening complications, such as infections and tumor progression. Among five patients (including our report), four patients died of intrapulmonary infection after chemotherapy as a result of myelosuppression, and one patient had an unknown prognosis. Several recent clinical studies showed CHOP could provide about 60% complete remission and a 30–50% five-year survival rate18–20 in PTCL-NOS treatment. No significant difference in survival was determined between those with bilateral or unilateral disease.

In summary, primary pulmonary T-cell lymphoma is a rare entity with generally nonspecific clinical and roentgenographic signs. Therefore, it's crucial to distinguish and diagnose as early as possible. Overall, these lymphomas are associated with a poor prognosis and more aggressive chemotherapy regimens may be considered for recurrent bilateral disease.

Disclosure

No authors report any conflict of interest.

References

  1. Cadranel J, Wislez M, Antoine M. Primary pulmonary lymphoma. Eur Respir J. 2002;20:750–762. doi: 10.1183/09031936.02.00404102. [DOI] [PubMed] [Google Scholar]
  2. Kurtin PJ, Myers JL, Adlakha H, et al. Pathologic and clinical features of primary pulmonary extranodal marginal zone B-cell lymphoma of MALT type. Am J Surg Pathol. 2001;25:997–1008. doi: 10.1097/00000478-200108000-00003. [DOI] [PubMed] [Google Scholar]
  3. Maehara T, Kobayashi H, Kaneko K, Naruse T. A case of T cell lymphoma of the lung. Nihon Kyobu Shikkan Gakkai Zasshi. 1991;29:469–476. (In Japanese.) [PubMed] [Google Scholar]
  4. Asano Y, Ohshima K, Yoshizawa S, Shiraishi G, Kato K. Modified CHOP in an aged patient with non-Hodgkin's lymphoma and probable extensive pulmonary involvement. Int J Hematol. 1991;54:327–329. [PubMed] [Google Scholar]
  5. Bernabeu Mora R, Sánchez Nieto JM, Nieto Olivares A. Bilateral pulmonary nodules as a manifestation of primary pulmonary T-cell lymphoma. Int J Hematol. 2009;90:153–156. doi: 10.1007/s12185-009-0372-8. [DOI] [PubMed] [Google Scholar]
  6. Boon ES, Graal MB, van Noord JA. Primary extranodal non-Hodgkin's lymphoma of the lung presenting with bilateral, patchy infiltrates dramatically improving after corticosteroid therapy. Chest. 1993;104:1292–1293. doi: 10.1378/chest.104.4.1292. [DOI] [PubMed] [Google Scholar]
  7. Hanada N, Abe T, Katagiri M, et al. A case of T-cell lymphoma showing multiple nodular shadows and an elevated titer of human T-lymphotropic virus type I (HTLV-I) antibody. Nihon Kyobu Shikkan Gakkai Zasshi. 1993;31:231–234. (In Japanese.) [PubMed] [Google Scholar]
  8. Hanawa T, Chiba W, Fujimoto T, et al. T-cell lymphoma presenting as recurrent bilateral pulmonary infiltrates over five years. Nihon Kyobu Shikkan Gakkai Zasshi. 1996;34:363–368. (In Japanese.) [PubMed] [Google Scholar]
  9. Karakus S, Yalcin S, Güler N, Cöplü L, Ayhan A. Angiocentric T-cell lymphoma of the lung mimicking metastatic carcinoma. Case report. J Exp Clin Cancer Res. 1998;17:371–373. [PubMed] [Google Scholar]
  10. de Torres JP, Kenney L, Celli B. Primary T-cell pulmonary lymphoma. A case report and review of the literature. Arch Bronconeumol. 2002;38:596–598. doi: 10.1016/s0300-2896(02)75297-6. (In Spanish.) [DOI] [PubMed] [Google Scholar]
  11. Laohaburanakit P, Hardin KA. NK/T-cell lymphoma of the lung: a case report and review of literature. Thorax. 2006;61:267–270. doi: 10.1136/thx.2004.025767. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. Minomo S, Takimoto T, Morimura O, et al. Primary pulmonary T-cell lymphoma in a human T-lymphotropic virus type-1 carrier showing atypical shadow. J Thorac Oncol. 2010;5:558–559. doi: 10.1097/JTO.0b013e3181d3cccb. [DOI] [PubMed] [Google Scholar]
  13. Liang WJ, Zhou XY, Xu SL. Air bronchogram sign in primary pulmonary T-cell lymphoma with multiple nodules. Iran J Radiol. 2010;7:105–108. [Google Scholar]
  14. Shin CH, Paik SH, Park JS, et al. Primary pulmonary T-cell lymphoma: a case report. Korean J Radiol. 2010;11:234–238. doi: 10.3348/kjr.2010.11.2.234. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Niitsu N, Okamoto M, Nakamine H, Aoki S, Motomura S, Hirano M. Clinico-pathologic features and outcome of Japanese patients with peripheral T-cell lymphomas. Hematol Oncol. 2008;26:152–158. doi: 10.1002/hon.853. [DOI] [PubMed] [Google Scholar]
  16. Weisenburger DD, Savage KJ, Harris NL, et al. Peripheral T-cell lymphoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood. 2011;117:3402–3408. doi: 10.1182/blood-2010-09-310342. [DOI] [PubMed] [Google Scholar]
  17. Swerdlow S, Campo E, Harris N, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue. Lyon: IARC Press; 2008. pp. 269–317. [Google Scholar]
  18. Avilés A, Castañeda C, Neri N, et al. Results of a phase III clinical trial: CHOP versus CMED in peripheral T-cell lymphoma unspecified. Med Oncol. 2008;25:360–364. doi: 10.1007/s12032-008-9046-2. [DOI] [PubMed] [Google Scholar]
  19. Morabito F, Gallamini A, Stelitano C, et al. Clinical relevance of immunophenotype in a retrospective comparative study of 297 peripheral T-cell lymphomas, unspecified, and 496 diffuse large B-cell lymphomas: experience of the Intergruppo Italiano Linformi. Cancer. 2004;101:1601–1608. doi: 10.1002/cncr.20531. [DOI] [PubMed] [Google Scholar]
  20. Sonnen R, Schmidt WP, Müller-Hermelink HK, Schmitz N. The International Prognostic Index determines the outcome of patients with nodal mature T-cell lymphomas. Br J Haematol. 2005;129:366–372. doi: 10.1111/j.1365-2141.2005.05478.x. [DOI] [PubMed] [Google Scholar]

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