Objective: A significant part of chronic lymphocytic leukemia (CLL) cases receive a diagnosis during the examination of routinely detected lymphocytosis or the investigation of the causes of lymphadenopathy or hepatosplenomegaly. Apart from these, CLL cases may rarely manifest as pulmonary involvement, which can include broncho-pulmonary infiltration, pleural effusion, or an endobronchial lesion. In the literature, cases presenting with CLL-associated broncho-pulmonary infiltration are extremely rare. Here, we present an elderly case with CLL presenting as pulmonary involvement.
Case report: An 82-year-old male patient presented to our hospital with progressive dyspnea, non-productive cough, and weight loss, which had persisted for one month. Chest X-ray radiography revealed opacity in the lower zone of the right lung. Contrast computed tomography (CT) of the chest visualized a soft-tissue density measuring approximately 74mm×75mm in maximal axial dimensions in the inferior segment of the right middle lobe with surrounding ground-glass density and some air bronchogram localized near the medial hilum. Laboratory test results were as follows: hemoglobin level, 13.4g/dL; total leukocyte count, 174×109/L; lymphocyte count, 148×109/L; platelet count, 192×109/L. Peripheral blood smear showed diffuse mature small lymphocytes and smudge cells. Peripheral blood flow cytometry revealed strong positivity for the CD5, CD20, CD19, and CD23 markers, consistent with CLL. A bronchoscopy was performed for diagnostic purposes and a transbronchial biopsy was taken from the lung parenchyma, and bronchoalveolar lavage (BAL) was performed. BAL cytology and microbiological tests were not diagnostic. On immunohistochemical examination of the parenchymal biopsy, neoplastic cells showed a CD20(+), CD5(+), CD23(+), CK(−), CK7(−), CK20(−), CD56(−), synaptophysin(−), chromogranin-A(−), CD3(−), TTF-1(−), Napsin A(−), and P63(−) staining pattern. The Ki67 proliferation index was 10%. The pathology clinic reported the result to be consistent with a chronic lymphocytic leukemia/small lymphoma infiltration. Cervical and abdominopelvic CT results of the patient were also considered and the CLL stage was determined as RAI 2 (moderate risk) and Binet B (moderate risk). However, in consideration of his weight loss and symptomatic extranodal involvement, a chemotherapy protocol with bendamustine and the CD20 antibody rituximab (BR) was initiated. BR treatment was administered every 28 days for up to 6 courses. The patient's symptoms demonstrated marked improvement after two cycles of chemotherapy. After a total of 4 courses, lymphocytosis in the peripheral blood showed complete remission and the involvement that had been visualized on direct chest radiography and CT showed nearly complete remission. After 6 cycles of chemotherapy, the patient was considered in complete remission and follow-up was started.
Conclusion: Pulmonary complications and involvement in CLL typically occur after the diagnosis, in the course of the disease, while there are cases who present as pulmonary involvement (broncho-pulmonary infiltrates, hilar and mediastinal lymphadenopathies, pleural effusion, etc.), although much less frequently. Pulmonary involvement must be considered in patients diagnosed with CLL who have symptoms associated with the respiratory system. Particularly in patients diagnosed with broncho-pulmonary lesions based on peripheral blood analysis or lymph node biopsy, CLL-associated involvement should certainly be included in the differential diagnosis when the most common causes are excluded.
