Pulmonary Manifestations of Systemic Disease: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II |

Pleural Effusion as a Rare Presentation of T-Cell Prolymphocytic Leukemia (T-PLL) FREE TO VIEW

Alyssa Ralph, MD; Bianka Eperjesiova, MD; Arvey Stone, MD
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Lutheran General Hospital, Chicago, IL

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1094A. doi:10.1016/j.chest.2016.08.1201
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SESSION TITLE: Student/Resident Case Report Poster - Pulmonary Manifestations of Systemic Disease II

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: T-cell Prolymphocytic Leukemia (T-PLL) is a rare post-thymic malignancy which comprises two percent of mature lymphoid malignancies1. It is a very aggressive disease with minimal response to conventional chemotherapy and a median survival of approximately seven months2. Common presentations include hepatosplenomegaly (40-73%), lymphadenopathy (53%) or cutaneous lesions (23%)2. Given the aggressive nature of this disease, prompt recognition and diagnosis is required to reduce mortality. We present a case of a 63 year old male who presented with pleural effusion and was subsequently diagnosed with T-PLL.

CASE PRESENTATION: A 63 year-old male with no past medical history presented with non-exertional dyspnea and mild non-productive cough of 2 weeks duration. Initial exam was pertinent for hypoxemia, decreased breath sounds and end expiratory wheezing in right lung fields, abdominal distension, splenomegaly, and axillary lymphadenopathy. CBC revealed leukocytosis of 196,000/mcL with 86% lymphocytes. Peripheral smear revealed cytoplasmic blebs. CXR and CT of chest showed large right pleural effusion. Thoracentesis removed a total of 4L. The fluid analysis was consistent with an exudative effusion with a nucleated cell count of 36,967/mcL and 89% lymphocytes. Flow cytometry and bone marrow analysis supported a diagnosis of T-PLL. He was started on treatment with alemtuzumab.

DISCUSSION: T-cell Prolymphocytic Leukemia is a rare post-thymic malignancy comprising two percent of all mature lymphoid malignancies1. It is an aggressive disease with rapid progression and short median survival2. Prompt recognition and correct diagnosis are therefore essential to reduce mortality. The rarity of this disease makes recognition difficult. Pleural effusion is an uncommon presentation of this disease but should be considered in patients who present with lymphocyte predominant pleural effusion and associated lymphadenopathy and leukocytosis2.

CONCLUSIONS: 1. This case illustrates pleural effusion as a rare presentation of T-cell Prolymphocytic Leukemia. 2. T-PLL is important to consider in the presentation of pleural effusion in the context of lymphadenopathy and leukocytosis. 3. Prompt pleural effusion analysis and cytology interpretation are essential in order to prevent a missed diagnosis.

Reference #1: Swerdlow, S. H., Campo E., Harris N. L., World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon: IARC Press; 2008.

Reference #2: Matutes E., Brito-Babapulle V., Swansbury J., et al. Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. Blood 1991;78:3269-74.

Reference #3: Graham, R. L., Cooper, B., & Krause, J. R. (2013). T-cell prolymphocytic leukemia. Proceedings (Baylor University. Medical Center), 26(1), 19-21.

DISCLOSURE: The following authors have nothing to disclose: Alyssa Ralph, Bianka Eperjesiova, Arvey Stone

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