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Primary Plasma Cell Leukemia Followed by a Pleuropulmonary Plasmacytoma FREE TO VIEW

Hernan Difilippo, AAS; Jaime Guillen, AAS; Silvia Quadrelli, AE-C
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Fundacion Sanatorio Guemes, Buenos Aires Argentina, Recreo, Argentina

Chest. 2015;148(4_MeetingAbstracts):512A. doi:10.1378/chest.2281613
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SESSION TITLE: Imaging Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Plasmacytomas are tumors of plasma cells. The cells are identical to those seen in multiple myeloma, but they form discrete masses of cells in the skeleton or in soft tissues (extramedullary plasmacytoma), not associated to systemic disease. Plasma cell leukemia (PCL) is associated to a very poor prognosis with a reported median survival of 6 - 8 months, even after autologous or allogeneic stem cell transplantation.

CASE PRESENTATION: A 65 year-old man presented to our Unit with progressive shortness of breath starting 15 days previous to admission. History of left renal cancer with surgical treatment, IRC in hemodialysis three times weekly secondary to drug toxicity. He had been diagnosed as having a PCL 6 months before. He was receiving treatment hyperCVAD regime. After chemotherapy was initiated, the patient entered complete remission. Computed tomography showed bilateral pleural effusion and a large mass (41 x 46 cm) adjacent to the pleura. Mediastinal and axillar nodal enlargement was present. Biochemical analysis of the pleural fluid confirmed that it was an exudate. A CT-guided biopsy was performed. The immune profile correspond to a well-differentiated myeloma plasma cells.

DISCUSSION: Solitary bone plasmacytoma (SBP) affects fewer than 5% of patients with plasma cell disorders. Extramedullary plasmacytoma (as the presented in our patient) represents approximately 3% of all plasma cell neoplasms. Only a few reports of pleural involvement by a plasmacytoma have been described. This case is unique because of the extremily rare occurrence of pulmonary and pleural plasmacytoma but mostly, because the extramedullary tumor followed the remission of a PCL. Plasmacytoma and plasma cell leukaemia are at opposite ends of a spectrum, with multiple myeloma in the middle, but all of them usually occur as isolated conditions. To our knowledge, only five cases have been previously described, none of them in the lung or pleura.If a pulmonary mass is diagnosed as a plasmacytoma, a thorough evaluation is essential to exclude systemic disease at initial presentation; and a close clinical follow-up to detect the conversion to a systemic disease.

CONCLUSIONS: This case is unique because of the extremily rare occurrence of pulmonary and pleural plasmacytoma but mostly, because the extramedullary tumor followed the remission of a PCL. Draws atention to the possibility of plasma cell neoplasias as a differential diagnosis of a pulmonary mass.

Reference #1: International Myeloma Working Group (June 2003). "Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group". Br. J. Haematol. 121 (5): 749-57

DISCLOSURE: The following authors have nothing to disclose: Hernan Difilippo, Jaime Guillen, Silvia Quadrelli

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