Recurrent Pleural Effusion Secondary to Multicentric Castleman Disease

Abstract

SESSION TITLE: Pulmonary Manifestations of Systemic Disease 4 SESSION TYPE: Affiliate Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Castleman’s disease is a rare disorder which results from unregulated lymphoproliferation. When involving one lymph node group (unicentric), it is usually benign and asymptomatic. However, when widespread involvement of lymph nodes is present (multicentric) it is often accompanied by numerous symptoms. Pleural effusion has been reported as an uncommon manifestation of this rare disease. CASE PRESENTATION: A 25 year old male with AIDS presented with abdominal pain for three weeks. He also complained of progressively worsening shortness of breath and night sweats but denied any fever, diarrhea, nausea, orthopnea or cough. Medical history was significant for opportunistic infections including MAC, PCP in the past one year and recently diagnosed Kaposi’s sarcoma from skin biopsy. On exam, he had mild diffuse abdominal tenderness, chest auscultation revealed decreased breath sounds and dullness to percussion bilaterally. Diffuse lymphadenopathy was felt in cervical, axillary and inguinal regions. CT scan of the abdomen showed significant bilateral pleural effusion, new from prior imaging one month ago, and intra-abdominal adenopathy. No obvious mass or infiltrate was seen on subsequent chest CT. Right sided thoracentesis on admission drained 1L of exudative pleural fluid per light’s criteria, however, cytology and microbiology was unremarkable. He was found to have leukopenia, macrocytic anemia and, elevated LFT, ESR and CRP. His last CD4 count one month ago was 5. Excision biopsy of axillary lymph node showed Kaposi’s sarcoma and HHV-8 associated multicentric Caslteman’s disease (MCD). IL-6 level was found to be significantly elevated. Patient continued to have recurrent symptomatic pleural effusions requiring repeated taps three times and eventually a pleurx catheter was placed. During this time, patient was continued on ART, MCD was treated with rituximab, etoposide, prednisone and valgancyclovir. After two cycles of chemotherapy, pleural effusion remained stable. DISCUSSION: Pleural effusion can be a result of lymphatic blockage in malignancies. However, in MCD IL-6 is hypothesized to cause capillary leak resulting in exudative pleural effusion. Management of HHV-8 associated MCD remains unclear and challenging as it involves chemotherapy in an already immunocompromised patient. Immunotherapy with Siltuximab is FDA approved for MCD. Due to availability, we treated our patient with anti-CD20 agent rituximab with steroids and cytotoxic agent etoposide. Due to aggressive nature of MCD in our patient, HHV-8 was treated with valgancyclovir. MCD remains a progressive disease despite treatment. CONCLUSIONS: This case highlights the pulmonary complication of Castleman’s disease. Long term catheters are recommended for recurrent pleural effusions, however, treating MCD aggressively may slow down reaccumulation. Reference #1: Reynolds SP, Gibbs AR, Weeks R, et al. Massive pleural effusion: an unusual presentation of Castleman's disease. Eur Respir J 1992; 5:1150-53 DISCLOSURE: The following authors have nothing to disclose: Abdurrahman Husain, Mekedim Bekele, Kibrewessen Tefera No Product/Research Disclosure Information