SESSION TITLE: Miscellaneous Cases IV
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Monday, October 28, 2013 at 04:15 PM - 05:15 PM
INTRODUCTION: Cutaneous T-cell lymphomas (CTCLs) represent a group of lymphoproliferative disorders characterized by infiltration of the skin by malignant T-lymphocytes. Although vasculitis is known to occur in those patients, it is usually confined to the skin and pulmonary vasculitis is exceedingly rare. To our knowledge, this is the first case of paraneoplastic isolated pulmonary capillaritis (IPC) in association with primary CTCL.
CASE PRESENTATION: A 29-year-old male presented with four days of severe, pleuritic chest pain associated with dyspnea, and dry cough. Two months prior, he had developed a diffuse and intensely pruritic skin rash. Upon admission, physical exam was significant for fever, tachypnea, and bilateral inspiratory crepitations. Skin exam showed a nummular rash involving large parts of the body. Initial labs demonstrated leukocytosis with eosinophilia and mildly elevated transaminases. Chest imaging showed bilateral pleural-based pulmonary nodules. Erythrocyte sedimentation rate was elevated at 51 mm/hr, and urinalysis was unremarkable. A trans-thoracic echocardiogram was negative for vegetations. Extensive workup for a possible infectious, connective tissue disease or vasculitic etiology (including ANCA) was negative. The patient was initially started on broad-spectrum antibiotics for possible septic pulmonary emboli without improvement. Three days later, he developed acute hypoxemic respiratory failure and worsening bilateral air-space densities and required mechanical ventilation. Bronchoalveolar lavage showed scant neutrophils and abundant hemosiderin-laden macrophages. An open lung biopsy demonstrated striking neutrophilic infiltration of pulmonary capillaries, and intra-alveolar fibrin deposits consistent with pulmonary capillaritis. He was started on high-dose methylprednisolone after which he dramatically improved and was extubated. Skin biopsy revealed features consistent with CTCL.
DISCUSSION: The pulmonary involvement in CTCL may include lymphomatous infiltration of the lung parenchyma. To our knowledge, pulmonary capillaritis in association with primary CTCL has never been reported. Our patient was diagnosed with IPC without clinical or serologic features of an associated systemic vasculitis. Management usually consists of aggressive immunosuppressive therapy.
CONCLUSIONS: IPC can be associated with primary CTCL.
Reference #1: Lara AR, Schwarz MI. Diffuse alveolar hemorrhage. Chest. 2010 May;137(5):1164-71.
Reference #2: Ioachimescu OC, Stoller JK. Diffuse alveolar hemorrhage: diagnosing it and finding the cause. Cleve Clin J Med. 2008 Apr;75(4):258, 260, 264-5 passim
Reference #3: Baser S, Onn A, Lin E, Morice RC, Duvic M. Pulmonary manifestations in patients with cutaneous T-cell lymphomas. Cancer. 2007 Apr 15;109(8):1550-5.
DISCLOSURE: The following authors have nothing to disclose: Waseem Barham, Mazen Al-Qadi, Yazan Migdady, Joseph Rabatin, Andrew Artenstein
No Product/Research Disclosure Information