INTRODUCTION:Castleman’s disease (CD), also known as Giant Lymph Node Hyperplasia, is a rare lymphoproliferative disorder that was first described by Dr. Benjamin Castleman in 1954. There are two clinical variants of Castleman’s disease: unicentric Castleman’s disease and multicentric Castleman’s disease.Patients with multicentric CD have widespread lymphadenopathy with organ involvement and constitutional symptoms. While those with unicentric CD, have a localized disease process, with no reportable symptoms.
CASE PRESENTATION:A 48 year old African American female with a past medical history significant for GERD, and hypertension presented with right sided pleuritic chest pain for one week and a nonproductive cough for two days. She was also complaining of dyspnea on exertion for the past few weeks. Patient denied fever, hemoptysis, and weight loss. Patient was a long-time non-smoker. On examination, patient had normal vital signs. Diffuse lymphadenopathy was noted in the cervical, axillary, and inguinal areas. Chest exam revealed dullness to percussion and decreased breath sounds at the right lung base. Cardiovascular and abdominal examinations were within normal limits. Patient was mildly anemic. She had no electrolyte abnormalities and had normal renal and hepatic function. Both Human Immunodeficiency Virus (HIV) and Human Herpes Virus 8 (HHV-8) tests were negative.Baseline pulmonary function testing showed a mild restrictive pattern with mild resting hypoxemia and normal oxygen saturation on room air. Chest x-ray showed right middle lobe disease with a right pleural effusion. CT chest showed a right middle lobe mass-like lesion with moderate right sided pleural effusion and bilateral axillary lymphadenopathy.A right thoracentesis revealed a lymphocytic pleural effusion (total pleural fluid WBC 3,100 with 53% lymphocytes). The pleural effusion cytology was negative for malignancy. Cultures were negative for bacterial and Mycobacterial infections.Transbronchial biopsy showed chronic bronchitis with fibrosis and hemorrhage. A subsequent right middle lobe open lung biopsy was consistent with lymphoid hyperplasia and lymphocytic interstitial pneumonitis.Submandibular excisional lymph node biopsy revealed CD, with negative immunohistochemical studies for HHV-8.
DISCUSSION:Lymphocytic pleural effusions are commonly found in patients with tuberculosis, pulmonary embolism, leukemia, lymphoma, and patients who are status post coronary artery bypass graft and status post cardiotomy syndrome. This patient presented with a moderate lymphocytic pleural effusion, which is a rare manifestation of CD.Pulmonary involvement in patients with CD is immune mediated. This patient presented with both pleural and parenchymal involvement, the latter in the form of lymphocytic interstitial pneumonitis.CD is divided into two histopathological variants: hyaline vascular and plasma cell. The triggers of the immune activation associated with CD remain elusive, although early studies linked over expression of interleukin (IL)-6 to the systemic manifestations of CD. Unicentric CD is usually the hyaline vascular variant, an isolated benign lymphoproliferative disorder. Patients with unicentric CD are mostly asymptomatic and this disease is not associated with HHV-8. Unicentric CD is generally curable with surgical resection of the lesion. Multicentric CD is typically the plasma cell variant. It is often associated with HIV and HHV-8 and is a systemic disease with generalized peripheral lymphadenopathy, hepatosplenomegaly, frequent fevers, and night sweats. Multicentric CD is progressive in nature and has no proven treatment modalities.
CONCLUSIONS:This patient had multicentric CD of the hyaline vascular variant with both pleural and lung involvement. She had a chronic clinical course with recurrent pleural effusions and worsening respiratory symptoms. This patient received symptomatic treatment and was followed closely.
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