An 18-year-old female presents with 5 months of pleuritic pain, cough, hemoptysis, 12 lb weight loss, reduced appetite and fever.
The cough,initially dry, later was blood streaked. She was of average size. Vitals signs were stable and she was afebrile. She reported no exposure to sick contacts or tuberculosis. Physical examination: rhonchi in right upper lung zone, no lymphadenopathy. Mild finger clubbing noted. She was PPD negative and anergic. Laboratory findings: Hemoglobin 11.6 gm%, Hct 35.1, Platelets 402K, WBC 24.1K(Neutrophils 85%, Bands 2%), ESR 110 mm/hr, CRP 8.5 mg/dL and ACE level 25 U/L. Chest radiograph demonstrated a large right upper lobe cavitary opacity and mediastinal widening. Chest CT showed the consolidation measuring 8.5 cm and extensive mediastinal lymphadenopathy. Bronchoscopy and bronchoalveolar lavage(BAL) without biopsy were negative for AFB on smear. She was treated with antibiotics and discharged on isoniazid, rifampin, ethambutol and pyrazinamide, pending cultures. Four weeks later the symptoms worsened. Cultures were negative to date. Repeat chest CT showed new patchy consolidations in the right upper and lower lobes with cavitation. The mediastinal nodes had enlarged. Repeat bronchoscopy with transbronchial biopsy revealed Hodgkin’s Lymphoma(HD), confirmed by immunohistochemical markers (CD45, CD15 and CD30). Staging revealed splenomegaly and positive bone marrow. She underwent chemotherapy and went into remission.
The differential diagnosis of multiple cavitating pulmonary densities includes: developmental, traumatic, thromboembolic,vasculitic and rheumatic diseases; sarcoidosis, silicosis, coal workers pneumoconiosis, infections(bacterial,tuberculosis, nontuberculous mycobacteria, fungal), cystic fibrosis, immunodeficiency, primary neoplasms and metastatic tumor. The lung in HD is a commonly involved extranodal site (1), and particularly the nodular sclerosing subtype (2). Secondary spread via lymphatic or hematogenous routes results in interstitial or mass lesions. Cavitation occurs in less than 1% of adults, developing initially or after treatment (3). Explanations for cavitation include central necrosis, secondary infection with liquefaction, and abscess formation. TNF-α may play a role in cavitating lung lesions in HD(1). Clubbing in a child with HD is unusual (3). Intrathoracic neoplasms are a major cause of clubbing and hypertrophic osteoarthropathy in adults but rare in adolescents. This patient had clubbing, but no clinical or radiographic evidence of osteoarthropathy. HD presenting as lung masses in the pediatric population is uncommon. Cavitation of those masses is even rarer. In a 10 yr retrospective analysis of 161 pediatric lymphoma patients by Blane et al (4) only 12% had HD involving the lung and, among all forms of pulmonary lymphoma (HD, Non-Hodgkins Lymphoma and post-transplant lymphoproliferative disorder) only 9% had cavitation. In personal discussion and review of the series with Dr. Blane, she did not find even a single case of cavitating HD of the lung.
The rarity of cavitary HD relative to cavitary tuberculosis in our adolescent population led the initial bronchoscopist to perform BAL only. Despite the negative BAL empirical antibiotic and anti-tubercular therapy was given for four weeks, allowing her condition to worsen. We are reporting this rare case of cavitating HD of the lung in an adolescent to demonstrate the diagnostic dilemma in our patient population. The delay in diagnosis with empirical treatment in spite of negative smears in the context of a known high yield of a BAL in cavitary tuberculosis emphasises the need for early lung biopsy.
Shubhra Ray, None.