Lymphomatoid granulomatosis (LYG) is a B-cell lymphoproliferative disease. The etiology of this disease is not clearly elucidated. We describe a case of LYG in a patient with HIV presenting with a localized mass.
A 48 year-old woman with HIV diagnosed since 1999 and was admitted to the hospital with progressive shortness of breath, fever, and productive cough for one week.Six was previously treated for pneumocystis jiroveci pneumonia (PJP) and herpes zoster. She was on antiretroviral therapy and prophylaxis with dapsone and azithromycin. She was a non-smoker and denied using illicit drugs. On physical examination her temperature was 100.4 F with oxygen saturation of 95% on room air. There was no lymphadenopathy. Chest examination showed a localized wheeze in the right infraclavicular area. Other examination was unremarkable. Laboratory results were notable for leukopenia with white blood count of 3.4 ×103 and hemoglobin of 6.4 gm/dl, CD4 count was 1 cells/UL with viral load 51,600 copeis/ml. Chest x-ray showed a new mass of the right upper lobe measuring approximately 6 cm diameter which was confirmed on CT chest. She was treated with vancomycin and Piperacillin-Tazobactam with no significant clinical improvement. A bronchoscopy revealed narrowing of the right upper anterior segment bronchus with no endobronchial lesion. Histopathology from transbronchial lung biopsies was suggestive of B-cell lymphoproliferative process but was not clearly diagnostic. Microbiological stains and cultures were all negative. A video-assisted thoracoscopic surgery (VATS) was performed which showed necrotic tissue with negative microbial cultures. A repeat chest CT showed progression of the mass with necrotic features and complete destruction of the right upper lobe. Hence a right upper lobectomy was performed. Gross examination showed the lobe was filled with grayish-white, necrotic tissue. The pathology showed granulomatous angitis with atypical lymphoid infiltrate consistent with LYG.
LYG is a B-cell lymphoproliferative disease. There is no known etiology but an association with immunocompromised states, such as AIDS, Sjögren’s syndrome, chronic viral hepatitis, rheumatoid arthritis, and renal transplantation has been described. In most cases LYG predominantly involves the lungs however it can affect any organ system including skin and nervous system. Pulmonary LYG presents with SOB and productive cough and is often associated with systemic symptoms such as fever and malaise. Massive hemoptysis and respiratory failure have also been reported. In 80% of cases radiographically LYG presents as peripheral bilateral nodular infiltrates involving the lower lobes. Rarely LYG may also present as mass, cavitations, atelectasis or lobar obstruction as in our case.Histology of LYG typically shows severe destructive inflammatory granulomatous angiitis with infiltration of large atypical B-cells. More recent data suggests that LyG is a type of B-cell lymphoma and the prognosis is not favorable. The treatment includes steroids and cytotoxic drugs. Anecdotal reports suggests that Interferon-alpha 2B and Rituximab may benefit in some cases. Radiation therapy may be help in cases that have a localized lesion.
LYG is a B-cell lymphoproliferative disease which often has a poor outcome. The diagnosis may be difficult as in the case described because of angiodestructive nature of LYG which lead to severe necrosis. Hence the TBLB and VATS biopsy were not sufficient to make a conclusive diagnosis. Additionally, the diagnosis depends on finding multiple pathologic features, including angiitis, granulomatosis, multiple types of inflammatory cells and the presence of atypical polyclonal B cells which were not all seen on the initial biopsies. Thus the diagnosis of LYG may be delayed and may be difficult in cases with localized lesion as in our case. This case also illustrates that in patients with HIV LYG should be considered in the differential diagnosis of lymphoproliferative diseases.
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