Granulomatosis, lymphomatoid

Granulomatosis, Lymphomatoid

Granulomatosis, lymphomatoid, also known as lymphomatoid granulomatosis, is a rare and potentially aggressive angiocentric and angiodestructive lymphoproliferative disorder. It is characterized by the presence of atypical lymphoid cells, often associated with Epstein-Barr virus (EBV) infection, and primarily affects the lungs, skin, and central nervous system.

Pathophysiology

Lymphomatoid granulomatosis is considered a B-cell lymphoproliferative disorder. The disease is associated with EBV, which is found in the majority of cases. The pathogenesis involves the proliferation of EBV-infected B-cells, which leads to the formation of granulomatous lesions. These lesions are characterized by a mixture of small lymphocytes, atypical lymphoid cells, and reactive histiocytes.

The disease is classified as a type of non-Hodgkin lymphoma, specifically falling under the category of diffuse large B-cell lymphoma (DLBCL) when it progresses to a higher grade.

Clinical Presentation

Patients with lymphomatoid granulomatosis typically present with respiratory symptoms such as cough, dyspnea, and chest pain due to pulmonary involvement. Skin lesions, which may appear as nodules or plaques, are also common. Neurological symptoms can occur if the central nervous system is involved, leading to headaches, seizures, or focal neurological deficits.

Diagnosis

The diagnosis of lymphomatoid granulomatosis is based on histopathological examination of affected tissues. A biopsy of the lung, skin, or other involved sites typically reveals angiocentric and angiodestructive infiltrates with atypical lymphoid cells. Immunohistochemistry is used to demonstrate the presence of EBV in the atypical cells.

Radiological imaging, such as a chest X-ray or CT scan, may show multiple nodular opacities in the lungs. PET scans can be useful in assessing the extent of the disease.

Treatment

The treatment of lymphomatoid granulomatosis depends on the grade of the disease. Low-grade disease may be managed with immunosuppressive therapy, such as corticosteroids. Higher-grade disease, which resembles DLBCL, often requires more aggressive treatment, including chemotherapy regimens like CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) or rituximab-based therapies.

Prognosis

The prognosis of lymphomatoid granulomatosis varies depending on the grade of the disease and the response to treatment. Low-grade disease may have a more favorable outcome, while high-grade disease can be aggressive and challenging to treat.

Also see

• Non-Hodgkin lymphoma
• Epstein-Barr virus
• Diffuse large B-cell lymphoma
• Granulomatous disease