Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature

Abstract

Lymphomatoid granulomatosis is a rare, Epstein Barr Virus (EBV)-associated systemic angiodestructive disorder that may progress to a diffuse large B cell lymphoma. Pulmonary involvement occurs in over 90 % cases followed by kidney, skin and brain. WHO classifies lymphomatoid granulomatosis under the generic heading of B cell proliferations of uncertain malignant potential. Radiologically, pulmonary lymphomatoid granulomatosis (PLG) presents with non specific findings making histopathology the gold standard for diagnosis. The histological diagnosis of PLG includes a triad of polymorphic lymphoid infiltrates, transmural infiltration of arteries and veins by lymphoid cells (Bangiitis^), and focal areas of necrosis within the lymphoid infiltrates. PLG should be distinguished from granulomatosis with polyangitis, as well as other forms of malignant lymphoma, like extranodal NK/T cell lymphoma, secondary diffuse large B-cell lymphoma and primary Non Hodgkin lymphomas of lung.

Clinical and misdiagnosed analysis of primary pulmonary lymphoma: a retrospective study

“…PPL, with a low incidence, comprises < 1% [ 1 ] of all non-Hodgkin’s lymphoma (NHL), and 0.5% of all primary pulmonary malignant tumors [ 2 , 3 ]. Traditionally, it has been defined as a clonal lymphoid proliferation affecting one or both lungs (parenchyma and/or bronchi) in a patient with no clinical, pathological, or radiographic evidence of lymphoma elsewhere, either in the past, or at present, or for 3 months after presentation [ 4 , 5 ]. The vast majority are type of low-grade mucosa-associated lymphoid tissue [ 6 – 10 ].…”

Respiratory tract involvement in primary Sjögren’s syndrome, focus on interstitial lung disease

“…Обнаружение признаков гранулематозного процесса в легочной ткани у пациентов с БШ является показанием для исключения сопутствующего инфекционного процесса (в особенности при проведении иммуносупрессивной терапии), саркоидоза, гранулематоза с полиангиитом [32,43] или онкогематологических заболеваний (солидные опухоли или лимфомы) [93,94].…”