Lymphomatoid granulomatosis.A clinicopathologic study of 152 cases

Abstract

A retrospective study of 152 cases of lymphomatoid granulomatosis (LYG) was undertaken to expand current knowledge about clinical manifestations and therapy, and to identify prognostic indicators in this disease. The major clinical and radiographic features and extra-pulmonary sites of involvement noted in previous studies were essentially confirmed in our study. Adverse prognostic factors included neurologic manifestations, and large numbers of atypical lymphoreticular cells within the pulmonary infiltrate. Unilateral chest lesions and large numbers of small lymphocytes and histiocytes within the infiltrate were associated with a better prognosis. Malignant lymphoma involving lymph nodes developed in 12% of patients. Almost two-thirds of patients have died and the median survival was only 14 months. No particular mode of therapy was found to be satisfactory for LYG, although there was some suggestion that corticosteroids may be useful. Randomized prospective studies are necessary for definitive recommendations regarding therapy. Cancer 43:360-373, 1979. YMPHOMATOID GRANULOMATOSIS (LYG) is L a unique form of pulmonary angiitis and granulomatosis which was first described in 1972 by Liebow et al. 17319 Histologically it is a necrotizing angiocentric and angiodestructive infiltrative process composed of small lympho-cytes, plasma cells, histiocytes and atypical lympho-reticular cells. It usually presents in middle age with bilateral pulmonary infiltrates ; skin, nervous system, and kidney are commonly involved, and the mortality is high. Since the initial report which included 40 patients , only a few additional cases of LYG have been reported in the literature'~2~3~7~8~"2.'5.'6.-22,253-33.35 and many questions remain un-answered regarding therapy, prognosis and natural history. The purpose of this paper is to expand the current knowledge about clinical manifestations and response to therapy in a large number of patients, and to identify clinical and pathologic features which may be useful prognostic indicators in this disease. MATERIALS A N D METHODS One hundred and fifty-seven patients with LYG were identified in the consultation files of A. A. Liebow. All of the cases had been referred to Dr. Liebow for consultation; 36 were included in the first description of LYG,lS and 12 others were subsequently pub-hematoxylin and eosin stained microscopic slides of biopsy or autopsy material were available for review in each case; paraffin blocks and wet tissue were available in some and a variety of special stains were done in many. Five of the patients were excluded from the present study because they manifested only extrapulmonary involvement (skin, central nervous system, tongue, nasal cavity and naso-pharynx, liver and mesentery), without evidence of lung involvement. Clinical data and follow-up information were obtained from the referring physicians or the patients themselves. Since we did not have access to hospital charts, details of clinical and laboratory data were not always complete. lished e l s e w h e r e. ...

Epstein-Barr virus–associated lymphoproliferative disorders

“…The most common site of involvement is the lung, with less frequent involvement of other extranodal sites such as the skin, kidney, liver, and central nervous system (CNS) [55]. Since it was first described in 1972 by Liebow et al [56] and for nearly 2 decades, lymphomatoid granulomatosis was thought to be a T-cell lymphoproliferative disorder because of the predominance of CD4-positive helper T cells and the failure to detect any associated B-cell gene rearrangements [57,58].…”

Pulmonary Lymphomatoid Granulomatosis- a Case Report with Review of Literature

“…Major sites of extrapulmonary involvement include kidney (32 %), skin (29 %), liver (29 %) and CNS (26 %) [2]. Upper respiratory tract, gastrointestinal tract, spleen and lymph nodes are rarely affected [3].…”

“…This is the largest study so far where they suggested increased prevalence of disease in patients on immunosuppressants, transplant patients and those with already existing autoimmune disorders. Hence, the role of B cell immune dysfunction and the atypical cell belonging to B cell lineage was proposed [3]. In 1990, in another study EBV genome was detected in 29 cases by polymerase chain reaction [4].…”

Pyrexia, Lung nodules, Granulomas: Pulmonary Lymphomatoid Granulomatosis

“…Lung is most commonly involved organ (80-90 %) followed by skin up to 50 % and nervous system in 30 %. They may occur together at presentation or in sequential or in isolation [3,4]. Patients may present with constitutional symptoms or related to the organ involvement.…”

“…The 5 year mortality is 60-90 % [7]. Poor prognostic predictors are age less than 25 years, high grade histology, CNS involvement and presence of hepatomegaly and leukocytosis [3]. Treatment of LYG is not well established owning to its rarity as well as unpredictable course after therapy.…”