Pitfalls in diagnosis of primary low-grade non-Hodgkin’s lymphomas of the spleen

Abstract

17559 Background: Primary low-grade (LG) non-Hodgkin’s lymphomas (NHL) of the spleen are indolent B-cell malignancies usually involving the liver, bone marrow (BM) and the blood while disseminating. The aim of the study was to outline diagnostic problems in primary LG NHL of the spleen, and to improve recommendations regarding the differential diagnosis between these lymphoproliferative disorders and benign abdominal organ pathology (BAOP). Methods: A series of 105 patients (pts) (age range of 15–82 years) with LG splenic NHL was enrolled in our study. In all pts the diagnosis was confirmed morphologically, with immunophenotyping in selected cases. The following histological types of splenic NHL had been identified: follicular (47.6%), small lymphocytic (37.1%), marginal-zone (11.4%), and mantle cell (3.9%) lymphoma. BM involvement and leukemic conversion (LC) were revealed at diagnosis in 102 (99.0%) and 85 (83.3%) pts, respectively. Results: Leading to diagnosis symptoms resulted from splenomegaly and hematological abnormalities. Of 41 (39.0%) pts with splenic NHL, that one had been initially interpreted and treated as chronic hepatitis with hypersplenism (17/16.2%), liver cirrhosis with portal hypertension and hypersplenism (11/10.5%), gastric ulcer/chronic gastroduodenitis (4/3.8%), chronic pancreatitis (3/2.9%), collagenosis (3/2.9%), or nephritis (1/0.9%). In 12 pts with a preliminary diagnosis of chronic hepatitis, or liver cirrhosis with portal hypertension and hypersplenism splenectomy was performed at the surgical departments of district hospitals as a treatment modality of choice. In these cases the initial histological examination of spleen specimens had failed to reveal the lymphomatous involvement, that led to delay in diagnosis. In 17 (16.2%) pts with stage IV disease LG splenic NHL was confirmed occasionally during prophylaxis follow up, or examinations axed on diagnosis of internal diseases. Conclusions: Primary LG NHL of the spleen exhibit the latent onset and nonspecific clinical picture, that usually lead to delay in diagnosis due to resemblance with BAOP. Progressive splenomegaly, LC, consequent liver, abdominal and peripheral lymph node enlargement may indicate the lymphoproliferative malignancy and require BM aspiration/biopsy for diagnosis. No significant financial relationships to disclose.