Brigitte Dragosics

Gastrointestinal malignant lymphomas of the mucosa-associated lymphoid tissue: factors relevant to prognosis.

Three hundred seven cases (244 gastric, 63 intestinal) of primary gastrointestinal non-Hodgkins lymphoma (NHL) in stages EI and EII, according to a modified Ann Arbor system, were examined retrospectively. The histological classification for mucosa-associated lymphoid tissue-derived lymphomas was applied. Gastric NHLs (male-female ratio, 0.97; mean age, 64.5 years) were stage EI in 51% and stage EII in 49% of cases. Histological grade of malignancy was low in 41% and high in 59% of cases; all NHLs were B-cell type. Tumors were radically resected in 87%, and overall 2-, 5-, and 10-year survival rates were 61%, 55%, and 46%, respectively. Early lymphomas (substage EI1) had best prognosis (5- and 10-year survival rates, 90% and 70%, respectively). Intestinal NHLs (male-female ratio, 1.1; mean age, 54.4 years) were stage EI in 30% and stage EII in 70% of cases. Histology was low grade in 21% and high grade in 79%, and all but 11 cases were B-cell type. In 58% of cases, radical tumor resection resulted in overall 2- and 5-year survival rates of 44% and 24%, respectively. Major prognosticators for survival in gastric location were low-grade histology, low depth of infiltration, and low stage and radical resectability of lymphoma; all factors were strictly intercorrelated. In intestinal site, radical tumor resectability was highly significant for survival. Cumulative proportion of relapses after 5 years was higher in intestinal than in gastric sites (44% vs. 22%). In conclusion, primary gastrointestinal tract NHLs may represent an entity with respect to characteristic histological features, focal tumor growth, and potential cure by radical resection. Because of late relapses, clinical follow-up is needed.

Wilson's disease in patients presenting with liver disease: A diagnostic challenge

BACKGROUND & AIMS In patients with Wilsons disease presenting with liver involvement, the correct diagnosis is often missed or delayed. The aim of this study was to find an algorithm for diagnosis of this difficult patient group. METHODS Clinical and laboratory findings of 55 patients with Wilsons disease were evaluated at diagnosis before treatment. Presenting symptom was chronic liver disease in 17 patients, fulminant hepatic failure in 5 patients, hemolysis in 3 patients, and neurological disease in 20 patients, and 10 patients were detected by family screening (siblings). Evaluation included neurological and ophthalmologic examination, routine laboratory tests, and parameters of copper metabolism including liver copper content in 43 liver biopsy specimens. RESULTS In the whole group, serum ceruloplasmin level was <20 mg/dL in 73%, urinary copper excretion was increased in 88%, and liver copper content was elevated in 91% at diagnosis. Kayser-Fleischer rings were detected in 55%. In contrast to patients with neurological disease (90% Kayser-Fleischer rings, 85% low ceruloplasmin), only 65% of patients presenting with liver disease were diagnosed by these typical findings. Ceruloplasmin levels were lower in patients with Kayser-Fleischer rings or with neurological disturbances than in patients without these symptoms. CONCLUSIONS The commonly used clinical and laboratory parameters are not sufficient to exclude the diagnosis of Wilsons disease in patients with liver disease of unknown origin.

Primary gastrointestinal non-Hodgkin's lymphomas. A retrospective clinicopathologic study of 150 cases

The records of 150 primary gastrointestinal (GI) lymphomas in adults collected from 1974 to 1982 at the Department of Pathology, University of Vienna, were reviewed. One hundred thirty‐three cases of malignant lymphomas (ML) were analyzed with respect to histologic type, presenting tumor stage, and clinical course, as well as for factors influencing prognosis. The histologic type of ML as assessed by the Working Formulation and the Kiel, the Lukes and Collins, and the Rappaport classifications showed only a minor influence on prognosis. MLs of follicular center cell origin prevailed in the stomach and large cell, immunoblastic MLs prevailed in the bowel. Immunoperoxidase studies indicated a B‐cell nature of GI MLs and demonstrated intracytoplasmic IgM kappa or lambda in most of the MLs of the small lymphocytic, plasmacytoid, and immunoblastic type, respectively. The 105 cases of gastric MLs represented 3.6% of all malignancies of the stomach collected during the study period. Clinical symptoms preceded the diagnosis by 4.4 months on average, and endoscopic biopsy specimens indicated malignancy in 78%. Presenting tumor stages of gastric MLs according to the Ann Arbor staging system were Stage I in 20%, Stage II in 76.2%, and Stage IV in 3.8%. The 28 cases of intestinal ML localized in the small and large bowel without any site prevalence presented with Stage I in 14%, Stage II in 82%, and Stage III in 4%. Tumor resection was performed in 90% of all cases and was followed by multiagent therapy in 53%. Radical tumor resection was obtained in 58% of the gastric MLs and only 28.6% of the intestinal MLs and was closely related to tumor stage. Statistical analysis demonstrated a significant influence of the presenting tumor stage on prognosis as expressed by the overall 2‐year survival rate of 70% for Stage I versus 39% for Stage II ML. In addition, Stage II1 according to Musshoff et al. run a better course than II2 as shown by the disease‐free 2‐year survival rate of 49% versus 15%, respectively. Radical tumor resection was a major determinant of survival and cure of disease as exhibited by the disease‐free 2‐year survival rate of 57% after radical resection versus 8% after nonradical resection of ML. Finally, diffuse tumor growth and tumor penetration of the gastric wall beyond serosa decreased the survival rates.

Expression of the mucosal homing receptor α4β7 in malignant lymphomatous polyposis of the intestine

Abstract Recent studies have identified the integrin α4β7 as a mucosal homing receptor that mediates lymphocyte migration to the intestinal mucosa by binding to MAdCAM-1, which is a vascular recognition molecule (adressin) selectively expressed on mucosal endothelium. The expression of the α4β7 mucosal homing receptor was studied in eight cases of malignant lymphomatous polyposis (MLP). This unusual presentation of non-Hodgkins lymphoma of mantle cell type is characterized by multifocal lymphomatous involvement of the gastrointestinal tract. Unlike nodal mantle cell lymphomas, cases of MLP showed expression of α4β7, suggesting that this homing receptor plays an important role in determining the characteristic mucosal dissemination pattern of MLP.