Immunoperoxidase study on paraffin sections of gastrointestinal lymphoreticular lesions.

Seventy-five cases of lymphoreticular lesions of the gastrointestinal tract were studied by the immunoperoxidase method on paraffin sections which demonstrated cytoplasmic markers, in particular immunoglobulin (Ig) in B-lymphocytes and alpha-1-antichymotrypsin in histiocytes. There were 4 cases of benign immunoproliferative disease (IPD), 1 in the stomach, 3 in the small intestine; their reactions were polyclonal. Large cell non-Hodgkin's lymphoma was subdivided into 21 cases with IPD ("Mediterranean Abdominal Lymphoma") and 29 cases without. Eight cases of lymphoma with IPD were gastric and 13 intestinal; 7 gastric and 12 intestinal lymphomas were of B-cell origin; 1 gastric tumour was histiocytic; 1 intestinal neoplasm had no detectable marker. Sixteen cases of lymphoma without IPD were gastric and 13 intestinal; 4 gastric and 4 intestinal lymphomas were of B-cell type; 3 gastric and 2 intestinal neoplasms were histiocytic; 1 intestinal tumour was a composite of B-cells and histiocytes, in separate but contiguous foci; 9 gastric and 4 intestinal lymphomas had no identifiable marker. One of 18 cases of mixed and small cell lymphoma was accompanied by IPD. In these types of lymphoma Ig was found only in 10-30% of cells, mainly with plasmacytoid differentiation.

Cardiac pathology in 47 patients with beta thalassaemia/haemoglobin E.

Autopsy protocols and heart slides of 47 patients with beta thalassaemia/haemoglobin E disease were reviewed. All but 1 patient had cardiac hypertrophy, accompanied by dilatation in 17; 5 of 9 patients (56%) with right ventricular and 11 of 22 patients (50%) with biventricular hypertrophy had chronic pulmonary thromboembolism. Cardiac iron deposition while present in 15 patients (32%) was very slight, in contrast to the amount of iron in their liver and pancreas. Four patients had fibrinous pericarditis, 2 with rheumatic heart disease. Twelve patients had chronic pericardial changes, 7 with adhesive pericarditis. The effects of cardiac pathology on the morbidity and mortality of patients with beta thalassaemia/haemoglobin E disease were discussed.

Burkitt's lymphoma in Thai children: an analysis of 25 cases.

Twenty five cases of Burkitt's lymphoma in Thai children were diagnosed at the Department of Pediatrics, Siriraj Hospital during the period of 13 years (January 1969 to April 1982). Males were more affected than females with the ratio of 1.7:1. The age ranged from 2 to 11 years with the median age of 4-5 years. The most common clinical manifestations were abdominal mass associated with nausea vomiting, abdominal pain, anorexia, weight loss and generalized lymphadenopathy which occurred in 50-60% of cases. Additional symptoms and signs included anemia, hepatosplenomegaly, edema and pleural effusion. Jaw tumor was found in only 37.5% of the patients. Definite diagnosis depended on the characteristic starry sky appearance of the lymph node biopsy or section of abdominal mass. In advance cases, the tumor cells could be discovered in bone marrow aspiration, ascitic fluid pleural fluid and cerebrospinal fluid. The typical blast cells were detected in the peripheral blood in 4 cases. Antibody to Epstein-Barr virus could be detected in almost all cases with high titers in some cases. Most patients responded very well to local irradiation and chemotherapy with prednisolone plus cyclophosphamide and vincristine or methotrexate. However, relapse occurred rapidly and 80% of the patients died within 3 months after diagnosis with the median survival of only 1 month. Five cases expired early before any specific treatment. The main causes of death were disease, sepsis, excessive bleeding and hyperkalemia.(ABSTRACT TRUNCATED AT 250 WORDS)

Pulmonary artery obstruction in thalassaemia.

A new feature has been encountered in review of a large species of autopsy materials of beta-thalassaemia/Hb E disease. Among 43 patients pulmonary arterial obstructive lesions were found in 19 (44%), of which 17 were splenectomised cases. The pulmonary arterial thromboembolism may have been due to circulating platelet aggregates. This newly discovered pathology may be an additional factor contributing toward dyspnoea and heart failure in thalassaemia besides anaemia and cardiac iron deposition. If it is proven that this pulmonary arterial thromboembolism is indeed due to circulating platelet aggregates, preventive measure by administration of drugs reducing platelet aggregation such as aspirin and Persantin may be indicated, especially after splenectomy.

Immunoproliferative disease of the gastrointestinal tract: a report of five cases.

Five patients with immunoproliferative disease of the gastrointestinal tract are presented. The main pathological feature was dense diffuse lymphoplasmacytic and immunoblastic infiltration of the mucosa and submucosa, of the proximal small bowel in 3 cases and the stomach in 2. Four of the five patients had concomitant immunoblastic sarcoma. The clinical presentation and pathological findings are compared and contrasted with those of "Mediterranean abdominal lymphoma" and alpha chain disease, and the possible role of ethnogeographical and environmental factors in the pathogenesis are discussed.