Pleomorphic large cell hemato-lymphoma (the so-called "malignant histiocytosis"): clinicopathological and immunophenotypic studies in 35 cases.

Thirty-five patients diagnosed with "malignant histiocytosis" from 1984 to 1989 were studied for clinical, laboratory, histopathological features as well as survival and response to therapy, Immunocytochemistry and immunophenotypic studies were performed in 12 cases using the paraffin immunoperoxidase method. The staining included alpha-1 antichymotrypsin, muramidase, immunoglobulins and monoclonal antibodies specific for T, B lymphocytes and macrophage. From the clinical features, responsiveness to therapy and survival, the patients were divided into 2 groups: the non-responders (25 cases) and responders (10 cases) groups.

Pulmonary microthromboemboli in thalassemic cases.

Lung specimens obtained from wet materials were reviewed for detection of microthrombi. Multiple microthrombi, which were composed mainly of platelets, were seen in the pulmonary arteriole and microcirculation in two autopsy cases with splenectomized thalassemic disease. These findings provide a rational explanation for the increased spontaneous platelet aggregation in the whole blood, and may be an additional factor to the progress of pulmonary dysfunction and hypoxemia seen in splenectomized thalassemic patients.

Pulmonary thromboembolism in thalassemic patients.

Chronic pulmonary thromboembolism plays an important role in cardiac failure which is a major cause of death in thalassemic patients over 20 years of age. This report is a study of autopsy lung tissue from 58 patients with beta-thalassemia/hemoglobin E disease (beta-thal/HbE), including whole lungs from five, 13 patients with hemoglobin H disease (HbH), and eight patients with beta-thalassemia major (beta-thal) including whole lung from one. Pulmonary thromboembolic lesions were found in 24 of 58 (41%) patients with beta-thal/HbE, of which 21 of 39 (54%) were splenectomized patients and 3 of 19 (16%) were nonsplenectomized patients, with the incidence increasing with age.

Hypoxemia in thalassemia.

Data are reviewed describing hypoxemia, a newly identified feature in thalassemia. Evidence indicates platelet aggregation in the pulmonary circulation as being a key factor leading to hypoxemia and cor-pulmonale with right heart failure.

Brain pathology in 6 fatal cases of post-transfusion hypertension, convulsion and cerebral hemorrhage syndrome.

Six fatal cases of post-transfusion hypertension, convulsions and cerebral hemorrhage syndrome were found in a series of 76 autopsies of thalassemic patients. Five of these cases were hypertensive. At autopsy the brains were all markedly edematous and congested.

Niemann-Pick disease: a case report.

The second case of infantile Niemann-Pick disease was reported in a Thai newborn baby who presented with respiratory distress and hepatosplenomegaly from birth and who succumbed from bronchopneumonia 20 days later. Autopsy examination showed generalized accumulation of foam cells in reticuloendothelial organs. The parenchymal cells of visceral organs as well as neurones in the central nervous system also showed cytoplasmic vacuolization.

Electron microscopic study of liver tissue from 30 thalassemic patients.

Electron microscopic study of liver tissue from 30 thal patients in advanced stages has been described. In all cases, regardless of the type of hemoglobin, electron microscopic observations gave identical results. Significant findings are ferroacidophilic bodies, ferroacidophilic degeneration of hepatocytes, interhepatocyte collagen, hemosiderin and ferritin, paracrystalline accumulations of ferritin molecules, and liver cell ballooning.

Histochemical study of liver tissue from thalassemic patients.

The livers of 30 cases of advanced thalassemia were studied pathologically. Severe degrees of hemosiderosis and fibrosis were found in liver cells and Kupffer cells in most cases. Various histochemical stains have been used to determine the characteristics of FAB and to grade degrees of iron deposit and fibrosis.

Cardiac pathology in 76 thalassemic patients.

Heart disease is a major cause of death in thal patients after the first decade of life. This study was carried out on autopsy material from 76 patients, six with beta-thal major, 58 with beta-thal/Hb E, and 12 with Hb H disease. Of the 58 patients with beta-thal/Hb E, which form the main group, all but one had cardiac hypertrophy, accompanied by dilatation in 17, five of ten patients with right ventricular and 14 of 25 patients with biventricular hypertrophy had chronic pulmonary thromboembolism.

Pathologic findings in 76 autopsy cases of thalassemia.

Pathologic findings in six beta-thal major, 58 beta-thal Hb E, and 12 Hb H patients were presented. The causes of death were mainly infection in the first and second decades and cardiac failure in older patients. There was accelerated intramedullary and extramedullary erythropoiesis, with a few cases showing focal fibrosis, some with decreased cellularity and occasional atypia.

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.

Intracranial extramedullary hematopoiesis inducing epilepsy in a patient with beta-thalassemia--hemoglobin E.

Generalized epileptiform seizures developed in a 23-year-old patient with beta-thalassemia-hemoglobin E. A computed tomographic scan suggested an intracranial mass. Surgery disclosed an extramedullary hematopoietic mass compressing the brain.

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.

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.

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.

A syndrome of hypertension, convulsion, and cerebral haemorrhage in thalassaemic patients after multiple blood-transfusions.

Various combinations of hypertension, convulsion, severe headache, and cerebral haemorrhage appeared in eight thalassaemic patients after they had received 3--7 units of blood in preparation for splenectomy. Intracranial haemorrhage definitely developed in four patients, three of whom died. At necropsy the brains showed changes similar to those of hypertensive cerebral haemorrhage and hypertensive encephalopathy.

Pathological findings in four cases of human angiostrongyliasis.

Three adults and a 3 1/2-year-old child presented with eosinophilic meningitis. A history of consumption of raw snails was elicited in 2; evidence of a constant diet of raw food, in the form of opisthorchiasis and taeniasis, as well as angiostrongyliasis, was present in the third; no history of raw food ingestion was obtained in the fourth, the child. Pathologic changes were similar in all 4 cases.