Malignant carcinoid tumor of the appendix with liver and lung metastasis: report of a case with a high level of serum carcinoembryonic antigen.

We report elevated serum carcinoembryonic antigen (CEA) in a case of malignant carcinoid tumor of the appendix with liver and lung metastasis. A 55-year-old Thai man was found to have multiple nodules in the liver by ultrasonography. Serum CEA was 7,387.9 ng/mL (normal 0-4.1 ng/mL) leading to a clinical impression of colonic carcinoma with liver metastasis. During the investigation, he developed acute abdomen caused by ruptured acute appendicitis. Malignant carcinoid tumor of the appendix, 1 cm in diameter and located proximal to the ruptured acute appendicitis, was identified. The tumor cells showed trabecular or insular growth pattern, some nuclear pleomorphism but typically fine nuclear chromatin, frequent mitoses and focal necrosis. They were immunoreactive for antibody to chromogranin, neuron-specific enolase, CEA, and cytokeratin. Tumor metastases were discovered in the liver, right lung, mediastinal and right supraclavicular lymph nodes. Electron microscopic study demonstrated pleomorphic neurosecretory granules of the midgut type of carcinoid tumor.

Clofazimine-induced crystal-storing histiocytosis producing chronic abdominal pain in a leprosy patient.

Clofazimine-induced crystal-storing histiocytosis is a rare but well-recognized condition in the literature. Besides the common reddish discoloration of the skin, clofazimine produces gastrointestinal disturbances-sometimes severe abdominal pain, prompting exploratory laparotomy, because pathologic and radiologic findings can produce diagnostic difficulties if the pathologic changes caused by clofazimine are not recognized. The authors report such a case in a leprosy patient to emphasize the importance of history taking, the radiologic abnormalities of the small intestine, and the pathologic findings in small intestine and lymph node biopsies. Clofazimine crystals are red in the frozen section and exhibit bright-red birefringence. However, they are clear in routinely processed histologic sections because they dissolve in alcohol and organic solvents. They also appear as clear crystal spaces during electron microscopic study, but some osmiophilic bodies can be observed. Histiocytosis caused by clofazimine crystals produces infiltrative lesions in radiologic studies mimicking malignant lymphoma or other infiltrative disorders. Associated plasmacytosis in the histologic sections can simulate lymphoplasmacytic lymphoma or multiple myeloma with crystal-storing histiocytosis. With the knowledge of this rare condition caused by clofazimine, appropriate management to avoid an unnecessary laparotomy is possible.

Pulmonary dirofilariasis in a patient with multisystem Langerhans cell histiocytosis--the first reported case in Thailand.

Despite a high prevalence of canine dirofilariasis, there is no case of pulmonary dirofilariasis reported from Thailand. We herein report a case of multisystem Langerhans cell histiocytosis who had an incidental pulmonary dirofilariasis found at the time of autopsy as a solitary nodule at the periphery of the right lower lobe. This is the first reported case in Thailand. Association between pulmonary dirofilariasis and Langerhans cell histiocytosis has not been described before in the literature.

Intestinal microsporidiosis in HIV infected patients with chronic diarrhea in Thailand.

Microsporidia have been recognized as emerging opportunistic agents affecting multiple organs. Intestinal microsporidiosis caused by Enterocytozoon bieneusi and Encephalitozoon intestinalis is a common disease which is associated with gastrointestinal symptoms, particularly in AIDS patients. So far, information on the frequency of this enteric disease in Thailand is not available. Therefore, the present study was undertaken to investigate the prevalence of intestinal microsporidiosis in HIV infected persons with chronic diarrhea. From 1995 to 1996, multiple diarrheal stool specimens were received and examined for the presence of the organism using Weber's modified trichrome staining method and transmission electron microscopy for confirmation. Twenty-two of 66 patients (33.3%) were positive for microsporidia which appeared as pink-red spores of 0.8-1.2 x 0.7-0.9 microm with the characteristic transverse or oblique band representing the coiled polar filament. Clinical features of these patients included chronic diarrhea (100%), weight loss (100%), abdominal pain (77%), fever (36%), vomiting (36%) and anorexia (18%). Transmission electron microscopic examination of fecal specimens from the 22 patients with positive staining results revealed E. bieneusi in 18 cases.

Liver injury due to iron overload in thalassemia: histopathologic and ultrastructural studies.

The livers of 30 cases of thalassemia (19/beta-thal/HbE, seven thal/HbH and four beta-thal major) were studied histopathologically and electron microscopically, in an effort to define the morphologic alterations due to iron overload. The results of light and electron microscopy were similar in most cases. Iron accumulation and fibrosis were the common features found in these patients, except that thal/HbH exhibited lesser hepatic damage. The degrees of iron deposition and fibrosis were found to be higher in splenectomized and cirrhotic than non-splenectomized and non-cirrhotic patients. The subcellular changes were swollen mitochondria, with the presence of an electron dense matrix and ruptured mitochondrial membrane. Proliferation of smooth endoplasmic reticulum (ER) and dilated rough ER was observed. Increases in lysosomal hemosiderin in hepatocytes and in Kupffer cells were demonstrated. The possible ways by which the iron compounds or free radicals mediated membrane damage are mentioned. The pattern of liver cell damage is similar to that of viral hepatitis.

Ultrastructural defects of the ciliary epithelium in a child with Kartagener's syndrome.

Kartagener's syndrome is a well known classical triad of presentations consisting of bronchiectasis, sinusitis and situs inversus. It is now recognized that the syndrome is an extreme presentation of primary ciliary dyskinesia, a large group of conditions with ultrastructural ciliary defects, leading to poor ciliary motility in various organ systems. A case of Kartagener's syndrome is presented in an eight year old Thai boy in whom the ultrastructural ciliary defects have been examined and described in detail for the first time in Thailand. Incomplete lack of dynein arms was recognized. In addition, disorientation of ciliary axis was noticed. Due to severe bronchiectatic changes of the right lower lobe and right lingular lobe which did not improve despite adequate antibiotics, these lobes were surgically removed. The child has done well since, but still suffers occasional and recurrent bouts of sinusitis.

Liver tissue injury secondary to iron overload in beta-thalassemia/hemoglobin E disease.

Liver tissues from 17 patients with advanced stages of beta-thal/Hb E disease were examined by light and electron microscopy, with emphasis on cellular damage due to excess iron overload. Various histochemical stains were performed to grade degrees of iron deposition and fibrosis, and to demonstrate hepatitis B surface antigen, in Kupffer cells as well as macrophages. The results of light and electron microscopic studies were correlated. There was a variation in the distribution of hepatic iron and the severity of fibrosis. In most of the cases ballooning degeneration of the hepatocytes, increase in number of lysosomes, hemosiderin and ferritin in hepatocytes as well as in the sinusoidal lining cells were found. The ferro-acidophilic body, representing another important form of hepatocellular necrosis also occurred. So far no precise mechanisms can be concluded.

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.

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 demonstrated characteristic intracytoplasmic electron-lucent membrane-bound bodies. The histologic and ultrastructural findings were similar to those described in the literature.

Cytopathic effects of measles virus on the human intestinal mucosa.

We reported a case of measles with diarrhea in an infant. Capsule biopsy samples of intestinal mucosa taken 14 days after onset of fever was damaged and had low disaccharidase activities. Virus particles were found in the damaged villous cell. Second intestinal mucosa biopsy samples taken 28 days after onset of fever was found to be normal in structure, but lactase and sucrase activities were still low. Mucosal damage by virus particles was the cause of diarrhea in our infant.

Type IV glycogen storage disease: first reported case in Thailand.

Glycogen storage disease type IV is rare. The disease is characterized by widespread storage of a polysaccharide similar to amylopectin. An autopsy case of a 6-year-old Thai boy with cirrhosis and deposits in the liver, heart, muscle and central nervous system was described along with histochemical and electron microscopic findings.

Muscular dystrophy with particular oculopharyngeal involvement.

The authors report the case of 33-year-old woman who presented with a 20 year history of external ophthalmoplegia with later involvement of pharyngeal and proximal muscles. Tensilon test excluded myasthenia gravis. Electromyography showed myopathy and muscle biopsy showed evidence of mitochondrial myopathy. Recent investigations revealed that the pathogenesis was probably due to abnormal genetic transmission in mitochondrial inner membrane respiratory chains. To the authors' knowledge, this is the first case reported in Thailand.

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. Iron deposition mirrored that of thal patients in the European and American literature, apart from minimal cardiac siderosis, and the correlation between the amount of iron deposited and dysfunction of the particular organ was not always apparent. Patients with Hb H disease showed a less extensive iron deposition and organ pathology than those with the other two types of thalassemia.

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. Iron deposition, while present in 18 patients, was very slight. Four patients had fibrinous pericarditis, two with diagnostic rheumatic heart disease; 15 patients had chronic pericarditis, with extensive fibrosis in half the cases. In comparison, the six patients with beta-thal major showed more severe cardiac changes, including more iron deposition. Of the 12 Hb H patients, two died of rheumatic heart disease, one had frank bilateral pulmonary embolism and striking right ventricular hypertrophy, while the remaining nine showed little cardiac pathology.

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. The correlation between hemosiderosis and fibrosis as well as the presence of FAB are described.

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. The ultrastructures of FAB, FAD, and balloon cells were similar to those seen in viral hepatitis, but no viral particles were found.

Persistent bacterial endocarditis caused by three isolates of satellite streptococci.

Three isolates of satellite streptococci were cultivated from the blood of a patient affected persistent bacterial endocarditis. They had distinguishable ultrastructural abnormalities. Their cell wall architecture changed from a fuzzy coat (first isolate) to a thick electron transparent layer covered with a rough fuzzy coat (second isolate), and to electron dense globular material which detached from the wall in small patches (third isolate). The antibiotics probably played an important role in changing their architecture. These three isolates were probably derived from the same strain, since they had common biochemical characteristics and they were isolated from the same patient during the course of his endocarditis.