1/66. A case of intra-abdominal multiple lymphangiomas in an adult in whom the immunological evaluation supported the diagnosis. A 60-year-old patient with intra-abdominal lymphangiomatosis is described. He presented with anaemia due to enteric haemorrhage, hypoproteinaemia with heavy hypogammaglobulinaemia and T-cell lymphopenia. Duodenal biopsy showed lymphangiectasia while a small bowel study revealed several filling defects in the terminal ileum. On exploratory laparotomy, numerous inoperable lymphangio-haemangiomata were found, involving the small and large intestine, appendix, mesenterium, gallbladder and main biliary tract. The importance of T-cell lymphopenia and hypogammaglobulinaemia in the diagnosis of intra-abdominal lymphangiomatosis with lymphangiectasia is stressed. ( info) |
2/66. Spontaneous regression associated with apoptosis in a patient with acute-type adult T-cell leukemia. We describe a 76-year-old man with acute-type adult T-cell leukemia, who demonstrated a spontaneous decrease in leukemic cell number, apparently coincident with apoptotic cell death. On admission the patient's white blood cell count was 38.9 x 10(9)/l with 77% abnormal lymphocytes. He also had hypoproteinemia (4.3 g/dl) from protein losing enteropathy. After admission the leukemic cell count decreased without chemotherapy, reaching 5.9 x 10(9)/l after 2 months. Studies of peripheral lymphocytes demonstrated appearance of the apoptotic cells and dna ladder formation from the beginning of regression. Same truncated proviral dna was recognized in primary ATL cells through the whole clinical course. The hypoproteinemia improved with intravenous nutrition, followed by increase of the leukemic cells. This case is the first report that demonstrates tumor-cell apoptosis induced clinical regression in adult T-cell leukemia. Further, we speculate that the hypoproteinemia may have been involved in the leukemic cell apoptosis. ( info) |
3/66. Lymph loss in the bowel and severe nutritional disturbances in Crohn's disease. A severe nutritional deficiency status is described in a 22-year-old patient with Crohn's disease. The clinical picture on admission was dominated by an episode of gastrointestinal bleeding secondary to clotting disturbances (vitamin k deficiency) and severe cachexia due to a protein energy malnutrition. The mechanisms of severe nutritional disturbances in Crohn's disease are multifactorial. In this patient, lymphatic leakage into the intestinal lumen was a major contributing factor in the pathogenesis of protein-losing enteropathy, fat malabsorption, and lymphocytopenia. The authors were able to demonstrate this intestinal lymph loss by nuclear imaging. ( info) |
| Malabsorption is a well-known complication of infection with giardia lamblia. However, selective protein-losing enteropathy is rare. We report a child with anasarca due to hypoalbuminemia as a result of gastrointestinal protein loss. Investigations established giardiasis as the etiology. The child returned to normal health after treatment with metronidazole. ( info) |
5/66. Stable reversal of pathologic signs of primitive intestinal lymphangiectasia with a hypolipidic, MCT-enriched diet. We report on a patient with protein-losing enteropathy due to primitive intestinal lymphangiectasia with an early reversal of clinical and biochemical signs and a stable late reversal of pathologic signs after treatment with a hypolipidic diet enriched with medium-chain triacylglycerols. ( info) |
6/66. Protein-loss into retroperitoneal lymphangioma: demonstration by lymphoscintigraphy and blood-pool scintigraphy with Tc-99m-human serum albumin. A rare, benign congenital lymphangioma has been reported to occur frequently in the neck and axilla, but rarely in the retroperitoneal space. We report a case of a retroperitoneal lymphangioma associated with hypoproteinemia caused by protein-loss into the tumor. In this case, lymphoscintigraphy with subcutaneously injected Tc-99m-human serum albumin (HSA) disclosed the communication between the tumor and the lymphatic system, and sequential abdominal scintigraphy with intravenously injected Tc-99m-HSA revealed the protein loss into the tumor. Abdominal scintigraphy with Tc-99m-HSA injected intravenously or subcutaneously is occasionally useful for determining the etiology of hypoproteinemia. ( info) |
7/66. Severe hypoproteinemia in a fetus after pleuro-amniotic shunts with double-basket catheters for treatment of chylothorax. The prognosis of a fetus with hydrothorax at mid-trimester is extremely poor. We encountered a fetus who developed bilateral chylothoraxes at 23 weeks of gestation. Bilateral pleuroamniotic shunts with double-basket catheters were successfully installed at 25 weeks of gestation. hydrothorax did not recur in this fetus. After the shunting, however, polyhydroamnios, fetal hypoproteinemia, and placental edema developed, and the hydrops worsened. The drainage of the fetal pleural effusion into the amniotic cavity was believed to have contributed to these complications. The infant, born at 29 weeks of gestation, died of cardiac failure and pulmonary hypoplasia. Thus, the shunts did not ameliorate the adverse conditions in this patient. ( info) |
| A 20-year-old woman who had daily contact with domestic herbivores presented with a painless and pruritic lesion in her neck; the lesion ulcerated to a black necrotic eschar from which bacillus anthracis grew. Rapidly expanding edema at the site of the ulcer was followed by shock, hematuria, hypokalemia, and hypoproteinemia. The latter symptoms - unusual for cutaneous anthrax - responded to intravenous penicillin therapy. ( info) |
9/66. A case of toxic shock-like syndrome presenting with serious hypoproteinaemia because of a protein-losing gastroenteropathy. A 37-year-old man was admitted to our hospital because of toxic shock-like syndrome (TSLS) induced by streptococcus pyogenes. After the pathogenic bacteria had been eradicated, serious diarrhoea appeared and a protein-losing gastroenteropathy developed. An immunohistochemical study of the biopsy specimens of both small and large intestines revealed the infiltration of t-lymphocytes, predominantly CD8 cells, into the lamina propria of affected mucosa, villus atrophy and crypt hyperplasia. Considering these histological findings, some immunological mechanism which lead the activation of cytotoxic t-lymphocytes may play an important role in the pathogenesis of this rare intestinal manifestation of TSLS. ( info) |
10/66. giardiasis as the cause of oedema and hypoproteinaemia in a child. This report describes a 3-year-old child who presented with generalised oedema and hypoproteinaemia owing to giardiasis, was treated with oral metronidazole and recovered fully 10 days after therapy. The importance of considering giardiasis in patients with hypoproteinaemia of obscure aetiology is emphasised. ( info) |