Progressive multifocal leukoencephalopathy in a kidney transplant recipient after conversion to mycophenolic acid therapy.

Progressive multifocal leukoencephalopathy in a kidney transplant recipient after conversion to mycophenolic acid therapy.

[Gastrointestinal hemorrhage needing blood transfusion as the first manifestation of small bowel carcinoid tumor]. / Transfusionspflichtige gastrointestinale Blutung als Erstmanifestation eines Dünndarmkarzinoids.

Carcinoid tumors arise from enterochromaffin or enterochromaffin-like cells that are present in the gastrointestinal tract, ovaries, and lungs. Over 90% of carcinoids originate in the gastrointestinal tract with the most common sites in order of frequency being the appendix, terminal ileum, rectum, and the remainder of the colon. Gastroduodenal and pancreatic carcinoids are infrequent. Carcinoid syndrome is associated with small intestine carcinoids in about 40%. Common symptoms include intermittent intestinal obstruction with crampy abdominal pain and vomiting, and weight loss. Upper gastrointestinal bleeding with melaena or hematochezia is a relatively rare early symptom of patients with small intestine carcinoid tumors. We report on a 69-year-old man, treated with acenocoumarol for previous thromboembolic complications of hereditary protein S deficiency. He was admitted to hospital because of an acute episode of hematochezia followed by melaena. Endoscopic evaluation of esophagus, stomach, duodenum and colonoscopy revealed no apparent source of bleeding. Selective angiographic evaluation of mesenterial arteries showed pathologic vasculature approximately in mid jejunum. Laparotomy revealed bleeding from a small submucosal malignant carcinoid tumor in small intestine and multiple large metastases within mesenteric tissue. Segmental resection of small intestine and exstirpation of the metastatic masses was performed. Postoperative period was uneventful. Cytotoxic chemotherapy in this adjuvant setting has not been recommended. Small intestinal carcinoid tumor has to be considered as a rare cause of gastrointestinal bleeding with melaena or hematochezia. Nevertheless, bleeding is a relatively rare early symptom of patients with small intestine carcinoid tumor.

[AL amyloidosis--a hematologic disease with initial cardiac manifestation. 2 case reports]. / AL-Amyloidose - Eine hämatologische Erkrankung mit kardialer Erstmanifestation. 2 Fallberichte.

Cardiac involvement in AL-amyloidosis due to a multiple myeloma is present in up to 90% of cases. We present two patients with cardiac symptoms in whom a hematologic disease could be diagnosed because of suspicious cardiac finding. The leading symptom was dyspnea. The routinely performed laboratory tests, especially the erythrocyte sedimentation rate and the electrophoresis, were normal. After exclusion of coronary artery disease an infiltrative cardiomyopathy was suspected because of the echocardiographic examination with marked left ventricular hypertrophy, the restrictive flow pattern at the mitral valve and the electrocardiogram with a low voltage in limb leads and absent R waves in left precordial leads. Further, hematologic workup confirmed the production of light chains due to a myeloma. If the echocardiographic examination and the electrocardiogram raises the suspicion of an infiltrative cardiomyopathy as the cause of dyspnea, an immunofixation should be done in spite of normal laboratory tests to confirm or rule out the presence of a light chain disease due to a myeloma.

Efficacy and toxicity of 2-Chlorodeoxyadenosine (Cladribine)--2 h infusion for 5 days--as first-line treatment for advanced low grade non-Hodgkin's lymphoma.

2-Chlorodeoxyadenosine (Cladribine) is a new purine analogue with high activity in pretreated low grade non-Hodgkin's lymphoma (NHL). To evaluate the efficacy of this drug in untreated patients with advanced NHL, we performed a prospective multicentre trial. Cladribine (0.12 mg/kg) was administered intravenously daily for 5 consecutive days in an out-patient setting. The treatment was repeated every 28 days for four cycles. Included were patients with a histological diagnosis of low grade NHL according to the Kiel classification and stage III or IV disease. Stage II patients were included when radiotherapy had failed. 55 patients were entered into the study. 50 patients were evaluable. The remission rate was 44/50 (88%; 95% confidence interval 82-100%), including complete remissions (CR) in 14 (28%) patients. Only 2 patients showed progression while on Cladribine treatment. The estimated overall survival, and time to treatment failure (TTF) were 85% and 51%, respectively, after a median observation time of 92 weeks. 11 (22%) patients showed grade 3 or 4 toxicity according to the WHO grading. Haematological toxicity was responsible for 86% of the overall toxicity and 100% of grade 3 and 4 toxicity. 7 patients (14%) had an infection, two of which were opportunistic. 12 (24%) patients did not experience any toxicity during the treatment. The results of this study clearly demonstrate the safety and considerable activity of this regimen. Cladribine is very effective even at lower doses than have been used so far.

Randomized open label phase III trial of CEOP/IMVP-Dexa alternating chemotherapy and filgrastim versus CEOP/IMVP-Dexa alternating chemotherapy for aggressive non-Hodgkin's lymphoma (NHL). A multicenter trial by the Austrian Working Group for Medical Tumor Therapy.

Primary end point of this trial was to reduce neutropenic infections during the treatment of aggressive NHL with CEOP/IMVP-Dexa (cyclophosphamide, epirubicin, vincristine, prednisolone ifosfamide, methotrexate, VP-16, and dexamethasone). Further, we studied the influence of filgrastim on dose intensity of CEOP/IMVP-Dexa, on the rate of complete remissions, on the time to relapse, and on survival. Eighty-five patients with untreated large-cell NHL were randomized to one of two treatment arms; 74 patients were eligible. Thirty-eight patients in arm 1 were treated with CEOP/IMVP-Dexa chemotherapy and filgrastim, 36 in arm 2 with CEOP/IMVP-Dexa chemotherapy alone. In arm 1 filgrastim was self-injected by the patients at 5 micrograms/kg body wt. s.c. daily, except on the days when cytotoxic drugs were given. During treatment we did weekly complete blood counts. Median leukocyte counts were 10.91 x 10(9)/l and 5.46 x 10(9)/l in arm 1 and 2, respectively (p = 10(-6)). Median neutrophil counts were 7.7 x 10(9)/l in arm 1 and 2.72 x 10(9)/l in arm 2 (p < 10(-6)). Median neutrophil nadirs were 0.199 x 10(9)/l and 0.213 x 10(9)/l in arm 1 and 2, respectively (p = 0.09). Mean platelet nadirs were 95 and 152 x 10(9)/l (p = 0.000004) and mean hemoglobin nadirs 83.95 g/l and 92.78 g/l (p = 0.00558) in arm 1 and 2, respectively. Dose intensity of CEOP/IMVP-Dexa was 82.3% and 76.2% in arm 1 and 2, respectively (p = 0.041). Forty-two percent and 58% of patients experienced a febrile neutropenia in arm 1 and 2, respectively (not significant, NS). Median time to first neutropenic infection was in treatment week 11 and 6 in arm 1 and 2, respectively (NS). There was no significant difference in rate, duration, and kind of infection, duration of hospitalization, or antibiotic treatment. Seven toxic deaths occurred, all due to neutropenic infection, 6 and 1 in arm 1 and 2, respectively (p = 0.0732). Four of the six patients, who died of infection in arm 1 were older than 60 years. Complete remission rate was 83% and 66.7% in arm 1 and 2, respectively (NS). After a median observation time of 3 years there was no difference in time to relapse or survival. Filgrastim increases leukocyte and neutrophil counts and dose intensity, if used with CEOP/IMVP-Dexa chemotherapy in high-grade lymphomas. There was no significant effect on febrile neutropenia or infections. The more frequent fatal neutropenic infection rate in the filgrastim arm was not statistically significant. It is most appropriate to explain it by the patient's age in combination with the high dose intensity. The small increase in dose intensity had no effect on survival but probably decreased hemoglobin levels and platelet counts in arm 1. We were unable to show a benefit for filgrastim in combination with CEOP/IMVP-Dexa.

[Mutism after posterior fossa tumor surgery. Report of 2 cases]. / Mudez após cirurgia para tumor da fossa posterior. Relato de dois casos.

The authors present two cases of mutism after posterior fossa surgery. Two patients aged 16 and 5 years old respectively with astrocytoma and medulloblastoma, developed mutism after the operation. Topographic aspects of the condition are discussed and its neurological expression.

Cerebral metastasis from choriocarcinoma and oncotic aneurysms. Case report.

Cerebral metastasis occur in 10 to 20% of patients with choriocarcinoma. We describe the twelfth patient with oncotic aneurysms from choriocarcinoma verified by cerebral angiography. The importance to consider this disease in a woman of childbearing age who develop an intracerebral hemorrhage or a lesion with mass effect is emphasized, as well as laboratial and radiological characteristics. Therapeutic approaches with chemotherapic agents, surgery and irradiation are discussed.

Cerebral metastasis from choriocarcinoma and oncotic aneurysms

Cerebral metastasis occur in 10 to 20% of patients with choriocarcinoma. We describe the twelfth patient with oncotic aneurysms from choriocarcinoma verified by cerebral angiography. The importance to consider this disease in a woman of childbearing age who develop an intracerebral hemorrhage or a lesion with mass effect is emphasized, as well as laboratial and radiological characteristics. Therapeutic approaches with chemotherapic agents, surgery and irradiation are discussed

[Plasmapheresis in Landry-Guillain-Barré-Strohl syndrome with or rapidly appearing respiratory insufficiency]. / Plasmaferese na síndrome de Landry-Guillain-Barré-Strohl com insuficiência ventilatória ou rapidamente ascendente.

The authors report three cases of Landry-Guillain-Barré-Strohl syndrome successfully treated by plasma exchange--the only cases in the Brazilian State of Rio Grande do Sul. The authors go through a brief literature revision and discuss more thoroughly the plasma exchange procedure, establishing criteria for its indication.

Relationships between portions of the third and fourth ventricles. An aid to stereotactic accuracy for posterior fossa stereotaxy.

The relationships between the third and fourth ventricles have been studied by examination of the angles formed by third and fourth ventricular projections. These have been subjected to statistical analysis and have proved useful for posterior fossa stereotactic surgery.