Use of tunnelled catheters in haematological malignancy patients with neutropenia.

This prospective study analysed 83 patients (age 45 +/- 17 years) with haematological neoplasms, implanted with 93 tunnelled catheters, who were neutropenic or developed neutropenia during treatment. Catheters were implanted in the right (n = 82) or left (n = 11) jugular vein by the same surgical team using the same technique. They remained in place for 124 +/- 88 days: 29% were removed due to infection; 18% due to treatment termination and 2% due to mechanical problems. Seventeen patients died with catheters in place. At 30, 60, 90, 120 and 200 days mean catheter duration rates were 82%, 75%, 65%, 60% and 35%, respectively, and freedom from catheter removal due to infection was 92%, 88%, 80%, 77% and 67%, respectively. Patient diagnosis and history of previous catheter infection did not increase catheter infection risk, but patients undergoing stem cell transplantation had an increased infection risk. Tunnelled catheters can be used in high-risk patients with neutropenia. Systemic infections can be managed in most patients without catheter removal.

Bilateral breast involvement in Sézary syndrome.

Cutaneous T-cell lymphoma is a term used for mycosis fungoides and Sézary syndrome, the distinct clinical entities where the skin is the primary organ of involvement. Sézary syndrome is the leukemic variant of mycosis fungoides, presenting with generalized erythroderma, lymphadenopathy, and atypical cells (the Sézary cells) in the peripheral blood and bone marrow. The dissemination of cutaneous T-cell lymphoma may occur with no exception of the organs; however, no prior report exists about the Sézary syndrome secondarily involving the breasts. We report the clinical and radiological findings of bilateral breast involvement in a case of Sézary syndrome.

Multicentric reticulohistiocytosis.

We describe a 42-year-old man with a five-year history of arthritis mutilans-like destructive joint changes and with a one-year history of nodules on the fingers, ears, oral mucosa, pharynx, larynx, vocal cords, some being ulcerated and haemorrhagic. He was diagnosed as having rheumatoid arthritis; however, biopsies from the nodules on the oral mucosa and ear revealed multicentric reticulohistiocytosis. The large nodule over the olecranon process, simulating a rheumatoid nodule but diagnosed as multicentric reticulohistiocytosis with biopsy; ulcerated and haemorrhagic nodules on the oral mucosa; and rapidly progressive joint destructions make our case interesting.

A new neurocutaneous syndrome: nevus sebaceus syndrome.

We describe a case of nevus sebaceus syndrome characterized by a linear sebaceous nevus, extending on the left half of the face from forehead to lower neck, including the auricle; alopecia on the temporoparietal region; generalized hypotonia; insufficiency of eyes in following moving objects and insufficiency in controlling the head; lipodermoid cysts in the left and right eyes; bilateral conjunctival masses; corneal vascularization in the right eye; coloboma of the left eyelid and right optic disc. This is a rare case of a sebaceous nevus occurring concomitantly with optical and central nervous system abnormalities.

[Lupus anticoagulant and antiphospholipid antibody values in a family with systemic lupus erythematosus]. / Lupus-Antikoagulans- (LA-) und antiphospholipide Antikörper- (APA-) Werte bei einer Familie mit systemischem Lupus erythematodes (SLE).

The Lupus Anticoagulant (LA) is an immunoglobulin directed towards the phospholipid portion of the prothrombin activator complex. This immunoglobulin because first identified in the plasma of patients with Systemic Lupus Erythematodes, was named as Lupus Anticoagulant. Although initially described in patients with SLE, it was subsequently observed in other diseases and also in patients without any manifest disease. SLE or similar diseases are present in 35% of LA (+) patients. The LA prevalence in SLE patients has been found as 34% whereas ACA prevalence was found as 44%. While searching for the presence of LA and the levels of APA in cases having unexplained recurrent fetal losses, a family (a mother and 3 daughters) was discovered, whose each member has been diagnosed as SLE at different times and with different symptoms. Data suggesting the presence of LA and high APA levels were determined in all the members of the family and also it was realised that cause who had recurrent fetal losses had the highest APA levels. In addition to immunoassay methods to detect APA, examination of coagulation tests in patients with unexplained thrombosis and/or fetal losses, would be of great help.