Hairy cell leukemia still an enigmatic disease.
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Biomedical subjects
Publications and source records attributed to J Jansen.
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Hairy cell leukemia is a chronic lymphoproliferative disorder that has been recognized as a separate clinical pathologic entity for the last 25 years. After a decade of discussions about the origin of the neoplastic cell, it has now been well established that hairy cells represent a certain, rather mature stage of B-cell differentiation. Evidence for this has been derived from studies using immunophenotyping with monoclonal and polyclonal antibodies, cytochemistry, and immunoglobulin gene rearrangement. For many years, splenectomy was the only therapy of proven value in hairy cell leukemia. For patients who showed insufficient response to the operation, chemotherapy with low-dose alkylating agents was moderately successful, whereas polychemotherapy often resulted in excessive toxicity. More recently, therapy with alpha-interferon has been shown to be very promising, whereas deoxycoformicin may be an attractive alternative. These new advances in immunology and therapy are reviewed.
Morphological studies were performed after intracerebral injection of Ethibloc (E.), an alcoholic prolamine solution, to prove the usefulness of E. as a drug depot in the local chemotherapy of brain gliomas. Organisation of E. starts 2-3 weeks after injection, but remains incomplete even 24 weeks later. This depot cannot be removed in case of diminished drug concentration because of the extremely close connection with the adjacent brain tissue. Further repeated injection of drug into the E. depot cannot reach the tumours periphery because of the surrounding glial scar and connective tissue.
Causes for operative revisions of CSF shunting systems are displacement of the distal catheter from the heart or the peritoneal cavity due to the patient's growth. This takes place in 40-50% of all revisions. Median time for the first revision is 2 years post implantation. Preliminary experience with a simple peritoneal "reserve" catheter lead us to expect that the revisions may eventually become redundant: the catheter is normally inserted 10 cm into the abdominal cavity and then fixed to the peritoneum. About 20 cm of a catheter tubing are coiled in a spiral line inside a silicone bag placed subcutaneously in the abdominal wall. As the child grows, the catheter uncoils, and this uncoiling may be observed radiologically.
The trichostrongylid and strongylid egg output in a group of ewes lambing in March, the middle of the usual lambing time in the Netherlands, was compared with counts in groups of older ewes and yearling ewes lambing in May-June and with counts in a group of barren ewes. The groups lambing in March showed only a rise related to parturition date as normally seen in the Netherlands. A few of the late lambing ewes showed a slight rise in spring, but all of them showed a peri-parturient rise related to parturition date. In the late lambing ewes and in the barren ewes egg counts due to reinfection were seen in summer. It is concluded that under the present circumstances a true spring rise, the calendar phenomenon, occurs very irregularly, while a peri-parturient rise related to the parturition date is a common phenomenon. The occurence of high egg counts in summer, excluding consideration on the barren ewes, in the late lambing ewes only, suggests that changing the traditional husbandry system may influence the epidemiological pattern of the trichostrongylid infections.
It is important to make the correct diagnosis of MDS and to exclude very carefully all other disorders that may induce dysplastic features in the bone marrow. In patients without excess of bone marrow blasts, cytogenetics and in vitro bone marrow cultures may aid in making the correct diagnosis. MDS patients without excess of bone marrow blasts or symptomatic cytopenia or cytogenetic abnormalities associated with poor prognosis should be followed on a regular basis with sequential examinations of blood counts and bone marrow specimens. In the absence of obvious disease progression, ie, increasing cytopenia or increasing percentage of marrow blasts, patients should only receive supportive care. An increase in RBC requirements alone is insufficient reason to start cytotoxic therapy. Once progression of the disease has been well documented, cytotoxic treatment is indicated. There is no reason to delay treatment until these patients have progressed to overt AML. In patients over the age of 50, the best available therapy is low-dose cytarabine with a 30% probability of a good response; this therapy requires careful supervision and the availability of intensive supportive care. In patients under 50 years with progressive disease, or with clear evidence of a poor prognosis, allogeneic BMT is the therapy of choice if a HLA-identical sibling can be identified. In those patients who lack a HLA-identical sibling, intensive combination therapy is the treatment of choice and should preferably include high-dose cytarabine. Intensive consolidation therapy will be necessary for a durable remission. Trials with inducers of differentiation remain experimental. Results to date have been disappointing.
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Anaesthesia was applied to 1,036 patients for diagnostic or therapeutic knee-joint arthroscopy, between 1983 and 1985. Different indications for general and regional anaesthesia, setbacks of spinal anaesthesia, and advantages likely to result from combined use of peridural anaesthesia and peridural analgesia are discussed in some detail.
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A survey was carried out in The Netherlands to determine the prevalence of benzimidazole resistance in sheep nematodes. It was shown that 47 per cent of the 59 investigated flocks harboured trichostrongylid populations with resistance to benzimidazoles, as demonstrated by an LD50 value of 0.12 micrograms ml-1 thiabendazole or higher in an in vitro egg hatch assay. On these farms Haemonchus contortus was found to be the dominant species. In two flocks resistant Ostertagia and, or, Trichostrongylus species populations were found. Farms with resistance were revisited to determine the anthelmintic efficacy in vivo by means of treatment and egg count. In only four of the 19 flocks which showed resistance in vitro was an efficacy of less than 95 per cent in vivo achieved. This means that there is a discrepancy between the in vivo and in vitro results. Resistance was found more frequently on pure sheep farms than on mixed farms. It is suggested that mixed farms offer more refuge for susceptible H contortus populations.
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The advantages, prevention of gastro-intestinal worm infections in lambs, the improvement in the condition of the ewes during lactation, and the disadvantages, the costs, a possible interference with the build-up of immunity and the risks of the occurrence of anthelmintic resistance are discussed. It is concluded that the advantages more than counterbalance the disadvantages. Providing that the dosage of anthelmintics is correct, that is to say that an overdosage is preferable to too low a dosage, and safe pastures are used, post-lambing anthelmintic treatment of ewes is an effective procedure in preventing gastro-intestinal infections in lambs. Moreover, treatment will eventually improve the condition of the ewes so that, for instance, as the result of a slightly higher milk yield, the gain in weight of the lambs will be promoted.
A patient with chronic B-cell leukemia in whom the malignant lymphocytes showed intracellular inclusions of immunoglobulin (Ig) G kappa molecules is described. Electron microscopy revealed filamentous material in the nuclear envelopes and in the cisternae of the rough endoplasmic reticulum. These in vivo surface Ig-negative, nonexcreting cells could be stimulated in vitro to excrete immunoglobulin-free light chain molecules into the supernatant, which were not found in the cytoplasm after stimulation.
A 33-year-old female dialysis patient suffered from osteomyelitis and luxation of the dens axis with cervical myelopathy. In the past she had had three attacks of anaphylaxis after treatment with dialyzers that had been sterilized with ethylene oxide. IgE-type antibodies directed against human serum albumin-ethylene oxide complexes could be demonstrated in the patient's serum by radioallergosorbent techniques. Immediately after an operation in which acrylic bone cement (Palacos-R) sterilized with ethylene oxide was implanted for stabilization of the cervical spine, the patient developed massive edema of the larynx, pharynx, and tongue, suggesting Quincke's edema. It is concluded that ethylene oxide present in acrylic bone cement may induce acute allergic reactions in sensitized patients. Dialysis patients may be at special risk, since the incidence of ethylene oxide allergy in this patient population is about 10%.
The small strongylid infections of two groups of three yearling female Shetland ponies and one yearling Shetland tracer pony were studied. One group was set stocked from April to November and was treated monthly with 5 mg kg-1 albendazole from two days before turnout until July. The other group grazed similar pasture until July, was treated with 5 mg kg-1 albendazole and subsequently removed to pasture grazed by sheep from April to July. The tracer ponies were added to both groups in September. The efficacy of both methods was not completely satisfactory probably because of low efficacy of anthelmintic treatment. There were no significant differences between the cyathostome burdens of the two groups. A high proportion of the cyathostome populations of all ponies consisted of inhibited early third stage larvae (L3). The finding of low numbers of immature fifth-stage worms in the tracer ponies indicated that the considerable adult burdens in the permanent ponies originated from infection picked up before the tracer ponies were added. In the group which was removed to sheep pasture after treatment in July it was likely that the majority of the adult worm burden had been ingested as infective larvae before treatment.
Alternate grazing of horses and sheep as a control measure for gastrointestinal helminthiasis was studied in three grazing experiments in 1981, 1982 and 1983. Each year a group of three mare yearling Shetland ponies, which were kept on a small pasture from spring to autumn, were compared with a similar group which grazed a similar or the same pasture until July and were subsequently removed to a similar pasture which had been grazed by sheep from April to July. In addition both groups were treated with an anthelmintic when the latter group was removed to the sheep pasture. Pasture larval counts and worm counts and, in 1982 and 1983, faecal egg counts, clinical condition, total protein, albumin and beta-globulin levels demonstrated that the groups removed to sheep pasture acquired considerably lower burdens of nematodes of the subfamilies Cyathostominae and Strongylinae, but considerably higher burdens of Trichostrongylus axei than the groups which were not moved. These T. axei infections resulted in higher serum pepsinogen levels in the former groups compared to the latter in 1981 and 1982. At necropsy an important part of the T. axei burdens and, in 1982 and 1983, the Cyathostominae burdens consisted of inhibited early third stage larvae. A total of 20 species of the subfamily Cyathostominae and 7 species of the Strongylinae were found. Generally the composition of species was in agreement with other observations in western Europe, the most common species being: Cylicostephanus longibursatus, Cylicostephanus minutus, Cylicostephanus calicatus, Cylicostephanus goldi, Cylicostephanus poculatus, Cyathostomum labratum, Cyathostomum coronatum, Cyathostomum catinatum, Cylicocyclus leptostomus, Cylicocyclus nassatus, Cylicocyclus insigne, Strongylus edentatus and Strongylus vulgaris.