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J Jansen

Publications and source records attributed to J Jansen.

At least 307 records · Page 17Linked to original sources

Clinical differential diagnosis of hairy-cell leukaemia.

The data on hairy-cell leukaemia (HCL) and resembling disorders in the literature and in our patients were analyzed to determine which clinical features and laboratory data are important for the recognition of HCL in an early stage. In pancytopenic patients the typical pattern of bone marrow involvement in HCL and the low number of monocytes in the peripheral blood appear to be essential for the differential diagnosis. In patients with many neoplastic cells in the peripheral blood, the presence of neutropenia and monocytopenia as well as tartrate-resistant acid phosphatase activity in the neoplastic cells, appears to be crucial for early diagnosis. Thus, the clinical features and routine laboratory data alone are sufficient in the majority of cases to suggest the diagnosis HCL. The monocytopenia proved to be most helpful in this respect. Nevertheless, in all patients, and certainly in patients presenting with atypical features, a bone marrow biopsy is indispensable for the correct diagnosis.

Adolescent↗

T lymphocyte function in hairy cell leukaemia.

The high incidence of infections characteristic of impaired cell-mediated immunity in patients with hairy cell leukemia led us to study T lymphocyte function in sixteen patients with the lymphocyte transformation test. All patients showed imparied responses to mitogens, attributable to one or more of the following causes: dilution of responsive T cells by inert hairy cells, shortage of monocytes to give adequate interaction with the T cells and a significant decrease in the number of T cells with Fcmu receptors proportional to the percentage of hairy cells in the peripheral blood. The response to antigens was severely depressed; PPD was one of the few antigens that induced positive reactions in half the cases. We conclude that in patients with hairy cell leukaemia, T lymphocyte function, as tested in a proliferative assay, is severely impaired and that this may contribute to the deficient resistance to infection.

Adult↗

Hairy-cell leukaemia: a B-lymphocytic disorder.

Fifteen cases of histologically proven hairy-cell leukaemia (HCL) were studied with immunofluorescence, rosette, and phagocytosis techniques. Unfixed hairy cells (HC) bound all kinds of labelled antiserum; but after fixation with formaldehyde a much more selective binding was observed. In two cases no surface-bound Ig was detected; four cases showed gamma and in nine cases two or three heavy chains were found, alpha and delta being the most frequent. Few cases were clearly positive for mu. The picture was invariably monoclonal with respect to light chains. Cytoplasmic Ig was present in only 3/15 cases; it was always IgM. HC did not form E-rosettes or react with a fluorescent anti-T cell antiserum. No EAIgMC-rosettes were formed. All cases showed Fc receptors, which were detected with EAIgG-rosettes (13/13) or with antigen-antibody complexes (6/6). The density of Fc receptors varied widely. Incubation with latex particles resulted in cell-associated particles in 16-63% of the HC; with Staphylococcus epidermidis, the percentage was 2-36. After enzyme treatment (lysostaphin), however, no ingested bacteria were found, which suggests that HC are essentially non-phagocytic. At least 13 cases were therefore classified as B-cell malignancies.

B-Lymphocytes↗

Phagocytic potential of hairy cells.

The hairy-cells (HC) of 10 patients with hairy-cell leukaemia were studied with several techniques to evaluate their phagocytic potential. Mononuclear cells from normal donors and from patients with acute monocytic leukaemia served as controls. Light microscopically HC seemed to have ingested bacteria or latex particles. Treatment of the cells with lysostaphin, an enzyme that kills extracellular Staphylococcus aureus, showed that almost all 'ingested' bacteria were extracellular. Lanthanum nitrate, added during the fixation procedure for electron microscopy, stained both the outer cell membrane and the membranes of the 'phagosomes' of the HC, also indicating that the 'ingested' particles were extracellular. HC showed no increased oxygen consumption on exposure to bacteria in the presence of serum. Furthermore, HC showed no lysozyme or peroxidase activity, whereas non-specific esterase activity was much weaker than in monocytes. These findings, which show that HC are essentially non-phagocytic, constitute strong evidence against a monocytic origin of the malignant cells of hairy-cell leukaemia.

Colony-Forming Units Assay↗

Primary plasmacytoma of lymph nodes. A case report.

Most plasmacytomas of lymph nodes are secondary metastases from myeloma or from primary plasmacytoma of the upper air passages. Primary plasmacytomas of lymph nodes are very rare. A case of primary plasmacytoma of mediastinal, cervical and para-aortic lymph nodes is reported. Bence Jones proteinuria of 8 g/24 h was present. Repeated bone marrow examinations were normal. When the patient died, 17 months after the first symptoms, the bone marrow was still not involved.

Adult↗

Distinct subtype within the spectrum of hairy cell leukemia.

Most cases of hairy cell leukemia represent malignancies of B cells. However, recent findings suggest that there is a spectrum of functional capacities within the entity hairy cell leukemia. Two patients with hairy cell leukemia, whose malignant cells in the peripheral blood showed both T- and B-cell features, are reported. The malignant cells of the spleens showed only B-cell characteristics. The hairy cells of both patients did not adhere to glass and lacked the la antigen. Both patients showed pronounced polyclonal hypergammaglobulinemia and developed frank leukemic blood pictures after splenectomy. Within the spectrum of hairy cell leukemia, these two cases probably represent a distinct subtype.

Adult↗

Spina bifida: epidemiological data from a pilot study.

The epidemiology of spina bifida has great geographical variation. A small unselected group of patients were registered as part of a pilot project on infantile hydrocephalus. Twenty-two patients, born 1963-69 in Vejle county, Denmark were identified, giving an incidence of 0.77 per 1000 births. The mothers had 26 pregnancies prior to the spina bifida children; 14 of these had resulted in normal viable infants (54%). Seven of the patients had other congenital malformations, unrelated to the neural tube defect. All unoperated patients died. Four of 10 operated children were alive at age 7-12 years, three of them without handicap.

Abnormalities, Multiple↗

Clonidine in the prophylaxis of migraine.

The prophylactic effect of clonidine in a dosage of 0.05 mg twice daily was investigated in 49 patients using a double-blind, crossover trial carried out in four Departments of Neurology. Seventy-one patients were originally included but 22 patients withdrew, two of them due to side effects,the remainder because of inability to keep the requisite diary, lack of drug compliance or refusal to attend the checkups. Approximately equal numbers withdrew during the clonidine and placebo periods. There was no statistically significant difference between the number of migraine attacks or between the number of severe attacks (8 hours' duration or more) during the placebo and clonidine periods. This also applied to the patients with foodstuff-provoked migraine attacks. Sixty-three patients carried through a double-blind, crossover trial with capsules containing either 125 mg tyramine or placebo. There was no significant difference between the number of patients who developed attacks after the ingestion of placebo and the number who did so after the ingestion of tyramine. The same is true of the group with foodstuff-provoked migraine. As a rule side effects were few and mild. This study has not confirmed that clonidine has any pharmacological effect in prophylaxis of migraine.

Adult↗

An epidemiologic survey of periodontal disease in Dutch adults.

An epidemiologic survey was carried out to assess the prevalence and severity of periodontal disease in randomly selected samples of employed populations in the Netherlands. Of the 1,337 persons examined by five examiners, 19.8% were edentulous; 61% of dentate persons had intense gingivitis in an average of 2.4 segments of their mouths; 53% had pockets of 3-6 mm and a further 10.1% had pockets of greater than 6 mm. The prevalence of gingivitis and pocketing increased with increasing age and decreasing levels of education. As no measures of predicting compliance were used and because treatment philosophies differ, no reasonable assessment of treatment needs could be made.

Adult↗

Hairy cell leukaemia. Clinical features and effect of splenectomy.

Hairy-cell leukaemia (leukaemic reticuloendotheliosis) is a well-defined clinical entity. Most of the recent reports are almost entirely concerned with the pathological and functional aspects of the disease. In the present retrospective study the clinical features and laboratory data of 12 patients were analyzed together with a series of 123 adequately clinically documented cases from the literature. The Hb level and the sex of the patient proved to be the only parameters having some prognostic value for the survival time after diagnosis. The effect of splenectomy was assessed in two comparable groups of 24 splenectomized and 51 non-splenectomized patients. The operation seemed to be beneficial, but after 2 years the difference was not significant(.05 less than P less than .10). Analysis of subgroups showed that splenectomy was definitely beneficial in women, in patients with a Hb level over 8.0 g/dl or a platelet level above 50 X 10(9)/1, in patients with leucocytes below 3 X 10(9/1, , and also in patients with hepatomegaly (P less than .05 in all cases). These findings suggest that splenectomy is beneficial in cases where anaemia and thrombocytopenia are not very severe; in severe cytopenia the operation does not increase the life expectancy.

Adult↗