[Acute pancreatitis in a patient with alpha 1-antitrypsin deficiency. Is there a causal relationship?]. / Acute pancreatitis bij een patiënt met alfa 1-antitrypsinedeficiëntie. Is er een oorzakelijk verband?

The clinical pattern and the pathophysiological mechanism of the hereditary disorder alpha 1-antitrypsin deficiency are outlined. It appeared that the patient described suffered not only from emphysema, cirrhosis of the liver and hepatocellular carcinoma, but also from acute haemorrhagic pancreatitis. The combination of acute pancreatitis and alpha 1-antitrypsin deficiency has not been described before. Screening of relatives is important because of possible treatment.

[Agranulocytosis caused by clozapine]. / Agranulocytose door clozapine.

This article describes clozapine-induced agranulocytosis in a psychotic patient. The importance of instructions to medical personnel is stressed especially if the patient is unable to mention symptoms. Leucocyte counts should be performed every week during the first 18 weeks of treatment, thereafter every month. In case of suspicion of infection immediate leukocyte count and differential are necessary.

Thrombocytopenia associated with mycoplasma pneumoniae infection.

The case of a patient with immune thrombocytopenia associated with mycoplasma pneumoniae infection is described. The thrombocytopenia was considered to be provoked by the infection and disappeared gradually after antibiotic treatment.

Monoclonal immunoglobulins with affinity for platelets and their relationship to malignant lymphoma.

Monoclonal immunoglobulins with affinity for platelets were detected in the blood of seven patients. Two of these had thrombocytopenia and non-Hodgkin's lymphoma (NHL). One patient had thrombocytopenia and possibly incipient NHL. The other four patients had pseudothrombocytopenia at the time of diagnosis but one of them developed NHL six years later. It is suggested that these monoclonal immunoglobulins may in some cases be associated with malignant lymphoma and that subjects presenting with these immunoglobulins should have a long term follow-up in order to elucidate the question whether or not lymphoma will develop.

Platelet antibodies in idiopathic thrombocytopenic purpura.

An immunofluorescence (IF) technique for the detection of antibodies was applied to idiopathic thrombocytopenic purpura (ITP). Serum platelet antibodies were found in thirteen out of twenty-two patients (59 percent) with active disease, but in only four out of fifteen patients (27 percent) who had attained remission. Direct tests for platelet-associated IgG were positive in 36 and 44 percent of these patients respectively. In two cases IgM was observed on the patients' platelet membranes. C3 was not detedted on patients' platelets. Platelet-associated IgG was also found in several other disorders and its occurrence is not therefore diagnostic of ITP. In addition, serum platelet antibodies do not indicate specifically ITP as they may also be due to previous isoimmunization. Antibodies in the sera of patients with ITP generally did not fix Clq and in most cases bound to platelets only in the presence of EDTA. In contrast, isoantibodies often fixed Clq and they had equal affinity for platelets suspended in ACD or EDTA plasma. This was confirmed by quantitative data on IgG binding by platelets obtained by measuring 125-I-labelled protein A uptake. The simplicity of the IF technique permits its routine application and the technique may give useful information with respect to the nature of the antibodies. It must, however, be considered of limited value in the diagnosis of ITP.

Pseudothrombocytopenia due to agglutinins.

Pseudothrombocytopenia may have any of a number of causes, one of which is agglutination in vitro. This phenomenon was found in samples of blood from six patients. A serum factor responsible for the agglutination was demonstrated. The factor was dependent upon the presence of EDTA and was more active at room temperature than at 37 C. It could be identified as an IgM immunoglobulin in four cases. In the other two cases definite characterization was not possible, but there was some evidence in favor of an IgM factor. All six patients had elevated serum IgM levels, but they had different and unrelated clinical disorders.

HLA antigens in idiopathic thrombocytopenic purpura.

A group of 40 patients with idiopathic thrombocytopenic purpura (ITP) was examined. Typing for HLA A and B antigens was performed. The frequencies of 24 HLA antigens in this group were compared with those of a group of 13 patients with drug-induced thrombocytopenia and two large groups of healthy controls. Differences of statistical significance between these groups were not found. Thus, associations of ITP and particular HLA antigens were not observed. The associations reported by others could not be confirmed.

Bacterial infections and thrombocytopenia in chronic idiopathic thrombocytopenic purpura.

A bacterial infection was considered to be responsible for provoking or containing episodes of thrombocytopenia in 5 patients with chronic idiopathic thrombocytopenic purpura (ITP). In 3 patients the course of the disease was continuous and in 2 of them remission was attained after eradication of the infection. In the other 2 patients the course was intermittent and a number of relapses was provoked by the infection. The possible causal connection of chronic ITP and bacterial infections is briefly discussed.

Myasthenia gravis and Werlhof's disease.

A case of Werlhof's disease (immune thrombocytopenia) associated with myasthenia gravis is described. The two disorders developed within a few months of each other. The immunological and practical aspects of the association are discussed.

The detection of platelet isoantibodies by membrane immunofluorescence.

A membrane ummunofluorescence test for the detection of platelet isoantibodies is described. Gel filtration of the incubation mixture was incorporated in the procedure and proved effective for the removal of serum proteins from the platelet suspension. With this technique isoantibodies were found in the serum of 13 out of a group of 16 patients who had received multiple transfusions. The results were checked by measuring the uptake of 125I-labeled anti-IgG fraction by gel-filtered platelets. Subsequently the membrane immunofluorescence method was also compared with established techniques described for the detection of isoantibodies such as the microtest for lymphocytotoxicity and a complement-fixation method and the procedures based on the release of labeled serotonin, the phagocytosis of chromium-tagged platelets, the increase of platelet factor 3 activity, and on platelet aggregation. We had the opportunity to investigate the serum of one patient for the presence of isoantibodies against platelets from HLA identical siblings both before and after the administration of their platelets. On the basis of this experience it is concluded that the membrane immunofluorescence test for platelet isoantibodies is a relatively simple method with a high degree of specificity and adequate sensitivity.