Biomedical subjects

W Miesbach

Publications and source records attributed to W Miesbach.

5 recordsLinked to original sources

PubMed2006

The catastrophic antiphospholipid (Asherson's) syndrome and malignancies.

The catastrophic antiphospholipid syndrome is characterised by the rapid chronological development of fulminant thrombotic complications that predominantly affect small vessels. It has been reported as frequently occurring in patients with underlying malignancies. We analysed the web site-based international registry of patients with catastrophic APS. The clinical characteristics of patients with CAPS and an underlying malignancy were evaluated. Of the 262 patients included in the CAPS registry, information on associated malignancies was available in 23 (9%) cases. Haematological malignancies were present in 6 (26%) patients. Four of the patients suffered from lung carcinoma (17%), and two patients (9%) from colon carcinoma. In most of the patients (61%), malignancy was the precipitating factor for CAPS. In 4 patients (17%), however, surgical procedures related to the carcinoma were noted as precipitating factors. In one patient CAPS occurred during allogenic stem cell transplantation after diagnosis of acute lymphoblastic leukemia (ALL). Cerebral manifestations were most common and consisted mainly of cerebral infarcts and encephalopathy. Recovery occurred in 9/23 (39%) patients. Malignancy may be an important risk factor for CAPS. 9% of patients with CAPS presented with an underlying malignancy. In most of these patients, the malignancy and/or surgical procedures were the precipitating factors for CAPS.

Adolescent↗

PubMed2006

High titres of IgM-antiphospholipid antibodies are unrelated to pathogenicity in patients with non-Hodgkin's lymphoma.

Heterogeneity in the mechanisms of coagulation may contribute to an increased thrombotic risk for patients with malignancies. The coincidence of malignancies and antiphospholipid antibodies (aPL) have been described in several important epidemiological studies. The pathological significance of aPL in patients with malignancies is, however, still unclear. In this study, we investigated the clinical manifestations of four patients with elevated IgM-aPL titres lying outside the region signifying 95% of normal cases and with a history of non-Hodgkin's lymphoma. The patients had elevated IgG- and IgM-anticardiolipin antibodies (aCL) and also tested positive for lupus anticoagulants. Other aPL were measured, and we found high positive results for all tested antibodies in three patients. The production of aPL, however, occurred in the absence of thrombotic complications. No thromboembolic manifestations occurred during the follow-up period either. It could also be demonstrated that the degree to which the aCL titre was elevated resembles the elevation of the non-classical antiphospholipid antibodies, but not that of beta2-GP-1 or anti-annexin antibodies. Therefore, it can be postulated that these extremely high levels of IgM-aCL antibodies do not enhance the risk of thrombosis and may be completely different from aCL antibodies in an antiphospholipid syndrome patient population without malignancies. In particular, haematological and lymphoproliferative malignancies may indeed be associated with the generation of aPL, but do not necessarily enhance the thrombophilic risk in these patients.

Aged↗

PubMed2004

[The antiphospholipid syndrome].

Antiphospholipid antibody syndrome (APS) is characterised by the presence of frequently recurring venous and/or arterial thrombosis, abortions and repeated evidence of lupus anticoagulants and/or anticardiolipin (aCL)-antibodies. The presence of lupus anticoagulants and/or high levels of aCL-antibodies in particular increases the risk of thrombosis. At present there are several models for the systemic and cell-damaging pathogenesis of phospholipid-antibodies. Where there are repeated occurrences of thrombo-embolic complications long-term anticoagulant treatment is indicated. For patients who have APS in pregnancy, the use of low-molecular heparin and acetylsalicylic acid (ASS) is recommended. According to recent research, a large number of antibodies can be found in APS patients, which are directed against phospholipids and plasmaproteins, but partly also against individual genetic polymorphism, which suggests focussing in future on the genetic basis of APS as well as on influencing "auto-immune thrombosis" by modulating the immune system.

Antibodies, Anticardiolipin↗

PubMed2004

Recurrent life-threatening thromboembolism and catastrophic antiphospholipid syndrome in a patient despite sufficient oral anticoagulation.

We report on a 32-year old female patient with primary antiphospholipid syndrome (PAPS) and several thromboembolic events despite stable doses of oral anticoagulation, good patient compliance and maintained INR values of >3. Over the preceding 3 years the patient had presented a wide spectrum of manifestations of APS, including recurrent venous and arterial thromboses, cardiac, gynecological (HELLP syndrome), neurological involvements, livedo reticularis, a mild thrombocytopenia and the most feared manifestation of the catastrophic antiphospholipid syndrome (CAPS). Life-threatening bilateral subdural bleeding occurred while she was anticoagulated. The clinical features appeared to be refractory to oral anticoagulation with phenprocoumon. They were life threatening on each occasion and she developed repetitive episodes of organ damage with cardiac insufficiency (NYHA III), pulmonary hypertension and other residual defects. Even during heparinization recurrent thromboembolism supervened as well as livedo reticularis of the extremities. Lupus anticoagulants (LAC), anticardiolipin (aCL) antibodies and anti-beta(2)-glycoprotein-1 (beta(2)GPI) titers were all markedly elevated. This case report shows that recurrent episodes of thrombosis can occur despite seemingly adequate anticoagulation in patients with CAPS.

Administration, Oral↗

PubMed2002

[Cardiac and cerebral manifestations of antiphospholipid syndrome].

UNLABELLED: We describe cardiac and cerebral manifestations in 27 patients with the antiphospholipid syndrome and give a review of the literature. PATIENTS AND METHODS: We studied 27 patients with either cardiac, cerebral or both manifestations, who also were diagnosed with the antiphospholipid syndrome (APS). All patients fulfilled the proposed classification criteria for the APS according to the Scientific Standardisation Committee of the International Society of Thrombosis and Haemostasis (1). Three patients died. RESULTS: Eighteen of the 27 patients had coronary artery disease with either thrombotic coronary occlusions, high grade stenosis or complete vessel occlusion. In three patients the left main artery was involved. Ten patients had involvement of the left anterior descending artery (LAD), 6 patients of the circumflex artery (RCX) and 5 patients had involvement of the right coronary artery (RCA). As valvular lesions have been described in association with the APS it should be noted that in 16 cases there was mitral- and/or aortic valve disease and in 3 cases tricuspid valve disease. Four patients underwent mitral- and/or aortic- and/or tricuspid valve replacement. 7 patients presented with a history of cerebrovascular involvement; 5 of these patients had cerebral infarction, one patient recurrent cerebral bleeding under oral anticoagulation and another patient presented with cognitive disorders. Three of the 7 patients had a prior history of myocardial infarction, whereas 3 patients underwent mitral or aortic valve replacement. CONCLUSION: The frequent occurrence of coronary disease, cardiac valvular disease and cerebral disease in patients diagnosed with APS may suggest a causative relationship between the presence of PL antibodies and vascular disease.

Adult↗