Exercise-induced fragmentation hemolysis: a simple in vivo test to evaluate heart valve hemolysis.

Differentiating heart valve-related fragmentation hemolysis from other causes of hemolysis can occasionally be difficult, especially when findings on transthoracic or transesophageal echocardiography are minimal. We report a case in which the cause of hemolysis remained in doubt after thorough hematologic and cardiologic evaluations. The decision to reoperate on the valve was finally made, based on the result of exercise-induced increase in serum hemoglobin. Hemolytic anemia promptly resolved after reoperation. We believe this to be the first reported use of this in vivo test to support the diagnosis of valve-related hemolytic anemia.

Streptococcus sanguis bacteremia and colorectal cancer: a case report.

Streptococcus sanguis, usually considered a nonpathogen of the oral cavity, was isolated from blood cultures from a patient who was subsequently found to have a cecal adenocarcinoma. Further studies are needed to determine if Streptococcus sanguis infections have diagnostic implications similar to those of Streptococcus bovis.

Expression of shared idiotypes by paraproteins from patients with monoclonal gammopathy of undetermined significance.

We have previously demonstrated the expression of shared idiotypes by the paraproteins from approximately one-quarter of patients with multiple myeloma (MM). We have now investigated whether similar cross-reactivity is expressed in the paraproteins of patients with monoclonal gammopathy of undetermined significance (MGUS), using a panel of 32 monoclonal antibodies (MAB) generated against follicular B cell lymphomas. The paraproteins from 76/409 (19%) patients with MGUS reacted with at least one of 23 different anti-idiotypic antibodies used in this study. 18 MABs demonstrated reactivity with more than one patient's paraprotein. Moreover, 10 MABs reacted frequently (with 5-22 paraproteins). Over half (41/76) of the reactive patients' paraproteins reacted with more than one MAB from this panel. This frequency of anti-idiotypic reactivity was similar to that of previously studied patients with myeloma, chronic lymphocytic leukaemia (CLL), and follicular B-cell lymphomas. There was no correlation between specific anti-idiotypic reactivity and the propensity to develop serious disease (MM, macroglobulinaemia, amyloidosis, or other lymphoproliferative disorders) in patients with MGUS. These results suggest that MGUS is derived from cells producing antibodies that are similar to those of other B-cell malignancies and that the pattern of idiotype expression is irrelevant to malignant potential.

Clinical syndromes associated with lupus anticoagulants.

Recent evidence suggests that lupus anticoagulants are immunologically distinct from the anticardiolipin antibodies. Nevertheless, the associated clinical complications exhibited by the two groups of antibodies are similar. They have been shown to have a strong association with a history of arterial and venous thrombosis, thrombocytopenia and neurological disease in patients with SLE or lupus-like disorders. The association between antiphospholipid antibodies and recurrent fetal loss is suggested by the currently available data but is not firmly established. Patients with lupus and antiphospholipid antibodies and an established history of recurrent fetal wastage are at high risk for experiencing subsequent fetal loss, but it is not yet known whether the same is true for patients without a history of fetal loss. The association of thrombosis, neurological disease, thrombocytopenia, and fetal loss in patients with non-SLE disorders has not been as extensively studied. Only recently have investigators such as Ginsberg and colleagues begun to show in prospective studies that there may, in fact, be a statistically significant risk of thrombotic events in otherwise healthy individuals with antiphospholipid antibodies. Many of the diverse minor manifestations reported in individual patients, case series, or cross-sectional studies such as livedo reticularis, leg ulcers, and hemolytic anemia may, alternatively, be due to coincidence or chance. Efforts to elucidate the mechanisms of thrombosis in patients with antiphospholipid antibodies is an area of active research. Most efforts have been based on the effects of these antibodies on endothelial cell and platelet function as well as on the fibrinolytic system. In addition, it has recently been shown that binding of antiphospholipid antibodies to phospholipids requires the serum "co-factor" beta 2-glycoprotein I. In patients with SLE selected for the presence of the lupus anticoagulant, thrombosis, or fetal loss, Viard and associates found that 17 of 47 (36%) patients had anti-beta 2-glycoprotein I antibodies. They were able to show, in their small retrospective study, that there was an association between the presence of these antibodies and anticardiolipin activity, lupus anticoagulant activity, and thrombotic events, but not with spontaneous abortion. Of patients with SLE and thrombosis (9 of 47) eight of nine were positive for anti-beta 2-glycoprotein I antibodies, seven of nine were positive for anticardiolipin antibodies, and eight of nine were positive for the lupus anticoagulant. The known inhibitory effect of beta 2-glycoprotein I on platelet aggregation, on platelet prothrombinase activity, and on the intrinsic pathway of coagulation supports the hypothesis that implicates beta 2-glycoprotein I in the pathogenesis of unwanted thrombotic events.(ABSTRACT TRUNCATED AT 400 WORDS)

Synovial sarcoma. A study of intensive chemotherapy in 14 patients with localized disease.

BACKGROUND: The effectiveness of adjuvant chemotherapy in soft tissue sarcomas remains a matter of controversy. The authors reviewed their experience with 14 patients with localized disease treated with intensive doxorubicin-cisplatin-ifosfamide-based chemotherapy. METHODS: Fourteen patients with newly diagnosed nonmetastatic synovial sarcoma seen between 1985 and 1992 received intensive chemotherapy and local radiation therapy before surgical resection, followed in most patients by intensive postoperative chemotherapy. Chemotherapy included high-dose cisplatin and doxorubicin or high-dose ifosfamide (14 g/m2) and cisplatin with doxorubicin. RESULTS: One (7%) patient had a local recurrence during the study interval and remains free of disease 35 months after re-excision and a second course of intensive chemotherapy. All other 13 (93%) patients remained continuously free of disease after a median follow-up of 37 months (range, 6-85 months). There were no deaths. General toxicity was the reason cited by seven patients who elected not to receive postoperative chemotherapy. For the remaining seven patients who elected to continue treatment, there were only two hospitalization admissions for neutropenia and fever. There was no significant cardiotoxicity, nephrotoxicity, or neurotoxicity. CONCLUSION: Additional studies using new intensive systemic adjuvant therapies are needed to determine whether the encouraging results of this experience can be translated into prolonged disease-free and overall survival.

[Therapy of the ocular form of Behçet disease with cyclosporin A]. / Therapie der okulären Form des Morbus Behçet mit Cyclosporin A.

Three patients with Behçet's disease and therapy-resistant posterior uveitis have been treated with cyclosporin A (CS-A) for four years. The uveitis has been controlled better, as indicated by the reduced frequency of inflammation, and there was an initial improvement in visual acuity. However, it has not been possible to prevent a slight, gradual loss of visual acuity over the years. Relapses were observed after dose reductions to blood levels below 400-500 ng/ml and following discontinuation of CS-A therapy. Few relapses occurred at higher CS-A concentrations. It was found that both types of relapse could be controlled by increasing the CS-A dose. An at least transitory increase in creatinine levels as a side effect of CS-A was observed in all patients. The authors' results and reports in the literature suggest that CS-A is an effective immunosuppressive therapy in Behçet's disease and is superior to other immunosuppressives used so far.