Thrombocytopenic thrombotic purpura: severe clinic with no CT, minor MRI, but a SPECT correlate.

A 28-year-old woman with primarily therapy refractory TTP was followed neuroradiologically over 6 months. Despite pronounced neurological and neuropsychiatric symptoms including hemiparesis and aphasia she had unremarkable CT scans on two occasions. Three MRI exams showed no correlate for her neurological symptoms except a small petechial cortical hemorrhage in the right parietooccipital gyrus which may account for her TTP-related anxiety disorder. A cerebral HMPAO-SPECT showed long-standing right-sided hypoperfusion compatible with residual vasculature changes. The possible causes for the clinico-neuroradiological discrepancies are discussed in view of the literature.

[Cryofibrinogenemia caused by monoclonal anti-fibrinogen antibodies]. / Kryofibrinogenämie durch monoklonale Anti-Fibrinogen-Antikörper.

HISTORY AND CLINICAL FINDINGS: For nine years a 54-year-old woman had been suffering from worsening treatment-resistant cold-dependent purpura of the limbs as well as cutaneous ulcerations and arthralgia, which recently had occurred even at a even slight decrease in room temperature. INVESTIGATIONS: A special form of cryofibrinogenemia was identified by affinity-chromatographic separation of a plasma cryoprecipitate. From this cryoprecipitate a monoclonal antifibrinogen antibody (IgG-kappa) was isolated which, in the cold, formed a precipitating complex with fibrinogen. Paraproteinaemia was not demonstrated by conventional serum and plasma electrophoresis. There was no evidence of neoplasma. TREATMENT AND COURSE: Attempted treatment with steroids, fibrinolytic agents and intravenous cyclophosphamide was unsuccessful. But long-term repeated plasmaphereses and anti-immunoglobulin adsorption improved the symptoms. After 5 years of this treatment-14 years after onset of symptoms-the patient died of the consequences of fulminant pulmonary embolism. CONCLUSION: To establish the diagnosis of monoclonal cryofibrinogenemia it is necessary, first, to identify the cryoprecipitate in plasma; secondly, to undertake affinity-chromatographic separation of the cryoprecipitate with subsequent analysis of its components.

Monoclonal cryo-antifibrinogenemia.

We report on a 54-year-old female patient with arthritis and a severe cold-induced leukocytoclastic vasculitis of the skin caused by a rare form of cryofibrinogenemia ("type II" cryofibrinogen). Affinity chromatography of cryoprecipitates from the patient's plasma revealed reversible cryoprecipitability of complexes composed of fibrinogen and a monoclonal antifibrinogen antibody (IgG3 kappa). Conventional serum and plasma electrophoresis did not detect the paraprotein. Control of symptoms was achieved by long-term plasmapheresis.

Treatment-free remission in severe systemic lupus erythematosus following synchronization of plasmapheresis with subsequent pulse cyclophosphamide.

OBJECTIVE: To investigate the effect of an intensified treatment protocol synchronizing plasmapheresis with subsequent pulse cyclophosphamide for severe systemic lupus erythematosus (SLE). METHODS: A protocol of plasmapheresis (3 x 60 ml/kg) and subsequent high-dose pulse cyclophosphamide (36 mg/kg) followed by 6 months of peroral immunosuppression was used to treat 14 patients with severe SLE. RESULTS: Rapid improvement was achieved in all patients. Immunosuppressants, including corticosteroids, were withdrawn at month 6 in 12 patients. Eight patients continued without treatment for a mean observation period of 5.6 years (46-91 months). CONCLUSION: The results demonstrate that treatment-free clinical remission can be achieved in some patients with severe SLE.

[Endogenous tracheobronchial foreign body. An indication for emergency bronchoscopy with a rigid bronchoscope]. / Endogene Tracheobronchialfremdkörper. Eine Indikation zur Notfallbronchoskopie mit starren Bronchoskopen.

We report two patients, a 3-year-old child and a 75-year-old woman, with a foreign body of the trachea, and of the entire tracheobronchial system respectively. We emphasise the importance of rigid endoscopes for foreign body extraction and for respiration. Also, we describe the differentiation of endogenous from exogenous foreign bodies of the tracheobronchial system.

[Intensified therapy of severe lupus erythematosus]. / Intensivierte Therapie des schweren Lupus erythematodes.

14 female patients (mean age 28 [18-56] years) with severe systemic lupus erythematosus (SLE) were treated after discontinuing previous immunosuppressive therapy, according to an intensified protocol, with three plasmaphereses (days 1-3), followed by pulse cyclophosphamide (12 mg/kg i.v. each on days 3-5) and then oral immunosuppression (cyclophosphamide 1-5 mg/kg daily, depending on white blood cell count; prednisone equivalent 2.0 decreasing to 0.1 mg/kg, according to response, for 6 months). The aim of "synchronization" of plasmaphereses with subsequent cyclophosphamide pulse-therapy is to damage pathogenic lymphocyte clones during maximal compensatory activation induced by plasmapheresis. In all patients there was rapid improvement from the nephritis, pneumonitis, cytopenia, CNS abnormalities and polyserositis. The lupus activity index (SLAM) fell clearly, from initially 28.4 (13-37) points to 8.9 (2-13) after 6 months. Treatment was discontinued after this fall in 12 patients. A recurrence was observed in two patients, at 12 and 39 months respectively. Another patient died from liver cirrhosis of unknown aetiology. Nine patients are under observation but without treatment at present, in essential remission after 2 years (5-51 months), with a SLAM of 2.8 (0-7) points. "Synchronization" of plasmaphereses with subsequent pulse cyclophosphamide achieved rapid improvement and it resulted, for the first time, repeatedly in long-term treatment-free clinical remissions.

[The thrombolysis treatment of acute myocardial infarct at the site of the emergency. A randomized double-blind study]. / Thrombolyse-Behandlung des akuten Myokardinfarktes am Notfallort. Eine randomisierte Doppelblindstudie.

Two million units of urokinase were administered intravenously as a bolus, either before (group I, n = 40) or after hospital admission (group II, n = 38), to 66 men and 12 women (mean age 55 +/- 8 years) with typical symptoms of acute myocardial infarction of less than 4 hours' duration. Time elapsed between onset of symptoms and urokinase administration averaged 85 +/- 51 min for group I and 137 +/- 50 min for group II (P less than 0.005). The complication rate was low, both during the pre-hospital and the hospital phases, without any significant differences between the two groups. The rate of open infarct vessels (by angiography before discharge from hospital) was 61% for group I and 67% for group II (no significant difference). Global left ventricular function, regional wall motion in the infarct area and maximal creatinekinase values did not significantly differ between the two groups (ejection fraction 51 +/- 10% and 53 +/- 14%, respectively; creatinekinase 838 +/- 634 U/l and 924 +/- 595 U/l, respectively). The data indicate that thrombolytic pre-hospital treatment carried a low risk. The gain of 45 min, however, seems to be of secondary importance in any significant diminution of the acute infarction size.

[Induction of remission in severe lupus erythematosus by intensified therapy]. / Remissionsinduktion bei schwerem Lupus erythematodes durch intensivierte Therapie.

11 patients with severe SLE were treated with plasmapheresis and subsequent pulse cyclophosphamide. In all patients rapid improvement was observed. After another 6 months of immunosuppression any treatment was withdrawn. The remissions remained stable in 6/8 patients during the follow-up period of 7-33 (mean: 21) months.

[Cyclophosphamide stosstherapy synchronized with plasmapheresis in rapidly progressive glomerulonephritis]. / Cyclophosphamid-Stosstherapie synchronisiert mit Plasmapheresen bei rapid progredienter Glomerulonephritis.

High-dosage stosstherapy of cyclophosphamide was administered, as far as possible optimally synchronized with plasmapheresis, to 12 patients with rapidly progressive glomerulonephritis. The basis for such synchronization lies in experimental data pointing to an increased antibody production after plasmapheresis (antibody rebound). Initial large-volume plasmapheresis was followed by high-dose immunosuppression in the phase of likely maximal proliferation of antibody-producing cells. The serum creatinine level in the 12 patients was 7.7 +/- 3.3 mg/dl before treatment. Eight patients required dialysis. After a period of observation of one year, ten patients are still alive. In one patient treatment had to be discontinued because she did not tolerate it. In the other nine patients renal function stabilized on a dialysis-free level. After one year the serum creatinine level averaged 2.5 +/- 1.2 mg/dl. With one exception no maintenance immunosuppressive treatment is required after one year. These preliminary results suggest that this treatment concept further improves the prognosis of rapidly progressive glomerulonephritis.