Plasma-exchange in chronic peripheral neurological disorders.

We investigated 19 patients affected by chronic peripheral neurological disorders treated with therapeutic plasma exchange (TPE) to verify the efficacy of the therapeutic protocol used in these diseases. Every patient was clinically considered after 5 TPE. Those who showed an improvement started chemotherapy and continued TPE at the rate of 2 procedures/week for 2 weeks, then 1 procedure/week for 1 month and finally 1 procedure every 2 weeks for 2 months. Intravenous immunoglobulins (IVIg) were infused at the end of apheretic treatment in one of the patients affected by neurological disorders due to monoclonal gammopathy undetermined significance. HCV-positive patients with cryoglobulins were treated with alpha-interferon (alpha-IFN) for 6 months before TPE. Eleven patients (58%) had a symptomatic improvement, 2 (1.5%) stopped TPE treatment owing to side effects and 6 (31.5%) did not respond to apheretic therapy. In order to improve the advantages of TPE we suggest using IVIg at the end of apheretic therapy, while in HCV-positive patients, at least one year of alpha-IFN therapy is required before initiating TPE.

Primary hyperfibrinogenolysis in a patient with anaphylactic shock.

Although systemic hyperfibrino(geno)lysis during hypotensive crisis is known, there do not seem to be recent reports of episodes of primary acute fibrinogenolysis during anaphylactic shock. We report the case of a 61-year-old male admitted to the hospital for anaphylactic shock due to an insect bite who presented a clinical and laboratory picture of severe acute generalized hyperfibrinogenolysis not secondary to disseminated intravascular coagulation (DIC). Without specific therapy, the clinical picture resolved itself spontaneously within 40 hours of onset. Careful clinical examination and the execution of simple laboratory tests permitted a rapid diagnosis and therapeutic success.

Presence of anti-Yta antibody in a Yt(a+) patient.

Since 1956 when Cartwright (Yt) was recognized as a new erythrocyte antigenic system, numerous studies about anti-Yta and anti-Ytb antibodies have been published. A number of these studies described the laboratory techniques utilized in antibody identification, while others investigated the clinical importance of anti-Yta, giving variable results. However, most authors agreed upon the homogeneity of expression in this antigenic system. In our study we have described a case regarding 1 Yt(a+) patient with anti-Yta antibody. Family studies indicated inheritance of a variant/variant expression of the Yta antigen from the father.

Combined factor VIII and factor XI congenital deficiency: a case report.

Combined congenital defect involving both Factor VIII and XI is a very rare disorder. We describe a case of combined Factor VIII and XI deficiency in which the propositus has a mild hemophilia A and inherited a Factor XI deficiency from the father. Moreover, we report on the benefit of DDAVP administration to the patient during a bleeding episode.

Erythrocyte endogenous proteinase activity during blood bank storage.

We studied proteolytic alterations of membrane proteins in ghosts derived from human red blood cells, preserved up to 35 days in the liquid state either as whole blood or with additive solution. The study was carried out by performing sodium dodecyl sulfate polyacrylamide gel electrophoresis of stromal proteins from erythrocytes, either previously treated with proteinase inhibitors or previously incubated in conditions promoting proteolysis. To differentiate the effect of erythrocyte from granulocyte proteinases, the investigation was also carried out in leukocyte-free red cell preparations. The results show: (1) the effects of endogenous proteinases on membrane proteins derived from red cells stored under blood bank conditions; (2) a decrease of proteolytic effects in ghosts derived from red cells which have been submitted to a longer storage; (3) a relevant influence of the red cell resuspending medium before lysis on the time-dependent onset and exhaustion of proteolysis in ghosts. The presence of increased proteolysis in ghosts could be regarded as a marker of molecular lesions induced in red cells by storage under blood bank conditions.

Normal fibronectin levels after surgical treatment of portal hypertension in liver cirrhosis.

We found a significantly higher plasma fibronectin concentration in a group of nine cirrhotic patients who underwent surgical treatment for portal hypertension (either shunting and non shunting procedures) when compared to twenty non operated patients. Significantly shorter prothrombin time and activated partial thromboplastin time in the operated patients were found as well. These results might be related to an increased breakdown of fibronectin during consumption coagulopathy taking place in the extended collaterals and reversed in part by surgical treatment of portal hypertension complicating liver cirrhosis.

Time-related shape control modifications during erythrocyte storage with additive solutions.

To obtain more detailed information on the reversibility of shape alterations in blood bank stored erythrocytes, we have studied shape recovery after chemical crenation and rheological properties in 8 PAGGS-sorbitol preserved erythrocyte concentrates during a five week storage period under blood bank conditions. Our results show that red cell capability to regain a normal discoid shape after chemical crenation decreases during storage but is not lost over a five week period. Moreover there is a significant but weak correlation between red cell ATP content and both shape recovery capability and viscosity. Our results confirm suspicious that red cell shape perturbations following blood bank storage are widely reversible. Two different mechanisms may be involved in reducing shape recovery capability during storage, namely an ATP-dependent mechanism and an energy-independent one. The energy dependent mechanism may be preserved by the previous addition of solutions which maintain higher energy levels during storage.