The role of erythrocytapheresis in secondary erythrocytosis therapy.

In chronic respiratory insufficiency secondary erythrocytosis (SPC), causing pulmonary hypertension and dx ventricular insufficiency, is often noticed. An alternative therapy to phlebotomy for SPC is isovolemic large volume erythrocytapheresis performed with cell separator (CSE) in order to quickly remove a large volume of red blood cells (RBC) while saving plasma proteins and clotting factors. In order to evaluate the efficiency and safety of CSE in SPC we reported a retrospective analysis of our experience with 61 SPC patients: from April 1996 to May 1998 we performed 208 CSE using Haemonetics MCS3P (TAE protocol). Before every apheresis procedure we verified Hb (in median 18.8 g/dl), Ht (in median 58.4%), viscometry, coagulation test, EGA, PFR and ECG. 11 patients were treated with 1 CSE, 12 with 3, 29 with 4 and 9 with 5. The mean volume of RBC removed was 576 ml (range 426-800); Hb post CSE averaged 14.4 g/dl and Ht post CSE averaged 42.7%; hematic viscosity post CSE was significantly reduced while tissue oxygen tension increased: the improvement of symptomatology and hematochemical parameters was maintained on the average for 6.5 months. All the procedures were well tolerated and light side effects (paresthesias citrate-depending in 27 apheresis) were easily controlled. CSE, compared to phlebotomy, has the advantage of selectively removing RBC without loss of clotting factors, platelets and plasma proteins. Although CSE has relatively high costs we noticed a decrease of hospital recurrence (about 50-65%) in SPC patients treated with apheresis.

Thrombin and platelet inhibition with low-dose calcium-heparin in comparison with ASA in patients with peripheral arterial occlusive disease at Leriche-Fontaine IIb class.

Sixty patients with a mean age of 64 years (range 57-77 years), 55 males and 5 females, with peripheral arterial occlusive disease (PAOD) at Leriche-Fontaine IIb class were randomly allocated to receive either subcutaneous calcium-heparin (12,500 IU once daily) or oral acetylsalicylic acid (ASA) (300 mg twice daily), each given for 3 months, followed by a 6-month follow-up period during which no treatment was given. The following parameters were evaluated before randomization, after 1 and 3 months of therapy and after 1, 3 and 6 months of follow-up: pain-free walking distance (PWD), maximum walking distance (WDmax), systolic and diastolic blood pressure, posterior tibial arterial pressure and ankle/arm index at rest and after exercise (treadmill), transcutaneous gas analysis at rest (TcPO2). Under both treatments a statistically significant increase (p < 0.01) in PWD/WDmax was observed after 3 months of therapy. Calcium-heparin was slightly but significantly more effective than ASA in prolonging WDmax (p < 0.05) after 3 months of therapy. Both treatment groups maintained the clinical improvement after 6 months of follow-up without any difference between each-other. Both treatments resulted in a statistically significant increase in TcPO2 and decrease in plasma fibrinogen but under calcium-heparin treatment these modifications were doubled in comparison with ASA (p < 0.01 and p < 0.05, respectively for TcPO2 and fibrinogen). The sustained effects on these parameters after 6 months of follow-up could suggest a more direct and multifactorial activity of calcium-heparin on microcirculation.(ABSTRACT TRUNCATED AT 250 WORDS)

Arterial thrombosis as clinical manifestation of congenital protein C deficiency.

A 49-year-old man was hospitalized for slight paresis of the upper left limb. Thrombosis of the right internal carotid artery was documented by arteriography and digital angiography, which showed evidence of an anastomotic blood flow. He went on anticoagulation treatment. Five years later, after an uneventful period, he was referred to our center for the occurrence of a superficial thrombophlebitis: diagnosis of congenital protein C deficiency was possible in the patient as well as in two of his relatives. Two other subjects with congenital protein C deficiency belonging to two different kindreds, whose illness was diagnosed in our center, suffered from myocardial infarction and TIA, respectively, as the only clinical manifestation; a fourth case, previously described, with recurrent superficial thrombophlebitis, suffered from a TIA when on treatment with stanozolol. These cases indicate that arterial thrombosis or TIA is not an uncommon event in congenital protein C deficiency, even in patients without other risk factors for arterial thrombosis.

Transient ischemic attack in a patient with congenital protein-C deficiency during treatment with stanozolol.

A patient with congenital protein-C deficiency was treated with stanozolol for 8 weeks to increase circulating levels of protein C. A rise in protein C was achieved, accompanied by an increase in factor II, factor X, antithrombin III, and protein S; but at the 8th week the patient suffered a transient ischemia attack.