Acquired factor V inhibitor in a woman following aortic aneurysm surgery.

A 67-year-old woman with nephrotic syndrome as a complication of membranous glomerulonephritis associated with chronic active hepatitis B virus infection developed factor V inhibitor following emergency aortic aneurysm surgery followed by massive blood transfusions and haemodialysis. On the second postoperative day, prothrombin time and activated partial thromboplastin time increased and were unresponsive to fresh frozen plasma. Epistaxis and urethral bleeding were observed, followed by mucosal mouth bleeding. A very low factor V activity less than 5% was found and a factor V inhibitor was detected at 7.76 Bethesda Units. Treatment with corticosteroids was successful. In this patient, several conditions known to predispose to the generation of factor V inhibitor occurred simultaneously. Four months later, factor V inhibitor (225 Bethesda Units) recurred and the patient died of intracerebral haemorrhage.

[Plasmapheresis in intensive therapy unit]. / Plazmafereza w oddziale intensywnej terapii.

Plasmapheresis or therapeutic plasma exchange is a procedure designed to deplete the body of blood plasma without blood cells. Whole blood is removed from the body, the plasma is separated from the cells, and the cells are suspended in saline before being returned to the patient. The procedure is used to remove excess antibodies, immunoglobulins, or cytokines from the blood in various clinical situations. The particles removed should be adequately large (>15 kDa) and have a relatively long half-life. The volume of removed plasma should be based on body weight and haematocrit, and in an adult patient be in range of 2.5-2.7 litres (30-40 mL kg(-1)). To remove 90% of a harmful substance, four to five exchanges are necessary. In the review, several modes of plasmapheresis are described. The most common indications for plasmapheresis in patients treated in an ITU are: thrombotic thrombocytopenic purpura, Waldenström macroglobulinemia, Guillain-Barre syndrome, chronic inflammatory poliradiculopathy, myasthenia gravis, or Lambert-Eaton syndrome.The procedure is safe, and complications are rare and not serious.

[Thrombotic thrombocytopenic purpura with myocardial ischaemia: two case reports]. / Zakrzepowa plamica maloplytkowa z objawami niedokrwienia miesnia sercowego--opis dwóch przypadków.

Thrombotic thrombocytopenic purpura (TTP) is mainly perceived by cardiologists as a rare complication of ticlopidine or clopidogrel treatment. However, this life-threatening disease is provoked not only by antiplatelet drugs and may lead to myocardial ischaemia and necrosis caused by microvascular thrombosis and anaemia. We present two thienopiridine-naive patients who had acquired TTP and myocardial ischaemia, and were successfully treated by plasma exchanges.

Acute respiratory failure in patients with Guillain-Barré syndrome and myasthenic crisis treated with plasmapheresis in the intensive care unit.

We present the cases of two patients with Guillain-Barré syndrome and one with myasthenic crisis who developed acute respiratory failure and needed mechanical ventilation in the intensive care unit. All the patients were treated with plasmapheresis, resulting in weaning from mechanical ventilation, and eventually complete functional recovery. Early treatment with plasma exchange shortens the treatment duration, whereas early intubation can prevent life-threatening complications.

The additive antiplatelet action of clopidogrel in patients with coronary artery disease treated with aspirin.

We searched for additional anti-platelet effects of clopidogrel in coronary artery disease (CAD) patients treated with aspirin. Response to clopidogrel was also stratified according to aspirin resistance. Out of 76 screened aspirin-treated CAD male patients, five were aspirin-resistant based on arachidonic acid (AA) and ADP aggregometry. These five patients and 15 aspirin-sensitive patients entered the proper study. Platelet function was assessed at baseline and after one week of additional clopidogrel treatment using aggregometry, flow cytometry (ADP, TRAP-6) and platelet reactivity index (PRI) based on VASP (vasodilatorstimulated phosphoprotein) expression. We evaluated the same markers in 15 healthy men after aspirin treatment. In healthy subjects aspirin did not affect resting or ADP-induced activated GPIIb/IIIa and P-selectin expression. The P-selectin expression on ADP-activated platelets was increased (p < 0.01) in aspirin treated ASA-resistant CAD patients as compared to ASA-sensitive group or aspirin-treated healthy subjects. Clopidogrel significantly decreased ADP and AA-induced platelet aggregation and overcame aspirin resistance in four of five patients. Expression of ADP-induced activation markers was significantly lowered after clopidogrel in all patients. Out of 20 patients, five did not respond to clopidogrel (<10% inhibition of ADP aggregation), and this group showed no change in expression of ADP-induced activation markers after clopidogrel. Clopidogrel treatment significantly reduced PRI only in the clopidogrel-sensitive group. In conclusion, the addition of clopidogrel to aspirin provides greater inhibition of platelets and can overcome aspirin resistance. Flow cytometric analysis of platelets is useful for monitoring of clopidogrel therapy.

Antithrombotic effects of aspirin based on PLA1/A2 glycoprotein IIIa polymorphism in patients with coronary artery disease.

OBJECTIVE: The diallelic glycoprotein IIIa polymorphism P1A1/A2 was attributed to be an inherited risk factor for coronary events. Whether this polymorphism affects response to aspirin in patients with coronary artery disease is not known. METHODS: We assessed thrombin generation (prothrombin fragment F1+2) in consecutive blood samples collected from bleeding-time wounds in 28 men with coronary artery disease; P1A2 carriers, n=9; P1A1/A1, n=19. Thrombin generation and bleeding time were measured before and after 2 weeks of aspirin 300 mg/day. RESULTS: Aspirin-depressed thrombin generation in A1 homozygotes (p=0.04), but not in A2 carriers. Bleeding time after aspirin was also prolonged in A1 subjects only (p=0.02). CONCLUSION: Genotyping for glycoprotein IIIa polymorphism might be helpful in predicting antithrombotic action of aspirin in secondary prevention of coronary artery disease.

[Recurrent crural phlegmonous abscesses as a sign of Münchausen syndrome in a young woman]. / Nawracajaca ropowica podudzia u mlodej kobiety jako manifestacja zespolu Münchausena.

We present a case of a 22-year-old woman chronically treated in many hospitals because of recurrent crural phlegmonous abscesses. Several months' follow up revealed Münchausen syndrome--a rare psychiatric disorder--manifesting with self-perpetuated and repeated fabrication of illness resulting in wandering from hospital to hospital for diagnosis and treatment. The above patient produced the abscesses by deliberately traumatizing her skin. The case points to Münchausen syndrome as a possible rare cause of some long, unsuccessfully treated diseases.

Anti-thrombotic action of clopidogrel and P1(A1/A2) polymorphism of beta3 integrin in patients with coronary artery disease not being treated with aspirin.

Individual variability in response to clopidogrel is known but its mechanism is poorly understood. We examined the relationship between glycoprotein IIIa polymorphism P1(A1/A2) and anti-thrombotic actions of clopidogrel. Clopidogrel (75 mg/d; 2 weeks) was administered to 48 normolipemic patients with coronary artery disease. Bleeding time, thrombin generation at the site of microvascular injury, platelet function under high shear, using PFA-100 with ADP cartridge, and platelet surface activation markers (P-selectin and fibrinogen binding sites on GPIIb/IIIa complex detected by PAC-1 antibody), were studied both before and after clopidogrel treatment. Both unstimulated and low-dose (0.02 microM and 1 microM) in vitro ADP-stimulated platelets were examined. GP IIIa polymorphism was assessed by polymerase chain reaction and restriction fragment length polymorphism analysis. We identified 32 P1(A1/A1) homozygotes, 15 P1(A1/A2 heterozygotes and one P1(A2/A2) homozygote. Clopidogrel significantly prolonged bleeding time in all subjects, but this effect was greater in P1(A2 carriers (p < 0.01). Furthermore, clopidogrel only depressed thrombin generation at the site of microvascular injury (p < 0.01) in P1(A2) patients and prolonged closure time measured in vitro by PFA-100 (p < 0.05). At baseline spontaneous expression of PAC-1 and P-selectin was higher in P1(A2) subjects as compared to P1(A1) homozygotes (p < 0.05 for both antigens). Clopidogrel lowered the expression of both markers affecting more P1(A2) carriers, so that the difference in binding PAC-1 antibody between platelets from P1(A1) and P1(A2) carriers disappeared, while the difference in P-selectin expression slightly diminished. Anti-thrombotic effects of clopidogrel are more pronounced in CAD patients carrying the P1(A2) allele than in P1(A1) homozygotes.

[Myocardial infarction as an initial symptom of antiphospholipid syndrome, factor V Leiden mutation, and hyperhomocysteinemia]. / Zawal serca jako przejaw pierwotnego zespolu antyfosfolipidowego. Wspóludzial czynnika V Leiden i hiperhomocysteinemii.

We present a case of a 32-year-old male survivor of two myocardial infarctions, without any classic risk factor of atherosclerosis. Laboratory and genetic diagnostic tests revealed primary antiphospholipid syndrome, mutation in blood coagulation factor V (Leiden) and mild hyperhomocysteinemia, which could be predisposing factors for coronary artery occlusions and should especially be considered in a young patient without apparent cardiovascular risk factors. Additional anticoagulation and substitutional treatment of the folic acid, vitamin B6 and B12 are effective and the continues to do well at home 3 years after discharge.

Increased carotid artery intima-media thickness as an indicator of the onset of atherosclerosis in patients with connective tissue systemic diseases.

BACKGROUND: Systemic connective tissue diseases have an autoimmunological background. Atherosclerosis is the main cause of ischaemic heart disease in patients with these disorders, particularly in young females. Atherosclerotic process begins in the intimal and medial layers of arterial wall. Early detection of these changes may have important clinical implications. AIM: To assess the intima-media thickness (IMT) in carotid arteries in patients with connective tissue disorders and to correlate IMT with the presence of antiphospholipid (aPL) antibodies. METHODS: The study group consisted of 74 patients (63 females, 11 males) with documented connective tissue disease and 75 (62 females, 13 males) control subjects without clinical symptoms suggesting atherosclerosis. The IMT values, measured using ultrasonography, and aPL (IgG and IgM) antibody titre were assessed in all subjects. RESULTS: Mean aPL IgG and IgM values were significantly higher in patients than in controls (9.22 GMP/ml vs 6.59 GMP/ml, p<0.01; and 18.59 MPL/ml vs 12.05 MPL/ml, p<0.01, respectively). Patients with connective tissue diseases had significantly higher IMT values than controls (0.82 mm vs 0.57 mm, p<0.01). The IMT values positively correlated with age, presence of aPL antibodies, hypercholesterolemia and duration of the disease. CONCLUSIONS: The presence of aPL antibodies and increased IMT may indicate atherosclerosis in young patients with connective tissue diseases, and identify those who need more intensive prophylactic treatment in order to decrease the risk of atherosclerosis-related complications and death.

[Atypical symptoms of Fabry's disease: sudden bilateral deafness, lymphoedema and Lown-Ganong-Levine syndrome]. / Nietypowe objawy choroby Fabry'ego--nagla obustronna utrata sluchu, obrzek limfatyczny i zespól Lowna, Ganonga i Levine'a.

A 40-year-old man with Fabry disease, confirmed by decreased leukocyte alpha-galactosidase A activity in 2001, complained of sudden bilateral deafness, as evidenced by clinical history and audiometry. Magnetic resonance of the brain revealed features typical of Fabry disease. Other clinical manifestations of the disease included: angiokeratoma, mild proteinuria with normal renal function, lymphoedema of the lower limbs, pre-excitation syndrome, myocardial hypertrophy.