Thrombotic microangiopathy (TMA) is a histopathological feature of various diseases including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). There are many secondary causes of TMA, many of them could mimic TTP or HUS. This article presents a short overview on TMA. In conclusion TMA is the result of various etiology reasons and pathologic reactions with various clinical entities. It is important to focus on a thorough history including family history when deciding on a diagnosis. Analysis of ADAMTS 13 and ADAMTS 13-antibodies may help to decide continued therapy.
Psoriasis is a common autoimmune chronic inflammatory skin disease that affects approximately 2% of the world's population; fundamental for its immunopathogenic mechanism is secretion of type 1 (Th1) cytokines by T cells and their activation. Since cytapheresis has been widely applied to autoimmune disorders, emphasizing the recently reported results of granulocyte and monocyte adsorption apheresis in psoriasis, a small series of psoriasis vulgaris (PV) patients underwent lymphocytapheresis (LCA) with the aim to remove lymphocytes. Five patients were submitted to weekly LCA. The severity of the disease had been evaluated through psoriasis area and severity index (PASI) score before LCA and one week after the last apheresis. PASI score before: patient A: 66; patient B: 33; patient C: 50; patient D: 56; patient E: 29. All the patients showed improvement of skin lesions. PASI score after LCA: patient A: 24; patient B: 8; patient C: 5; patient D: 36; patient E: 2.1. No side effects linked to apheresis were reported. LCA seems to produce interesting results in PV, and PASI improvement related to apheresis is clinically significant. Further studies to address its mechanism of action and potential long-term side effects are needed. It could become a valuable therapeutic alternative or a complementary tool, which might even be used to reduce the dosages of conventional pharmacological therapies adopted for this chronic disease.
Centers from 9 countries have applied for a login code to the WAA apheresis registry. Of these 9 centers from 4 countries have been actively entering data during 2004. Registered are at the moment a total of 347 patients (55% women, mean age 54 y, and 45% men, mean age 52 y). Main indications were neurological (33%), vasculitis (9%), and lipid apheresis (8%). In 92% of the patients therapeutic apheresis was performed while in 8% retrieval of components was the reason. A total of 1808 procedures were registered. More than 88% of the patients had acute indications. Access was mainly by peripheral vessels (65%), central dialysis catheter through jugular route (11%) or subclavian (9.4%) while femoral vein and AV fistula were used in 4.3 versus 0.5% of occasions. Anticoagulation was performed by ACD (80.5%), CPD citrate (2.5%), ACD and heparin (7%), ACD and LMW heparin (4%) and only heparin (5.8%). Replacement fluids for therapeutic apheresis removing plasma was mainly by albumin (85%) while plasma (22%) and cryoprecipitate poor plasma (5%) was used to less extent. Adverse events (AE) were registered in 6% of procedures. Distribution was for mild AE (1.4%), moderate (3.4%) and severe (0.6%) In 21 treatments there were 2 AEs while in 2 patients there were 3 AEs. Most frequent AEs were hypotension (35%), tingling around the mouth (24%), chill and fever (5.5%) and urticaria (5.5%). There were significant differences between the centers. The incidence of hypotension varied between 0.36–3.23% between centres. There was no significant difference in severe AEs between the centres. Main procedure used was centrifugation technique for conventional plasma exchange by centrifugation or filtration, while other modes were leukapheresis, erythrapheresis, platelet apheresis, LDL‐apheresis, photopheresis and adsorption. Conclusion: Data from the registry show that centres have various approaches to apheresis. One can learn from each others experience to reduce side effects and improve efficacy.
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