The therapy of the idiopathic hypereosinophilic syndrome (HES) is largely directed at controlling symptoms and preventing organ damage. Universally accepted therapeutic protocols for HES are still lacking. Patients are treated by different drugs and drug doses, and therapy appears to be mostly aimed at treating symptoms. However, in order to prevent organ damage the roles of the variously employed agents need to be clarified. Interferon was reported to be an effective agent in 44 patients, and our evaluation of five new cases suggested that IFN-alpha may be suitable as a first-line 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.
Thrombocytosis has been reported during chemotherapy with gemcitabine (GEM). A 71-year-old male patient who developed asymptomatic thrombocytosis (1,749 x 10(3)/microl), secondary to GEM chemotherapy for lung carcinoma, was submitted to therapeutic plateletpheresis (TP). We adapted the software of the productive dual-needle plateletpheresis of our cell separator in order to reduce the procedure time and the plasma volume harvested. With 2 low-volume TP in 24 h, we achieved a 55% global reduction of the platelet count. While there are no accepted indications for prophylactic plateletpheresis in asymptomatic patients and the measured count should not, by itself, determine whether platelet reduction is indicated, a drastic reduction of an extremely high platelet count, especially if obtained with a relatively short procedure, can be important to prevent acute severe sequelae.
Background: These last 20 years, the activity of Italian blood banks and transfusion services has been modified and widened, following the evolution of transfusion medicine from the primary, prevailing interest for immunohematology towards new activities in diagnostic, clinical-therapeutic and research field. Notwithstanding this gradual transformation, blood collection activity and the production of hemocomponents in quantities proper to reach and/or keep a balance of self-sufficiency on a local and national level, keeps beeing a prior target, as it is the main task of National Health Department plans. The aim necessary to guarantee hemocomponent safety is very important also, as such as to meet public opinion expectations and law provisions in an efficient way. Blood safety can only be joined to adopting severe methodological criteria even upon donor’s selection stage, in order to avoid hemocomponents donation in the “window period” of HBV, HCV or HIV infections. Nevertheless the temporary donation suspension has a negative impact on subsequent donations by the deferred donors. Donors who do not meet any of the selection criteria in the screening process, may undergo quarantine plasmapheresis (QP) as an alternative to deferral, and QP units may be stocked, validated and released only if repeated donor’s screening tests are negative up to a maximum of 12 months after the risk event. Methods: Since 01/01/98 we have been collecting 600 mL QP units from donors who should have been deferred for one of the following reasons: 1) minor dental surgery (DS); 2) major or minor surgery (MMS); 3) sexual risk behaviours (SRB) (with a limit to: change of sexual partner or more than one partner); 4) health care work risk (HW); 5) other risk events (OR) (with a limit to: tattoo application, skin or ear piercing, acupuncture); 6) endoscopic examination (EE). QP units were daily listed, labelled and physically located and quarantined in monthly dedicated containers within a specific freezer. Results: From 01/01/98 to 06/30/04 we have been collecting 16,555 plasmapheresis units and 1,531 (9.25%) of these were QP donated by 1,053 donors. First time apheresis donors were 350 and 45% of them became repeat plasmapheresis donors. The causes for including donors into QP group have been in percentage respectively: 1) DS: 29.5; 2) MMS: 26; 3) SRB: 20.5; 4) OR: 19; 5) EE: 3; HW: 2. None of the QP units has been eliminated for seroconversion during or at the end of the quarantine period; QP units elimination percentages (0.69) for sanitary reasons (high ALT, HCV positive) soon after donation appear to be substantially superimposable in comparison to non-QP units (0.71). Conclusions: Our data shows that the provisions of this strategy, with a limit to some specific exclusion causes and joined to a proper logistical organization: 1) Does not generate an increase of elimination sanitary causes of plasmapheresis soon after donation. 2) Is highly efficient in improving plasma production safely, without sacrificing whole blood donations. 3) Greatly increases repeat plasmapheresis donor’s recruitment. 4) Prevents the negative impact on subsequent donations by the deferred donors. 5) Is a donor’s recruitment and a retention strategy which contributes to raise plasma production safely, even though restrictive changes to donation selection criteria continue to increase deferrals.
Autoimmune lymphoproliferative disease (ALD) is a rare familial disorder. Clinical and laboratory features of this disease include a generalized lymphadenopathy, splenomegaly, increased levels of circulating CD3+ with low levels of CD4+, CD8+ T-cells, and autoimmune phenomena, characteristics that the autoimmune lymphoproliferative syndrome (ALPS) have in common. Treatment usually consists of different supportive therapies. We report on the case of a young man affected by ALD who became resistant to steroids and was unresponsive to cyclosporine. Nevertheless, he was successfully treated with interferon (IFN)-alpha, resulting in a long-lasting, clinically complete remission.
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