High-dose dexamethasone in adult refractory idiopathic thrombocytopenic purpura.
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Adult↗
Biomedical subjects
C Schiavotto
Publications and source records attributed to C Schiavotto.
Failure of repeated courses of high-dose intravenous immunoglobulin to induce stable remission in patients with chronic idiopathic thrombocytopenic purpura.
Repeated courses of HD IVIg are reported to induce stable remission in a significant proportion of adults with chronic refractory ITP. We have treated 14 such patients obtaining a remission rate quite comparable to the 5-10% of spontaneous remission.
Adult↗
A follow-up study of 49 adult patients with idiopathic thrombocytopenic purpura treated with high-dose immunoglobulins and anti-D immunoglobulins.
BACKGROUND: We analyze here short- and long-term results in 49 patients with idiopathic thrombocytopenic purpura (ITP), consecutively treated with high-dose (h.d.) immunoglobulins (Ig) or anti-D Ig. The major aims of this study were to assess the prognostic power of some patient characteristics and to verify the possibility that repeated courses of treatment can induce a stable remission. Moreover, the relative efficacy and safety of these two treatments were compared. PATIENTS AND METHODS: Group A included 28 patients with chronic ITP and 17 with ITP of recent onset who were receiving h.d. Ig; Group B included 5 patients with ITP of recent onset and 7 with chronic ITP treated with anti-D Ig. Eight cases, receiving both treatment, were included in both groups. Response to treatment was defined as any increase in platelet count above 30 x 10(9)/l, when the platelet count was less than 10 x 10(9)/l, or any doubling of the basal platelet count otherwise. Remission was defined as any platelet count higher than 100 x 10(9)/l lasting for 3 months or longer without therapy. RESULTS AND CONCLUSIONS: Cumulative response and remission rate was not statistically different in the two groups. Multivariate logistic regression analysis showed no influence of sex, previous therapy or duration of disease. Patients older than 60 years were definitely less responsive to h.d. Ig (58% vs 92%, p = 0.03). In Group A, two patients obtained remission after the first course of h.d. Ig. No additional remissions were obtained by repeated courses of h.d. Ig, apart from a single case of ITP of recent onset.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Fulminant sepsis in adults splenectomized for idiopathic thrombocytopenic purpura.
BACKGROUND AND METHODS: Splenectomy is still a mainstay in the treatment of idiopathic thrombocytopenic purpura (ITP). Although the risk of fulminant sepsis after splenectomy in children and in adults has been known for a long time, the true prevalence of fulminant sepsis in adults splenectomized for ITP has still not been well established, despite many anecdotal reports. In this paper we have tried to estimate this risk by reviewing the available literature and by analyzing a large cohort of adults splenectomized for ITP at our Institute since 1970. RESULTS: Two cases of fulminant sepsis (a 21-year-old man and a 24-year-old woman) were traced in a group of 133 splenectomized adults, with a crude incidence of 1.5% or, equivalently, of 0.17 cases per 100 patient-years. No case of sepsis occurred in a control group of 323 non splenectomized ITP adults. This estimation seems sufficiently precise for the large population analyzed and the duration and completeness of follow-up (1,126 patient-years, mean 101 months, 2 patients lost from follow-up), and it is in agreement with the estimations yielded by a literature review (1.8%). CONCLUSIONS: The risk of fulminant sepsis seems to be a real hazard in adults splenectomized for ITP, and a conservative approach in recommending splenectomy seems advisable. Antipneumococcal vaccination should also be recommended.
Adolescent↗
Twenty years experience with treatment of idiopathic thrombocytopenic purpura in a single department: results in 490 cases.
BACKGROUND: All patients with idiopathic thrombocytopenic purpura (ITP), diagnosed in our Department between June 1970 and December 1989, have been analyzed to evaluate the efficacy of treatment, incidence of spontaneous remission and see whether or not there are some parameters that may be indicative of the clinical outcome of these patients. PATIENTS AND METHODS: A total of 93 children and 397 adults have been evaluated. Response to treatment was any increase in platelet count to above 30 x 10(9)/1, when the platelet count was < 20 x 10(9)/l, or any doubling of the basal platelet count otherwise. Remission was defined as any platelet count higher than 100 x 10(9)/l lasting for 3 months or longer without treatment. For duration of the response and remission, the last check-up with a favorable platelet count was considered or the midpoint between the last favorable check-up and relapse. RESULTS AND CONCLUSIONS: In children, short-term responses to prednisone were obtained in 78% of acute patients and 67% of chronic patients, while stable remissions or long-term responses were found in 49% and 17% (p = 0.01). In adults, a short term response to prednisone was obtained in 71% of acute and in 72% of chronic patients, while stable remissions or long-term responses in 30% and 5% (p = 0.000003). In 40/45 children (89%), splenectomy induced a stable remission or a long-term response, while in adults, remissions or a long-term responses were achieved in 100/133 (75%) (p = 0.003). Age below 40 was found to be a significant determinant of a better response (p = 0.0013). Of the 92 evaluable adult patients with borderline thrombocytopenia, 8 developed a severe thrombocytopenia (9%). Spontaneous remissions or spontaneous rises to safe levels of platelet counts were recorded in 9% of adult cases presenting with severe thrombocytopenia: among refractory patients, spontaneous remission occurred significantly more frequently in younger patients (p = 0.003). An overall rate of 19% of adult patients continue to need therapy or have become refractory to any treatment. Among these cases, 7 died from cerebral hemorrhage, 6/7 were older than 50 years. Thus, the overall crude rate of thrombocytopenia-related death for our series of patients was 1.8%.
Adolescent↗
Treatment of idiopathic thrombocytopenic purpura (ITP) in patients with refractoriness to or with contraindication for corticosteroids and/or splenectomy with immunosuppressive therapy and danazol.
BACKGROUND: The best treatment for patients with idiopathic thrombocytopenic purpura (ITP) who are refractory to or have contraindications for splenectomy and corticosteroid remains uncertain. We report here our experience with vinca alkaloids (VA), azathioprine (Azp) and danazol in 33 such patients (6 M/27 F), median age 66 (23-83). PATIENTS: Group A (n = 19), Group B (n = 11), Group C (n = 17) patients were treated with VA, Azp and danazol. Fourteen patients were given more than one immunosuppressor agent. Sixteen patients were given 2 mg/week bolus infusions of vincristine (Vcr), while weekly slow infusions of vinblastine (Vnb, 0.1 mg/kg), for 2-4 weeks, were administered to the remaining 3 cases of Group A. Azp was administered at a daily dose of 150 mg for a median duration of 6 months. Danazol was administered at a median daily dose of 400 mg (400-800 mg), for a median length of 5 months. Response was defined as any increase of platelet count to higher than 30 x 10(9)/l, when platelet count was < 20 x 10(9)/l or any doubling of the basal platelet count otherwise. Remission, any increase of platelet count to higher than 100 x 10(9)/l lasting for 3 months or longer without therapy. RESULTS AND CONCLUSIONS: In Group A, there was a response rate of 63%, with 2 remissions (10%). All responses were observed after the first infusion. Two additional patients, who responded transitorily to VA, went into spontaneous remission 19 and 51 months after the last infusion of VA. In Group B, the response rate was 45%, with 1 remission (9%). The response was never observed before one month. One additional patient went into spontaneous remission 60 months after stopping Azp. In Group C, the response rate was 56% with 2 remissions (12%); 2 patients relapsed while on therapy, 4 continue to require therapy and 1 died from a stroke while on therapy. Four patients in Group A and two in Group B discontinued the therapy because of severe side effects. Danazol was generally well tolerated but for one patient was interrupted after only 5 days because of severe dyspepsia. In conclusion, the clinical usefulness of VA and Azp is very limited and burdened by severe side-effects. Danazol seems to be safer but no more effective and its long-term toxicity is not known. There were two hemorrhagic deaths in this series of patients.
Adrenal Cortex Hormones↗
Adverse reactions after high-dose intravenous immunoglobulin: incidence in 83 patients treated for idiopathic thrombocytopenic purpura (ITP) and review of the literature.
BACKGROUND: High dose intravenous immunoglobulin (h.d. Ig) is increasingly used in several hematological diseases. There are few data on the safety and no review of side effects after this treatment is available. We review here the literature on reported adverse reactions after h.d. Ig and report our own experience in the management of 83 patients with idiopathic thrombocytopenic purpura (ITP). PATIENTS AND METHODS: Computer assisted (Medline) and manual searches of the cumulative Index Medicus were undertaken to retrieve articles reporting side effects after infusion of h.d. Ig, published between 1981 and August 1993. Our own experience is based on clinical records of 83 patients, 56 females, 27 male, with a median age of 61 years (range 8-84), treated with h.d. Ig for acute or chronic ITP between 1985 and June 1993. RESULTS AND DISCUSSION: Major adverse reactions included aseptic meningitis (14 cases), hemolytic anemia (8 cases) and renal dysfunction (12 cases). The most frequently reported mild side effect was headache. We have had 2 cases of severe skin reactions, 1 case of acute renal failure, 3 cases of severe headache and 1 case of chills and fever. For other 3 cases, an anaphylactic/oid reaction, caused by a too rapid infusion rate, occurred. Thus, 10/83 (12%) patients, 7 female and 3 male, developed side effects, but they were unpredictable and severe in only 3 cases (4%). Side effects developed only in 21/480 (4%) infusions.
Acute Kidney Injury↗
The clinical significance of the antiplatelet antibody test based on results for 265 thrombocytopenic patients.
BACKGROUND: The usefulness of measuring antiplatelet antibodies by ELISA or cytofluorimetric techniques for the diagnosis of idiopathic thrombocytopenic purpura is still uncertain. METHODS: We evaluated the clinical significance of two widely applicable antiplatelet antibody tests for a series of 265 patients evaluated consecutively in our Department for the diagnosis or follow-up of thrombocytopenia. Flow cytofluorimetry was used to measure platelet-associated immunoglobulins (PAIg) and the enzyme-linked immunosorbent assay (ELISA) was used to detect antiplatelet antibodies in patients sera (S-PBIg). The significance of antiplatelet antibody testing was addressed by studying the factors that influence test positivity, the diagnostic value of a positive test and the prognostic implication of a positive test. RESULTS: The platelet count was found to be strongly associated with positive PAIg or S-PBIg (P < 0.001), while neither splenectomy nor corticosteroid treatment affected PAIg or S-PBIg positivity. Both PAIg and S-PBIg were limited diagnostic value for the differential diagnosis of idiopathic thrombocytopenic purpura (ITP) from secondary thrombocytopenia for patients with platelet counts between 25,000 and 100,000 platelets/microliters, but the percentages of misclassified patients based on only the PAIg or S-PBIg test were 32 and 54%. We found no relationship between PAIg and/or S-PBIg at time of diagnosis and the patient's clinical response to corticosteroid therapy. CONCLUSIONS: We conclude that antiplatelet antibodies are strongly correlated with platelet counts, discriminate poorly between ITP and secondary thrombocytopenia and have negligible prognostic value. Therefore, we do not recommend performing antiplatelet antibody tests as a routine laboratory test in the diagnostic workup of thrombocytopenia.
Adolescent↗
Gestational thrombocytopenia: a prospective study.
Gestational thrombocytopenia (GT) is commonly observed in pregnancies with otherwise limited obstetric and hematologic complications. However, few data are available on the natural history of the disease, and on the recurrence of thrombocytopenia in subsequent pregnancies. From June 1987 to December 1993, 37 consecutive patients with GT were enrolled in a prospective study, with a total of 41 pregnancies observed. Vaginal delivery was carried out in 33/41 (80%); two patients were transfused fused with packed red cells for obstetric hemorrhage (post-partum uterine atony). Neonatal bleeding did not occur. In all newborns platelet count was performed within 24 hours after delivery: 2 newborns had mild (80 and 75 x 10(9)/L) and 1 severe thrombocytopenia (12 x 10(9)/L) at birth; all of them recovered to a normal platelet count within 10 days without treatment. Twenty-eight out of 3% patients were followed for 12 months after delivery; in 23 a normalization of platelet count occurred within 1-5 months from delivery; in 5 mild thrombocytopenia (100-120 x 10(9)/L) persisted during follow-up. Four patients had a second pregnancy and recurrence of thrombocytopenia was observed in all of them. GT is rarely associated with bleeding episodes during pregnancy and partum, and recovers spontaneously within few months after delivery but thrombocytopenia can recur in subsequent pregnancies. Severe thrombocytopenia is not observed in newborns so that a conservative management is warranted.
Adult↗
Pilot study on the safety and efficacy of desmopressin for the treatment or prevention of bleeding in patients with hematologic malignancies.
BACKGROUND AND OBJECTIVES: Desmopressin is the treatment of choice for patients with von Willebrand's disease and mild hemophilia A. This compound is also useful in other congenital and acquired disorders of hemostasis, reducing the need for blood derivatives with the inherent risks of infections and alloimmunization. The following article presents a pilot study on the safety and efficacy of desmopressin for the treatment or prevention of bleeding in 15 patients with thrombocytopenia associated with hematologic malignancies. METHODS: Cases were consecutively recruited from February to June 1995. Fifteen patients were treated with desmopressin for prevention or treatment of bleeding. Desmopressin was diluted in 100 mL of isotonic saline and infused for 30 minutes. Bleeding time (BT) was carried out using the Simplate II device, making two standardized incisions on the forearm: the mean between the two incisions was recorded. RESULTS: Significant reduction of BT was observed in three out of four patients with myelodysplastic syndrome who were successfully treated for active bleeding or dental extraction. In the remaining patients, the effect of desmopressin on BT was not tested. Nevertheless, in all of them bleeding mainly due to epistaxis or persistent gum oozing was stopped by a single infusion of desmopressin. In three patients, desmopressin infusion had been successfully administered on a different occasion. No side effects were observed. INTERPRETATION AND CONCLUSIONS: Desmopressin could be a safe and immediately effective option for the treatment or prevention of bleeding in selected patients with hematologic malignancies.
Adult↗