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Biomedical subjects

L M Aledort

Publications and source records attributed to L M Aledort.

At least 55 records · Page 3Linked to original sources
PubMed1997

Intravenous anti-D treatment of immune thrombocytopenic purpura: experience in 272 patients.

We report the results of intravenous anti-D (WinRho, WinRho SD) therapy in 261 non-splenectomized patients treated at the New York Hospital-Cornell Medical Center over the period from 1987 to 1994. Children (n = 124) and adult patients (n = 137) with classic immune thrombocytopenic purpura (ITP; n = 156) or human immunodeficiency virus (HIV) related thrombocytopenia (n = 105) and acute (n = 75) or chronic (n = 186) disease at the time of the initial anti-D treatment were studied. In addition, 11 previously splenectomized patients were treated as a separate group. Our objectives were to evaluate the following. (1) Efficacy of anti-D: The response after the initial infusion was analyzed according to clinical parameters, such as patient's age, HIV status, gender, disease duration, pretreatment platelet count, and hemoglobin value, as well as treatment-related factors, including the dose of anti-D, the solvent detergent treatment of the preparation, and the type of administration. (2) Use of anti-D as maintenance therapy: The duration of response after the initial infusion and the results of subsequent treatments were evaluated. (3) Safety/toxicity of anti-D: Postinfusion reactions and hemoglobin decrease after treatment were studied. Anti-D is a safe treatment providing a hemostatic platelet increase in greater than 70% of the Rh+ non-splenectomized patients. The group with the best results is HIV- children, but all patient groups respond and the effect lasts more than 21 days in 50% of the responders. Duration of response is not influenced by HIV status; furthermore, HIV+ patients show no adverse effects on hemoglobin decrease or HIV disease progression. Patients with chronic ITP after splenectomy have minimal or no response to intravenous anti-D.

Adult↗
PubMed1997

von Willebrand disease: from the bedside to therapy.

Von Willebrand's disease was recognized by a careful observant clinician. Research advances in blood banking technology and molecular biology, in addition to astute clinical observation, have led to unraveling the factor VIII molecule, classification of a heterogenous disease, and rational therapy.

Deamino Arginine Vasopressin↗
PubMed1996

Serum HIV-1 RNA levels and time to development of AIDS in the Multicenter Hemophilia Cohort Study.

OBJECTIVE: To determine if the long-term incidence of the acquired immunodeficiency syndrome (AIDS) is related to human immunodeficiency virus type 1 (HIV-1) RNA levels measured early in HIV-1 infection. DESIGN: Epidemiologic cohort study. SETTING: Five hemophilia treatment centers in the United States. SUBJECTS: A total of 165 subjects with hemophilia and HIV-1 infection (age at HIV-1 seroconversion, 1-66 years) followed from 1979 to 1995. METHODS: The HIV-1 RNA level was measured by polymerase chain reaction over a range of 200 to 1 million or more HIV-1 RNA copies/mL in archived serum specimens collected 12 to 36 months (median, 27 months) after the estimated date of HIV-1 seroconversion. Kaplan-Meier methods were used to examine the risk of AIDS and proportional hazards models were used to estimate relative hazards. RESULTS: The HIV-1 RNA values were similar in subjects younger than 17 years at seroconversion (median, 5214 copies/mL) and those 18 to 34 years old (median, 4693 copies/mL), but higher in those 35 years or older (median, 12069 copies/mL) (P = .02 compared with each younger group). At 10 years after seroconversion, the proportions of subjects with AIDS were 72% among subjects with 100,000 or more HIV-1 RNA copies/mL measured 12 to 36 months after HIV-1 seroconversion (n = 9), 52% among subjects with 10,000 to 99,999 copies/mL (n = 55), 22% among subjects with 1000 to 9,999 copies/mL (n = 82), and 0% among subjects with fewer than 1000 copies/mL (n = 19) (P < .001). The age-adjusted relative hazard for AIDS for subjects with 10,000 or more copies/mL was 14.3 (95% confidence interval, 1.9-105.6) compared with subjects with fewer than 1000 copies/mL. CONCLUSIONS: The HIV-1 RNA level during early chronic HIV-1 infection is a strong, age-independent predictor of clinical outcome; low levels define persons with a high probability of long-term AIDS-free survival.

Acquired Immunodeficiency Syndrome↗
PubMed1996

Long term evaluation of septic arthritis in hemophilic patients.

Before 1983, septic arthritis was rare in patients with hemophilia. With the advent of human immunodeficiency virus infection in the hemophilia population, many centers noted an increasing incidence of patients with septic arthritis. Fifteen septic joints in 10 patients with severe hemophilia were documented. Eight patients were human immunodeficiency virus positive, 1 was human immunodeficiency virus negative, and 1 was not tested. The diagnosis was delayed in 5 patients because the symptoms are similar to an acute hemarthrosis. An elevated temperature was common. The white blood cell count was elevated in only 1/3 of the infections, being modified by human immunodeficiency virus infection. Associated risk factors included infected angioaccess catheters (2), pneumonia (2), and generalized sepsis (1). All but 1 joint responded to appropriate antibiotics and either repeated aspiration or arthrotomy. However, 6 patients died of acquired immunodeficiency syndrome from 2 to 109 months after infection. Three patients are alive 29, 86, and 96 months, respectively, after infection.

Adolescent↗
PubMed1996

AIDS-associated non-Hodgkin's lymphomas as primary and secondary AIDS diagnoses in hemophiliacs. Hemophilia Malignancy Study Group.

We studied the characteristics and temporal trends of AIDS- associated non-Hodgkin's lymphoma (AIDS-NHL) in individuals with hemophilia. Prospective data were collected on 33 HIV-positive hemophiliacs with AIDS-NHL enrolled in the Hemophilia Malignancy Study (HMS), of whom 21 had primary and 12 had secondary or subsequent AIDS-defining illnesses, and analyzed for frequency and temporal trends. As compared with primary AIDS- NHL, secondary AIDS-NHL occurred at an older mean age, 37 versus 29 years (p = 0.12); at a lower mean CD4 count, 46 versus 154 (p = 0.07); after a longer period of immunosuppression (CD4 < 200/microl), 41 versus 16 months (p = 0.03); and with shorter median survival, 2 versus 7 months (p = 0.09). The presence of EBV in tumor tissue was associated with shorter survival, 1 versus 7 months (p = 0.17). Between 1981 and 1988 and 1989 and 1994, the proportion of primary AIDS diagnoses that were AIDS-NHL changed minimally, 4.6 versus 6.1%, whereas there were significant decreases in Pneumocystis carinii pneumonia (PCP, p = 0.02) and wasting (p = 0.07), and an increase in Candida (p = 0.004). These findings confirm that an increasing proportion of AIDS-NHL in hemophiliacs are occurring as secondary or later AIDS diagnoses, and they are associated with prolonged duration of immunosuppression.

AIDS-Related Opportunistic Infections↗
PubMed1996

Prophylaxis and continuous infusion for hemophilia: can we afford it?

Little is known about the ideal factor regimen for hemophiliacs nor how treatment should be administered. A prospective multicenter study evaluated different dosage regimens and defined orthopedic outcomes, based on factor consumption and a comparison of prophylaxis with on-demand therapy. Prophylaxis led to better outcomes, whether joints were initially normal or previously affected. Factor use was substantially more costly if prophylaxis was used, but a major reduction could be achieved if continuous infusion were feasible.

Cost-Benefit Analysis↗
PubMed1995

A prospective study of treatment of acquired (autoimmune) factor VIII inhibitors with high-dose intravenous gammaglobulin.

A decrease in inhibitor titer has been reported in some patients with acquired factor VIII inhibitors treated with intravenous gammaglobulin (IGIV). We have conducted a prospective, multicenter study of high-dose IGIV in treatment of this disorder to determine efficacy. Nineteen patients received induction therapy with IGIV, 1,000 mg/kg x 2 consecutive days or 400 mg/kg x 5 consecutive days, followed by maintenance doses at intervals as clinically indicated. A > or = 25% reduction in inhibitor titer was observed in 8 of 16 assessable patients, six of whom met the criteria for response. A rapid decline in inhibitor titer over 3 to 4 days was observed in two patients, but in four additional responding patients--two of whom received repetitive maintenance doses of IGIV--the decline was more gradual, with final nadirs being reached several weeks to many months after treatment. The inhibitor disappeared in three patients with low-level inhibitors, less than 1 Bethesda unit (BU). Concomitant therapy with prednisone may have contributed to the response in two of the patients but was not a factor in four patients; the response rate is, therefore, estimated to be between 25% and 37.5%. These results confirm the beneficial effect of IGIV in treatment of some patients with acquired autoimmune factor VIII inhibitors.

Adult↗
PubMed1995

Idiopathic CD4+ T-lymphocytopenia in HIV seronegative men with hemophilia and sex partners of HIV seropositive men. Multicenter Hemophilia Cohort Study.

Persons with hemophilia or other HIV-1 risk factors may be more likely to have idiopathic CD4+ T-lymphocytopenia (ICL). We determined the frequency of ICL in prospectively followed cohorts of HIV-1 seronegative hemophilic men and seronegative female sex partners of HIV-1 infected hemophilic men, and examined factors potentially associated with ICL. Seven of 304 (2.3%) seronegative hemophilic men and one of 160 (0.6%) female partners met the ICL definition, but the condition resolved for two of the men and for the sole female partner. All five men with persistent ICL had lymphocytopenia (< 1,200 total lymphocytes/microliters) and < 300 total CD4+ lymphocytes/microliters; only one had a low CD4+ percentage. On the most recent measurement, 14.5% of the 304 seronegative hemophilic men had lymphocytopenia. Compared with matched hemophilic controls, men with persistent ICL more often had a history of liver disease (3/5 cases, 0/21 controls, P = 0.007) or splenomegaly (3/5 cases, 4/21 controls; P = 0.04), but not severe hemophilia, greater clotting factor concentrate exposure, high alanine aminotransferase levels, hepatitis B virus antigenemia, or detectable hepatitis C virus RNA in plasma. All five cases and 20/21 controls had antibodies to hepatitis C virus present in their serum. In this cohort of hemophilic men, ICL was related to lymphocytopenia associated with liver disease rather than selective loss of CD4+ lymphocytes.

Acquired Immunodeficiency Syndrome↗
PubMed1995

Hemophilia home treatment. Economic analysis and implications for health policy.

This analysis describes the development of technology for home self-infusion of factor VII in the treatment of hemophilia and its clinical, economic, and social consequences, and uses the case study of such home care treatment to illustrate the potentials and pitfalls of formal economic analyses of programs to treat chronically ill children. A comprehensive review of all original data on hemophilia programs, their related costs, and outcomes, conducted from 1966 through 1993, examined the economic outcomes for two hypothetical cohorts, one aged 0-4 years and the other aged 30-34 years. Including the measurement of treatment effects on the productivity of parental caregivers substantially increases the benefit-cost relationship of an intervention directed at chronically ill children. Increased economic productivity and societal return resulting from such a program for young adults exceeds those for a cohort of children, primarily due to assumptions related to discounting. However, estimation of quality-adjusted life years favors the younger age cohort, since children survive for a longer period of time and with each year survived comes a higher quality of life. Unlike simpler instances in which economic benefits can be shown to outweigh resource costs, policy decisions concerning services for chronically ill children raise an additional set of complex analytic issues. Inclusion of the benefits in productivity experienced by family caregivers provides an important added dimension to such analyses. The development of cost-benefit or cost-effectiveness analyses of these programs illustrates the importance of careful measurement of outcomes and explicit statements of underlying assumptions. Such an analysis of home care for children with hemophilia therefore demonstrates both the strengths and the limitations of this approach.

Adult↗
PubMed1995

Low risk of viral infection after administration of vapor-heated factor VII concentrate or factor IX complex in first-time recipients of blood components. International Factor Safety Study Group.

BACKGROUND: Vapor-heated human factor VII concentrate and human factor IX complex are both obtained from prothrombin complex, undergo similar methods of manufacture, and are subjected to an identical two-step vapor-heating process for virus inactivation. STUDY DESIGN AND METHODS: Intermediate-purity vapor-heated human factor VII concentrate and vapor-heated human factor IX complex were monitored for safety with regard to viral infection in the context of an International Factor Safety Study, a prospective study that follows the revised recommendations from the International Congress of Thrombosis and Hemostasis (ICTH). Because the rarity of the respective hereditary deficiencies would have made separate analyses unrealizable, the results were combined for the final analysis. Entry required that patients have no history of transfusion with any blood derivative. After the first infusion of the study drug, patients were monitored for 6 months for the development of non-A, non-B hepatitis (NANBH) and infection with hepatitis B virus (HBV) and for 15 months for infection with hepatitis C virus (HCV) and human immunodeficiency virus (HIV). An event was defined as a positive result on any test for any infection. An alanine aminotransferase level more than 2.5 times the upper limit of normal on two consecutive occasions was defined as an event for NANBH. HBV infection was monitored with tests for three different HBV markers: the HBV surface antigen, antibody against the HBV surface antigen, and antibody against HBV core antigen. HCV and HIV infection were monitored with tests for HCV and HIV antibodies. RESULTS: The 25 patients who completed the study (1 has not completed the study and 1 dropped out) received a total of 434 infusions comprising 17 different production lots of the concentrates. Twenty patients were analyzable for NANBH and 25 for HCV and HIV infection. Since most patients had been given HBV vaccination, only 4 patients were analyzable for this end point. None of the patients showed evidence of having developed an event. These data satisfy ICTH criteria when the products are considered together, but vapor-heated factor VII concentrate does not qualify alone because there were only five patients in this group. CONCLUSION: Vapor-heated factor VII concentrate and vapor-heated factor IX complex are associated with a low risk of viral infection. Preliminary results are also presented, indicating that the concentrates are safe with regard to inhibitor development.

Adult↗
PubMed1995

Comparison of the recovery and half-life of a high-purity factor IX concentrate with those of a factor IX complex concentrate. Factor IX Study Group.

BACKGROUND: Recovery and half-life estimations were carried out to compare a high-purity factor IX concentrate with an established factor IX complex concentrate. STUDY DESIGN AND METHODS: Two high-purity factor IX concentrates, which are identical except for the presence or absence of heparin (Immuninehep-plus and Immuninehep-minus), were evaluated in two independent crossover studies using an intermediate-purity factor IX complex concentrate (Bebulin) as reference drug. RESULTS: In the Immuninehep-plus crossover study (n = 27), Immuninehep-plus and Bebulin had, respectively, a recovery of 0.90 +/- 0.26 and 0.84 +/- 0.23 IU per dL per IU per kg, a compartmental half-life of 17.11 +/- 6.18 and 15.94 +/- 4.69 hours, and an effective half-life of 16.51 +/- 3.48 and 16.48 +/- 4.26 hours. In the Immuninehep-minus crossover study (n = 26), Immuninehep-minus and Bebulin had, respectively, a recovery of 0.92 +/- 0.31 and 1.02 +/- 0.36 IU per dL per IU per kg, a compartmental half-life of 17.42 +/- 5.60 and 18.77 +/- 6.27 hours, and an effective half-life of 16.39 +/- 4.44 and 16.48 +/- 4.28 hours. Equivalence tests indicated that the recovery and half-life of Immunine, with or without heparin, are equivalent to those of Bebulin. CONCLUSION: The equivalence in pharmacokinetics and bioavailability indicates that the dosage schedule for Immunine should be the same as or very similar to that of Bebulin. The high specific activity of the former, however, allows administration at lower volumes.

Adolescent↗