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D Bodensteiner

Publications and source records attributed to D Bodensteiner.

14 recordsLinked to original sources

Cryoprecipitate poor plasma does not improve early response in primary adult thrombotic thrombocytopenic purpura (TTP).

Thrombotic thrombocytopenic purpura (TTP) is a potentially fatal disease that is treated with plasma exchange and typically with replacement with fresh frozen plasma (FFP). This approach results in an approximate 50% response rate following 1 week of therapy and 80% survival. Cryoprecipitate poor plasma (CPP) is plasma from which the cryoprecipitate fraction is removed. CPP has been reported to be successful as salvage therapy in refractory TTP and has been suggested to be superior to FFP in retrospective studies. The present report compares initial therapy of TTP with exchange using replacement with either FFP or CPP in a multi-institutional prospective randomized study performed by the North American TTP Group (NATG Group) from 1993 to 1995. Initial therapy also included corticosteroids. Antiplatelet drugs or vinca alkaloids were not employed. A severity score index, response score, and individual clinical parameters (platelet count, LDH x upper limit of normal, hemoglobin level, and creatinine) were compared at their nadir or peak values, baseline, and days +6 and +13 of therapy. Thirteen patients were randomized to FFP exchange and 14 to CPP exchange. Results were equivalent for all parameters. Survival was equal with three deaths in each group. These data indicate that the efficacy of FFP and CPP are the same in the initial treatment of TTP in adults.

Adult↗

Pharmacoeconomic analysis of liposomal amphotericin B versus conventional amphotericin B in the empirical treatment of persistently febrile neutropenic patients.

PURPOSE: In a randomized, double-blind, comparative, multicenter trial, liposomal amphotericin B was equivalent to conventional amphotericin B for empirical antifungal therapy in febrile neutropenic patients, using a composite end point, but was more effective in reducing proven emergent fungal infections, infusion-related toxicities, and nephrotoxicity. The purpose of this study was to compare the pharmacoeconomics of liposomal versus conventional therapy. PATIENTS AND METHODS: Itemized hospital billing data were collected on 414 patients from 19 of the 32 centers that participated in the trial. Hospital length of stay and costs from the first dose of study medication to the time of hospital discharge were assessed. RESULTS: Hospital costs from the time of first dose to discharge were significantly higher for all patients who received liposomal amphotericin B ($48,962 v $43,183; P =.022). However, hospital costs were highly sensitive to the cost of study medication ($39,648 v $43,048 when drug costs were not included; P =.416). Using decision analysis models and sensitivity analyses to vary the cost of study medications and the risk of nephrotoxicity, the break-even points for the cost of liposomal therapy were calculated to range from $72 to $87 per 50 mg for all patients and $83 to $112 per 50 mg in allogeneic bone marrow transplant patients. CONCLUSION: The cost of liposomal amphotericin B and patient risk for developing nephrotoxicity play large roles in determining whether liposomal amphotericin B is cost-effective as first-line empirical therapy in persistently febrile neutropenic patients.

Adult↗

Liposomal amphotericin B for empirical therapy in patients with persistent fever and neutropenia. National Institute of Allergy and Infectious Diseases Mycoses Study Group.

BACKGROUND: In patients with persistent fever and neutropenia, amphotericin B is administered empirically for the early treatment and prevention of clinically occult invasive fungal infections. However, breakthrough fungal infections can develop despite treatment, and amphotericin B has substantial toxicity. METHODS: We conducted a randomized, double-blind, multicenter trial comparing liposomal amphotericin B with conventional amphotericin B as empirical antifungal therapy. RESULTS: The mean duration of therapy was 10.8 days for liposomal amphotericin B (343 patients) and 10.3 days for conventional amphotericin B (344 patients). The composite rates of successful treatment were similar (50 percent for liposomal amphotericin B and 49 percent for conventional amphotericin B) and were independent of the use of antifungal prophylaxis or colony-stimulating factors. The outcomes were similar with liposomal amphotericin B and conventional amphotericin B with respect to survival (93 percent and 90 percent, respectively), resolution of fever (58 percent and 58 percent), and discontinuation of the study drug because of toxic effects or lack of efficacy (14 percent and 19 percent). There were fewer proved breakthrough fungal infections among patients treated with liposomal amphotericin B (11 patients [3.2 percent]) than among those treated with conventional amphotericin B (27 patients [7.8 percent], P=0.009). With the liposomal preparation significantly fewer patients had infusion-related fever (17 percent vs. 44 percent), chills or rigors (18 percent vs. 54 percent), and other reactions, including hypotension, hypertension, and hypoxia. Nephrotoxic effects (defined by a serum creatinine level two times the upper limit of normal) were significantly less frequent among patients treated with liposomal amphotericin B (19 percent) than among those treated with conventional amphotericin B (34 percent, P<0.001). CONCLUSIONS: Liposomal amphotericin B is as effective as conventional amphotericin B for empirical antifungal therapy in patients with fever and neutropenia, and it is associated with fewer breakthrough fungal infections, less infusion-related toxicity, and less nephrotoxicity.

Adolescent↗

Plasminogen activator inhibitor (PAI-1) antigen levels in primary TTP and secondary TTP post-bone marrow transplantation.

Our objectives were to measure and compare plasminogen activator inhibitor levels (PAI-1) in primary adult thrombotic thrombocytopenic purpura (TTP) and in secondary TTP associated with bone marrow transplantation (BMT)-TTP. PAI-1 antigen levels were measured by an enzyme linked immunosorbent assay on platelet poor plasma samples obtained from patients at the time of diagnosis of the TTP disorder and from a group of normal volunteers. The samples were frozen at -70 degrees C. Patients with TTP secondary to bone marrow transplantation had their grade determined by percentage fragmented cells and lactate dehydrogenase levels. The primary TTP samples were contributed by investigators in the multi-institutional North American TTP Group, and the bone marrow transplant samples were obtained from an adult bone marrow transplant program. Nineteen patients with adult TTP, and 47 patients with bone marrow transplant-TTP were evaluated. Of the latter, 14 had Grade 2, 13 had Grade 3, and 20 had Grade 4 BMT-TTP. PAI-1 levels were elevated compared to control volunteers in both primary adult TTP and BMT-TTP, P < 0.001. Levels did not differ from normal in Grade 2 BMT-TTP (median = 16 ng/ml; quartiles = 9-20). PAI-1 levels were similar in primary TTP (median = 32 ng/ml; quartiles = 25-51) and Grade 3 BMT-TTP (median = 35 ng/ml; quartiles = 19-48 ng/ml), P = 0.7. However, PAI-1 levels were significantly higher in Grade 4 BMT-TTP (median = 83 ng/ml; quartiles = 60-143) than Grade 3 BMT-TTP, and primary TTP, P < 0.001. PAI-1 levels are high in primary TTP and secondary bone marrow transplant-TTP (Grades 3-4). In contrast, normal levels are seen in Grade 2 BMT-TTP, which is a self-limited disorder. Therefore, high PAI-1 levels may contribute to hypofibrinolysis in the pathogenesis of primary TTP and of moderate to severe TTP (Grades 3-4) following bone marrow transplantation.

Adult↗

Effect of age on survival in acute leukemia. 1950-1990.

BACKGROUND: Four decades ago, survival of patients with acute leukemia was brief at any age. Since 1950, survival of children has increased remarkably but has changed little for the elderly. The nature and magnitude of this age difference in survival are not understood. METHODS: Median survival was determined in 2364 Kansas residents diagnosed with acute leukemia from 1947 until 1990. Of this number, 1032 had acute lymphocytic leukemia and 1227 had acute myelocytic leukemia diagnosed between 1950 and 1989. These patients were stratified by age and decade of diagnosis. Survival was computed by life-table analysis with significance determined by log-rank, Wilcoxon, and Fisher exact tests. RESULTS: During the 40 years of the study, highly significant increases occurred in median survivals for all groups with either acute lymphocytic leukemia or acute myelocytic leukemia occurring in patients younger than 60 years of age (P < 0.0001 for some age groups), but no significant increases for those 61 to 80 years of age and those 81 years of age and older. Viewed in another dimension, for each decade from the 1960s to the present in acute lymphocytic leukemia and from the 1970s to the present in acute myelocytic leukemia, age at diagnosis was inversely correlated with median survival in a highly significant manner (P < 0.001). CONCLUSIONS: Increasing age is inversely related to survival in patients with acute leukemia. The lack of significant improvement in median survival in the last 40 years for those older than 60 years of age stands in stark contrast to the remarkable improvement for younger patients. Acute leukemia in older patients demands new and probably different therapeutic strategies.

Adolescent↗

Decreased fibrinolytic potential in patients with idiopathic avascular necrosis and transient osteoporosis of the hip.

A thrombotic etiology has been suggested as the cause of idiopathic avascular necrosis of the hip, although the underlying pathophysiological mechanisms are unknown. Transient osteoporosis of the hip has also been suggested to represent bone marrow edema that may be related to ischemia. We evaluated four patients with idiopathic avascular necrosis and one patient with transient osteoporosis of the hip for thrombotic potential placing a special emphasis on the fibrinolytic system. All five patients had identifiable abnormalities of fibrinolysis. Four patients had elevated levels of plasminogen activator inhibitor (PAI-1) and one patient had an inadequate increase in tissue plasminogen activator (tPA) post venous occlusion. Serum triglycerides were increased in three of the patients. These findings suggest an association between decreased fibrinolytic potential and the subsequent development of avascular necrosis and transient osteoporosis of the hip. These patients should have an evaluation of the fibrinolytic system with tPA and PAI-1 levels as well as a lipid profile.

Adult↗

In vitro growth modulation by L-ascorbic acid of colony-forming cells from bone marrow of patients with myelodysplastic syndromes.

In vitro colony growth was studied on bone marrow cells from 51 patients with myelodysplastic syndromes (MDS), using a cell culture method with the unique feature of daily feeding, in an effort to gain insight into the pathophysiology of MDS and to assess the clinical utility of this cell culture assay. The colony growth pattern of MDS marrow cells is remarkably similar to that of acute myeloid leukemia but quite dissimilar from that of normal marrow, in support of a common pathophysiological mechanism for these two disorders. In particular, L-ascorbic acid (LAA) enhanced colony growth in 30% and suppressed growth in 16% of cases, a finding also similar to that in acute myeloid leukemia, indicating a unique growth requirement which may be explored for therapeutic purposes. Further, these LAA effects have prognostic value, with LAA-sensitive (both LAA-enhanced and LAA-suppressed) cases displaying shorter survivals than LAA-insensitive cases (median survival of 5 months versus 18 months; P = 0.011). This prognostic value is independent of, and more powerful than, bone marrow blasts; the median survival was 18 months for less than 5% bone marrow blasts and 8 months for greater than 5% bone marrow blasts (P = 0.044). These two risk factors can be used together to identify patients with an extremely good or an extremely poor prognosis. This study establishes the clinical usefulness of the LAA effect in MDS as a prognostic factor and provides a new lead to explore in understanding differential biochemical/molecular events and, possibly, a new therapeutic approach to the management of MDS.

Adolescent↗

Flow cytometry of needle aspirates from bone and soft tissue tumors.

Fine needle aspiration specimens from 29 patients with bone and soft tissue neoplasms were analyzed by flow cytometry for DNA index and cell cycle analysis to determine whether such studies were helpful in cytologic diagnosis. Of 15 cases initially cytologically diagnosed as benign, 14 had a DNA index of 1.0, indicating a diploid population. The remaining case diagnosed as cytologically benign had a DNA index of 1.3. Further tissue from this tumor revealed an osteogenic sarcoma. Of the 14 cases initially diagnosed as malignant, 12 were hyperdiploid. Cell cycle analysis showed that malignant tumors had a higher proportion of cells in S phase (15.2% +/- 8.7%) than benign tumors (6.9% +/- 1.6%). Furthermore, high-grade malignancies had a significantly greater number of cells in S phase (18.5% +/- 1.5%) than low-grade tumors (9.9% +/- 6.3%).

Adolescent↗

Elevated platelet-surface-bound IgM in thrombotic thrombocytopenic purpura.

The level of platelet-surface-bound IgM (PSIgM) was measured during the course of therapy in two patients with thrombotic thrombocytopenic purpura (TTP). Before therapy, both had significantly elevated PSIgM. In both cases the PSIgM dropped after the institution of treatment with plasmapheresis, steroids, and dipyridamole, correlating with a complete response to therapy in one patient and a partial response in the second. This is further evidence that in some cases of TTP, an immune mechanism is present.

Adult↗

Platelet satellitism: a possible mechanism.

Markedly increased platelet surface-bound IgG was found on platelets collected in citrate and heparin in a patient with platelet satellitism. The level on platelets collected in EDTA was mildly increased. However, neutrophils from this patient collected in EDTA had markedly increased surface IgG due to attached platelets, while no surface IgG was seen in citrate or heparin neutrophils, indicating that the anticoagulant EDTA in some way modified the surface IgG or neutrophil membrane resulting in platelet attachment. This is the first case in which a possible mechanism for platelet satellitism has been identified.

Blood Platelets↗

Chronic relapsing pancreatitis with pseudocyst erosion into the portal vein and disseminated fat necrosis.

A 63-year-old man with the triad of chronic relapsing pancreatitis, disseminated fat necrosis with subcutaneous nodular panniculitis, and polyarthritis was found to have a pseudocyst which had eroded into the portal vein. These lesions were associated with markedly elevated serum pancreatic enzymes. Although recent immunological studies by others suggest a possible contribution of immune-mediated tissue injury, clinical and pathological evidence in the present case including direct tissue immunofluorescent findings do not support an immune pathogenesis of this association. The present study further substantiates the concept of physiochemical tissue injury by circulating pancreatic enzymes as the primary pathogenetic mechanism.

Amylases↗

The enzyme-linked immunosorbent assay: accurate detection of red blood cell antibodies in autoimmune hemolytic anemia.

The enzyme-linked immunosorbent assay (ELISA) was employed in the study of red blood cells from patients with autoimmune hemolytic anemia. The ELISA was more sensitive and correlated with severity of hemolysis better than the direct antiglobulin test (DAT). It was helpful in diagnosing and following the clinical course in these patients. This was particularly true in the DAT-negative group, since the ELISA can detect smaller increases in red blood cell IgG than are required for a positive DAT.

Adult↗

Sickle cell anemia.

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Anemia, Sickle Cell↗