bluuebunny/crossref_metadata_2025_split huggingface dataset:
Antinuclear antibodies in patients with chronic idiopathic autoimmune thrombocytopenia followed 2–30 years
Authors: Panzer Simon , Penner Edward , Graninger Wolfgang , Schulz Elisabeth , Smolen Josef S. 
Published: February 2007

AbstractWe determined whether serological investigations can assist to distinguish between chronic idiopathic autoimmune thrombocytopenia (cAITP) and immune‐mediated throm‐bocytopenia in patients at risk to develop systemic lupus erythematosus (SLE); 82 patients were seen in this institution for the evaluation of immune thrombocytopenia. In nine of these patients a diagnosis of SLE was made at presentation or shortly thereafter. In the remaining 73 patients with an established diagnosis of cAITP we obtained serum samples at a median disease duration of 7 years; 45 patients were investigated again 2 years later. cAITP patients suffered from their disease for up to 30 years (median 7 years, range 2–30 years) without developing clinical features of SLE. Elevated antinuclear antibodies were found in all patients with a diagnosis of SLE but also in seven patients with established cAITP. All patients who had thrombocytopenia in association with SLE had additional antibodies to anti‐nDNA and/or antibodies to soluble cellular antigens. Neither in the initial, nor in the follow‐up investigation, such antibodies were discernible in patients with cAITP. We conclude that patients with cAITP do not develop SLE and do not form antibodies to defined cellular antigens, even if suffering from the disease for many years. The ANA titer does not help to differentiate cAITP from thrombocytopenia of SLE. A differentiation, however, between the two diseases is possible by the combined screening for antinuclear antibodies, anti‐nDNA, and antibodies to defined soluble cellular antigens.
Resolution of pure red cell aplasia and lymphoma: Response to intravenous gammaglobulin and combination chemotherapy
Authors: Vukelja Svetislava J. , Krishnan Jayashree , Link Christina M. , Salvado August J. , Knight Robert D. 
Published: February 2007

AbstractPure red cell aplasia (PRCA) rarely occurs in nonthymic lymphoproliferative disorders. This report describes the clinical course and therapy of a patient with a history of Sjogren syndrome who developed well‐differentiated lymphocytic lymphoma associated with PRCA and severe hemolytic anemia. Life‐threatening hemolytic anemia combined with the presence of multiple antibodies and lack of erythroid precursors was treated successfully with a single dose of intravenous gammaglobulin. A sustained, complete remission and normalization of the bone marrow was achieved following six courses of an aggressive chemotherapy regimen. Thus, occasionally low‐grade lymphomas can produce life‐threatening complications, requiring a more aggressive therapeutic intervention than those routinely applied.
Chronic myelogenous leukemia and sweet syndrome
Authors: Cohen Philip R. , Kurzrock Razelle 
Published: February 2007

AbstractWe report a 64‐year‐old woman with chronic myelogenous leukemia of 3 years duration who developed Sweet syndrome. Improvement in her blood counts after hydroxyurea was not associated with a decrease in size of the skin lesions. However, the cutaneous lesions of Sweet syndrome quickly resolved with systemic prednisone. Sweet syndrome has only been documented in the literature for five other chronic myelogenous leukemia patients. The characteristics, treatment, and differential diagnosis of this disorder in chronic myelogenous leukemia patients are reviewed.
Simultaneous lupus anticoagulant and anticardiolipin assays and clinical detection of antiphospholipids
Authors: Rosove Michael H. , Brewer Petronella M. C. , Runge Alice , Hirji Karim 
Published: February 2007

AbstractLupus anticoagulants and/or anticardiolipin antibodies were detected in 100 patients with autoimmune disorders, thrombosis, or pregnancy loss. Significant agreement between tests for these two antipnospholipid activities was lacking. Performing both assays is thus important in maximizing the likelihood of detecting antiphospnolipids that may have clinical relevance.
Selected indices of micronutrient status in adult patients with sickle cell anemia (SCA)
Authors: Tangney C. C. , Phillips G. , Bell R. A. , Fernandes P. , Hopkins R. , Wu S.‐M. 
Published: February 2007

AbstractIn 24 adults with hemoglobin SS followed at the Duke University Comprehensive Sickle Cell Center, we have studied the following nutritional parameters: reduced ascorbic acid; dehydroascorbic acid; alpha and beta carotenes; cryptoxanthin; and alpha and gamma tocopherols in whole blood, washed red blood cells, plasma, or serum. In the same population we also examined reduced glutathione (GSH) and oxidized gluthathione (GSSG). Fifteen of these 24 patients also were interviewed for usual dietary intakes using a 28‐day dietary history. Data obtained from patients with hemoglobin SS, sickle cell anemia (SCA) were compared to those found for seven healthy normal black adults of similar age. Plasma alpha tocopherol levels were significantly lower in SCA individuals than those of the controls (P< 0.004). Alpha and gamma tocopherol levels in sickle RBCs were significantly higher than those from RBC suspensions of control subjects (P< 0.007, andP< 0.001, respectively). All serum values for carotenoids examined, specifically, beta carotene, alpha carotene, and cryptoxanthin were also markedly depressed when compared to those of healthy controls (P< 0.001,P< 0.002, andP< 0.001, respectively). No other statistically significant differences were found between the two groups for any of the remaining variables, including dietary estimates. Dietary analyses suggest that dietary intakes of SCA individuals exceeded the recommended daily allowances (RDA) of all macro‐ and micronutrients measured, and intakes of most nutrients exceeded those of black controls interviewed. These results suggest that in individuals with SCA, several micronutrients vital to maintaining reducing capacity are present in diminished quantities in plasma/serum. These anomalies exist in SCA patients even though their intake of these micronutrients are similar to those of healthy black men and women.
Splenectomy in patients with AIDS
Authors: Mathew Anna , Raviglione Mario C. , Niranjan Usha , Sabatini Maria T. , Distenfeld Ariel 
Published: February 2007

AbstractTo establish the indications for splenectomy in patients with human immunodeficiency virus (HIV) infection we retrospectively analyzed 12 patients who underwent splenectomy. Patients with HIV infection who had immune thrombocytopenic purpura (ITP) were excluded as they had no splenomegaly and a definite indication for splenectomy exists in some of these patients. All 12 patients were anemic; 6 were thrombocytopenic and 6 leukopenic. All patients had splenomegaly and all were febrile. At surgery 3 patients were found to have Mycobacterium avium intracellulare (MAI) infection; 2 had splenic abscess due to Salmonella group D; 1 each had cytomegalovirus (CMV) splenitis and localized Kaposi's sarcoma (KS) of the spleen. No definite histopathologic diagnosis could be made in five patients, all of whom had evidence of extramedullary hematopoiesis. The degree of splenic enlargement did not correlate with the outcome. Both clinical and hematologic improvements were achieved in patients with splenic abscess and in patients who had splenomegaly, anemia, and thrombocytopenia. The presence of either of these findings constitutes an indication for splenectomy. Anemia and/or leukopenia without thrombocytopenia failed to improve; the presence of MAI and active CMV infection also resulted in failure. The presence of either of these conditions may be considered a contraindication to splenectomy.
Development of anti‐idiotypic antibodies in a patient with a factor VIII autoantibody
Authors: Tiarks Cheryl , Pechet Liberto , Humphreys Robert E. 
Published: February 2007

AbstractAntibodies against coagulation factor VIII were detected in a 33‐year‐old who developed severe bleeding 2 months postpartum. After treatment with plasmapheresis and immunosuppressive agents, the inhibitor was no longer detectable, but anti‐idiotypic antibodies were detected, as demonstrated by binding in immunoradiometric assays, and by in vitro neutralization of the initial factor VIII autoantibodies. The anti‐idiotypic titer subsequently declined but was still detectable 1 year later. Three commercial immunoglobulin preparations and pooled multiparous IgG also bound to (but did not neutralize) the patient's factor VIII antibody. These studies suggest that anti‐idiotypic antibodies, arising in the face of immunosuppressive therapy, might suppress autoimmunity to factor VIII and confirm the presence of anti‐idiotypic antibodies in pooled normal immunoglobulins.
Case report: Allergic reaction with immune hemolytic anemia resulting from chlorambucil
Authors: Thompson‐Moya Luis , Martin Thomas , Heuft Hans‐Gert , Neubauer Andreas , Herrmann Richard 
Published: February 2007

AbstractIn a 73‐year‐old female with chronic lymphocytic leukemia we observed an acute illness with shivering, high fever, and hemolytic anemia following chlorambucil administration. Reexposure in a controlled clinical situation suggested an allergic drug reaction. In an in vitro assay, we were able to demonstrate chlorambucil as the causative agent of immune hemolysis.
Use of recombinant activated factor VII for treatment of a retropharyngeal hemorrhage in a hemophilic patient with a high titer inhibitor
Authors: Macik B. Gail , Hohneker John , Roberts H. R. , Griffin Anne M. 
Published: February 2007

AbstractTreatment options during an acute hemorrhage for a hemophilic patient who has an inhibitor (antibody) to factor VIII (FVIII) are limited. If the inhibitor titer is high, even massive doses of FVIII are not sufficient to neutralize the antibody. Likewise, immunoadsorption techniques or plasmapheresis cannot remove enough antibody to permit treatment with FVIII. Allergic reactions and cross‐reacting inhibitors complicate therapy with porcine FVIII. Prothrombin complex concentrates (PCC) may be effective but the mechanism is unclear. The theory that activated factor VII (FVIIa) is the active principle in PCC prompted our treatment of a patient with a recombinant FVIIa (rFVIIa) product (NOVO Industries). The patient presented with a large retropharyngeal hemorrhage and an initial inhibitor titer of 129 Bethesda units (BU). Despite Autoplex therapy (100U/kg), tracheal compression by the hematoma increased and asphyxiation was imminent. RFVIIa therapy (60 μg/kg) was substituted for Autoplex and nine doses were given without complication. The hemorrhage was controlled. By 18 hr breathing was normal and swallowing and speech were greatly improved. Clinically, the patient dramatically responded to rFVIIa. In addition, the purity, the lack of known infectious agents, and the ease of administration make rFVIIa a potentially attractive new therapy. Use of this product also promises to further our understanding of in vivo hemostasis.
Post‐transfusion purpura and isoimmune neonatal thrombocytopenia in the same family
Authors: Cobos Everardo , Gandara David R. , Geier Larry J. , Kirmani Saeeda 
Published: February 2007

AbstractPost‐transfusion purpura and isoimmune neonatal thrombocytopenia are rare and unusual syndromes leading to severe thrombocytopenia. In both disorders the PLA1 platelet antigen is involved in the pathogenesis. A 41‐year‐old woman with an obstetrical history of isoimmune neonatal thrombocytopenia in two of her children developed post‐transfusion purpura following a transfusion of packed red blood cells. Despite the apparent link in these two disorders associated with the PLA1 platelet antigen, this is the first reported case of post‐transfusion purpura and isoimmune neonatal thrombocytopenia occurring in the same family.
Carrier detection for hemophilia B: Evaluation of multiple polymorphic sites
Authors: Mariani G. , Chistolini A. , Hassan H. J. , Gallo E. , Xigen Gu , Papacchini M. , Di Paolantonio T. , Fantoni A. 
Published: February 2007

AbstractDNA analysis was performed in families with hemophilia B. Restriction fragment length polymorphisms (RFLPs) produced by endonucleases Taql, Xmnl, and Ddel were studied by two factor IX genomic probes, F9(Vlll) and F9(Xlll). Fifty‐seven subjects from ten families were investigated; of them, 31 were carriers (11 obligate and 20 potential). Of the potential carriers, ten displayed laboratory features allowing for a phenotypic diagnosis of heterozygosity. Segregation analysis of the markers was informative in 19/20 potential carriers, which belong to nine of the ten studied families. Among the potential carriers, Taql allowed the carriership assessment in 15 (78.9%), Xmnl in 15 (94.7%), and Ddel in two (10.4%). Diagnosis was not possible in one family since a homozygosity in the key individuals with all the employed enzymes (Taql, Xmnl, Ddel, + BamHI) was found. Hemophilia B syndrome in two families likely results from a new mutation. In one family, a first‐trimester prenatal diagnosis was performed. The use of RFLP analysis allowed us to improve genetic counseling as compared with the phenotypic evaluation by clotting factor assays. Indeed, evaluation of RFLP increased by 26% the carriership assessment of the potential carriers of the hemophilia B trait.
VAD‐based regimens as primary treatment for multiple myeloma
Authors: Alexanian Raymond , Barlogie Bart , Tucker Susan 
Published: February 2007

AbstractAn alternating VCAD‐VAD regimen, combining vincristine‐doxorubicin by continuous infusion with cyclophosphamide and pulse dexamethasone, or VAD alone, was given to 175 previously untreated patients with multiple myeloma. The response rate with primary VAD‐based regimens of 55% was virtually identical to the 54% in comparable patients treated previously with similar programs by using bolus vincristine‐doxorubicin. Despite responses to VAD that were more rapid in onset than any previous treatment, remission and survival times were similar. This may be due to major differences in drug sensitivity between progenitor and differentiated plasma cells. A VAD‐based regimen seems better for newly diagnosed patients when rapid control of multiple myeloma is necessary.
Orthotopic liver transplantation in a patient with combined hemophilia A and B
Authors: Delorme Michael A. , Adams Paul C. , Grant David , Ghent Cameron N. , Walker Irwin R. , Wall William J. 
Published: February 2007

AbstractA 38‐year‐old man with severe factor IX and mild factor VIII deficiencies complicated by cirrhosis secondary to chronic non‐A non‐B hepatitis underwent orthotopic liver transplantation as treatment for both the cirrhosis and his congenital coagulopathy. Intraoperative hemostasis was obtained with factor VII‐depleted prothrombin complex concentrate and fresh frozen plasma. Factor VIII and factor IX levels were assayed frequently in the perioperative period, and both returned to normal within 24 hr and remained normal postoperatively. Liver transplantation can be considered as definitive therapy for hemophilia A and/or B with transfusion‐related liver disease.
Multiple myeloma: Relationship between survival and cellular morphology
Authors: Pasqualetti Paolo , Casale Raffaele , Collacciani Antonio , Abruzzo Benjamin P. , Colantonio Domenico 
Published: February 2007

AbstractMyeloma cells in bone marrow aspirates from 121 patients with multiple myeloma at time of diagnosis were evaluated, by assigning them into morphological categories. Cox's model was used to calculate the coefficients for total myeloma cells and “plasmablasts” in respect to survival. Applying these coefficients, the Multiple Myeloma Cellular Score (MMCS) was calculated. MMCS significantly divides the patients into three stages. In fact, there are significant differences both between mean survivals (P< 0.001) and between survival curves (P< 0.00001). There is also a significant correlation (P< 0.00001) between the calculated MMCS and survival in the whole patient group.
Induced expression of A monocytoid B lymphocyte antigen phenotype on the reh cell line
Authors: Patel Bimal , Mohammad Ramzi M. , Blaustein John , Al‐Katib Ayad 
Published: February 2007

AbstractA human common acute lymphoblastic leukemia (ALL) cell line, REH, was treated in vitro with γ‐interferon (γ‐IFN) or 12‐O‐tetradecanoylphorbol 13‐acetate (TPA). Untreated (control) and treated cells were analyzed for changes in growth patterns, morphology, cytochemistry, surface phenotype, and terminal transferase (TdT) activity. TPA but not γ‐IFN induced further maturation of REH cells along the B‐cell lineage. There was a dramatic decrease in CALLA expression, loss of TdT activity, induction of Leu M5, and increase in Leu 14 expression. TPA also induced monocytoid morphological features on REH cells. Enzymatically, the induced cells strongly expressed acid phosphatase (tartrate sensitive), α‐naphthol acetate esterase (NAE), and periodic acid Schiff (PAS). We conclude that TPA induced monocytoid B‐lymphocyte features on REH cells within the B‐cell lineage, which should not be confused with monocytes/macrophage. The phenotype of cells in this stage is Leu 14 +, Leu M5 +, BL1 +, Leu 12 +, AcP +, PAS +, NAE +, CALLA‐, TdT‐, MO1‐, and MO2‐.
Precise quantitation of palgg: A new radiometric microtechnique
Authors: Schwartz Kenneth A. , Gauger John A. , Davis John M. 
Published: February 2007

AbstractWe report the development of a radiometric assay for platelet‐bound IgG that is both sensitive and quantitative. The assay utilized 96‐well millititer plates incorporating a 0.2 μm filter membrane in the bottom. A 125l‐labeled monoclonal antihuman IgG, as a secondary antibody, detected the platelet‐bound human IgG. Since 5 ± 106 platelets were used for each assay, tests for platelet‐bound IgG can be performed on persons with severe thrombocytopenia. For the detection of circulating antiplatelet alloantibodies, as little as 10 μl of platelet‐free plasma per assay is required.Antiplatelet IgG was quantitated by using anti‐PlA1 antibody that was purified with affinity and elution and DEAE chromatography. This purified antiplatelet antibody was labeled with 125l and was used to determine the binding ratio of secondary antibody to primary antibody. Under our standard conditions, this ratio was found to be stable at approximately 0.35 over the sensitivity range of the assay. The assay can detect approximately 200 molecules of human IgG per platelet (0.1 ng of secondary antibody bound per 5 ± 106 platelets). It has a linear range from ) to 7,000 molecules per platelet.Quantitation of anti‐PlA1 binding for platelets stored for up to 6 months under refrigeration showed no change in number of PlA1 binding sites. Clinical studies showed that 18 of 19 ITP patients had an increased number of IgG molecules per platelet as did patients with malignancy and drug‐induced immune thrombocytopenia. Patients who had received multiple platelet transfusions had antiplatelet antibody in their plasma. Normal amounts of PAlgG were observed in platelets and plasma of patients with nonimmune thrombocytopenia. The advantages of this method are that it is: 1) a more precise quantitation of PAlgG via direct measurement of binding ratio with PlA1 antibody; 2) performed with small amounts of platelets and plasma; 3) both sensitive and specific; and 4) reliably reproducible with both fresh and stored platelets.
Alternation of two doses of intravenous gammaglobulin in the maintenance treatment of patients with immune thrombocytopenic purpura: More is not always better
Authors: Bussel James B. , Fitzgerald‐Pedersen Julie , Feldman Charles 
Published: February 2007

AbstractIntravenous infusion of gammaglobulin (IVGG) has been shown to be effective in the maintenance treatment of children and adults with immune thrombocytopenic purpura (ITP). The objective of this treatment is not to increase the platelet count into the normal range but rather to keep the platelet count above a safe level, usually 20,000—30,000/μI. This use requires periodic infusion whenever the platelet count falls. The dose of gammaglobulin in previous studies was 1 gm/kg/Infusion in the majority of patients. This study compared doses of 0.5 and 1.0 gm/kg/Infusion by alternating the two doses in the maintenance treatment of 11 patients with ITP. There was no significant difference in the duration of response following single IVGG infusion between these two doses. This finding suggests that there is no advantage to the greater dose of IVGG and will substantially lower the cost of as well as facilitating IVGG maintenance treatment. It was not possible to determine whether a lower dose would impact adversely on the curative potential of IVGG. This effect of IVGG has never been proved and, if it exists, may be mediated by a different mechanism from the acute platelet response.
Durable complete remissions after 2′‐deoxycoformycin treatment in patients with hairy cell leukemia resistant to interferon alpha
Authors: Blick Mark , Lepe‐Zuniga Jose L. , Doig Robert , Quesada Jorge R. 
Published: February 2007

AbstractThe efficacy of interferon alpha in treatment of hairy cell leukemia is well established. Our experience with low doses of 2′‐deoxycoformycin, a competitive inhibitor of adenosine deaminase, showed that it was also effective against hairy cell leukemia in 10 of 11 patients studied whose disease was resistant to interferon alpha. Ten patients entered complete remission after five to 12 doses of 2′‐deoxycoformycin (4 mg/m2 every other week), and one patient did not respond. No relapses were observed after median followup of 18.5 months; unmaintained complete remissions lasted from more than 10 to more than 30 months. The study demonstrated that 2′‐deoxycoformycin induces a high rate of durable, unmaintained complete remissions in patients with hairy cell leukemia resistant to interferon alpha.
Treatment of platelet‐alloimmunization with cyclosporin a in a patient with aplastic anemia
Authors: Yamamoto Masahiro , Ideguchi Hiroshi , Nishimura Junji , Nawata Hajime , Inaba Shoichi , Tokunaga Kazuo , Kiyokawa Hiroyuki , Maeda Yoshiaki 
Published: February 2007

AbstractThe development of alloantibodies to platelets is a major problem in the supportive management of thrombocytopenia in patients with severe aplastic anemia. We report here a case of aplastic anemia refractory to platelet transfusion. An immunosuppressant, cyclosporin A, which was used for the therapy of aplastic anemia, modulated alloimmunization to platelets in this patient, followed by repeated platelet transfusion. The treatment reduced platelet alloantibodies detected by anti‐human immunoglobulin lymphocytotoxicity test, with change of the CD4/CD8 ratio in T lymphocytes in peripheral blood. These results suggest the usefulness of cyclosporin A for the prevention of platelet alloimmunization.