bluuebunny/crossref_metadata_2025_split huggingface dataset:
Inappropriate increase in erythropoietin titers during chemotherapy
Authors: Piroso Ettore , Erslev Allan J. , Caro Jaime 
Published: February 2007

AbstractSerial erythropoietin measurements by RIA were performed in six patients with acute leukemia treated by intensive chemotherapy. In all cases erythropoietin titers increased after the onset of treatment, although the hemoglobin concentration remained at stable values. Subsequently the erythropoietin titers gradually returned to baseline levels. In same patients this reduction occurred at the end of chemotherapy, in others coincident with infections and antibiotic therapy. In four patients this decrease occurred at the time of bone marrow recovery.The explanation for this inappropriate increase in erythropoietin titers is not clear but may be related to a direct or indirect effect of a suppressed marrow on sites of erythropoietin production or catabolism.
Severe hyponatremia after repeated intravenous administration of desmopressin
Authors: Weinstein Ralph E. , Bona Robert D. , Altman Arnold J. , Quinn John J. , Weisman Steven J. , Bartolomeo Ann , Rickles Frederick R. 
Published: February 2007

AbstractDesmopressin (DDAVP) has recently been found to improve hemostasis in patients with congenital or acquired disorders of coagulation and to reduce operative blood loss in patients with normal hemostasis undergoing certain surgical procedures. Despite its potent antidiuretic effect, severe hyponatremia after the intravenous administration of DDAVP is felt to be rare. We report four cases of severe hyponatremia with serious clinical sequelae occurring in patients with underlying coagulopathies who were treated prophylactically with DDAVP to improve hemostasis prior to surgical procedures. Each patient received multiple (3–22) doses of DDAVP and was given intravenous hydration with hypotonic solutions before developing clinical signs and laboratory evidence of hyponatremia. We believe that the risk of significant hypoantremia after treatment with intravenous DDAVP may be higher than is generally appreciated and that patients undergoing surgical procedures, who often receive multiple doses of DDAVP and intravenous hydration, are at particular risk for this complication. Hypotonic intravenous solutions should be avoided and serum sodium levels should be monitored frequently in those patients receiving multiple doses of DDAVP.
Two‐year evaluation of clinical and laboratory variables of immune function in 117 hemophiliacs seropositive or seronegative for HIV‐1
Authors: Teitel Jerome M. , Freedman John J. , Garvey M. Bernadette , Kardish Margaret 
Published: February 2007

AbstractFifty‐nine HIV‐1 antibody positive and 58 antibody negative hemophiliacs were evaluated over a 2 year study period to gain insight into the natural history and prognosis of HIV‐1 disease in members of this risk group. Mean CD4 (Leu 3+) cell counts calculated at 6 month intervals decreased gradually in seropositive patients (from 403 to 311/μl), whereas CD8 (Leu 2+) counts remained stable but above the normal range. CD4 cell counts correlated closely with advancing CDC clinical stage; CD8 numbers showed no such association, but were markedly lower in the six patients with overt AIDS. Serum P24 antigenemia was associated with low CD4 cell counts and with advanced clinical stage (58% of antigenemic and 14% of non‐antigenemic seropositive patients were in stage IV). In addition to CD4 cell counts, significant reductions in Leu 11+ natural killer cell (NK) subsets and in Leu 3+8‐ cells occurred in seropositive patients over the study period; Leu 2 + DR+ cells increased significantly. When expressed as a percentage of lymphocytes, the reduction in Leu 19+ NK cells was also significant, as were the increases in Leu 4 + DR+ cells and Leu 12+8+ B cells. In summary, declining CD4 cell numbers and percentages are valuable markers of progressive HIV‐1 disease in hemophiliacs, but may not always accurately reflect the degree of disease activity. Progressive changes in additional variables such as serum P24 antigen, and numbers and percentages of NK cell subsets and (as AIDS supervenes) CD8 cell numbers, may allow more precise monitoring of HIV‐1 disease. This will, in turn, facilitate the design of optimal individualized strategies for therapeutic intervention.
Natural killer lymphocyte blast crisis of chronic myelogenous leukemia
Authors: Warzynski M. J. , White C. , Golightly M. G. , Steingart R. , Otto R. N. , Podgurski A. E. , Johnson M. L. , Glynn P. , Smith D. E. 
Published: February 2007

AbstractWe describe for the first time a case report documenting a chronic myelogenous leukemia (CML) patient who developed a blast crisis of natural killer (NK) lymphocytes. Many of the blasts exhibited large granular lymphocytic (LGL) morphology. Single parameter immunophenotyping results determined that the granulated as well as the agranulated blast cells were NK lymphocytes (CD45, NKH1, CD2, LEU 17, and CD16 positive; CD3, CD8, and LEU 7 negative). Dual parameter flow cytometric testing also determined that some of the blasts expressed the CD11b and CD11c markers as reported for some types of NK lymphocytes. Approximately 10% of the cells were in the S phase of the cell cycle as determined by a modified Vindelov DNA content analysis test and may theoretically reflect some of those cells expressing CD11b and CD11c. The cells did not express in vitro NK lymphocyte functional activity against a K562 target and therefore similar to other reported cases of presumably immature NK lymphocytic leukemias. The NK lymphocyte blast crisis was successfully treated with vincristine and prednisone. The patient's disease eventually relapsed and transformed to a progenitor stem cell before she died (CD45, 13, CD38, and CD34 positive). The flow cytometric immunophenotyping results contributed significantly as an important adjunct in determining the appropriate diagnosis, helping to select the type of therapy, and monitoring the patient with this unusual type of blast crisis.
Hemoglobin Villejuif [β 123(H1) Thr→lle]: A new variant found in coincidence with polycythemia vera
Authors: Wajcman H. , Mrad A. , Blouquit Y. , Parmentier C. , Riou J. , Galacteros F. 
Published: February 2007

AbstractA new abnormal hemoglobin, Hb Villejuif [β 123 (H1) Thr→IIe] has been discovered during the exploration of a polycythemia in a 87‐year‐old patient of French origin. The isoelectric focusing of the lysate revealed the presence of a variant hemoglobin with an isoelectric point very close to that of HbA. The oxygen binding properties of the patient's red blood cells being normal, it was clear that the polycythemia was not a consequence of the presence of this hemoglobin. In fact, the red blood cell morphology and the involvement of the other blood cell lines, demonstrating excessive hemopoiesis, led to the diagnosis of polycythemia vera.
Hereditary sideroblastic anemia with associated platelet abnormalities
Authors: Soslau Gerald , Brodsky Isadora 
Published: February 2007

AbstractA 62 year old male (R.H.) presented with a mild anemia (Hb 11–12 gm%) and a history of multiple hemorrhagic episodes. The marrow had 40–50% sideroblasts. Marrow chromosomes were normal. His wife was hematologically normal, while one daughter, age 30 years, had a sideroblastic anemia (Hb 11–12 gm%) with 40–50% sideroblasts in the marrow. Her anemia was first noted at age 15 years. Administration of vitamin B6 did not correct the anemia in either the father or daughter. Platelet abnormalities inherited jointly with this disorder are described for the first time. Both R.H. and his daughter had prolonged bleeding times, with normal PTT, PT times, fVIII:C, fVIII:Ag levels, and vWF multimers, which may rule out a von Willebrand's disease. They have normal platelet numbers but abnormally low platelet adhesiveness and greatly depressed ADP, collagen, and epinephrine responsiveness. Response to ristocetin was in the low normal range, and aggregation with thrombin was normal. While desmopressin completely normalized R.H.'s bleeding time, none of these platelet parameters were improved. No differences in the SDS PAGE protein patterns of RH platelets could be detected in comparison to normal samples. His platelets took up and released serotonin (5HT) normally, and electron micrographs defined no morphological abnormalities. However, no ATP was released from platelets activated with collagen, and when followed by thrombin about fourfold greater ATP was released by control platelets as compared to RH platelets. The dense granule fraction derived from RH platelets contained about 20% the level of ATP, 40% the level of ADP, and 50% the level of 5HT detected in a normal sample. The results indicate that the bleeding disorder is related to a non‐classical heritable storage pool defect. The connection between the inherited sideroblastic anemia and platelet defects is obscure.
Nonsecretory multiple myeloma in a 26‐year‐old man with acquired immunodeficiency syndrome, presenting with multiple extramedullary plasmacytomas and osteolytic bone disease
Authors: Karnad Anand B. , Martin Alvin W. , Koh Howard K. , Brauer Mark J. , Novich Marilyn , Wright Jonathan 
Published: February 2007

AbstractHigh grade B‐cell lymphoma and leukemia have been well described in patients with the acquired immunodeficiency syndrome (AIDS). Malignant transformation of more differentiated lymphoid cells has not been well described in these patients. We report a 26‐year‐old man with AIDS‐associated multiple myeloma, who had a highly unusual presentation and clinical course. A review of the literature indicates that monoclonal gammopathy in patients seropositive for the human immunodeficiency virus (HIV) is common. Multiple myeloma and extramedullary plasmacytomas, diseases that are extraordinarily rare in young persons, are now being reported in patients with AIDS and should be added to the list of neoplastic diseases now associated with HIV infection.
Procollagen type III aminoterminal peptide in serum in idiopathic myelofibrosis and allied conditions: Relation to disease activity and effect of chemotherapy
Authors: Hasselbalch H. , Junker P. , Hørslev‐Petersen K. , Lisse I. , Bentsen K. D. 
Published: February 2007

AbstractThe serum concentration of the aminoterminal propeptide of type III procollagen (PIIINP) was measured serially in patients with idiopathic myelofibrosis and other myeloproliferative syndromes. Two different assays were applied, the RIA‐gnost assay (Hoechst, Frankfurt, FRG) and a new equilibrium RIA for the N‐terminal propeptide of human type III procollagen (Farmos Diagnostica, Oulu, Finland).A positive correlation was found between the results obtained by the two RIA's (rho = 0.90. P < 0.001). The highest propeptide levels were recorded in patients with idiopathic myelofibrosis, particularly in those with active disease. Elevated serum PIIINP levels decreased during treatment with various cytotoxic drugs, including intensive chemotherapy. By contrast, serum PIIINP was unchanged or increased in patients undergoing interferon alpha‐2b therapy.Gel filtration of sera on Sephacryl S‐300 column (Pharmacia, Sweden) showed that smaller PIIINP related peptides dominated in healthy subjects and in osteomyelosclerosis with stable disease. Conversely, the relative proportion of intact propeptide increased in accelerating disease stages and acute myelofibrosis.In conclusion, the present study implicates serum PIIINP as a useful indicator of disease activity in idiopathic myelofibrosis. The propeptide also appears to be a sensitive sero‐marker of chemotherapy effect on fibrogenesis related to clonal myeloproliferation. Finally, the propeptide is suggested as an early predictor of relapse during cytotoxic therapy.
Intranuclear inclusions in myeloma cells in a case of nonsecretory multiple myeloma
Authors: Feng Chi‐Shun 
Published: February 2007

AbstractInclusions in the nucleus, compared with those in the cytoplasm, are rare in myeloma cells but have been reported in all electrophoretic varieties of multiple myeloma except the nonsecretory type. In this unusual case, a 54 year old Chinese woman had a pathological fracture of the left femur, and biopsy of the fracture site revealed a round cell tumor compatible with plasmacytoma. A bone marrow aspirate revealed 50% plasma cells, many of which contained intranuclear inclusions. Protein electrophoresis was normal with no paraprotein, and urine was free from Bence‐Jones protein. Under electron microscopy, the plasma cells showed electron‐dense spherules not circumscribed by a membrane. The absence of a membrane was unusual, because according to all reported cases, these intranuclear inclusions were invariably membrane‐bound. The association of nonsecretion of paraprotein in myeloma, which is rare, and the absence of a membrane enclosing the intranuclear inclusions, which is heretofore unreported, is probably not coincidental but causally related in that paraprotein produced in the nucleus of myeloma cells (stored in the form of intranuclear inclusions) fails to be detected in serum and urine because of noninteraction between these inclusions and the membranes of the nucleus and endoplasmic reticulum.
Acute pancreatitis complicating therapy of Hodgkin disease: A case report
Authors: Batson Oliver A. , Branda Richard F. 
Published: February 2007

AbstractAcute pancreatitis developed in a young woman with disseminated Hodgkin disease coincident with cytotoxic treatment. Despite concerns about possible drug‐related causes, chemotherapy was continued without further gastrointestinal consequences. This case indicates that pancreatitis need not necessarily alter therapeutic plans for Hodgkin disease. Causes of pancreatitis in the setting of malignancy are reviewed.
Deficient elevation of the cytoplasmic calcium ion concentration by epinephrine in epinephrine‐insensitive platelets of patients with myeloproliferative disorders
Authors: Ushikubi Fumitaka , Okuma Minoru , Ishibashi Takafumi , Narumlya Shuh , Uchino Haruto 
Published: February 2007

AbstractElevation of the cytoplasmic Ca2+ concentration ([Ca2 +]i) by epinephrine and epinephrine‐induced inhibition of prostaglandin E1 (PGE1)‐stimulated cyclic adenosine monophosphate (CAMP) accumulation were assessed in platelets from three groups of subjects: normal controls (NS, n = 11) and patients with myeloproliferative disorders whose platelets were either sensitive (ES, n = 9) or specifically insensitive (El, n = 7) to the aggregatory effect of epinephrine. The inhibition by epinephrine of CAMP accumulation in the platelets exposed to 500 nM PGE, was not significantly different between the three groups. Therefore, despite the detective aggregation response to epinephrine, platelets from the El group seemed to retain normal response, which was attained through cα2‐adrenergic receptors, guanine nucleotide binding regulatory protein, and the adenylate cyclase system. However, in aequorin‐loaded, washed platelets, the epinephrine‐stimu‐lated rise in [Ca2+]i showed significant decrease in the El group compared with the other groups (P < 0.01). Thus the mechanism for the impaired aggregation response to epinephrine in platelets from the El group could include the defect that exists in the pathway from receptor binding of epinephrine to the aggregation response through [Ca2+]i elevation.
Prevalence of a shortened prostacyclin half‐life in plasma in healthy adults: Results of a screening investigation
Authors: Sinzinger H. , Rauscha F. , Fitscha P. , Granegger Susanne 
Published: February 2007

AbstractOut of a total of 1,981 adults (956 males, 1,025 females; aged 16‐71 yr) undergoing a screening investigation for elevated cholesterol only 3 healthy participants with pathologically enhanced prostaglandin 12 (PG12) degradation in plasma in vitro were discovered. The PG12 half‐life in these three screenings was shorter than 60 sec and persisted during several follow‐up checks. In one patient a familial defect was discovered. Diseases known to be associated with this disturbance in PG12 metabolism have been excluded. The mean half‐life of PG12 in 1,978 people amounted to 10.71 ± 1.93 min. It still needs to be clarified whether this rare disturbance in PG12 metabolism is causally related to, or only associated with, thromboembolic events. A possible predisposition should be elucidated by regular monitoring of affected persons.
Stimulation of myelopoiesis in chronic lymphocytic leukemia and in other lymphoproliferative disorders by recombinant human granulocyte‐macrophage colony‐stimulating factor
Authors: Vadhan‐Raj Saroj , Velasquez William S. , Butler James J. , Lemaistre Anne , Lepe‐Zuniga Jose , Keating Michael J. , Koller Charles A. , Gutterman Jordan U. 
Published: February 2007

AbstractIn an attempt to stimulate granulopoiesis, we administered recombinant human granulocyte‐macrophage colony‐stimulating factor (GM‐CSF) to 11 patients with lymphoprolif‐erative disorders. Ten patients had neutropenia, six of whom had severe neutropenia (<500 neutrophils), including two with agranulocytosis. GM‐CSF (60‐250 μg/m2/day) was administered by continuous intravenous infusion daily for 14 days at 2‐week intervals. Significant increases in white blood cell counts (1.3‐ to 11.7‐fold) and neutrophils (1.7‐ to more than 29‐fold) were seen in 10 of 11 patients, including one patient with agranulocytosis. Eosinophils (3.9‐ to > 65‐fold) and monocytes (1.3‐ to >5‐fold) increased as well. In contrast, no significant increases were seen in total lymphocytes or in different phenotypic subsets of lymphocytes during treatment. The overall proportion of myeloid and lymphoid elements in bone marrow remained stable. These results indicate that GM‐CSF is effective in stimulating myelopoiesis in neutropenic states associated with lymphopro‐liferative disorders. Further studies will be necessary to determine whether the correction of neutropenia ultimately translates into clinical benefit.
The transferrin receptor system is not involved in the pathogenesis of hematological disorders with 3q inversion
Authors: Abe Yasunobu , Muta Koichiro , Yufu Yuji , Takahira Hiroyuki , Nishimura Junji , Nawata Hajime 
Published: February 2007

AbstractTo clarify the idea that an alteration of the transferrin receptor (TF‐R) gene, localized to 3q26, may be of pathogenetic significance in hematological disorders with 3q anomaly, we studied the TF‐R systems of erythroblasts from both functional and genetic aspects. The patient described here had refractory anemia with an excess of blasts (RAEB), with paracentric inversion, inv(3)(q21q26). The patient had the characteristic findings of mi‐ cromegakaryocytosis and thrombocytosis, with giant platelets. There was no functional abnormality of TF‐R as far as number of binding sites, affinity, molecular weight, or recycling kinetics were concerned. Furthermore, we could not recognize any rearrangement of the TF‐R gene with Southern blot analysis. These data suggest that TF‐R is not involved in the pathogenesis of leukemogenesis and thrombocytosis of the disease.
Reduced serum haptoglobin values in hemophiliacs receiving monoclonally purified factor viii concentrates
Authors: Buchanan George R. , Holtkamp Christine A. , Johnson Andrea 
Published: February 2007

AbstractHemophiliacs often have mild anemia, and hemolysis has been suggested as the likely mechanism on the basis of the reduced serum haptoglobin values frequently observed in these patients. It has been suggested that hypohaptoglobinemia results from isohemagglutinins or other contaminating proteins in the infused factor concentrates. The advent and increased utilization of Factor VIII concentrates that are highly purified by use of monoclonal antibodies have provided the opportunity to study whether proteins other than Factor VIII contained in the concentrate induce hemolysis. Of 49 consecutively studied Factor VIII‐deficient hemophiliacs, 19 (39%) had a reduced serum haptoglobin level (< 27 mg/dl). In particular, 16 of 35 (46%) of patients receiving only monoclonally purified Factor VIII products (Monoclate or Hemofil‐M) had a reduced serum haptoglobin value. Haptoglobin measurements were variable on repeat measurement in 8 patients. Haptoglobin levels did not correlate with type or severity of hemophilia, hemoglobin value, or alterations in liver function. Low serum haptoglobin values were also observed in children with leukemia, without apparent hemolysis, who had extensive cutaneous hemorrhage associated with thrombocytopenia. We propose that reduced serum haptoglobin values in hemophiliacs do not result from immune‐mediated hemolysis due to contaminating proteins in the concentrate. Moreover, hypohaptoglobinemia may not be due to hemolysis at all but may instead result from dissolution of hematomas and other foci of internal hemorrhage.
Successful treatment of hairy cell leukemia with β‐ser interferon
Authors: Wiernik Peter H. , Schwartz Bradford , Dutcher Janice P. , Turman Nancy , Adinolfi Crestina 
Published: February 2007

AbstractTwelve patients with hairy cell leukemia underwent treatment with beta‐ser interferon, a type I interferon with potent antiproliferative activity. Ten of the 12 patients had had prior therapy and the median time from diagnosis to initiation of treatment was 8.5 months (range 1 month to 18 years). Bone marrow involvement ranged from 90‐100% hairy cells. Beta‐ser interferon was administered as an intravenous injection of 90 million units per dose, three times weekly. Dose reductions were by dose and by frequency in individual patients. Ten of 12 patients (83%) responded, including 7 complete (CR) and 3 partial (PR) responses. The median time to at least PR was 6 months and the median time to CR was 12 months. The median duration of overall response (PR + CR or PR) is 31+ months (range 26+‐40+ months). Of the 6 patients who underwent dose reductions due to toxicity or poor tolerance, 4 CR patients reverted to PR, and 2 PR patients have increased the number of hairy cells in the marrow, while maintaining normal peripheral blood counts. One each of these has regained CR and PR status after an increase in dose.
In vivo aging of red cell enzymes: Study of biotinylated red blood cells in rabbits
Authors: Zimran A. , Forman L. , Suzuki T. , Dale G. L. , Beutler E. 
Published: February 2007

AbstractIt is generally recognized that the activities of some of the red cell enzymes decline as the cell ages. However, there is still a controversy regarding the rate at which this aging occurs. In the present study we applied newly developed technology for the specific isolation of maturing reticulocytes/erythrocytes for a more comprehensive study of in vivo aging of red cell enzymes in rabbits. Anemia was induced by repeated phlebotomy, and reticulocyte‐rich erythrocytes were labeled with N‐hydroxy succinimido‐biotin and then transfused into a normal rabbit. These biotinylated cells were isolated at various time points by their affinity for an avidin support, and the enzymatic activity of 19 red cell enzymes was measured. We observed a biphasic pattern of decay for the activity of six age‐dependent enzymes—aldolase, glutamate‐oxaloacetate transaminase, glucose 6‐phosphate dehydrogenase, hexokinase, pyrimidine 5′‐nucleotidase, and pyruvate kinase.