bluuebunny/crossref_metadata_2025_split huggingface dataset:
Intracranial extramedullary hematopoiesis in β°‐thalassemia/hemoglobin E disease
Authors: Fucharoen Suthat , Tunthanavatana Chalermchai , Sonakul Dhira , Wasi Prawase 
Published: February 2007

AbstractA 27‐year‐old woman with β‐thalassemia/hemoglobin E disease presented with an intracranial mass of 10 × 9 × 1.2 cm firmly attached to the dura and the falx cerebri extending over the central part of both hemispheres, with pressure atrophy of the underlying brain. Histopathologic examination revealed that the mass consisted entirely of hematopoietic cells. We have seen 1,315 cases with β‐thalassemia/hemoglobin E, but this was the first case with intracranial extramedullary hematopoietic mass.
Polycythemia vera in identical twins
Authors: Friedland Michael L. , Wittels Edward G. , Robinson Rita J. 
Published: February 2007

AbstractThe occurrence of polycythemia vera in family members has been a rare event, with little or no documentation in the literature to suggest a common genetic defect. We report here on the first case of polycythemia vera documented in identical twins. No chromosomal abnormalities have yet been detected in these patients. The criteria for the diagnosis of polycythemia vera are reviewed, and the possible implications of our findings are discussed.
Terminal deoxynucleotidyl transferase‐positive acute myeloblastic leukemia
Authors: McGraw T. P. , Folds J. D. , Bollum F. J. , Stass S. A. 
Published: February 2007

AbstractTerminal deoxynucleotidyl transferase (TdT) is a biochemical marker for acute lymphoblastic leukemia (ALL). In studies of ALL at diagnosis, there are usually greater than 40% TdT‐positive cells by indirect immunofluorescence, whereas acute myeloblastic leukemia (AML) shows less than 1% TdT‐positive cells. Rare cases of TdT‐positive AML have been reported. We present here three AML patients with TdT in 15%, 45%, and 90% of the leukemic blasts. The diagnosis of AML was established on the basis of morphology and cytochemistry, and the cases included one patient with Auer rods. Myeloperoxidase was present respectively in 20%, 90%, and 5% of the blasts. There was no Philadelphia chromosome present in the three cases. These results may indicate the simultaneous presence of lymphoid and myeloid populations, or the presence of a blast cell with both lymphoid and myeloid markers.
Juvenile and adult types of chronic granulocytic leukemia of childhood: Growth patterns and characteristics of granulocyte‐macrophage colony forming cells
Authors: Barak Y. , Levin S. , Vogel R. , Cohen I. J. , Wallach B. , Nir E. , Zaizov R. 
Published: February 2007

AbstractPeripheral blood and bone marrow cells from three children with the juvenile (Ph1 negative) type of chronic granulocytic leukemia and from one with the adult (Ph1 positive) type were cultured in soft agar, and their specific growth patterns were evaluated. Greatly increased numbers of colonies were obtained in all cases, particularly from peripheral blood cells. By morphologic, cytochemical and ultrastructural criteria, colonies from one juvenile type and from the single adult type patients were found to be almost exclusively granulocytic, whereas in the other two juvenile type leukemia patients colonies were either granulocytic or macrophage. Moreover, both growth patterns were obtained in the same patients on different occasions. It appears that the leukemic cell populations of the juvenile and the adult forms of chronic granulocytic leukemia do not arise from different cell lines. Rather, both are the progeny of the common monocyte‐granulocyte progenitor cell, whose abnormal proliferation and differentiation along either the granulocytic or the monocytic pathway is probably directed by fluctuations in humoral and/or microenvironmental factors.
Human platelet factor 4: Preparation from outdated platelet concentrates and application in cerebral vascular disease
Authors: Levine Peter H. , Fisher Marc , Fullerton Albert L. , Duffy Celeste P. , Hoogasian James J. 
Published: February 2007

AbstractPlatelet factor 4 (PF4), the platelet antiheparin protein, was isolated from both the supernatant and the cells of recently outdated platelet concentrates. Following purification by affinity chromatography, a competitive binding radioimmunoassay was developed to detect this protein in human plasma. The normal range was determined to be 9.4 ± 4.7 ng/ml (mean ± SD for 52 healthy adults). In order to determine whether individuals with transient ischemic attack (TIA) or stroke had measurable increments of PF4 in their plasma, radioimmunoassay studies were performed on 11 patients with well‐documented TIA, 10 patients with well‐documented stroke and on 16 age‐matched controls hospitalized on a neurology service with disorders unrelated to arterial thrombosis. The 16 hospitalized controls had PF4 levels of 10.3 ± 9.1 ng/ml, a value not significantly different from the 52 normals (P > 0.50). Patients with TIA had PF4 levels of 24.6 ± 12.1 ng/ml, a value significantly higher than both the 52 normals (P < 0.001) and the 16 hospitalized control patients (P < 0.005). Patients with stroke had PF4 levels of 35.4 ± 29.2 ng/ml, a value significantly higher than both the 52 normals (P < 0.001) and the 16 hospitalized control patients (P < 0.005).Outdated platelet concentrates facilitate the development of a reproducible radioimmunoassay for PF4. The elevation of this platelet‐derived protein in the plasma of patients with stroke and TIA provides evidence for recent or ongoing platelet activation in the cerebral vascular disease population.
Thrombotic thrombocytopenic purpura: Prolonged coma with recovery of neurologic function with intensive plasma exchange
Authors: Frankel Arthur E. , Rubenstein Martin D. , Wall Robert T. 
Published: February 2007

AbstractA 45‐year‐old‐woman was presented with fever, microangiopathic hemolytic anemia, thrombocytopenia, purpura, and mental status changes. She was diagnosed as having thrombotic thrombocytopenic purpura (TPP). She was treated with daily plasma exchange and antiplatelet drugs, steroids, and vincristine. This patient had a remarkable course with 18 days of coma on full therapy followed by essentially complete recovery coincident with an increase of the plasma exchange dose to two plasma volumes processed per procedure. The patient has remained well with discontinuation of plasma exchange. We conclude that prolonged coma without evidence of major central nervous system structural lesions in TTP should be treated vigorously and continuously with plasma exchange.
Acute lymphoblastic leukemia – hand mirror variant – viral immune interrelationship as demonstrated by ultrastructural studies
Authors: Schumacher Harold R. , Thomas William J. , Strong Michael , Creegan William J. , Rowden Geoffrey , Phillips Terry M. , More Newton S. , Perlin Elliott , Stass Sanford A. 
Published: February 2007

AbstractAcute lymphoblastic leukemia – hand mirror variant – was extensively restudied in a 22‐year‐old white female who survived for 22 months without therapy. Immune complexes to the baboon endogenous virus (BaEV) were found in the bone marrow plasma of the relapse specimen in 1977, but not in the bone marrow plasma from the terminal state in 1979. Immunoperoxidasetagged IgM antibody prepared from the patient's bone marrow plasma revealed BaEV antigen on the tip of the uropod of the HMC at the time immune complexes were found in the marrow. Absence of immune complexes in the bone marrow in the terminal state suggested a failure of the patient's immune surveillance system and/or possible immune suppression by chemotherapy.
Treatment of graft‐versus‐host disease in human allogeneic marrow graft recipients: A randomized trial comparing antithymocyte globulin and corticosteroids
Authors: Doney K. C. , Weiden P. L. , Storb R. , Thomas E. D. 
Published: February 2007

AbstractThis prospective randomized trial compared the effect of antihuman thymocyte globulin (ATG) versus corticosteroids as treatment of graft‐versus‐host disease (GVHD) in recipients of HLA‐identical allogeneic bone marrow transplants. Patients undergoing transplantation as therapy for either hematologic malignancies or aplastic anemia were given methotrexate as postgrafting immunosuppression. Patients who nevertheless developed acute GVHD of moderate severity were randomized to receive either corticosteroid therapy or ATG therapy.Thirty‐seven patients were randomized: 20 patients received corticosteroids, and 17 received ATG. Both ATG and corticosteroids were in general well tolerated, although all patients receiving ATG developed fever and chills. Both treatment modalities were associated with a mild decrease in severity of GVHD after therapy. There was, however, no significant difference between treatment groups, whether assessed by improvement in specific organ involvement, improvement in the overall grade of GVHD, need for additional therapy for acute GVHD, or the proportion of patients who developed chronic GVHD. Infectious complications and survival were also not different between treatment groups. Thus, corticosteroids were as effective as ATG for the treatment of acute GVHD in recipients of HLA‐identical marrow transplants and, therefore, appear to be a reasonable choice as primary therapy for acute GVHD.
Activation and partial characterization of a human reticulocyte heme‐dependent elF‐Z α kinase
Authors: Franco R. S. , Hogg J. W. , Martelo O. J. 
Published: February 2007

AbstractAn inhibitor of globin translation initiation that is activated under heme‐deficient conditions and whose activity is associated with phosphorylation of the 38,000 dalton (α) subunit of the initiation factor eIF‐2 has been previously described in rabbit reticulocytes. We describe here an analogous enzyme in human reticulocytes. The human enzyme can be activated in intact reticulocytes under conditions of heme synthesis inhibition or by incubation of lysates in the absence of hemin. Addition of hemin to lysates prevents activation of the eIF‐2 a kinase. The partially purified kinase is associated with inhibition of globin synthesis in a rabbit cell‐free system and phosphorylates the small subunit of highly purified rabbit eIF‐2. We conclude that human reticulocytes contain a protein kinase that is analogous to the rabbit HCR and that may play a role in clinical heme deficiency states.
Evidence that platelet buoyant density, but not size, correlates with platelet age in man
Authors: Mezzano Diego , Hwang Kai‐Lun , Catalano Patricia , Aster Richard H. 
Published: February 2007

AbstractFollowing infusion of 51Cr‐labeled autologous platelets into normal subjects, high‐density (HD) and low‐density (LD) platelet cohorts were isolated by prolonged centrifugation in isosmotic arabino‐galactan (Stractan). Specific radioactivity of LD platelets declined rapidly post‐infusion (T1/2 = 1.5 days), but specific radioactivity of HD platelets remained constant or increased over a 3–4‐day period and gradually declined for 6–7 days thereafter. These differences were exaggerated when platelet cohorts enriched in LD or HD cells by slow centrifugation in high‐density albumin were labeled and transfused. Mean survival of a platelet cohort enriched with HD cells was significantly (P < 0.02) shorter (7.73 days) than that of a cohort enriched with LD cells (9.33 days). In normal subjects treated with aspirin, capacity for thromboxane synthesis was regained more rapidly (P < 0.05) in LD than in HD platelets. HD and LD platelets differed only slightly in mean volume (HD platelets = 7.57 μ3, LD platelets = 6.87 μ3, 0.05 < P < 0.01).We believe the most logical interpretation of these findings is that under normal conditions in man, newly formed platelets are less dense on the average than total platelets and become more dense as they age in the circulation. Thus, specific radioactivity of LD platelets declines rapidly as these platelets move into a more dense compartment and are replaced by newly formed, un‐labelled cells; specific radioactivity of HD platelets remains constant or increases as labelled platelets enter this compartment in numbers equal to or greater than the number leaving it at the end of their life span. The similarity in mean volumes of LD and HD platelets suggests that platelet size is unrelated to platelet age under normal conditions.
Human lgG antigranulocyte antibodies: Comparison of detection by quantitative antiglobulin consumption and by binding of 125I staph protein A
Authors: Blumfelder Theresa , Logue Gerald 
Published: February 2007

AbstractThe amount of IgG in the serum of patients with suspected immune neutropenia that binds to normal paraformaldehyde‐fixed human granulocytes was measured simultaneously by a quantitative antiglobulin consumption assay and by binding of 125I‐staphylococcal protein A (SPA). There was a significant linear relationship between the results of these two assays for the sera of 42 different patients. However, SPA binding appeared more sensitive than the quantitative antiglobulin assay for determining IgG antigranulocyte antibodies in serum. In a patient with Felty's syndrome who underwent splenectomy, the results of both assays on sequential serum samples correlated with clinical improvement. Thus, SPA binding appears to be a sensitive and reliable technique for measuring antigranulocyte antibodies, and there is a close correlation between antibody measured by antiglobulin consumption and those detected by SPA binding.
Bone marrow transplantation followed by normal pregnancy
Authors: Jacobs Peter , Dubovsky D. W. 
Published: February 2007

AbstractA full‐term normal male child was delivered using forceps fifteen months after successful allogeneic bone marrow transplantation in a woman who presented with severe acute aplastic anemia. The uneventful course of this pregnancy demonstrates that the high‐dose cylcophosphamide administration used to prepare the patient for the procedure need not irreversibly suppress ovarian or endocrine function. This observation has relevance for the increasing numbers of young patients with aplastic anemia or acute leukemia undergoing bone marrow transplantation.
Prothrombin gainesville. A dysprothrombinemia in a pair of identical twins
Authors: Smith Laura G. , Coone Leah A. H. , Kitchens Craig S. 
Published: February 2007

AbstractA pair of identical twins was evaluated for menometrorrhagia and mild post‐operative bleeding. Each had a slightly prolonged partial thromboplastin time and prothrombin time. Full hemostatic evaluation was normal, except that plasma prothrombin activity was 23% and 25% of normal with a Russell's viper venom assay. Each twin had 70% of normal plasma prothrombin antigen. Prothrombin kinetic data were obtained before and during hysterectomy. In these twins, infused prothrombin (Konyne®) was found to have a volume of distribution of approximately one and one‐half times the plasma volume and a half‐life of 34 hours. The previously reported dysprothrombins are briefly reviewed. Prothrombin Gainesville is the twelfth such disorder reported.
Evaluation of the platelet storage pool deficiency in the feline counterpart of the chediak‐higashi syndrome
Authors: Meyers K. M. , Seachord C. L. , Holmsen H. , Prieur D. J. 
Published: February 2007

AbstractCats with the Chediak‐Higashi (CH) syndrome have abnormal hemostasis with prolonged bleeding times and normal coagulation times. Platelet aggregation induced by serotonin, ADP, and collagen was impaired. Platelets from normal and CH cats were incubated with 14C‐adenine and then gel‐filtered. Gel‐filtered platelets (GFP) from CH cats contained 63% of the ATP, 38% of the ADP, 100% of the Ca2+, and 75% of the Mg25 of normal platelets. Serotonin could not be detected in CH platelets. Acid hydrolase and total platelet protein of CH platelets was similar to normal platelets. Gel‐filtered platelets were treated with thrombin to induce maximal secretion. Secretion of ATP, Ca2+, and Mg2+ was 1.9%, 12.4%, and 16% respectively of normal platelets. ADP secretion by CH platelets was not detectable. The ATP/ADP ratio in the 14C‐labeled metabolic pool of normal platelets was similar to that of total measured nucleotide pool of CH platelets. These findings suggest that in feline CH platelets, as in platelets from CH mink and cattle, there is storage pool deficiency that is virtually complete, and the virtual absence of ADP and 5HT may in part account for the abnormal hemostasis.Aggregation of platelets from CH cats was impaired, but these platelets did aggregate to arachidonate, serotonin‐induced biphasic aggregation, and the aggregation response to ADP and collagen varied according to the amount of serotonin‐induced TxB2 formed. These findings support a major role for arachidonate in platelet activation.
Extramedullary blast transformation in the central nervous system in idiopathic myelofibrosis
Authors: Barnes Harry M. , Prchal Josef T. , Scott C. W. 
Published: February 2007

AbstractIdiopathic myelofibrosis (IM) is a myeloproliferative syndrome characterized by anemia, fibrosis of the bone marrow, variable proliferation of megakaryocytes, myelocytic and erythrocytic precursors, and splenomegaly. Extramedullary hematopoiesis can develop in diverse areas. Unlike in chronic granulocytic leukemia, transformation to acute leukemia is uncommon in IM. Transformation to acute leukemia is usually accompanied by the findings of progressive increase in blasts in peripheral blood or bone marrow [1]. We have recently treated a patient with an unusual transition from IM to acute leukemia in whom leukemic transformation began in the central nervous system.
Alternative pathway of complement in multiple myeloma
Authors: Kraut Eric H. , Sagone Arthur L. 
Published: February 2007

AbstractThe alternative pathway of complement was studied in 16 newly diagnosed and 2 previously treated patients with multiple myeloma. Functional activity of this pathway was abnormal in 10 of these 18 patients (55%). These abnormalities correlated with depressed levels of alternative pathway components in 3 of 10 patients, but in the remaining 7, no etiology was demonstrated. There was an inverse correlation between activity and concentration of the monoclonal protein. This defect is an additional factor that may predispose patients with myeloma to overwhelming infection.
Differing platelet aggregating effects by two tumor cell lines: Absence of role for platelet‐derived ADP
Authors: Bastida Eva , Ordinas Antonio , Jamieson G. A. 
Published: February 2007

AbstractTwo different mechanisms of aggregation of heparinized human platelet‐rich plasma have been identified with two tumor cell lines: In neither case are these mechanisms dependent on platelet‐derived ADP. U87MG cells from a glioblastoma line of human origin caused a single irreversible wave of aggregation simultaneously with the onset of platelet secretion, and this was inhibited by heparin and hirudin but not by apyrase or phospholipase D. In contrast, Hut 20 cells from an undifferentiated tumor cell line of murine origin gave an initial reversible wave followed by a second irreversible wave, which then led to secretion. The first wave of platelet aggregation was unaffected by heparin or hirudin but was inhibited by apyrase, and the second wave was inhibited by phospholipase D. Citrate caused irreversible inhibition with either cell line, and aggregation did not occur with gel filtered platelets. These results suggest that platelet aggregation by the Hut 20 line is initially dependent on ADP released from the tumor cells, whereas aggregation induced by the U87MG line is dependent on a procoagulant activity of the tumor cell surface.
Identification and quantitation of embryonic and three types of fetal hemoglobin produced on induction of the human pluripotent leukemia cell line K‐562 with hemin
Authors: Fuhr J. E. , Bamberger E. , Lozzio C. B. , Lozzio B. B. , Felice A. E. , Altay G. , Webber B. B. , Reese A. L. , Mayson S. M. , Huisman T. H. J. 
Published: February 2007

AbstractThe hemoglobins synthesized by the pluripotent K‐562 leukemia cell line of human origin after induction with hemin have been isolated by DEAE‐cellulose chromatography and characterized by electrophoresis, high pressure liquid chromatography, and a radio‐immunological assay. Six hemoglobin zones have been observed with the following likely compositions. Zone 1: α2ϵ2, or Hb Gower‐2; zone 2: ζ 2ϵ2, or Hb Gower‐1; zone 3: ζ2γ2, or Hb Portland‐I; zone 4: Hb F, or α2γ2; zone 5: a mixture of acetylated Hb Portland‐I and Hb F; zone 6: Hb Bart's, or γ4. The embryonic Hbs (zones 1, 2, and 3) constituted 50%–75% of the total Hb present; the quantities varied from one experiment to the other. Both Hb Gower‐1 and Hb Gower‐2 were present. The γ chain was heterogeneous and contained the Gγ,AγI, and AγT types in a ratio of about 4:2:1, indicating a heterozygosity for the Ile → Thr substitution at position γ75. The methodology used can be applied for additional studies evaluating quantitative changes in Hb types due to in vitro manipulations.
Immunologic evaluation of long‐term effects of childhood all chemotherapy: Analysis of in vitro NK‐ and K‐cell activities of peripheral blood lymphocytes
Authors: McGeorge M. B. , Russell E. C. , Mohanakumar T. 
Published: February 2007

AbstractTwenty‐five children with acute lymphoblastic leukemia (ALL) were tested for natural killer (NK) and K‐cell activity in vitro using the nonsensitized myeloid/erythroid cell line K562 and the K562 sensitized with rabbit antithymocyte globulin, respectively. The patients consisted of two groups: 1) 13 patients in continuous first remission undergoing maintenance chemotherapy and 2) 12 patients in remission for at least five years in whom chemotherapy had been discontinued at least six months before this study. The first group consistently demonstrated a marked depression in their NK activity and antibody‐dependent cell‐mediated cytotoxicity (K‐cell activity), as compared with normal controls. In contrast, normal levels of cytotoxicity were found in the second group of patients off of all chemotherapy. One patient studied while on chemotherapy and on two occasions following discontinuation of maintenance medications demonstrated that while NK and K‐cell activity was depressed during therapy, normal activity returned within days when immunosuppressive therapy was stopped. Thus, present modes of chemotherapy clearly had a profound effect on the in vitro NK and K‐cell activity; however, no long‐term effect on these functions was noted in our studies.
Acute monocytic leukemia: A surface marker study
Authors: Mirchandani Ila , Tabaczka Pamela , Palutke Margarita 
Published: February 2007

AbstractThirteen cases of acute monocytic leukemia (AMoL) were studied morphologically, cytochemically, and immunologically in an attempt to determine similarities and/or differences between acute monocytic and lymphocytic leukemias. Single class surface immunoglobulins (IgG kappa) were seen in six cases. These markers are thought to represent serum immunoglobulins attached to the leukemic monocytes via the receptors for the Fc portion of immunoglobulin and are not synthesized by the cells as is the case with B lymphocytes. Receptors for complement were also noted. It appears that surface immunoglobulins and receptors for the Fc portion of the immunoglobulin and complement, which are found on several classes of normal lymphocytes and monocytes, may also be found on leukemic monocytes. Antisera to B lymphocytes, detecting HLA‐DRw antigens, also reacted with some of the leukemic monocytes.
Agranulocytosis associated with chronic oral administration of cloxacillin for suppression of staphylococcal osteomyelitis
Authors: Shah I. , Kumar K. S. , Lerner A. M. 
Published: February 2007

AbstractOral cloxacillin was used for chronic suppression of a 59‐year‐old woman with staphylococcal osteomyelitis. She received 380.5 gm of cloxacillin over 263 consecutive days. Severe agranulocytosis followed, reverting rapidly to normal with cessation of drug. The absence of signs of an allergic reaction such as rash, fever, or eosinophilia suggests that cloxacillin, like other semisynthetic penicillins, may exert a direct bone marrow toxicity.