bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nonclonal hemopoietic progenitors in a G6PD heterozygote with chronic myelogenous leukemia revealed after long‐term marrow culture
Authors: Hogge Donna E. , Coulombel Laure , Kalousek Dagmar K. , Eaves Connie J. , Eaves Allen C. 
Published: February 2007

AbstractWhen marrow cells from Philadelphia chromosome (Ph) positive chronic myelogenous leukemia (CML) patients are placed into long‐term culture, Ph‐positive progenitor numbers typically decline rapidly. Karyotypically normal cells derived from persisting, but previously undetectable, precursors can then frequently be demonstrated. To investigate the origin of these cytogenetically normal progenitors, longterm cultures were established with marrow from a girl with CML who was also heterozygous for the glucose‐6‐phosphate dehydrogenase (G6PD) enzyme variants A and B as shown by studies of her skin fibroblasts. Erythroid and granulocytic colonies generated in methylcellulose assays of fresh marrow from this patient were all Phpositive and expressed the enzyme G6PD‐A as found in the mature blood cells. In assays of 4‐ and 6‐ week‐old long‐term cultures, numbers of progenitors present were low and most were Ph‐positive, but some (2/11) were Ph‐negative. G6PD analysis showed that a similar proportion was not part of the neoplastic clone (4/30 were G6PD‐B and 26/30 were G6PD‐A). Thus, long‐term marrow culture in conjunction with G6PD analysis was able to reveal the presence of a nonclonal hemopoietic progenitor population in a CML patient where such cells would not otherwise have been detected. This approach may offer a novel strategy for the demonstration and selection of populations of normal hemopoietic progenitors in other patients where the malignant cells cannot be distinguished by chromosome analysis.
Severe thrombocytopenia in iron deficiency anemia
Authors: Berger Mark , Brass Lawrence F. 
Published: February 2007

AbstractMild‐to‐moderate thrombocytopenia has been reported as an occasional finding in patients with iron deficiency. The present case describes a multiparous woman who presented with increased menorrhagia, severe anemia (3.0 g/dl) and thrombocytopenia (9,000 platelets/mm3). Her bone marrow examination showed iron deficiency, megakaryocytopenia, and erythroid hypoplasia but no other evidence of a primary marrow disorder. Her symptoms, the thrombocytopenia and the megakaryocytopenia, resolved with iron replacement. This case demonstrates the profound degree to which thrombopoiesis can be affected in iron deficiency.
Screening for sickle cell trait: The veterans administration national sickle cell program
Authors: Meyer Leo M. , Adams Junius G. , Steinberg Martin H. , Miller Inez E. , Stokes Norma 
Published: February 2007

AbstractResults of the Veterans Administration Sickle Cell Program for a period of 10 years are presented. We screened 370,250 patients; 404,341 attended educational sessions, and 38,347 had individual counseling sessions. Sickle cell trait was present in 6.4% of patients, and HbC trait was present in 1.8%. The clinically significant disorders HbSC disease, sickle cell anemia, and sickle β thalassemia were present in 0.41% of individuals screened. A large number of uncommon variants were detected. The program enhanced the awareness of and the approach to evaluation of hemoglobinopathies.
Spontaneous hemoperitoneum in two patients with myeloproliferative disorders
Authors: Storniolo Anna Maria , Fitzgerald Denis B. , Rowe Jacob M. , Olson John P. 
Published: February 2007

AbstractSpontaneous hemoperitoneum developed in two patients with hitherto undiagnosed myeloproliferative disorders. No other known etiology could be attributed to the two patients described. The report of these two patients suggests that the diatnosis of a myeloproliferative disorder should be considered in the evaluation of patients with spontaneous hemoperitoneum.
Decreased numbers of chemotactic factor receptors in chronic neutropenia with defective chemotaxis: Spontaneous recovery from the neutrophil abnormalities during early childhood
Authors: Yasui Kozo , Yamazaki Munehiro , Miyagawa Yukiaki , Komiyama Atsushi , Akabane Taro 
Published: February 2007

AbstractChildhood chronic neutropenia with decreased numbers of chemotactic factor receptors as well as defective chemotaxis was first demonstrated in an 8‐month‐old girl. Chemotactic factor receptors on neutrophils were assayed using tritiated N‐formyl‐methionyl‐leucyl‐phenylalanine (3H‐FMLP). The patient's neutrophils had decreased numbers of the receptors: numbers of the receptors were 20,000 (< 3 SD) as compared with those of control cells of 52,000 ± 6,000 (mean ± SD) (n = 10). The neutropenia disappeared spontaneously by 28 months of age parallel with the improvement of chemotaxis and increase in numbers of chemotactic factor receptors. These results demonstrate a transient decrease of neutrophil chemotactic factor receptors as one of the pathophysiological bases of a transient defect of neutrophil chemotaxis in this disorder.
Refractory aplastic anemia: Concomitant therapy with antithymocyte globulin and high‐dose corticosteroids
Authors: Pulver Kerry P. , Flaum Morris A. 
Published: February 2007

AbstractBone marrow transplantation is possible for only a minority of patients with severe aplastic anemia. There has been successful treatment in some patients with immunosuppressive agents: high‐dose 6‐methylprednisolone, antilymphocyte globulin, and antithymocyte globulin. We report the successful treatment oftwo patients with severe aplastic anemia with the simultaneous adminstration of antithymocyte globulin and high‐dose 6‐methylprednisolone after failure with antithymocyte globulin and low‐dose corticosteroids.
Hermansky‐pudlak platelets: Further studies on release reaction and protein phosphorylations
Authors: Rendu Francine , Maclouf Jacques , Launay Jean‐Marie , Boinot Catherine , Levy‐Toledano Sylviane , Tanzer Joseph , Caen Jacques 
Published: February 2007

AbstractPlatelets from a patient with the Hermansky‐Pudlak syndrome were studied. These platelets had decreased amounts of serotonin and adenine nucleotides, and a decreased number of mepacrine‐labeled dense bodies. β‐Thromboglobulin and acid hydrolases contained in alpha‐granules and lysosomes respectively were present in normal amount. Platelets in platelet‐rich plasma did not respond to collagen, but arachidonic acid and ionophore A 23187 induced normal aggregation and normal thromboxane (TX) synthesis. Alpha‐granule release was found impaired and remained subnormal even with high doses of inducers. In response to thrombin aggregation, release and TX synthesis of isolated metrizamide gradient platelets were found at lower than normal levels. Phosphorylation of P20 and P43 proteins was normal. Only a combination of ADP plus thrombin could restore a normal aggregation, with normal alpha‐granule and lysosome release and normal TX synthesis. These results indicated that in the absence of dense bodies: 1) the release of other granules is impaired; 2) the TX synthesis is delayed except when induced by arachidonic acid and A 23187 ionophore; 3) the absence of dense bodies could be compensated for by the addition of ADP which restores the impaired release reaction and TX formation; and 4) P20 and P43 polypeptides were phosphorylated as rapidly as those in normal platelets.
Severe acute thrombocytopenia following infusion of plasma containing anti P1A1
Authors: Nijjar Tirath S. , Bonacosa Ines A. , Israels Lyonel G. 
Published: February 2007

AbstractAn 18‐year‐old girl with a preoperative platelet count of 216,000/cmm received 3 units of stored plasma at the time of surgery. Within 6 hours her platelet count had fallen to 5,000/mm3 and hemorrhagic manifestations appeared. One of the plasma donors was found to be P1A1 negative with an anti P1A1 antibody. The recipient, who was P1A1 positive, recovered normal platelet counts by day 8 post plasma infusion.
Concomitant occurrence of disseminated intravascular coagulation and factor VIII inhibitor in a patient with prostatic cancer
Authors: Singal Usha , Mammen Eberhard F. 
Published: February 2007

AbstractAn 86‐year‐old man, diagnosed as having carcinoma of the prostate, stage D, was admitted to the hospital. Soon after admission, he developed bleeding from various sites, including intravenous puncture sites and gastrointestinal and urinary tracts. A clinical diagnosis of disseminated intravascular coagulation (DIC) was made, which was corraborated by laboratory data. A factor VIII inhibitor of 12.5 Bethesda units was also identified in the patient's plasma. Concomitant occurrence of disseminated intravascular coagulation and an acquired factor VIII inhibitor has not been reported previously.
Formation and disappearance of pocked erythrocytes: Studies in human subjects and laboratory animals
Authors: Buchanan George R. , Holtkamp Christine A. , Horton Jureta A. 
Published: February 2007

AbstractThe pocked or “pitted” RBC count is being increasingly utilized as a test of splenic function. Since little is known about patterns of formation and removal of the characteristic organelles in the pocked RBC, we performed serial pocked RBC counts following splenectomy in six patients and in three animal species (dogs, rats, and rabbits). In the patients, pocked RBC counts began to rise within 1 week following splenectomy and reached a plateau (40‐60%) by 60‐100 days. Similar results were obtained following splenectomy of dogs, except that the plateau value was less. Pocked RBCs in splenectomized rats rose initially, but after the sixth week there was a progressive decline in their numbers; splenosis or accessory spleens were not visualized at autopsy. Rabbits had only a slight and inconsistent rise in pocked RBCs after splenectomy. When the rate of removal of pocked RBCs from the circulation was determined by transfusion of blood from splenectomized dogs in eusplenic animals, the pocked RBC count rapidly decreased within 3 to 6 hours. Pocked RBCs did not disappear when crosstransfused into a splenectomized recipient animal. Prior treatment of the recipient dog with either corticosteroids or vincristine did not affect the pattern of removal of pocked RBCs. We conclude that pocked RBCs rise slowly following splenectomy, disappear rapidly from the circulation in the presence of a normal spleen, and vary in pattern of rise and peak levels following splenectomy of different laboratory animals.
Philadelphia chromosome‐positive acute leukemia: Morphologic and clinical correlations
Authors: Helenglass Gilla , Testa Joseph R. , Schiffer Charles A. 
Published: February 2007

AbstractTwenty‐eight adult patients with Philadelphia chromosome positive (Ph+) acute leukemia were studied to determine if additional chromosomal changes were related to specific morphologic and clinical features. Twenty patients had chronic myeloid leukemia in blast crisis (CML‐BC), three had Ph+ de novo acute nonlymphocytic leukemia (ANLL), and five had de novo acute lymphoblastic leukemia (ALL). Chromosomal abnormalities in addition to a single Ph were noted in 90% of patients with CML‐BC and included a second Ph (five patients), +8 or duplication of part of 8q (five patients), dicentric isochromosome 17 (two patients), and +19 (two patients). Octaploidy with 4 Ph was seen in one patient with megakaryoblastic transformation. One of two patients with a progranulocytic blast crisis had a t(15;17) abnormality. Hypodiploidy was noted in 4 of 20 patients with CML‐BC. Each of the four patients had prominent extramedullary manifestations of blast crisis. All had received intensive chemotherapy prior to the detection of hypodiploidy, and the cytogenetic findings were similar to those often seen in patients with therapy‐related leukemia. An inv(3)(q21q26) was noted in two patients (one CML‐BC, one de novo Ph+ ANLL), one of whom had hypolobulated micromegakaryocytes. Additional cytogenetic abnormalities in de novo Ph+ ANLL (especially +19) were similar to those in CML‐BC. In contrast, the additional karyotypic changes in de novo Ph+ ALL (eg, +4, –7, –20, markers) were those commonly seen in ALL without a Ph and were generally different from those seen in CML‐BC.
Nonleukemic granulocytic sarcoma of the heart: A report of a fatal case
Authors: Foucar Kathy , Foucar Elliott , Willman Cheryl , Horvath Andrew , Gerety Richard L. 
Published: February 2007

AbstractWe report the clinical and pathological features of a cardiac fatality caused by granulocytic sarcoma in a young adult man with no evidence of leukemia involving bone marrow or peripheral blood. At autopsy, walls of the four cardiac chambers were massively infiltrated by tumor, resulting in a cardiac weight of almost three times normal. Routine cytochemical stains plus immunoperoxidase staining for OKM1 confirmed the diagnosis. Although subclinical cardiac infiltrates are commonly detected at autopsy in patients with acute nonlymphoblastic leukemia (ANLL), massive cardiac granulocytic sarcoma in the absence of bone marrow disease has not been previously described. Cytochemical and immunological methods of diagnosing granulocytic sarcoma in fixed tissues are reviewed.
Modulation of monocyte Mo2 surface antigen expression by exposure to respiratory syncytial virus
Authors: Baumann M. A. , Paul C. C. , Stokes C. , Bernstein J. M. 
Published: February 2007

AbstractThe Mo2 antibody recognizes a monocyte‐specific cell surface antigen of unknown function. Upward modulation of Mo2 surface epitope density was demonstrated in response to 72‐hr culture of monocytes with respiratory syncytial vcirus (RSV), but this was not seen after culture with phytohemagglutinin or other respiratory viruses. Monoclonal antibody probes for RSV proteins were used to demonstrate probable replication of RSV proteins in peripheral blood monocytes and pulmonary alveolar macrophages. These data provide possible clues to the biologic role of the Mo2 antigen.
Essential thrombocythemia associated with recurrent abortions and fetal growth retardation
Authors: Falconer John , Pineo Graham , Blahey Walter , Bowen Tom , Docksteader Brian , Jadusingh Inderman 
Published: February 2007

AbstractEssential thrombocythemia (ET) is one of the myeloproliferative disorders characterized by an elevated platelet count, usually greater than 1,000 × 109/L. It may be associated with either hemorrhagic or thrombotic tendencies. Although it usually affects older people, we have recently encountered two young women with ET who had a total of six spontaneous abortions before the end of the first trimester. During her most recent pregnancy, one of the patients underwent plateletpheresis on four separate occasions before the 12th week. She reached 34 weeks but demonstrated evidence of fetal growth retardation and pre‐eclampsia. At delivery there were scattered villus infarcts in the placenta. Based on this patient and previous reports in the literature, we postulate that the spontaneous abortions, fetal growth retardation, and pre‐eclampsia may be related to the thrombocytosis and suggest that the use of antiplatelet agents throughout pregnancy should be seriously considered in such cases.
Should platelets be radiolabelled in plasma medium?
Authors: Tsan Min‐Fu , Hill‐Zobel Robin L. 
Published: February 2007

AbstractBecause of the low labelling efficiency of platelets with In‐111 oxine in plasma containing media, most investigators have labelled platelets in plasma‐free media. However, labelling of platelets with In‐111 oxine in plasma‐free media may result in either: 1) irreversible damage leading to rapid clearance from the circulation and permanent hepatic sequestration; or 2) reversible damage with transient hepatic sequestration without affecting the in vivo survival. Since human platelets can be labelled with In‐111 oxine in plasma in sufficient efficiency for routine clinical use, they should be labelled in plasma whenever possible. The use of newer In‐111 chelates, tropolone and mercaptopyridine‐N‐oxide, which label platelets with high efficiency in plasma as well as in plasma‐free media, may offer another alternative.
Megakaryocyte progenitors in the bone marrow and peripheral blood of patients with myeloproliferative diseases
Authors: Grossi Alberto , Vannucchi Alessandro M. , Rafanelli Daniela , Vannucchi Letizia , Ferrini Pierluigi Rossi 
Published: February 2007

AbstractWe studied the behavior in culture of megakaryocyte progenitor cells (CFU‐Mk) from peripheral blood (PB) and bone marrow (BM) cells in eight patients with myeloproliferative diseases (MPD). In seven patients we observed megakaryocyte (Mk) colony formation from PB cells, which were generated in the absence of any added stimulator and which did not increase after the addition of a source of Mk‐colony stimulating activity (CSA‐Mk). The number of BM CFU‐Mk was significantly higher in patients than in controls, and in seven out of eight patients the responsiveness to added CSA‐Mk was retained. Plasma obtained from six patients did not stimulate normal donors' BM target cells to form Mk colonies. These data demonstrate an expansion of the CFU‐Mk pool in MPD patients without increased plasma levels of CSA‐Mk, and suggest that PB and BM CFU‐Mk of MPD patients might have different kinetic properties.
Effects of α‐thalassemia‐2 on the developmental changes of hematological values in children with sickle cell disease from georgia
Authors: Felice A. E. , McKie K. M. , Cleek M. P. , Marino E. M. , Kutlar A. , McKie V. C. 
Published: February 2007

AbstractThe hematology and pathophysiology of sickle cell disease during the postnatal development of younger hemoglobin (Hb) S homozygotes (SS) could be considerably affected by a variability of α globin gene numbers. We have documented longitudinal developmental changes of hematological values and hemoglobin composition on 147 patients with SS (αα/αα), 64 with SS (‐α/αα), and 9 with SS (–α/–α) between the ages of 1 and 15 years. Non‐steady‐state data were excluded from these analyses. The number and organization of α globin genes was established by gene mapping. As anticipated, mean corpuscular volume and erythrocyte counts correlated with α globin gene numbers throughout the 15‐year age interval. On the other hand, SS children with αα/αα, –α/, –α had similar hemoglobin concentrations up to the ages of 5–10 years. Around the age of 7, the SS patients with –α/–α developed a higher Hb concentration than that of the SS (–α/αα), which in turn was higher than that of the SS (αα/αα). The emergence of this difference coincided with a developmental increase of the mean corpuscular hemoglobin concentration (MCHC) in patients with SS (αα/αα) and the decline of Hb F % under 15%. This newly observed developmental change of the MCHC could lead to increased hemolysis and anemia after the age of 5‐10 years. It occurs to a smaller extent among SS (–α/αα) or not at all among SS (–α/αα) such that these two categories of patients have less severe hemolysis and higher hemoglobin levels at older ages. Although the proportion of Hb F was independent of α globin gene numbers, the absence of Hb Bart's suggested that α‐thalassemia promotes the intracellular assembly of Hb F over Hb S tetramers. Thus, the interaction of α‐thalassemia and Hb F in young SS patients may be more complex than revealed by Hb F levels in cell lysates. Among older SS children (> 7 years) α‐thalassemia and Hb F levels exceeding 15% appear to have additive effects in diminishing the rate of hemolysis.
Malignant granular lymphoproliferation after Epstein‐Barr virus infection: Partial immunologic reconstitution with interleukin‐2
Authors: Aronson Frederick R. , Dempsey Roy A. , Allegretta Mark , André‐Schwartz Janine , Poldre Peeter A. , Hillyer Christopher D. , Parkinson David R. , Rudders Richard A. , Schwartz Robert S. , Mier James W. 
Published: February 2007

AbstractThis report describes a patient who developed a malignant proliferation of granular lymphocytes following Epstein‐Barr virus (EBV) infection. For many months, his illness resembled prolonged infectious mononucleosis with persistent fatigue, fever, leukocytosis, and serologic evidence of recent primary EBV infection. After approximately I year, however, he developed progressive granular lymphocytosis and extensive lymphocytic infiltration of the bone marrow and liver. Tests for EBV DNA in pre‐ and postmortem tissue samples using a sensitive DNA hybridization technique were negative. Southern blot analysis of DNA prepared from blood mononuclear cells demonstrated clonal T‐cell antigen receptor gene rearrangement. Despite increased numbers of circulating lymphocytes with the morphology and surface phenotype of normal donor natural killer (NK) cells, the patient's NK activity was consistently depressed in a standard in vitro assay. However, in vitro incubation with interleukin‐2 (IL‐2), but not with α‐ or ‐γ‐interferon, increased the NK activity of the patient's lymphocytes. Intravenous recombinant IL‐2 treatment transiently increased the patient's blood NK activity and was associated with seroconversion to EBV nuclear antigens but failed to affect the progression of his disease. Our findings indicate that clonal granular lymphocytic proliferation may develop after EBV infection and confirm the utility of DNA hybridization analysis in distinguishing monoclonal from benign immunoreactive lymphoproliferation. Furthermore, our results suggest that certain functionally inert neoplastic granular lymphocytes acquire NK activity when exposed to IL‐2.
In vitro immunoglobulin production, proliferation, and cell markers before and after antithymocyte globulin therapy in patients with aplastic anemia
Authors: Lum Lawrence G. , Seigneuret Margaret C. , Doney Kristine C. , Storb Rainer 
Published: February 2007

AbstractPeripheral blood lymphocytes from 16 aplastic anemia patients were studied for in vitro biosynthesis of immunoglobulins (Ig), proliferative responses, and cell markers before and after antithymocyte globulin (ATG) treatment in an attempt to identify immune functions that would be useful in predicting responses to ATG therapy. Six of the 16 aplastic anemia patients were complete responders to ATG therapy, two were partial responders, and eight failed to respond to ATG therapy. The proportion of E+, CD4, CD8, and surface Ig‐positive cells did not correlate with in vitro lymphocyte functions nor clinical responses before or after ATG therapy. Lymphocyte proliferative responses to phytohemagglutinin, tetanus toxoid, alloantigens, or pokeweed mitogen were generally present before and after ATG therapy. When pokeweed mitogen, herpes simplex type I virus, and tetanus toxoid were used as probes to elicit in vitro Ig production using a hemolytic plaque assay, some patients had 1) B cells that failed to produce Ig, 2) T cells that failed to provide helper activity, and 3) T cells that exhibited excessive suppressor activity in the various antibody production systems. These measures of immune function, however, did not correlate with clinical responses to ATG therapy.