bluuebunny/crossref_metadata_2025 huggingface dataset:
Analysis of rates of globin synthesis in normal human fetuses and neonates and in twins
Authors: Smith Merran B. , Cauchi M. N.
Published: February 2007

The factors responsible for the switch from γ‐to β‐globin synthesis, which occurs in man in the perinatal period, are still unknown. In this study the relationship of fetal maturity (as measured by gestation age and fetal weight) to changing rates of globin synthesis was analyzed. The relationship of rates of erythropoiesis to the switch was also analyzed. Rates of globin chain synthesis were studied in blood from fetuses from 10‐41 wk gestation, in neonates up to 6 mo of age, and in a series of twins from 29‐40 wk gestation. The β/γ‐globin synthesis ratio in fetal blood remained relatively constant until 30‐34 wk gestation when an increase in β‐globin production was observed. Adult levels of globin synthesis were present in neonates at 6‐9 wk after birth. The γ/α‐globin synthesis ratio showed a reciprocal change, falling after 30 wk gestation. Globin synthesis ratios in fetuses and neonates from 32‐41 wk post‐conception showed a significant correlation with age (β/γ: p < 0.001; β/α: p < 0.01; γ/α: p < 0.05), and there were significant correlations between the β/α and β/γ synthesis ratios and weight (p < 0.05 for both ratios). Globin synthesis ratios in 12 sets of twins from 29‐40 wk gestation were generally within the normal range for gestational age. However, the switch to β‐globin production was more advanced than expected from weight for some of these babies.
Functional characterization of the cells in chronic neutrophilic leukemia
Authors: Dotten Dale A. , Pruzanski Waldemar , Wong Dean
Published: February 2007

Light and electron microscopy of neutrophils from chronic neutrophilic leukemia (CNL) did not reveal differences from normal mature neutrophils. However, functional characterization of CNL cells showed marked differences when compared to normal cells. CNL neutrophils were much less viable in suboptimal conditions. Their survival was further reduced by autologous serum and was corrected by normal human serm. CNL cells showed very active phagocytosis, but their bactericidal activity was reduced in suboptimal conditions. The total content of lysozyme and β‐glucuronidase was lower in CNL cells compared to normal neutrophils, but the release of these enzymes from stimulated cells was much higher than normal. This observation is compatible with a marked lysosomal lability. Cells from the patients' peripheral blood and bone marrow showed excessive growth in CFU‐C assays. Marked susceptibility of CNL cells to cytotoxic activity of cold agglutinins, SLE sera, and CSFs was observed and may signify qualitative and/or quantitative differences in the membrane structure of CNL neutrophils, as compared to normal cells.
Synthesis of the minor fetal hemoglobin Fic in colonies of erythropoietic precursors isolated from human umbilical cord blood
Authors: Abraham E. C. , Reese A. L. , Stallings M. , Abraham A. , Garbutt G. J. , Huisman T. H. J.
Published: February 2007

The biosynthesis of the minor hemoglobin FIc, which contains acetylated γ chains, and the major hemoglobin Fo was studied during erythroid cell differentiation and maturation in cultures of erythroid precursors isolated from five human umbilical cord blood samples. A gradual decrease in the synthesis of Hb FIc was observed during the maturation of the erythroid cells when cultured from 7 to 15 days. The synthesis of Hb Fo did not show a consistent change; however, the relative synthesis of Hb FIc was the highest in the 7‐day‐old colonies and decreased at day 15 to levels observed in the reticulocytes. The addition of sodium butyrate, which is known to promote histone acetylation, significantly increased the synthesis of Hb FIc in 7‐day‐old colonies.
Fluoresceinated lectins as probe for cell surface changes associated with lymphocyte transformation
Authors: Strauchen James A.
Published: February 2007

Lectins are plant‐derived proteins with specific affinities for simple or complex sugars and are potentially useful tools for investigation of the carbohydrate structure of the cell surface. A panel of fluoresceinated lectins was used to study the cell surface changes associated with lymphocyte transformation. The major determinants detected on peripheral blood lymphocytes were αD‐mannose,β‐D‐galactose and N‐acetyl‐glucosamine. Phytohemagglutinin transformation was associated with increased binding of lectins specific for N‐acetyl‐galactosamine. MOLT‐4, a T‐lymphoblast cell line in continuous culture, was associated with increased binding of lectins specific for N‐acetyl‐galactosamine and L‐fucose. Application of fluoresceinated lectins may be a useful tool in the classification of lymphoid cells.
Surface antigenic phenotypes of human T‐cell leukemia corresponding to those of post‐thymic T cells
Authors: Ishii Yoshifumi , Fujimoto Junichiro , Kon Shinichiro , Ogasawara Minoru , Koshiba Hirofumi , Mikuni Chikara , Kikuchi Kokichi
Published: February 2007

Leukemic cells from eight adult patients with various types of T‐cell leukemias, including one patient with lymphosarcoma cell leukemia (T‐LSL), two patients with chronic lymphocytic leukemia (T‐CLL), and five patients with adult T‐cell leukemia (ATL), were analyzed for their surface antigenic phenotypes with a series of monoclonal antibodies directing to human T‐cell differentiation antigens. All of the leukemic T cells studied were regarded as being of post‐thymic T‐cell origin because of their ability to form rosettes with sheep red cells under the condition at 4oC but not 37oC as well as the expression of human Ly‐1‐like but not TL‐like antigen on their cell surfaces. By using monoclonal antibodies to a variety of human T‐cell antigens (Leu‐1, Leu‐2a, and Leu‐3a), all eight cases could be divided into either group of three distinct categories. Thus, one patient with T‐LSL had cells with Leu‐1+2a+3a+ phenotype, which might reflect possible post‐thymic precursor T cells, whereas one patient with T‐CLL had cells with the same phenotype (Leu‐1+2a+3a‐) as normal cytotoxic/suppressor T cells. The latter cells also expressed Ia antigens as defined by monoclonal antihuman Ia antibody. The remaining six cases, including one T‐CLL and five ATL patients, had leukemic cells with the same phenotype (Leu‐1+2a‐3a+) as normally found on helper/inducer T cells, despite distinct clinical and immunological features between T‐CLL and ATL. Some clinical findings observed in those patients may reflect functional activities retained by their leukemic T cells.
A rapid quantitation of platelet‐associated igg by nephelometry
Authors: Morse Bernard S. , Giuliani Dennis , Nussbaum Murray
Published: February 2007

Platelet‐associated IgG (PAIgG) was measured by a simple rapid nephelometric technique using washed solubilized platelets and commercially available, prestandardized reagents. Normal subjects with normal platelet counts had PAIgG levels of 2.1–6.7 fg/platelet. Subjects with idiopathic immune thrombocytopenic purpura (ITP) had levels of 7.2–43.3 fg/platelet. Ninety percent of ITP patients had values exceeding 2 SD units of the mean of normal subjects. Elevated values were also found in 17% of patients with recovered ITP, patients with SLE with and without thrombocytopenia, patients with thrombocytopenia occurring during septicemia, and patients with IgG myeloma. Results can be obtained within several hours of receipt of blood specimen, and are similar to the reports that used more complex techniques.
Thrombotic thrombocytopenic purpura: Recovery after plasmapheresis, corticosteroids, splenectomy, and antiplatelet agents
Authors: Rothberg Harvey , Pachter Ilana , Kosmin Martin , Stevens D. Barton
Published: February 2007

A 68‐year‐old woman severely ill with thrombotic thrombocytopenic purpura (TTP) made a complete recovery after splenectomy was added to a program of corticosteroids, antiplatelet agents, and plasmapheresis. The authors review the subject of therapy of TTP. They advise high‐dose corticosteroids, antiplatelet agents, and plasmapheresis, with splenectomy to be considered as an effective alternative.
Simultaneous evaluation for terminal deoxynucleotidyl transferase and myeloperoxidase in leukemia
Authors: Folds J. D. , Bollum F. J. , Dean L. , Chan J. , Stass S. A.
Published: February 2007

A technique for dual staining of cells using terminal deoxynucleotidyl transferase (TdT) and myeloperoxidase (MPO) is described. The technique has been applied to cells of two patients. One patient had chronic myelomonocytic leukemia evolving into acute myelomonocytic leukemia. The other patient had chronic myelogenous leukemia in blast crisis. Our findings indicate that TdT and MPO are exclusive markers except for a rare precursor cell with dual staining in one patient. This study supports the concept of acute mixed leukemia.
Dr antigen positive monocyte‐macrophages control granulocyte‐macrophage colony‐stimulating activity and burst promoting activity elaboration in man
Authors: Verma D. S. , Spitzer G. , Beran M.
Published: February 2007

To investigate the mechanisms that modulate granulocyte‐macrophage colony‐stimulating activity (GM‐CSA) and burst promoting activity (BPA) elaboration, we studied human peripheral blood‐derived monocyte‐macrophage (MØ) and T‐lymphocyte (TL) interaction. Coincubation of live MØ with autologous TL at a 1:3 ratio in the presence of 1% phytohemagglutinin synergistically increased GM‐CSA (6 of 6 experiments) and BPA (4 of 6 experiments) (P <0.002). Prior treatment of MØ with cycloheximide or actinomycin D significantly (P < 0.002) diminished this MØ's capacity to collaborate with TL. Mitomycin C treatment did not. Live MØ also enhanced TL‐derived GM‐CSA (P <0.002) and BPA (P <0.001). This enhancement was again compromised by prior cycloheximide or actinomycin D treatment, but not by mitomycin C treatment. Further experiments in which we blocked DR antigen on MØ membrane with monoclonal anti‐DR antibodies suggested that MØ required their membrane DR antigen to collaborate with TL in elaborating GM‐CSA and BPA.
Change of enzyme properties caused by cross‐linking treatment of human erythrocytes
Authors: Ikawa M. , Yoshida A.
Published: February 2007

Aragon et al [4] reported that rat erythrocytes can be cross‐linked and permeabilized without significant inactivation or alteration of several enzymes in the glycolytic pathway. If this is the case, in situ kinetic analysis of the red cell enzymes in normal human red cells and abnormal red cells associated with hemolytic problems could be performed. However, we found that the treatment of human red cells with several bifunctional cross‐linking reagents under various conditions always caused a certain extent of inactivation and a change in kinetic properties of glucose‐6‐phosphate dehydrogenase, 6‐phosphogluconate dehydrogenase, and lactate dehydrogenase. Thus, the cross‐linking and permeabilization method, as it stands, is not satisfactory for in situ kinetic analysis of red cell enzymes.
Periodic neutropenia and monocytopenia
Authors: Adams William H. , Liu Yong K.
Published: February 2007

A patient with periodic neutropenia exhibited simultaneous monocytopenia, and epinephrine infusion revealed no monocytes in the marginating pool during neutropenia. Lymphocytes, eosinophils, and platelets also fluctuated periodically, but serial bone marrow studies and epinephrine infusion data indicate these fluctuations could have represented epiphenomena rather than a more global form of periodic hematopoiesis. Bone marrow descriptions of most cases of periodic neutropenia have indicated a “maturation arrest” at the promyelocyte or myelocyte stage prior to development of neutropenia; peripheral blood monocytes are usually normal or fluctuate out of phase with neutrophils. In the present case, “maturation arrest” occurred at the myeloblast stage, and neutrophils and monocytes cycled together. Morphologically normal eosinophilopoiesis with a mean eosinophil to erythroid ratio in the marrow of 0.27 ± 0.10 (SD) persisted despite a sustained disappearance of promyelocytes.
Chlorpromazine‐induced lupus anticoagulant and associated immunologic abnormalities
Authors: Canoso Rosa T. , Sise Herbert S.
Published: February 2007

Chronic administration of chlorpromazine is associated with the development of a lupus‐like circulating anticoagulant and a variety of immunological abnormalities. The prevalence of these findings was studied in 123 psychiatric patients. The anticoagulant was present in 11 of 30 patients receiving chlorpromazine (CPZ), in none of 17 patients who had been off phenothiazine therapy for over a year and in none of 53 controls. It was also seen in 5 of 13 patients who had been switched from CPZ to another phenothiazine even after several years being off CPZ. The anticoagulant was characterized by prolongation of the partial thromboplastin time, thromboplastin dilution test, and Russell's viper venom time. Washed frozen platelets partially corrected the abnormality induced by the anticoagulant. In all but one case the anticoagulant was associated with positive antinuclear antibody test and/or increased serum IgM. Six of 16 patients also had decreased complement levels, and two had a positive direct Coombs' test. None of these patients manifested bleeding, hemolysis, splenomegaly, or other clinical features of systemic lupus erythematosus.
Fetal hemoglobin in women with normal and with hydatidiform molar pregnancy
Authors: Lee John C. , Hayashi Robert H. , Shepard Marguerite K.
Published: February 2007

The quantity and the rate of synthesis of fetal hemoglobin (HbF); the level of HbF‐containing erythrocytes (F cells); and the levels of progesterone, human chorionic gonadotropin (HCG), and human chorionic somatomammotropin (HCS) in serial blood samples from women at different stages of pregnancy were determined. An increase was observed in the synthesis and the quantity of maternal HbF, reaching a peak at about 9–12 weeks gestation. A major peak in F‐cell level was also detected at about 9–12 weeks, with two minor peaks appearing at about 16–20 and 22–24 weeks. Levels of HCG, HCS, and progesterone also varied during pregnancy. Timing of the rise and fall of HCG levels corresponded to that of maternal HbF levels, whereas levels of HCS and progesterone did not reach their peak until later stages of gestation when HbF level had returned to normal.

Similar analysis conducted on patients with hydatidiform molar pregnancy revealed that the majority (>95%) of these patients showed significant increases in HbF synthesis at the time of molar evacuation, but decreased to normal levels in approximately 30 days postevacuation. A similar profile in the levels of HCG, but not progesterone and HCS, was observed. A positive correlation between levels of HCG and HbF can be established (correlation coefficient = 0.94). These data are in agreement with the hypothesis that HCG may act as a stimulus for the enhanced HbF synthesis in adults.
An assessment of the fibrinolytic system in hemophilia A
Authors: Eyster M. Elaine , Wahl Gary , Ballard James O. , Sanders Jeffrey
Published: February 2007

The fibrinolytic system was assessed in 28 hemophiliacs using the chromogenic substrate H‐D‐Val‐Leu‐Lys‐pNA. To determine whether a state of hyperfibrinolysis might be associated with Factor VIII replacement therapy, 14 patients with severe disease who were intensively treated with Factor VIII concentrates were compared with 14 patients with mild disease who were receiving infrequent transfusions with cryoprecipitate or fresh frozen plasma. Seventeen normal males served as controls. With the exception of an elevated level of plasminogen activator and a decreased level of immediate antiplasmin in the mild group only, no evidence of enhanced fibrinolysis was found. Other components of the fibrinolytic system were either normal (plasmin) or increased (progressive antiplasmin containng both α2PI and α2M, and plasminogen). The elevated plasminogen levels were found only in the severe intensively transfused group. The elevated progressive antiplasmin levels were found in both groups of patients and did not appear to be related to transfusions. These findings do not support the concept of enhanced fibrinolysis associated with intensive Factor VIII replacement therapy.
Neonatal screening of β‐thalassemias by thin layer isoelectric focusing
Authors: Cossu Gianfranco , Manca Mario , Pirastru M. Gavina , Bullitta Renza , Bosisio Adriana Bianchi , Gianazza Elisabetta , Righetti Pier Giorgio
Published: February 2007

As an alternative to the recently proposed screening for β‐thalassemias by isoelectric focusing (IEF) of denatured globin chains in urea‐detergent gels [6], IEF of intact hemoglobins, obtained from umbilical cord blood in neonatal units, is reported here. For maximum separation, IEF should be performed in nonlinear pH gradients, in gels containing 0.2 M β‐alanine and 0.2 M 6‐amino caproic acid, which flatten the pH gradient around pH 7, thus increasing the resolution between HbA and HbFac. The method can unambiguously detect homozygous and heterozygous β‐thalassemic conditions. A bimodal distribution of HbA at birth has been found: In heterozygous patients, HbA values of 9.02% (range 6.8–9.98%) have been found while in normal newborns HbA levels of 20.34% (range 11.02–30.6%) have been demonstrated.
Tumours of the mononuclear phagocyte system: A review of clinical and pathological features
Authors: Burns Bruce F. , Evans William K.
Published: February 2007

Cells of the mononuclear‐phagocyte system (MPS) may give rise to a wide variety of tumours of diverse morphology ranging from acute leukemia to small benign tumours of the skin. This review of tumours of the MPS was prompted by recent advances in understanding of tumour morphology and immunologic features. It is now possible to differentiate tumours of the MPS more precisely from tumours of lymphoid origin with which they have often been confused; however, controversy still exists as to the origin of the malignant cell of Hodgkin disease. Current evidence is reviewed which favours the view that the malignant cell of Hodgkin disease is of monocyte origin. A classification of MPS tumours based on organ system of origin and disease extent is presented.
The treatment of acute myelocytic leukemia in patients 30 years of age and younger
Authors: Preisler H. D. , Brecher M. , Browman G. , Early A. P. , Walker I. R. , Raza A. , Freeman A.
Published: February 2007

The treatment of acute myelocytic leukemia in childhood and young adults has lagged behind that for acute lymphocytic leukemia. The studies described here were directed towards evaluating the role of intensive chemotherapy in the treatment of this illness. Intensive remission induction therapy combining cytosine arabinoside with an anthracycline antibiotic produced a complete remission rate comparable to that achieved in acute lymphocytic leukemia (45 of 49 patients or 92%). Intensive consolidation chemotherapy produced a median duration of complete remission of 160 weeks with 40% of patients projected to be in remission at 4 years. By contrast, the median duration of remission for patients treated with moderate consolidation/maintenance therapy was 23 weeks with only 10% of patients in remission at 4 years. These studies demonstrate that intensive chemotherapy can be administered to pediatric patients and young adults and that this approach to therapy produces a high remission rate with a 3 year median duration of remission.
Clinical pilot study of a cytotoxic antiserum in myeloblastic leukemia
Authors: Heyworth M. F. , Wainscoat J. S. , O'Hea A. M. , Schifferli J. A.
Published: February 2007

A large dose of equine antilymphocytic globulin (ALG, 120 mg/kg/day) was given intravenously to four patients with myeloblastic leukemia. Leukemic myeloblasts from each patient were lysed by the ALG in vitro, in the presence of the same patient's serum complement. In each patient, ALG infusion was followed by a reduction in the count of circulating leukemic myeloblasts, and a fall in the serum levels of C3 and C4 complement. It is likely that complement‐mediated lysis of circulating leukemic myeloblasts occurred in these patients, as a result of ALG administration.
Skeletal muscle uptake of Tc 99m pyrophosphate in amyloidosis
Authors: Johnston James B. , Rayner Harry L. , Trevenen Cynthia , Greenberg David
Published: February 2007

In a patient with amyloidosis involving the heart, kidneys, and skeletal muscles, a Tc 99m pyrophosphate “bone” scan demonstrated intense uptake of tracer by the skeletal muscles; but not by the heart or kidneys. Using the alizarin red stain, prominent calcium deposits were detected histologically in association with the skeletal muscle amyloid. These findings suggest that certain amyloid deposits will take up Tc 99m pyrophosphate, but only if sufficient amounts of calcium are associated with the amyloid.
The effect of cell hydration on the deformability of normal and sickle erythrocytes
Authors: Gulley Margaret L. , Ross Dennis W. , Feo Claude , Orringer Eugene P.
Published: February 2007

The deformability of the erythrocyte (RBC) is greatly influenced by its state of hydration. The purpose of this investigation is to quantitate this relationship by measuring the deformability of an RBC population over a broad range of cell water content. By manipulation of the ion content of the RBC, we performed all of the experiments in media which were isotonic with plasma. To raise ion and water content, RBC were incubated in a Li2CO3 medium. To lower cell ion and water content, RBC were exposed to the K ionophore, valinomycin. The range of cell water content achieved during the entire experiment was 900–3200 g/kg cell solid (normal in vivo cell water content being 1800–1950 g/kg cell solid). By using the Ektacytometer, an automated cylindrical viscometer, we were able to measure deformability of the RBC sampled at various points along this range of cell water content. We found that optimal rheologic behavior was exhibited by normal RBC when their water content was in the normal range. A rise or a fall in cell hydration resulted in a decrease in cell deformability. By contrast, the deformability of freshly drawn, well‐oxygenated sickle RBC was well below that found for normal RBC. Upon volume expansion, however, the deformability of these sickle RBC improved markedly. This observation suggests that sickle RBC are suboptimally hydrated and that their abnormal rheology is at least in part a consequence of cell dehydration.
Hemoglobin H disease and multiple congenital anomalies in a child of Northern European origin
Authors: Hjelle Brian , Charache Samuel , Phillips John A.
Published: February 2007

Hemoglobin H (HbH) disease was recently described in three unrelated northern European boys with mental retardation. We have studied a somewhat similar patient, in whom HbH disease was associated with multiple congenital anomalies. Restriction endonuclease analysis of DNA from this proband yielded a pattern consistent with the α‐/– genotype commonly associated with the HbH phenotype in Asians. His parents both carry α thalassemia, in contrast to the previously described families in which only one of the two parents was a carrier.
Hemoglobin queens: α34 (B15) Leu‐Arg structural and functional properties and its association with Hb E
Authors: Moo‐Penn W. F. , Jue D. L. , Johnson M. H. , McGuffey J. E. , Simpkins H. , Katz J.
Published: February 2007

Hemoglobin Queens: α34 (B15) Leu‐Arg was found in association with Hb E in a Vietnamese boy. The observed microcytosis and hypochromia were due to Hb E, which also occurred in other members of this family. The functional properties of Hb Queens in the absence and presence of allosteric effectors were normal. The abnormal hemoglobins appear to be unrelated to the hyperbilirubinemia that was observed at birth.
Childhood monosomy 7 syndrome
Authors: Gyger Martin , Bonny Yvette , Forest Lorraine
Published: February 2007

In recent years, chromosomal aberrations in various hematologic disorders have raised a great deal of interest. In fact, several nonrandom chromosomal abnormalities are now recognized to be responsible for a specific type of dyshemopoiesis while others are closely associated with characteristic hematologic features. Monosomy C, later shown to be monosomy 7 by different banding methods, has been described in children in relation to a peculiar myeloproliferative disorder. Retrospective analysis of early cases published in the literature and a recent observation that we wish to report suggest that the most consistent phenotypic expression of monosomy 7 is an increased susceptibility to bacterial infections related to a preleukemic dyshemopoiesis. Acute nonlymphocytic leukemia is the terminal event of this peculiar preleukemic syndrome, and thus suggests that monosomy 7 involves a stem cell already committed to myeloid differentiation.
Factor VIII inhibitors: A clinical overview
Authors: White Gilbert C. , McMillan Campbell W. , Blatt Philip M. , Roberts Harold R.
Published: February 2007

There is much evidence to indicate that inhibitors to Factor VIII in patients with classical hemophilia are the result of an immunological response to exposure to material (VIII:C or VIII:CAg) that is absent or present in reduced amounts in these patients. The inhibitor is an antibody that is usually restricted in immunochemical composition and in many instances contains predominantly or exclusively γG3 or γG4 heavy chains. Exposure to Factor VIII in many inhibitor patients leads to typical anamnestic responses with marked increases in the level of the inhibitor. The tendency to develop inhibitors and the clinical characteristics of the inhibitor may be affected by genetic factors, basal levels of Factor VIII:C and/or VIII:CAg, and the nature and amount of the “immunizing” material. Currently accepted therapeutic modalities are aimed primarily at the management of acute bleeding episodes.
Mechanism of canine cyclic hematopoiesis: The role of prostaglandin E in feedback regulation
Authors: Hammond William P. , Robertson R. Paul , Dale David C.
Published: February 2007

Prostaglandin E inhibits granulocyte‐macrophage colony formation in vitro in man and mouse, suggesting that it plays a role in feedback regulation of granulocyte production in vivo. Therefore, we examined the role of PGE in normal canine hematopoiesis and its potential role in the pathogenesis of cyclic hematopoiesis in grey collie dogs. The prostaglandin synthesis in hibitors indomethacin and ibuprofen (10−5 M) increased CFU‐C growth to 194 and 160% of control, respectively, while PGE2 addition caused a dose‐dependent inhibition of bone marrow CFU‐C growth in both normal and grey collie dogs. These concentrations of indomethacin and ibuprofen decreased bone marrow cell elaboration of PGE measured by radioimmunoassay to less than 5% of control values. The levels of PGE in leukocyte conditioned medium prepared from grey collies correlated with the number of monocytes in the conditioning cell suspension (r=0.78, n=10, p<0.05) so that PGE production per monocyte was no different in normal and grey collie dogs. The effect of PGE2 upon CFU‐C was to inhibit formation of macrophage, but not neutrophil colony subtypes. These findings make aberrant PGE‐mediated inhibition of precursor cells an unlikely mechanism to cause cyclic hematopoiesis, and show that PGE produced by monocytes acts as a feedback inhibitor for precursor cells destined to produce monocytes but not for those destined to form neutrophils.
Correlation of in vitro culture pattern and Q‐banded karyotype in acute nonlymphocytic leukemia
Authors: Richman Carol M. , Rowley Janet D.
Published: February 2007

Both cytogenetic abnormalities and in vitro growth patterns of leukemic cells have prognostic significance in acute nonlymphocytic leukemia (ANLL). The present study was undertaken to analyze the interrelationship between these two factors and response to therapy. Blast cells from 43 patients with de novo ANLL, four patients with secondary ANLL, and two patients with ANLL following a preleukemic phase were studied using both in vitro culture in methylcellulose and quinacrine chromosome banding techniques. In 19 patients with de novo ANLL, minimal growth in culture (≤ 5 colonies without prominence of small clusters) was noted (Pattern I). Blast cells from the remaining 24 patients formed numerous abnormal clusters and colonies in a continuum distribution (Pattern II). Sixty‐three percent of Pattern I patients had completely normal karyotypes (NN). Only 29% of Pattern II patients were NN. No Pattern I patient had only abnormal karyotypes (AA), while 25% of Pattern II patients were AA (p<0.05). All six patients with secondary leukemia or leukemia following a preleukemic phase demonstrated both Pattern II growth and cytogenetic abnormalities. Fifty‐six percent of Pattern I patients with de novo ANLL responded to chemotherapy, 35% of Pattern II patients responded, and 0% of patients with secondary or post‐preleukemic ANLL responded. The correlation between the presence of cytogenetic abnormalities in leukemic cells and a pattern of excessive, abnormal growth in vitro coupled with the trend toward poor prognosis in these patients suggests that cytogenetically abnormal cells may have a proliferative advantage both in vitro and in vivo.
Febrile Transfusion Reaction Following Initial Transfusion in a Man With Immunoblastic Lymphadenopathy and Granulocyte Autoantibodies
Authors: Barnes Harry M. , Poon Man‐Chiu , Huang Shu Tsong , Conrad Marcel E. , Lin John , McGowan Eoline I.
Published: February 2007

A 69‐year‐old man with immunoblastic lymphadenopathy and autoimmune hemolytic anemia who had no previous exposure to blood products developed a severe febrile nonhemolytic transfusion reaction following the initial infusion of packed red blood cells. The reaction recurred with transfusion of packed red blood cells, but not when freeze‐thawed red blood cells were used. Immunofluorescence techniques demonstrated granulocyte antibodies in his serum and on the surface of his granulocytes. Circulating immune complex, HLA, and platelet antibodies were not present. The granulocyte antibodies fluctuated in titers with disease activities, and could be completely removed from the serum by autologous granulocyte absorption. We conclude that our patient had granulocyte autoantibodies which probably produced febrile transfusion reactions.
Schistosomal stimulation of eosinophil production by human bone marrow in vitro
Authors: Robinson William A. , Entringer Maureen A. , Mahmoud Lotfy Ablel Naby , Drebing Carla J.
Published: February 2007

These studies were undertaken to determine if schistosomal and ragweed antigens incubated with human peripheral blood mononuclear cells (MNC) would stimulate the production of an eosinophil colony stimulating factor (CSF) which was active on human bone marrow. These studies have shown that schistosoma mansoni antigen in a concentration of 5 μg/ml incubated with MNC's from unsensitized individuals lead to production of a conditioned medium which did not stimulate an increased number of total colonies but did increase the number of eosinophil colonies (44 to 61 colonies). Ragweed antigen also stimulated increased eosinophil CSF (from 44 to 63 colonies).

The granulocyte response to parasitic antigens almost invariably involves the eosinophil system. Eosinophils play a vital role in the control of these diseases in humans. Further understanding of the interaction of parasitic antigens with the granulopoietic system will hopefully give new and important clues as to how such parasites interact with man and how they are controlled.
Clostridial species in the pathogenesis of necrotizing enterocolitis in patients with neutropenia
Authors: Hopkins D. Gregg , Kushner James P.
Published: February 2007

The role of gram‐negative bacteria in the pathogenesis of necrotizing enterocolitis has been stressed. We describe a 21‐year‐old woman with chronic neutropenia with cyclic fluctuations who died of necrotizing enterocolitis due to Clostridium septicum. Seven patients with cyclic neutropenia and necrotizing enterocolitis have been described in the literature and in each case in which bacteriologic data were reported, a Clostridial infection was documented. Patients with cyclic neutropenia should be treated with antibiotics effective against Clostridial species when abdominal symptoms complicate neutropenic periods.
Burkitt cell leukemia with abnormality of chromosome no. 1
Authors: Solomons H. D. , Mendelow B. , Levinstein A. , Johnson A. E. , Bernstein R.
Published: February 2007

A case of L3 (Burkitt cell) leukemia with chromosome 1 abnormality but no detectable abnormalities of chromosomes 8 or 14 is reported. This is the first time that L3 leukemia has been shown to be associated with a primary abnormality of chromosome 1. The implications of these findings relative to the development of B‐cell ALL and the role of chromosome 1 in human neoplasia is discussed.
Biochemical characterization of three mutant isozymes of erythrocyte pyruvate kinase: PK‐“Gainesville,” PK‐“San Juan,” and PK‐“Cape Canaveral”
Authors: Paglia Donald E. , Keitt Alan S. , Valentine William N. , Gordon Shirley
Published: February 2007

Pyruvate kinase was partially purified from erythrocytes of three unrelated, nonconsaguineous patients with chronic hemolytic anemia of differing clinical severities. Characterization of the defective PK isozymes by internationally standardized criteria indicated that one (PK‐“Gainesville”) had severely impaired substrate affinity, another (PK‐“San Juan”) had markedly reduced residual activity, and the third (PK‐“Cape Canaveral”) had a combination of milder defects. Each appears representative of subsets emerging from the heterogeneous molecular defects that make up pyruvate kinase deficiency.
Aging of the erythrocyte. 23. Changes in the permeation of spin‐labeled electrolytes
Authors: Bartosz Grzegorz , Gwozdzinski Krzysztof
Published: February 2007

The transport of spin‐labeled anions and cations was studied in bovine erythrocyte fractions of various ages. Permeation of a spin‐labeled anion, 3‐carboxy‐2,2,5,5‐tetramethylpyrrolidine‐1‐oxyl into the cells was found to be significantly slower in older cells, suggesting a functional impairment of the band 3 protein of the erythrocyte membrane during red cell aging in vivo.
Percentages of abnormal hemoglobins in adults with a heterozygosity for an α‐chain and/or a β‐chain variant
Authors: Huisman Titus H. J.
Published: February 2007

The author reviews quantitative data from blood samples involving more than 700 simple and compound heterozygotes for one of 21 types of α‐chain and 36 types of β‐chain variants. All samples were analyzed during the past few years by the same analytical procedure. Various factors influencing the percentages of the different abnormal hemoglobins are discussed; these include the variability of the method, the number of active α‐chain genes, the location of the substitution, the type of substitution, the effect of the loss of a critical amino acid residue, and the introduction of a new residue on the physicochemical and/or functional properties of the protein. The use of a single accurate method is promoted; it may well be that high‐pressure liquid chromatographic procedures will be the recommended methods for future analyses.
Impaired pentose phosphate shunt function in sickle cell disease: A potential mechanism for increased heinz body formation and membrane lipid peroxidation
Authors: Lachant Neil A. , Davidson Warren D. , Tanaka Kouichi R.
Published: February 2007

The red cells' antioxidant defense mechanisms were compared between individuals with sickle cell disease and those with hemolytic anemia and reticulocytosis. In sickle cell disease, there was a significant increase in incubated Heinz body formation (p < .001), a decrease in reduced glutathione concentration (p < .01), an increase in glucose‐6‐phosphate dehydrogenase activity (p < .01), and a decrease in glutathione reductase activity (p < .005). The patients with sickle cell disease had an absolute increase in the activity of the pentose shunt in the intact red cell after methylene blue stimulation (p < .05) and in red cell hemolysates (p < .025). Heinz body formation (r = .75) and pentose shunt activity in red cell hemolysates (r = .83) were strongly related to the degree of reticulocytosis. Although there was a correlation between the pentose shunt activity in the stimulated red cell and in red cell hemolysates for the patients with hemolytic anemia (r = .58), stimulated shunt activity did not increase as the hemolysate shunt activity increased for the patients with sickle cell disease. There were very strong relationships between the ATP concentration and the reticulocyte count (r = .80) and the hemolysate pentose shunt activity (r = .77) in sickle cell disease. These data suggest that in spite of an absolute increase in stimulated pentose shunt activity, there is a relative suppression of stimulated shunt activity in the youngest sickle erythrocytes. This may be related, in part, to the inhibitory effects of high concentrations of ATP on the activity of glucose‐6‐phosphate dehydrogenase.
Acute leukemia and infections: Perspectives from a general hospital
Authors: Balducci Lodovico , Halbrook John C. , Chapman Stanley W. , Vance Ralph B. , Thigpen J. Tate , Morrison Francis S.
Published: February 2007

The incidence and etiology of infections in 210 acute leukemics at the University of Mississippi Medical Center between 1962 and 1978 were reviewed. Infectious episodes occurred 269 times in 148 patients. In 193 infections, potential pathogens were cultured. Infection was a contributing cause of death in 89 patients. E. Coli, S. aureus, K. pneumoniae, and P. aeruginosa accounted for 58% of the isolates. No unusual pattern of antimicrobial resistance were observed. The outcome of the infections was related to the absence or resolution of neutropenia. Among 48 patients febrile on first admission, four cases of gram‐negative pneumonia, two cases of fungal pneumonia, and two cases of pseudomonas cellulitis were diagnosed.

We conclude that the etiology of infections was similar to that of cancer centers; multidrug‐resistant gram‐negative organisms were not prevalent; absence or resolution of neutropenia indicates a good prognosis for outcome of infection; and untreated acute leukemics may acquire opportunistic infections.
Effect of splenic congestion associated with hemolytic anemia on mortality of rats challenged with haemophilus influenzae b
Authors: Chen L. T. , Moxon E. R.
Published: February 2007

The effect of splenic congestion associated with acute hemolytic anemia on susceptibility to bacterial infection was investigated in rats inoculated with Haemophilus influenzae b by intranasal (in) or intravenous (iv) challenge. The rats were made anemic by phenylhydrazine treatment and were challenged with 106 (in) or 5 × 107 (iv) H influenzae b. Forty‐eight hours after in inoculation of bacteria, the number of bacteria in the blood was 10 times greater in the anemic rats with extensive splenic congestion than in controls. After iv inoculation of bacteria, a significantly (P < 0.001) higher mortality rate was found in the anemic rats with extensive splenic congestion and fewer bacteria were present in the spleens but not the livers, as compared to normal rats. Since phenylhydrazine‐induced hemolytic anemia exhibited an extensive congestion in the spleen, higher mortality rate observed in the anemic rats challenged with H influenzae b may result, in part, from decreased intravascular clearance of bacteria by the spleen.
Calmodulin level in whole blood correlates with the percentage of reticulocytes
Authors: Warth James A. , Brewer George J. , Gnegy Margaret E. , Treisman Glen , Near Karen
Published: February 2007

We studied the calmodulin‐like activity (CaM) level in hemolyzed samples of whole blood after determining the percentage and absolute number of reticulocytes present. Twenty‐six samples from 25 people with a range of reticulocyte counts were studied. CaM levels correlated with the percentage (r = 0.63, P < .01) and absolute number (r = 0.64, P < .01) of reticulocytes present, indicating that the calmodulin‐like activity level in whole blood was inversely related to erythrocyte age.
The clonogenic assay as a reproducible in vitro system to study predictive parameters of treatment outcome in acute nonlymphoblastic leukemia
Authors: Browman George , Goldberg Jack , Gottlieb Arlan J. , Preisler Harvey D. , Azarnia Nozar , Priore Roger L. , Brennan James K. , Vogler William R. , Winton Elliott F. , Miller Kenneth B. , Grunwald Hans
Published: February 2007

Seven institutions studied the cloning pattern of leukemic cells from pretherapy bone marrows of 273 patients with newly presenting and relapsed acute nonlymphoblastic leukemia. The cloning assay was done in all centers using an identical double‐layer agar method with a common source of colony‐stimulating factor. Cells were incubated for seven days, and clones were identified visually with an inverted microscope. All centers were able to obtain clonal growth in a substantial proportion of patients. Differences in growth pattern were observed between the major contributing center and the pooled results for all other centers. However, an analysis of clinical results suggested that in vitro differences were more likely related to differences in the patient populations than to variability in laboratory technique. The proportion of marrows in which leukemic cells formed colonies (>40 cells) and large clusters (20‐40 cells) was greater in relapsed patients than in newly presenting patients (P < .06). A progressive improvement in induction treatment outcome was seen with decreasing clonal growth. Patients whose marrows did not produce clones had a complete remission (CR) rate of 83% versus 50% for those patients whose marrow leukemic cells formed colonies and/or large clusters (P=.05). In vitro drug sensitivity studies with cytosine arabinoside and adriamycin were performed on bone marrow cells of patients treated with this combination clinically. The percent killing of clonogenic cells in this assay correlated with remission induction outcome. Complete remission was obtained in 73% of 15 patients whose bone marrow leukemic cells showed >30% killing by in vitro drug exposure, and CR was obtained in only 30% of 23 patients whose marrows showed < 30% killing (P< .01). The results indicate that the clonogenic assay correlates with treatment outcome and can be used for drug sensitivity testing in patients with acute nonlymphoblastic leukemia. The limitations of the assay are discussed.
Treatment of acute sickle cell crises with a vasopressin analogue
Authors: Charache Samuel , Moyer Martha A. , Walker W. Gordon
Published: February 2007

Eight patients with sickle cell anemia were treated for acute painful crises with DDAVP and intravenous fluids; five were treated with placebo and the same regimen of fluid administration. Although hyponatremia was produced in both treatment groups, duration of hospitalization did not differ between them, nor did it differ from concurrent hospitalization of other patients who received conventional treatment. Safe induction of hyponatremia required intensive laboratory surveillance, and serum sodium could be lowered without use of DDAVP. These data suggest that a controlled trial hyponatremia for acute sickle cell crises should not be performed.
A variant 8;21 translocation in acute myeloblastic leukemia
Authors: Oguma Nobuo , Misawa Shinichi , Testa Joseph R.
Published: February 2007

A three‐way rearrangement involving chromosomes 1, 8, and 21 was observed in bone marrow cells from a female with acute myeloblastic leukemia, subtype M2 (AML‐M2). The translocation break points in chromosomes 8 and 21 were identical to those found in the standard t(8;21) that is associated with this disease type. A review of this and four previously reported cases involving variant 8;21 translocations indicates that a consistent pattern of exchange may be involved in these complex rearrangements even though the third chromosome affected may differ.
Adult T‐cell lymphoma leukemia in western countries
Authors: Farcet Jean Pierre , Kuentz Mathieu , Andre Chantal , Darves Jean Marc , Reyes Félix , Dreyfus Bernard , Rochant Henri
Published: February 2007

A new T‐cell disorder has recently emerged: the so‐called adult T‐cell lymphoma leukemia (ATLL) initially described in Japan. Subsequently, ATLL cases were recognized in patients from the Caribbean. We summarize the clinical and hematological features of 19 published cases from Western countries, in addition to a new case we encountered. The leukemic cells display characteristic morphological features and a T3+ T4+ T8− T6− surface antigenic phenotype. Overall survival is of short duration, but remission could be obtained in our case despite a subsequent relapse in skin and CNS. Geographic clusters of ATLL cases have led to the discovery of the possible role of a new retrovirus, HTLV, in the genesis of this rare malignancy.
Evidence that less replacement therapy is required for dental extractions in hemophiliacs
Authors: Steinberg Stephen E. , Levin Jack , Bell William R.
Published: February 2007

The requirements for factor VIII (AHF) or factor IX (PTC) of hemophilic patients undergoing dental extractions were evaluated to determine the minimum effective regimen. Sixteen patients underwent 19 operative procedures. The mean total dose of factor VIII or IX was 45.8 U/kg for 11 procedures with preoperative replacement therapy and 34.5 U/kg for the 8 without. Four patients received no replacement therapy at all.

Our results compared favorably to published studies employing factor replacement alone or in combination with antifibrinolytic agents such as epsilon‐amino‐caproic acid (EACA), with respect to blood products utilized and duration of hospitalization. However, our patients received less factor replacement than usually suggested. General anesthesia with intubation, a common recommendation, was not required in any patients.

Dental extractions can be carried out in hemophiliacs using less replacement therapy than currently recommended. In some circumstances, no replacement therapy may be necessary. The reported efficacy of antifibrinolytic agents in reducing the requirement for replacement therapy is difficult to assess because of the relatively large amount of prophylactic factor replacement used in conjunction with these agents.
Transferrin receptors on human reticulocytes: Variation in site number in hematologic disorders
Authors: Shumak Kenneth H. , Rachkewich Rose A.
Published: February 2007

Assays of binding of 125iodine‐labeled (125I) human transferrin were used to study transferrin receptor sites on reticulocytes from 15 normal subjects and from 66 patients with various hematologic disorders. In normal subjects, few or no transferrin receptors were detected whereas the average number of receptors per reticulocyte varied greatly from patient to patient, ranging from 0 to 67,700 in samples, from 35 patients, on which Scatchard analysis of binding of [125I]‐transferrin was done. Marked heterogeneity in the number of reticulocyte transferrin receptors in different hematologic disorders was also found in assays with [125I]‐OKT9 (monoclonal antibody to the human transferrin receptor). The number of receptors was not correlated with either the reticulocyte count or the hemoglobin.