bluuebunny/crossref_metadata_2025_split huggingface dataset:
Mean platelet volume is increased in hyperthyroidism
Authors: Ford H. C. , Toomath R. J. , Carter J. M. , Delahunt J. W. , Fagerstrom J. N. 
Published: February 2007

AbstractIn 28 unselected patients with hyperthyroidlsm, platelet count, platelet hematocrft, mean platelet volume, and platelet distribution width were measured at the time of presentation and again when the patients were euthyroid. On return to the euthyroid state, there were highly significant falls in the mean values of the mean platelet volume (16% decline, P < 0.001) and the platelet hematocrit (16% decline, P < 0.001) and a slight but highly significant increase in the mean value of the platelet distribution width (2% increase, P < 0.01). A decline in mean platelet volume was observed in 24 of 28 patients (86%); three patients showed no change. There was no significant change in the mean value of the platelet count. The observed effects of thyrotoxicosis on platets appeared to be largely independent of the well‐known effects on erythrocytes and white blood cells. An increase in mean platelet volume is a regular feature of hyperthyroidism that has not previously been described.
Cerebral thrombosis in a newborn with a congenital deficiency of antithrombin III
Authors: Brenner B. , Fishman A. , Goldsher D. , Schreibman D. , Tavory S. 
Published: February 2007

AbstractAn Israeli Arab family with type I antithrombin III (AT‐III) deficiency with several affected symptomatic members in three generations is reported. The propositus presented with deep vein thrombosis and pulmonary emboli associated with gestation.The propositus infant presented at the age of 2 weeks with superior sagittal and rectus sinus thrombosis.Hereditary AT‐III deficiency should be considered in infants with cerebral thrombosis, especially if they have a family history of thromboembolism. The role of prophylactic therapy by AT‐III concentrates in these infants should be further assessed.
Myelopoietic effect of bone marrow fibroblasts cultured from patients with myelofibrosis
Authors: Wang Jen C. 
Published: February 2007

AbstractBone marrow fibroblasts (BMF) have been shown to be able to support granulopoiesis. The present studies were designed to see whether BMF in patients with agnogenic myeloid metaplasia (AMM) are also able to support granulopoiesis. Myeloid colonystimulating activity (CSA) was assayed in BMF derived from the three groups of patients. 1) Group I: Patients with hip fracture but without underlying hematological disease; patients with solid tumor but without bone marrow metastasis; and patients with iron deficiency anemia. 2) Group II: Patients with myeloproliferative disorders other than AMM. 3) Group III: Patients with AMM or myelofibrosis with prior history of polycythemia vera. CSA was determined in a bilayer agar culture system inwhich BMF served as a feeder layer and either mouse or human marrow cells were employed as target cells. There was no difference of CSA production by BMF among these three patient groups. These studies suggest that BMF cultured from patients with myelofibrosis exhibit similar myeloid stimulating activity as do BMF from other patients. A significant correlation was also found between CSA production by BMF and white blood cell counts in patients with myelofibrosis (group III). This suggests that BMF may have a role in either supporting or producing CSA for granulopoiesis in myelofibrosis.
Red cell vacuoles: Their size and distribution under normal conditions and after splenectomy
Authors: Reinhart Walter H. , Chien Shu 
Published: February 2007

AbstractThe frequency of occurrence of vacuoles in red blood cells was studied by transmission electron microscopy. Small vacuoles were found in about 13% of the cell sections, and they had a mean diameter of 130 ± 72 nm (mean ± SD). It can be estimated that there were about 20 small vacuoles per erythrocyte. The frequency of vacuoles was similar in density‐separated cell fractions. In splenectomized patients, the small vacuoles were 4 times more frequent; there was again no difference in vacuole density between top and bottom fractions of density‐separated red blood cells. The bottom fraction of red blood cells from splenectomized patients, however, had a high incidence of large vacuoles (> 300 nm in diameter) and clustering of small vacuoles. These large vacuoles were probably the result of aggregation and fusion of small vacuoles, and their size allowed detection by light microscopy. Hence, the well‐known “pocked” or “pitted” red blood cells of splenectomized individuals were more frequent in the bottom fraction. We conclude that small vacuoles occur normally in erythrocytes, that they tend to cluster and fuse during cell aging, and that the spleen is capable of removing these structures when they reach a certain size.
Platelet‐derived growth factor is decreased in patients with myeloproliferative disorders
Authors: Katoh Osamu , Kimura Akiro , Kuramoto Atsushi 
Published: February 2007

AbstractPlatelet‐derived growth factor (PDGF) has been suggested to play some role in the pathogenesis of myelofibrosis frequently encountered in patients with myeloproliferative disorders (MPD). In this study we measured PDGF activity and PF4 content in circulating platelets of patients with MPD. Both factors were lower than those of normal controls. PDGF activity in patients with myelofibrosis was slightly lower than in those without fibrosis. However, when adjusted to whole blood volume, there was a positive correlation between platelet count and PDGF activity per ml whole blood. Nevertheless, no correlation was found between activity and grade of bone marrow fibrosis. These results may support the idea that an abnormal release of PDGF occurs from platelets or megakaryocytes in the bone marrow environment, resulting in the stimulation of fibroblast proliferation, and hence, the occurrence of myelofibrosis in patients with MPD.
Procainamide‐associated pancytopenia
Authors: Shields Anthony F. , Berenson Jeffrey A. 
Published: February 2007

AbstractA 42‐year‐old male developed thrombocytopenia and anemia along with agranulocytosis during treatment with procainamide. Bone marrow hypoplasia was evident on biopsy, and the patient was without evidence of systemic lupus erythematosis. He had prompt marrow recovery on drug withdrawal. While procainamide‐induced agranulocytosis has been reported on a number of occasions, this is only the second case of pancytopenia.
Prognostic value of clonal chromosomal abnormalities in patients with primary myelodysplastic syndromes
Authors: Gyger Martin , Infante‐Rivard Claire , D'Angelo Giovanni , Forest Lorraine , Lussier Pauline 
Published: February 2007

AbstractChromosome analyses were carried out on bone marrow cells from 43 consecutive patients with primary myelodysplastic syndromes (MDS), classified according to the French‐American‐British (FAB) cooperative group criteria. The objective was to evaluate the prognostic value of clonal chromosomal abnormalities and of an excess of blasts for early death from acute nonlymphocytic leukemia (ANLL) and/or bone marrow failure (BMF). Patients were subdivided into two main groups: (1) refractory anemia without an excess of blasts (RAWEB), grouping patients with refractory anemia (RA) and refractory anemia with ringed sideroblasts (RARS), and (2) refractory anemia with an excess of blasts (RAEB), grouping patients with refractory anemia with an excess of blasts (RAEB) and refractory anemia with an excess of blasts in transformation (RAEBt). There were 29 patients with RAWEB and 14 with RAEB. The median time of observation was 26 months for RAWEB and 12 months for RAEB. Ten RAWEB patients (34%) and 11 RAEB patients (78%) had clonai chromosomal abnormalities. Among the ten RAWEB patients with clonal abnormalities, one (10%) died from ANLL, while of 19 RAWEB patients with a normal karyotype, two (10%) died from ANLL or BMF. The median survival for patients with RAWEB and an abnormal karyotype was not reached. In contrast, eight of the 11 RAEB patients with clonal chromosomal abnormalities (74%) died from ANLL or BMF. The median survival in this sub‐group was 7 months. By using a Cox proportional hazard regression analysis, it was determined that a karyotype abnormality was not a significant predictory of survival once the contribution of the RAWEB/RAEB variable was taken into account. Being in the RAEB group was associated with a relative risk of 10.6 of dying from ANLL or BMF (β = 2.36, standard error (SE) = 0.68,P= .0001). We conclude that classifying patients according to an excess of blasts will lead to a better prediction of survival than determining karyotype abnormality.
DNA damage in intact cells induced by bacterial metabolites of chloramphenicol
Authors: Isildar Mike , Jimenez Joaquin J. , Arimura Grace K. , Yunis Adel A. 
Published: February 2007

AbstractFour chloramphenicol (CAP) metabolites known to be produced by intestinal bacteria were examined with respect to their capacity to induce DNA damage in intact cells. The induction of DNA single‐strand breaks in Raji cells, activated human lymphocytes, and human marrow cells was assayed by the alkaline elution technique. One of the four compounds tested, dehydro‐CAP, was capable of inducing DNA single‐strand breaks in all three cell systems at concentrations of 10−4 M. This effect is comparable to that observed previously with nitroso‐CAP, the nitroreduction intermediate of CAP. The nitroreduction of dehydro‐CAP by human bone marrow cell homogenate was detected by the production of the corresponding amino derivative amounting to 5.6 × 10−5M from 2 × 10−3M substrate under aerobic conditions. In sharp contrast, nitroreduction of CAP by bone marrow could not be demonstrated. The genotoxicity of dehydro‐CAP, its relative stability compared to the nitroso‐CAP, and its nitroreducibility by bone marrow suggest that this bacterial metabolite of CAP may play a key role as a mediator of aplastic anemia in the predisposed host.
Ehrlichiosis: A cause of bone marrow hypoplasia in humans
Authors: Pearce Carolyn J. , Conrad Marcel E. , Nolan Patrick E. , Fishbein Daniel B. , Dawson Jacqueline E. 
Published: February 2007

AbstractInfection with Ehrlichia canis should be suspected in patients with fever, headache, malaise, leukopenia, thrombocytopenia, and a history of recent exposure to ticks. The cytopenia is caused by bone marrow hypoplasia which may be severe. The disease may be confused with spotless Rocky Mountain spotted fever but can be differentiated from this infection serologically with acute and convalescent sera. In humans, recovery has occurred with and without antibiotic therapy. However, prompt antibiotic therapy is advised prior to serologic studies, especially in immunocompromised individuals, splenectomized persons, and patients with AIDS—who may develop a more overwhelming rickettsial infection.
Absence of immunoglobulin and T‐cell receptor gene rearrangements in myelodysplastic syndromes and acute nonlymphocytic leukemias
Authors: Wainscoat J. S. , Fey M. F. , Pilkington S. , Summers C. , Oscier D. G. 
Published: February 2007

AbstractThe arrangement of the immunoglobulin and T‐cell receptor genes has been analysed in 72 cases of primary myelodysplastic syndrome and 17 cases of acute nonlymphocytic leukemia. DNA was extracted from bone marrow aspirates, digested with at least two restriction enzymes, and hybridised with probes for the joining region of the immunoglobulin heavy chain gene, the constant region of the T‐cell receptor β chain gene, and the joining region of the T‐cell receptor γ chain gene. All cases showed germline arrangements of the immunoglobulin and the T cell receptor genes. Thus, true interlineage infidelity, myeloid to lymphoid, is a rare occurrence in myelodysplasia and in myeloid leukemias.
Neonatal alloimmune thrombocytopenia: Spontaneous in utero intracranial hemorrhage
Authors: Burrows Robert F. , Caco Chris C. , Kelton John G. 
Published: February 2007

AbstractNeonatal alloimmune thrombocytopenia is an uncommon but important cause of thrombocytopenia in infants. Because of the severity of the thrombocytopenia, some of these infants will have intracranial hemorrhage with resultant long‐term disability. Obstetricians and neonatologists have recommended delivery by caesarean section and the rapid institution of appropriate treatment for the infant; however, it is theoretically possible that a hemorrhagic event could precede the delivery and consequently not be prevented by these perinatal interventions. In this report we describe a neonate in whom the diagnosis of alloimmune neonatal thrombocytopenia was suspected because of antenatal ultrasound evidence of intracerebral hemorrhage. This case demonstrates the importance of antenatal fetal assessment and indicates the need for the development of therapeutic strategies to maintain fetal hemostasis.
Early transfusion reactions due to acquired anti‐IgA antibodies in a patient with acute lymphoblastic leukemia
Authors: Parikh P. M. , Iyer Y. S. , Mehta B. C. 
Published: February 2007

AbstractWe report transfusion reactions due to acquired anti‐IgA antibodies in a 32‐year‐old woman with acute lymphoblastic leukemia. Previously, such reactions have been reported only after several hundred transfusions. In our patient they commenced within the first few transfusions.
Plasmin‐α2‐plasmin inhibitor complex in plasma of patients with disseminated intravascular coagulation
Authors: Takahashi Hoyu , Hanano Masaharu , Takizawa Shin‐Ichiro , Tatewaki Wataru , Shibata Akira 
Published: February 2007

AbstractPlasmin‐α2‐plasmin inhibitor (α2PI) complex, an indicator of in vivo plasmin generation, was measured in the plasma of 48 patients with disseminated intravascular coagulation (DIC), by enzyme‐linked immunosorbent assay (ELISA). Plasmin‐α2PI complex was markedly elevated to 7.9 ± 4.26 mg/liter (mean ± SD) in DIC at presentation, with normal values at less than 0.8 mg/liter. The concentration of plasmin‐α2PI complex changed in parallel with the course of DIC and decreased to 1.9 ± 1.49 mg/liter (n = 28) in remission. Among various underlying disorders, DIC, in patients with acute promyelocytic leukemia, had the highest complex levels (mean 10.8 mg/liter), and septic patients had the lowest levels (mean 3.4 mg/liter). No significant correlation was found between FDP and plasmin‐α2PI complex. These results indicate that the quantitative assay of plasmin‐α2PI complex in plasma would be valuable in the assessment of hyperfibrinolysis in DIC.
Copper deficiency and sideroblastic anemia associated with zinc ingestion
Authors: Simon Sheryl R. , Branda Richard F. , Tindle Barbara H. , Burns Stanley L. 
Published: February 2007

AbstractAnemia and neutropenia developed in a man who took pharmacologic doses of supplemental zinc. Laboratory investigation showed high zinc level, hypocupremia, low ceruloplasmin level, and ringed sideroblasts. All resolved after withdrawal of zinc. Self‐administered zinc appears to have caused severe copper deficiency, with secondary anemia and neutropenia. Physicians should be aware of this deleterious and completely reversible effect of megadose mineral therapy. Zinc ingestion or exposure should be considered in the differential diagnosis of unexplained anemia, leukopenia, or sideroblastic anemia.
Plasmapheresis in the management of heparin‐associated thrombocytopenia with thrombosis
Authors: Nand Sucha , Robinson John A. 
Published: February 2007

AbstractHeparin‐associated thrombocytopenia with thrombosis (HATT) is fatal in 29% and leads to limb amputation in another 21% of patients. Patients with arterial thrombosis do worse than do those with venous thrombosis alone. Heparin‐associated thrombocytopenia is mediated through IgG or IgM immunoglobulin fractions and is believed to be an immune phenomenon, with heparin acting as a hapten. In addition, endothelial injury may be responsible for the development of thrombosis. Heparin‐induced platelet aggregation persists for weeks to months after its withdrawal in these patients. Acute management of HATT includes surgical thrombectomy, thrombolytic therapy, dextran, ancrod, lowmolecular‐weight heparin, and antiplatelet agents, but overall results remain unsatisfactory. We report the use of plasmapheresis, along with aspirin and dextran, in a patient with HATT. The patient experienced pain relief in 1 day; the heparin‐associated platelet aggregation test became negative in 5 days, and there was functional salvage of the affected limb. We suggest that plasmapheresis may be an effective therapy in the management of HATT.
Acetylcholinesterase in the human erythron. I. Cytochemistry
Authors: Koekebakker Marijke , Barr Ronald D. 
Published: February 2007

AbstractThe successful demonstration and localisation of acetylcholinesterase (AChE), in cells by a cytochemical technique requires maximal expression of enzyme activity, minimal loss of AChE and precise, quantitative generation of reaction product at the actual site of the protein in vivo. These requirements are addressed in a standard technique that has been modified to avoid or optimise fixation and to exhibit enzyme activity under close‐to‐physiological conditions of osmolality, pH, and temperature. With these refinements and with the use of a variety of substrates and enzyme inhibitors of different specificities, true AChE was demonstrable on the membrane of erythrocytes and in the nucleus and cytoplasm of erythroblasts in bone marrow and of the constituent cells of erythroid clones in vitro. The activity in erythrocytes from umbilical cord blood was less than that in corresponding cells from the peripheral circulation of adults. AChE was observed also in human megakaryocytes and in leucocytes at all levels of differentiation, including the components of granulocyte‐macrophage clones. Pseudocholinesterase was detected likewise across the spectrum of erythroid (and leucocyte) ontogeny, suggesting that these enzymes may exercise an important function in hematopoiesis.
Acetylcholinesterase in the human erythron. II. Biochemical assay
Authors: Barr Ronald D. , Koekebakker Marijke , Lawson Alison A. 
Published: February 2007

AbstractAcetylcholinesterase (AChE) is an integral erythrocyte membrane protein. A role for the enzyme in the developing human erythron is being explored. Assays of AchE by the standard Ellman technique overestimate the amount of enzyme by failing to account for the contribution of hemoglobin to the optical density of the reaction mixture. Furthermore, reliance on substrate selection alone for specificity is unsatisfactory. Incorporation of inhibitors of “true” AchE and of pseudocholinestearase confer greater ability to distinguish one enzyme from the other. In our experience, the inhibitor constant (K1) for edrophonium, which is highly specific for AChE, is approximately 5 × 10−5M against adult human erythrocytes that contain significantly more total cholinesterase activity than do erythrocytes from umbilical cord blood. This consists of both “true” and “pseudo” enzyme, the former predominating and accounting for 0.75–1.65 (mean 1.02, median 0.87) femtomoles of substrate hydrolysed per min per cell in adult blood, with values of 0.15–1.04 (mean 0.71, median 0.73) obtained on cord blood. Moreover, the enzyme activity in neonatal erythrocytes has a rather different inhibitor profile from that of adult cells. AChE was also demonstrated in fresh (ALL) and cultured (K562 and HL60) human leukemic cells, as well as in primitive granulocyte‐macrophage and erythroid cells cloned from normal human bone marrow. In the erythroid colonies the enzyme activity was 0–3.76 (mean 1.20, median 0.76) femtomoles per min per cell, apparently the first successful measurement of AChE in such cells.
T(8;21) With a phenotype of chronic myeloid leukemia
Authors: Raskind Wendy H. , Papayannopoulou Thalia , Hammond William P. 
Published: February 2007

AbstractA myeloproliferative disorder having the phenotype of chronic myeloid leukemia (CML) with t(8;21) is reported. Neither t(9;22) nor a DNA rearrangement in the bcr region was detected. The presence of t(8;21), in contrast with previous experience, does not appear to prevent full maturation of the granulocytic series.
Gray platelet syndrome in the elderly
Authors: Berrebi Alain , Klepfish Abraham , Varon David , Shtalrid Mordechai , Vorst Eliakim , Nir Emanuel , Lahav Judith 
Published: February 2007

AbstractA 68‐year‐old male who suffered from thrombocytopenia and mild splenomegaly for 18 years was found to present agranular gray platelets on peripheral blood smear. Bone biopsy revealed a mild, diffuse, reticular fibrosis with no collagen, and electron microscopy of the platelets showed an absence of almost all the α‐granules. Platelet thrombospondin and fibronectin analysed by SDS‐polyacrylamide gel electrophoresis and Rocket immunoelectrophoresis were absent. Follow‐up of 4 years showed the same parameters with no evidence of active myeloproliferative or dysmyelopoietic disorders. Hemorrhagic diathesis was limited to ecchymoses and postprostatectomy bleeding, necessitating platelet transfusion. This led us to conclude that our patient probably had a constitutional primary α‐granule deficiency or gray platelet syndrome. This extremely rare defect has been described in less than 10 patients, all of them very young. Our observation shows that these patients may have a long, uneventful survival.
Recombinant interferon gamma in hairy cell leukemia, multiple myeloma, and Waldenstrom's macroglobulinemia
Authors: Quesada Jorge R. , Alexanian Raymond , Kurzrock Razelle , Barlogie Barthel , Saks Samuel , Gutterman Jordan U. 
Published: February 2007

AbstractFifteen patients with multiple myeloma, five with hairy cell leukemia, and five with Waldenstrom's macroglobulinemia were treated with recombinant interferon gamma (rINF‐gamma) to determine the antitumor activity of this agent. The rIFN‐gamma was administered by daily intramuscular injection at doses ranging from 0.125 to 0.5 mg/m2. No responses were observed in patients with multiple myeloma, although in one patient the disease has remained stable for over 16 months. Minimal improvement in some hematologic indexes were observed in three of five patients with hairy cell leukemia. One partial remission and one minor response were documented in two of the five patients with Waldenstrom's macroglobulinemia. In five patients, an increase in normal serum immunoglobulins was observed. These results suggest that there is only minimal activity of rIFN‐gamma as a single agent in neoplasms of B‐cell origin.
Acute lymphoblastic leukemia in a patient with longstanding polycythemia vera: Cytogenetic analysis reveals two distinct abnormal clones
Authors: Anastasi John , Pettenati Mark J. , Le Beau Michelle M. , Kwaan Hau C. , Weil Susan C. 
Published: February 2007

AbstractA 68‐year‐old female patient is described in whom acute lymphoblastic leukemia followed a long course of polycythemia vera. Chromosomal analysis of a peripheral blood specimen at the time of blastic transformation revealed two distinct clones: one characterized by a chromosomal abnormality frequently noted in polycythemia vera and the other by a rearrangement characteristically observed in lymphoid malignancies. These findings suggest the existence of two independent hematologic diseases: this phenomenon would not support the speculation from previous reports that lymphoproliferative disorders in patients with polycythemia vera arise from clonal evolution.