bluuebunny/crossref_metadata_2025 huggingface dataset:
Plasma cobalamin‐binding and serum cobalamin in patients with folate deficiency
Authors: Lewis M.J. , Dawson D.W.
Published: August 2010

Plasma unsaturated R‐binder and transcobalamin 2 (TC 2) levels were measured in 62 patients with folate deficiency and compared with 80 control subjects and 52 patients with pernicious anaemia. An increase in unsaturated R‐binder concentration was found in the majority of patients with folate deficiency and with PA. In folate deficiency, however, the unsaturated R‐binder was often elevated whether the serum cobalamin (Cbl) was low or normal, more frequently when the serum Cbl was normal. Results of a separate in vivo study of plasma retention of injected 57Co cyanocobalamin were consistent with these findings. An elevated TC 2 was found in a small number of patients with folate deficiency and with PA. The serum Cbl appears to the maintained at a normal level in some patients with folate deficiency by an increase in R‐binder, which is caused by folate deficiency itself.
Cyclosporin A pretreatment of both donor and recipient undergoing allogeneic bone marrow transplantation
Authors: Jacobs Peter
Published: August 2010

51 patients received allogeneic marrow from histocompatible and MLR non‐reactive siblings. Post‐transplantation methotrexate was associated with acute refractory graft‐versus‐host disease (GVHD) in 10/23 (43%) and caused death in 6. When cyclosporin was substituted for the methotrexate fulminating GVHD occurred in 2/28 (28%): both responded to methylprednisolone. When both donor and recipient were pretreated with cyclosporin GVHD of only mild degree developed in 6/20 (30%): 2 responded rapidly to methylprednisolone and in the remaining 4 mild cutaneous lesions persisted. The latter regimen was associated with no donor morbidity and approximately 50% of the recipients had easily reversible renal dysfunction. Thus, cyclosporin A appears to marginally reduce the incidence of acute GVHD and to facilitate response when additional methylprednisolone is required: additional pretreatment of the donor appears to reduce the severity of the acute syndrome. In none of these regimens was a beneficial effect observed on de novo GVHD.
Surface immunoglobulin restriction in B‐non Hodgkin's lymphomas in cell suspension and on frozen tissue sections
Authors: Kluin Philip M. , de Weger Roel A. , Schuurman Henk‐Jan , Peters Peter P. J. , Spies Philomé I. , Bast Bert J. E. G. , van Unnik Jan A. M. , de Gast Gijsbert C.
Published: August 2010

The diagnostic relevance of different tests for detection of surface immunoglobulin on tumour cells of B‐type non‐Hodgkin's lymphomas (B‐NHL) was investigated by comparison of the direct antiglobulin rosetting reaction (DARR) in suspension with two‐colour direct immunofluorescence (DIF) on frozen tissue sections. In benign lymph nodes (n = 27) the κ/λ ratio by DARR test ranged from 0.9 to 2.8. Tested by suspension and frozen tissue analysis, light chain restriction was found in 24 and 27 of 31 cases of B‐NHL, respectively. Heavy chain restriction was found in half of the cases (14 of 26) studied in suspension and in almost all (28 of 31) tested on sections. In 9 cases DARR tests showed restriction of more than one Ig class on tumour cells, which was infrequent (2 of 28) in frozen section analysis. Although both tests appeared valuable for routine diagnostic purposes, we found the DIF analysis on tissue sections somewhat more discriminative, especially in detection of heavy chain restriction in B‐NHL.
Expression of an antigen defined by a monoclonal antibody (SA‐1) on normal and malignant cells
Authors: Avnstrom Søren , Plesner Torben , Ralfkier Elisabeth , Wilken Michael , Griffin James D. , Nissen Nis. I.
Published: August 2010

A monoclonal antibody (SA‐1) has been characterized. The antibody was derived from the fusion of the X‐63 myeloma cell‐line with splenocytes from a mouse immunized with human acute megakaryoblastic leukaemia cells. The antibody reacted with blast cells from 65% of patients with AML without correlation to morphologic classification. The antibody further reacted with a subset of myeloid cells from normal bone marrow, and with peripheral neutrophil granulocytes. In lymph nodes the antibody showed reactivity with subsets of dendritic reticulum cells. In skin biopsies the antibody reacted with subsets of Langerhans cells, subsets of indeterminate cells and activated T‐lymphocytes. The antigen was not expressed on non‐activited T‐lymphocytes. Neither was it expressed on B‐lymphocytes, erythrocytes, platelets nor blast cells from patients with ALL. The antigenic target for the antibody SA‐1 was a surface exposed polypeptide (mol. wt. 15000 D).
Intermittent melphalan in the treatment of essential thrombocytosis with haemorrhage or thrombosis
Authors: de Pauw B. E. , van Bergen A. N. L. , Haanen C. , Steenbergen J.
Published: August 2010

178 episodes of essential thrombocytosis with symptoms of haemorrhage or thrombosis, were treated in 15 patients with melphalan (5 mg/m2 orally during 4 d). An average reduction in the platelet count of 77% was achieved by oral melphalan in 14 responding patients. 1 patient appeared to be refractory to oral therapy, but a decrease of the thrombocyte count was achieved after intravenous administration of melphalan. All responding patients experienced a relief of the thrombocytosis‐associated clinical symptoms. Reduction of the thrombocyte count persisted for 3–4 wk.
Does plasma transferrin regulate iron absorption?
Authors: Aran J. , Baynes R. , Bothwell T. H. , Lyons G. , Graham B. , Torrance J. D.
Published: August 2010

It has been suggested that transferrin that has recently donated its iron to receptor sites is ‘activated’ to take up iron more avidly from donor tissues. The hypothesis was tested in vitro in a system in which use was made of the different electrophoretic mobilities of normal and desialated transferrin. Recently desaturated transferrin and native apotransferrin were added in equal amounts to a solution of radioactive ferric citrate to produce various end saturations. The resultant mixture was electrophoresed on 5.4% polyacrilamide gel, which was then sliced and counted for 59Fe counts. The size of the 2 radioactive peaks was then compared and expressed as a ratio. Using this in vitro system no supporting evidence could be found for the hypothesis that diferric transferrin which has just donated its iron is able to bind available iron more avidly than native apotransferrin.
Cobalamin binding proteins (haptocorrin and transcobalamin) in human cerebrospinal fluid
Authors: Hansen Mads , Brynskov JøSrn , Christensen Peter Anders , Krintel Jens Jakob , Gimsing Peter
Published: August 2010

The unsaturated cobalamin binding capacity of transcobalamin and haptocorrin was studied in cerebrospinal fluid (CSF) and plasma (P) from 37 reference individuals. These comprised 27 males and 10 females who underwent minor surgery in spinal anaesthesia.

The 5th and 95th percentiles were as follows:

P‐Transcobalamin 300 ‐ 870 pmol/l (median 550 pmol/l)

CSF‐Transcobalamin 90 ‐ 540 pmol/l (median 194 pmol/l)

P‐Haptocorrin 75 ‐ 290 pmol/l (median 159 pmol/l)

CSF‐Haptocorrin 10 ‐ 41 pmol/l (median 21 pmol/l)

No sex difference was found between the levels of haptocorrin or transcobalamin in plasma or cerebrospinal fluid. A positive correlation between P‐Transcobalamin and CSF‐Transcobalamin was found, whereas no correlation between P‐Haptocorrin and CSF‐Haptocorrin values was found. The plasma/CSF ratios of transcobalamin, haptocorrin, albumin and IgG indicated that the binders may be synthetized into the cerebrospinal fluid or are actively being transported into the cerebrospinal fluid.
The effects of ethanol and anticonvulsants on erythroid δ‐aminolaevulinic acid synthase
Authors: Tikerpae J. , Samson Diana , Lim C. K. , Peters T. J. , Reynolds E. H.
Published: August 2010

The activity of δ‐aminolaevulinic acid (ALA) synthase was measured in bone marrow from 13 control subjects, 12 chronic alcoholic patients and 9 patients on long term anticonvulsant therapy. The majority of patients in both groups had macrocytic red cells in the absence of megaloblastic changes in the marrow. There was a significant increase in ALA synthase activity in the alcoholic patients but no significant increase in enzyme activity in the patients on anticonvulsants and overall there was no correlation of activity with MCV. The macrocytosis associated with these drugs does not therefore appear to result from accelerated erythroid haem synthesis.
A study of disturbances in granulocytopoiesis in humans using a diffusion chamber system
Authors: Stojanović Nevenka , Jovčic Gordana , Ruvidić R. , Hajduković S.
Published: August 2010

Data are presented demonstrating that bone marrow granulocytic cells from normal persons continuously proliferate and mature during 5 d of cultivation in a diffusion chamber system (DC). Bone marrow granulocytopoiesis in 3 patients with different granulocytopoietic disorders was investigated using DC. In patient 1, with drug‐induced agranulocytosis, bone marrow aspirated on the “day of recovery” revealed intensive proliferation in DC resulting in a highly significant increase of mature granulocytes. Patient 2 suffered from chronic neutropenia of unknown aetiology. After 5 d cultivation of bone marrow in DC an increase of metamyelocytes was observed, indicating delayed maturation of granulocytic cells. Patient 3 had neutropenia probably due to the splenic sequestration. Data obtained with DC suggest stimulated granulocytopoiesis in this patient. The results obtained using DC reflect the particular phase of the disease in which bone marrow was aspirated. It may be concluded that this method could provide additional data in quantitative and functional disorders of granulocytes.
Low dose cytosine arabinoside in the treatment of acute non‐lymphocytic leukaemia
Authors: Jensen Mogens Krogh , Ahlbom Göran
Published: August 2010

Complete remissions were obtained with low dose cytosine arabinoside (ARA‐C) in 8 of 21 previously untreated patients with ANLL above 60 years of age and in 2 of 4 previously treated patients below 60 years of age. 7 of the patients remain in remission after 1–26 months. The results demonstrate that low dose ARA‐C with rather low toxicity may induce remissions in elderly patients with ANLL.
Expression of the platelet‐common acute lymphoblastic leukaemia associated antigen on normal eosinophils
Authors: Ohto H. , Shibata Y. , Takeuchi A. , Chen R‐F. , Maeda H.
Published: August 2010

A murine monoclonal antibody (TP82) raised against normal platelets was found to be reactive with eosinophils and common acute lymphoblastic leukaemia (cALL) cells, and some blast cells from patients with non‐lymphoid leukaemia. The antibody showed that the platelet‐cALL associated antigen (p24) is also expressed on normal eosinophils, and may prove useful for studying the function as well as the differentiation of eosinophils.
Isolation of leukaemia‐associated inhibitor (LAI)‐producing cells from normal peripheral blood
Authors: Olofsson Tor , Cedergren Britt‐Marie , Person Elisabeth
Published: August 2010

LAI, leukaemia‐associated inhibitor, has previously been shown to be produced by a subpopulation of null cells in myeloid leukaemia, and has the capacity to suppress the proliferation of normal granulopoietic stem cells, CFU‐GM, in vitro. In the present study, low density mononuclear cells from normal peripheral blood were separated into adherent/non‐adherent, phagocytic/non‐phagocytic, T‐lymphocytes/non‐T cells, and Fc‐receptor positive and negative cells in search for LAI‐producing cells in normal blood. Cell fractions enriched for NK‐cells were isolated from Percoll gradients and NK‐activity and LAI‐production were assayed in the different fractions. Anti‐Leu‐2, anti‐Leu‐3, and anti‐HLA‐DR were used to deplete mononuclear cells of cells positive for these monoclonals using a panning technique. It is concluded from these studies that normal LAI‐producing cells belong to a non‐adherent, non‐phagocytic, non‐T, non‐B, Fc‐receptor positive population which does not express NK‐activity, and which is Leu‐2, Leu‐3 and HLA‐DR negative. The results imply that LAI may be a novel feedback regulator of the proliferative rate of granulopoietic stem cells and that LA1 is produced by a small subpopulation of cells in both blood and bone marrow.
Erythrocyte involvement in chronic lymphocytic leukaemia
Authors: Meyerstein N. , Oppenheim U. , Yirmiahu T. , Hatskelson L. , Dvilansky A.
Published: August 2010

Chronic lymphocytic leukaemia (CLL) is a lymphoproliferative disorder which sometimes also affects the erythrocytes. In this study we investigated erythrocyte involvement in 23 CLL patients. We found increased erythrocyte osmotic fragility in 15 CLL patients, but this finding was not accompanied by increased permeability to acidified glycerol (decreased AGLT50). Only those CLL patient who had positive direct antiglobulin test (DAT) had significantly decreased AGLT50. AGLT cannot serve as a predictor test for the future development of autoimmune haemolytic anaemia in CLL patients. ATP content was unaffected by the lymphoproliferative disease, but whole blood filterability was markedly decreased in CLL patients. Our study supports the hypothesis that erythrocytes are indeed affected by the lymphoproliferative disorder, even in the absence of overt autoimmune haemolytic processes.
Defective recloning capacity of granulocyte‐macrophage colony‐forming cells in chronic myeloid leukaemia
Authors: Olofsson Tor , Nilsson Bo
Published: August 2010

We investigated the recloning capacity of normal and chronic myeloid leukaemia granulocyte‐macrophage colony‐forming cells (CFU‐GM) after 7 d culture in methyl‐cellulose. Normal CFU‐GM were recloned with the formation of 3.52 ± 1.12 secondary CFU‐GM per primary d‐7 colony. A frequency distribution of the colony‐forming cells within d‐7 colonies showed a heterogeneous distribution with the majority of d‐7 colonies containing 1 or zero CFU‐GM and a few colonies containing more than 30 CFU‐GM. Separation of marrow cells by velocity sedimentation demonstrated that the recloning capacity was primarily expressed by the smallest colony‐forming cells. In contrast, marrow or blood cells from 18 patients with Ph1‐chromosome‐positive CML in the chronic phase produced colonies with defective recloning capacity. Only 1 patient had a recloning value within the normal range; the others had a mean value of 0.35 (range 0–1.28) secondary colonies per primary d‐7 colony. The recloning defect was not related to WBC or treatment, since 5 newly diagnosed patients with CML also showed defective recloning before any treatment was given, which is compatible with the defect being an inherent property of CML.
Evolution of a terminal deoxynucleotidyl transferase‐positive lymphoma from a chronic T cell lymphocytosis
Authors: Oscier D. G. , Mufti G. J. , Hamblin T. J. , Jones D. B. , Smith J. L.
Published: August 2010

A 56‐yr‐old Caucasian man presented with a generalised scaly rash and a peripheral blood lymphocytosis of 5.6 times 109/l. 5 yr later he developed cutaneous nodules, lymphadenopathy and hepatosplenomegaly. Cells with convoluted nuclei and prominent nucleoli were seen in the peripheral blood. He underwent splenectomy and received intensive chemotherapy but died 6 months later with CNS infiltration. At presentation the peripheral blood lymphocytes were E‐ve, UCHT1 + ve, and OKT8 + ve. Following transformation, cells in blood, spleen and CSF were E‐ve, OKT11 + ve, DR + ve and Tdt + ve. A proportion of these cells had a Sézary‐like appearance at E/M. The splenic cells showed functional suppressor activity. This is the first reported case of the evolution of a Tdt + ve lymphoma from a post‐thymic T cell lymphocytosis.
Bone marrow histology in myelodysplastic syndromes
Authors: Frisch B. , Bartl R.
Published: August 2010

Bone biopsies of 300 patients with myelodysplastic syndromes (MDS) were studied. The bone marrow findings fell into one of six categories: 1. MDS sideroblastic; 2. MDS megaloblastoid; 3. MDS proliferative; 4. MDS blastic; 5. MDS inflammatory; 6. MDS hypoplastic, which were then correlated with defined clinical entities. A comparison was made between the findings in bone marrow histology and the groups of the FAB classification based on aspirate cytology. No correspondence was found between the 5 groups of the FAB classification and the histological categories. The possible underlying bone marrow histologies for the FAB subgroups are given. Follow‐up and sequential biopsies were used to establish the subsequent clinical course for each of the histological categories. The conclusion is drawn that aspirate cytology and bone marrow histology are complementary procedures and that both are required for the diagnosis and evaluation of prognosis in patients with MDS.
Natural course of myelodysplastic syndromes — Helsinki experience
Authors: Teerenhovi L. , Lintula R.
Published: August 2010

Between the years 1969 and 1984 162 new patients with myelodysplastic syndromes were diagnosed in the Helsinki University Central Hospital. The median survival was 29 months. The survival was best in the FAB‐groups with < 5 % bone marrow blast cells: median 48 months in the RA‐group and 41 months in the RARS‐group. In the FAB‐groups with > 5 % bone marrow blasts, RAEB and RAEBt, the median survival was 21 and 13 months, respectively. In the CMML‐group the survival was almost as short as that in the RAEBt‐group, median 16 months. These differences in survival between the FAB‐groups were highly significant (p = 0.003). Other variables that predicted short survival were Hb < 10 g/1, platelets < 150X 109/1, and the presence of peripheral blood blast cells.

26.5 % of all patients progressed to ANLL. The patients in the FAB‐groups with > 5 % bone marrow blasts cells (RAEB, RAEBt) expired more often in ANLL when compared with patients with < 5 % bone marrow blast cells (RA, RARS) and with patients with CMML (p < 0.001). Other variables that predicted progression to ANLL were the presence of peripheral blood blast cells (p = 0.01) and the age < 65 years (p < 0.01). Besides ANLL, infection and/or bleeding were common causes of death. Two thirds of all deaths were due to these causes.
ACUTE MYELOGENOUS LEUKEMIA OF UNFAVOURABLE PROGNOSIS TREATED WITH RETINOIC ACID, VITAMIN D3, ALPHA‐INTERFERON AND LOW DOSES OF CYTOSINE ARA‐BINOSIDE
Authors: Robèrt Karl‐Henrik , Hellström Eva , Einhorn Stefan , Gahrton Gösta
Published: August 2010

Eight patients with AML of unfavourable prognosis were treated with combinations of low doses of ara‐C, retinoic acid, vitamin D3 and alpha‐interferon. 2 complete remissions and 3 partial responses were achieved. The good response in this patient group, together with the observation that 3 of the responders (1 complete remission) had no prior marrow hypoplasia, are suggestive of mechanisms of action that are separate from those in conventional chemotherapy. Possible alternatives are the induction of differentiation of leukemic cells, stimulation of normal stem cells, a slowly active selective cytotoxicity on leukemic stem cells with only minor side effects on normal stem cells, or combinations of these mechanisms.
A transitional variant of a T‐lymphoproliferative malignancy
Authors: Jønsson V. , Wantzin G. Lange , Badsberg E. , Menné T. , Videbæk Aa.
Published: August 2010

2 cases of malignant T‐lymphoproliferative disease are reported. The proliferating cell was a large blast expressing E and Fcγ receptors but no helper or suppressor phenotypes and no SmIg. Skin infiltrates were the dominant clinical sign with conspicuous perivascular aggregations of T, E, Fcγ lymphocytes, though both patients initially had disseminated disease with mild lymphadenopathy, splenomegaly and, in case 2, also hepatic infiltrations. Accordingly, DNA measurements on skin biopsies, taken early in the course, showed a dominating hypotetraploid clone (case 1) and a pronounced population in S‐phase (case 2). The patients were alive for 6 and 2 yr, respectively, with a final fatal course of about 6 months duration involving a rather sudden progression of the skin infiltrates, increasing lymphadenopathy and splenomegaly, leukaemic transformation of the neoplastic T, E, Fcγ lymphocyte and practically no response to cytostatic treatment.
Molecular defects in 2 examples of severe Hb H disease
Authors: Trent R. J. , Wilkinson T. , Yakas J. , Carter J. , Lammi A. , Kronenberg H.
Published: August 2010

Severe Hb H disease presented in unexpected ways in 2 families of Greek origin. In 1, Hb H disease led to neonatal death. The underlying molecular defect was double‐heterozygosity for the —Med/ α thalassaemia haplotype and a nondeletional α thalassaemia defect (ααT'Karditsa’/). The 2nd family requested antenatal diagnosis. The husband had mild nondeletional α thalassaemia. Initial investigations in the wife demonstrated unexpected gene mapping patterns. These have recently been shown to result from the (−α)Med 20.5/haplotype.
Megakaryoblastic leukaemia and myelofibrosis complicating Fanconi anaemia
Authors: Dharmasena F. , Catchpole M. , Erber W. , Mason D. , Gordon‐Smith E. C.
Published: August 2010

A 9‐yr‐old boy with a 2‐yr history of Fanconi anacmia developed worsening pancytopenia that was unresponsive to oxymetholone therapy. Bone marrow was difficult to aspirate but showed the presence of megakaryoblasts. Bone marrow trephine was hypercellular with large clusters of abnormal megakaryocytes and a small population of megakaryoblasts, giving a diagnosis of megakaryoblastic leukaemia.
Pancytopenia and other haematological findings in brucellosis
Authors: Aysha Marei H. , Shayib Mohammed A.
Published: August 2010

The haematological findings in 212 patients with brucellosis are presented, analysed, and the possible underlying pathogeneses discussed. Pancytopenia and other haematological abnormalities were frequently found among the clinical features. As brucellosis is common in our region, and pancytopenia is a common presentation, it should be included in the differential diagnosis of all those clinical conditions leading to pancytopenia.
Binding of murine monoclonal antibodies to human MHC class I or II antigens increases binding sites for either antibody
Authors: Neppert Jürgen , Mueller‐Eckhardt Christian
Published: August 2010

Membrane redistribution of major histocompatibility complex (MHC) class I and II antigens on human B lymphocytes and monocytes was studied quantitatively by fluorescence flow cytometry using monoclonal antibodies (mAb) w6.32 (class I‐specific) and 2MC3 (class II‐specific). It was shown that the surface density of class I and II antigens to which corresponding mAb had been bound decreased rapidly upon incubation at 37°C. However, the total amount of class I antigens remained practically unchanged, while that of class II antigens increased substantially. This augmentation of class II antigens could be elicited not only by the corresponding class II‐specific mAb but, similarly, also by non‐corresponding class I‐specific mAb. New expression of class I antigens only compensated for removed antigens. The speed of disappearance was comparable for antigens of either class. We conclude that the more pronounced expression of new class II antigens is not due to a greater surface mobility, but to a faster recycling and/or larger intracellular deposits of the antigens. This uneven redistribution of class I and II antigens is likely to reflect differences of function known to be dependent on MHC products.
Androgen therapy in aplastic anaemia: A comparative study of high and low‐doses and of 4 different androgens
Authors: Najean Yves ,   French cooperative group for the study of aplastic and refractory anaemias
Published: August 2010

A prospective randomized study of androgen therapy in aplastic anaemia (AA) was performed: 2 androgens (fluoxymesterone and norethandrolone) at high (1 mg/kg/d) and low (0.2 mg/kg/d) dose were studied on 110 patients; and 4 androgens given at high doses were objectively compared in 125 other cases. When patients are matched for AA severity there is an obvious efficiency of the high‐dose androgens in the survival of the less severe cases of aplastic anaemia and in their haematological improvement. The data further show that, among the 4 androgens tested, fluoxymesterone is the most efficient and stanozolol the least. We conclude that androgen therapy is truly efficient in moderately. severe aplastic anaemia and that fluoxymesterone (1 mg/kg/d) for 18 months should be chosen as reference drug to any new androgen therapy assay.
Hairy cell leukaemia: effects of phorbol ester on cell morphology and adherence and comparison with other B‐cell neoplasias
Authors: Polliack Aaron , Gurfel Dorit , Or Reuven , Leizerowitz Rachel , Gazitt Yair
Published: August 2010

This report documents phorbol ester‐induced changes in cell morphology in 9 patients with hairy cell leukaemia (HCL) and contrasts them with those obtained in cells from 20 patients with B‐type chronic lymphocytic leukaemia (CLL) and non‐Hodgkin's lymphoma in leukaemic phase (NHL). The phorbol ester caused hairy cells to adhere strongly and produce thin elongated cytoplasmic extensions. Other cells showed marked surface ruffling, and contained increased numbers of phagolysosomes, although cells still retained ribosomal lamellar complexes. This in vitro growth pattern differed from that observed in B‐CLL and NHL cells, which readily detached in clumps with minimal adherence. CLL and NHL‐cells did not show macrophage features but developed plasmacytoid and hairy cell‐like features. It is suggested that these different growth patterns may aid in distinguishing HCL from other B‐cell neoplasias.
Platelet‐associated IgG in childhood idiopathic thrombocytopenic purpura: Measurements on intact and solubilized platelets and after gammaglobulin treatment
Authors: Ljung R. , Nilsson I. M. , Frohm B. , Holmberg L.
Published: August 2010

An immunoradiometric assay was developed for determining platelet‐associated IgG (PAIgG) both on intact and solubilized platelets. In 20 healthy subjects PAIgG was 0.28 ± 0.20 ng/106 platelets and 2.2 ± 1.1 ng/106 platelets on intact and on solubilized platelets, respectively. 13 children with acute ITP all had increased concentrations of PAIgG, but no correlation was found between the severity of thrombocytopenia and PAIgG concentrations, either on intact or on solubilized platelets. Neither was there any correlation of the increase in PAIgG between intact and solubilized preparations. 3 out of 4 children who received high‐dose i.v. gammaglobulin showed a concomitant normalization of the platelet count and of the PAIgG concentration both on intact and lyzed platelets. The remaining child did not respond to gammaglobulin and continued to have abnormal PAIgG.
Coexpression of T‐ and B‐markers in a lymphoproliferative disorder
Authors: Miguel J. F. San , Foroni L. , González M. , del Cañizo M. C. , Salazar J. , Rios A. , Matutes E. , Catovsky D.
Published: August 2010

An atypical case of lymhoproliferative disorder in which T‐ and B‐cell antigens were coexpressed in the neoplastic cells is described. The disease was characterised by hepatosplenomegaly, lymphadenopaty, a low WBC (5 × 109/1) and bone marrow infiltration. The predominant cell population (> 70%) comprised lymphoid cells with a range of nuclear irregularities and included some blast cells. 90% of the peripheral blood lymphocytes showed a mature T‐helper phenotype (E+, T11+, T3+, T4+, T8‐, T6‐, TdT‐) with coexpression of the specific B‐markers Bl and FMC7, in 90% and 50% of cells, respectively. HLA‐DR antigens were present in 55% of cells while surface and cytoplasmic immunoglobulins (Ig) were detected in < 10% of cells. Molecular investigations with appropiate probes showed evidence of T‐cell receptor gene rearrangement but no rearrangement for the genes of the Ig‐heavy and ‐ light chains. Cytogenetic studies revealed a translocation t(10;19) (p12; q13) in all the metaphases analyzed. This case demonstrates that the study of neoplastic cells with a battery of monoclonal antibodies may disclose the existence of a hitherto unrecognised lymphoid cell population with atypical expression of B‐ and T‐cell antigens. On the other hand, the presence of T‐cell receptor gene rearrangement indicates that this is a T‐cell disorder with the aberrant co‐expression of specific B‐cell markers.
Expression of monocytic and T‐lymphocytic phenotype in a variant form of acute promyelocytc leukaemia (M3)
Authors: Sato Tadashi , Abe Rokuo , Kawaguchi Michiko , Uchida Tatsumi , Kariyone Shigeo
Published: August 2010

A 46‐yr‐old woman was diagnosed as having M3 variant leukaemia in FAB sub‐classification with disseminated intravascular coagulation (DIC) and the 15;17 translocation. Immunologic examinations revealed that the blast cells exhibited monocytic (Ia +, Mo1 +, Mo2 +, My4 +, My7+ and My9 +) and T‐lymphocytic (OKT11 +, Leu4+ and Leu5b +) immunophenotype, with probably a biphenotypic population of cells. Expression of monocytic phenotype on the blast cells suggests that M3 variant includes a subtype with monocytic characteristics, and may be related to the morphologic monocyte‐like feature of that of M3 variant.
Hyperactive phagocytosis by circulating neutrophils and monocytes in Chèdiak‐Higashi syndrome
Authors: Komiyama Atsushi , Saitoh Hiroharu , Yamazaki Munehiro , Kawai Hiroshi , Miyagawa Yukiaki , Akabane Taro , Ichikawa Makoto , Shigematsu Hidekazu
Published: August 2010

Phagocytic capacity of circulating neutrophils and monocytes was studied by electron micrsocopy in 2 children with Chèdiak‐Higashi syndrome (CHS). Apparent phagocytosis of autologous leucocytes by the CHS neutrophils and monocytes was demonstrated when they were coincubated with Staphylococcus aureus in vitro, indicating their capacity for hyperactive phagocytosis. The hyperactive phagocytic capacity of the CHS phagocytes was a cellular abnormality. CHS neutrophils and monocytes are known to be defective in Chemotaxis and bactericidal capacity, and the hyperactive phagocytic capacity is another functional abnormality of CHS phagocytes.
Immunocytochemical detection of normal and abnormal megakaryocytes using monoclonal antibody to glycoprotein IIb‐IIIa (TP80): The quantitative assay in normal and in leukaemic patients
Authors: Sato Tadashi , Shichishima Tsutomu , Kimura Hideo , Uchida Tatsumi , Kariyone Shigeo , Ohto Hitoshi , Maeda Hiroo
Published: August 2010

We identified bone marrow megakaryocytes by an immunocytochemical technique using a monoclonal antibody (TP80) against platelet glycoprotein IIb‐IIIa (GPIIb‐IIIa). The immunocytochemical technique using TP80, specific to megakaryocytes, enabled us to observe cellular morphology and immunological reaction under light microscopy, and permitted quantitative assessment of megakaryocytes. In normal marrow, TP80 labelled 22 ± 5 megakaryocytes / 104 mononuclear cells (mean ± 1 SD, n = 14). In addition to typical large megakaryocytes, small immature megakaryocytes (≤ 20 μm) were recognized in 10–15% of total megakaryocytes. 4 out of 18 patients with acute myeloblastic leukaemia and 9 of 10 patients with myelodysplastic syndrome showed increased numbers of megakaryocytes. In these patients, cell size distribution was abnormal, i.e., most of the megakaryocytes consisted of small, atypical megakaryocytes. None of the patients with acute lymphoblastic leukaemia showed increased megakaryocytes. Immunocytochemical identification of megakaryocytes using a specific antibody is useful to quantitate the megakaryocytes and to detect the proliferation of atypical megakaryocytes in several leukaemic conditions.
Meningitis and septicaemia in adults splenectomized for Hodgkin's disease
Authors: Baccarani Michele , Fiacchini Mauro , Galieni Piero , Gherlinzoni Filippo , Fanin Renato , Fasola Gianpiero , Mazza Patrizio , Tura Sante
Published: August 2010

Splenectomized and asplenic persons are at risk of developing sudden episodes of meningitis and/or septicaemia, frequently due to Streptococcus pneumoniae (overwhelming postsplenectomy infection, or OPSI). The application of staging splenectomy to patients with Hodgkin's disease (HD) generated a new population at risk for OPSI. We reviewed the cases of sudden meningitis and/or septicaemia in a series of 491 consecutive adults with HD. No case was recorded among 149 non‐splenectomized patients. 6 episodes of OPSI were observed in 5 of 342 splenectomized patients (crude frequency 1.75%, actuarial frequency at 10 yr 3.87%). The infecting organisms were Streptococcus pneumoniae (5 cases) and Salmonella enteritidis (1 case). 2 episodes (1 fatal) occurred during first complete remission (CR), 15 and 120 months, respectively, after splenectomy. 4 episodes (2 fatal) occurred after first relapse. For splenectomized patients in continuous CR, the cumulative risk of OPSI was 2.30% (95% CL 0–6.10%), and it was 15.25% (95% CL 0–35.37%) for splenectomized patients who had relapsed. These data suggest that, in adults with HD, the risk of OPSI should not limit the indication to staging splenectomy and does not warrant systematic prophylaxis. However, in splenectomized relapsed patients, OPSI can be a troublesome complication and appropriate prophylaxis could be useful.
Cerebellar hemangioblastoma with erythropoietin in cerebrospinal fluid
Authors: Janković G. M. , Ristić M. S. , Pavlović‐Kentera V.
Published: August 2010

A patient with cerebellar hemangioblastoma and secondary erythrocytosis is presented. Erythropoietin, indirectly determined by measuring 59Fe incorporation in polycythemic mouse bioassay, was not detected in serum and urine, but was found in cerebrospinal fluid. The significance of erythropoietin determination in cerebrospinal fluid in patients with cerebellar hemangioblastoma is stressed.
Erythropoietin activity in the serum of beta thalassemic patients
Authors: Manor Daphna , Fibach Eitan , Goldfarb Ada , Rachmilewitz Eliezer A.
Published: August 2010

Serum erythropoietic activity was determined in 32 patients with beta thalassemia major and intermedia. Quantitation was performed by an in vitro bioassay using rabbit erythroid precursor cells (CFU‐E) either by colony assay or by 3H‐thymidine uptake. 20 polytransfused beta‐thalassemic major patients had erythropoietic activity (mean 89.3 ± 36 milliunits/ml) which was not significantly different (p > 0.2) from normal individuals (51.3 ± 32 milliunits/ml). 12 untransfused patients with beta thalassemia intermedia were found to have comparable serum erythropoietic activity (p > 0.1). These levels were much lower than those found in patients with aplastic anemia who had a comparable degree of anemia. We have shown that the low EPO activity in thalassemic patients was not due to experimental conditions (excess of ferritin, low transferrin) nor to specific inhibitors appearing in this disease.

No correlation was found between the erythropoietic activity and sex or other clinical parameters of the patients such as severity of the anemia, splenectomy, iron chelation or transfusion therapy. 4 young thalassemic children (1–2 yr of age) studied had high erythropoietic activity ranging from 661 to 5793 milliunits/ml ‐ significantly different from normal children of the same age. It is suggested, therefore, that a decrease in serum erythropoietin levels develops during the course of the disease.
Significance of in vitro cultures in myelodysplastic syndromes
Authors: Haak H. L. , Kerkhofs H. , van der Linden J. S. M. , Schonewille H. , van der Sanden H. S. Gonggrijp , van der Meer  , Hermans J.
Published: August 2010

Bone marrow samples from 20 controls, 41 patients suffering from various types of myelodysplasia and 19 suffering from ANLL were investigated by in vitro cultures. The cultures were stimulated by various concentrations of leucocyte conditioned medium (LCM) and PHA‐stimulated conditioned medium (PCM) and were examined after 7 and 14 d. We found that, in clinically stable MDS, growth patterns and dose‐response to CM's were mostly within the normal range. With progressive blastic transformation, these features became abnormal with an increase in cluster growth. Clusters responding to a high dose of LCM, persisting after 14 d and enhanced by PCM may represent ‘early’ clonogenic cells. These clusters were found in progressive MDS with increased numbers of blast cells. Clusters formed by ‘late’ clonogenic cells were found in normal bone marrow and stable MDS. In ANLL the disturbance of proliferation and maturation seems to be much more pronounced than in progressive MDS with blastic transformation. We conlcude that the interpretation of in vitro bone marrow culture data in terms of a disorderly arrangement of clonogenic cells in MDS and ANLL is facilitated by comparing different conditioned medium stimulations and by scoring after different time intervals.
T‐cell chronic lymphocytic leukaemias and T‐cell lymphoma‐leukaemia: Heterogeneity and anomalous cell markers
Authors: Spiers A. S. D. , Lawrence D. A. , Levine Marilyn , Weitzman H.
Published: August 2010

We studied cell surface markers in 8 men with T‐cell chronic lymphocytic leukaemia, using fluorescein‐conjugated monoclonal antibodies and a cell analyser/sorter. 1 patient's cells had a T‐suppressor/cytolytic phenotype (T4‐, T8+) and in 3 patients the phenotype was a T‐helper cell (T4+, T8‐) as described in retrovirus‐associated adult T‐cell lymphoma‐leukaemia. In 4 patients, the cell phenotype was anomalous, with both helper and suppressor antigenic markers (T4+, T8+). Terminal deoxynucleotidyl transferase was positive in 1 of the 4 cases with dual markers. Clinical features and cytological characteristics, including cell size and the density of T8 antigen on the cell surface, showed no obvious correlation with the immunophenotypes, but study of additional cases is needed to evaluate this further.
The efficacy and toxicity of VAD in the treatment of myeloma and related disorders
Authors: Sheehan T. , Judge M. , Parker A. C.
Published: August 2010

Impressive results have recently been reported in 29 cases of advanced refractory myeloma treated with 4‐d infusions of Vincristine and Adriamycin (VAD). We report our own experience with this protocol in 13 cases of myeloma and related lympho‐proliferative disorders. In 2 of these cases, VAD was used as first line treatment. Objective responses were seen in two‐thirds of cases and the major complication of therapy was infection, predominantly bacterial in nature.
Clinical application of the chromogenic assay of factor VIII in haemophilia A, and different variants of von Willebrand's disease
Authors: Lethagen Stefan , Østergaard Hans , Nilsson Inga Marie
Published: August 2010

A chromogenic substrate kit for determination of factor VIII activity (COATEST Factor VIII) was compared to a one‐stage clotting assay and the correlation was evaluated in different genetic variants of mild and moderate haemophilia A, in severe haemophilia A and in all known variants of von Willebrand's disease. In all these cases a high correlation between the two methods was obtained. A good correlation was also obtained after intranasal administration of DDAVP (1‐desamino‐8‐D‐arginine vasopressin) to patients with von Willebrand's disease. The chromogenic substrate method was performed using a microtray technique.
Myeloperoxidase‐deficient polymorphonuclear leucocytes (VI): Relation to cytogenetic abnormalities in primary myelodysplastic syndromes
Authors: Bendix‐Hansen Knud , Kerndrup Gitte , Pedersen Bent
Published: August 2010

Relations between cytogenetic status, FAB‐classification and an abnormal subpopulation of myeloperoxydase (MPO)‐deficient polymorphonuclears (PMN) in 45 patients with myelodysplastic syndrome (MDS) are reported. Clonal abnormalities were demonstrated in 85% of the patients, with a lower incidence in the RA+ group (refractory anaemia with ring sideroblasts) compared to the others (p = 0.004). In 12 patients a spontaneous progression in cytogenetic aberrations occurred and in 7 of these (60%) a simultaneous progression in FAB‐subtype was seen. The appearance of MPO‐deficient PMNs was observed in 6 of these patients (55%). A progression in FAB‐subtype was noted in further 4 patients and 2 additional patients developed MPO‐deficient PMNs. Only one sufficient cytogenetic investigation was available in these patients. Thus 100% of the fully studied patients showed progression in cytogenetic abnormalities when a progression in FAB‐subtype or a development of MPO‐deficient PMNs was seen. 3 (49%) of the 8 patients developing MPO‐deficient PMNs too showed a progression in FAB‐subtype. Although no significant correlation to specific categories of structural aberrations or abnormalities in specific chromosome pairs could be demonstrated, clonal cytogenetic aberrations seem to be involved when the disease progresses and when MPO‐deficient PMN develop.
Monoclonal Bence Jones proteinuria in chronic lymphocytic leukaemia
Authors: Pezzoli Antonio , Pascali Enzo
Published: August 2010

A series of 52 consecutive patients with newly diagnosed chronic lymphocytic leukaemia (CLL) was investigated for the presence of serum and urine monoclonal immunoglobulins. The overall incidence of monoclonal gammopathy (MG) was 69%. Serum M components belonging to the IgG and IgM classes were found in 27% of cases with a concentration ranging from 1.7 to 40.3 g/l (mean 10 g/l). A monoclonal free light chain, i.e. Bence Jones protein (BJP), was documented in the urine of 65% of cases. In 42% of the 52 patients the urinary excretion of BJP represented the sole detectable monoclonal immunoglobulin abnormality.

The occurrence of serum monoclonal immunoglobulins was not found to correlate with the stage or progression of the disease. Conversely, the isolated urinary excretion of BJP showed a relationship with the tumour load, as evaluated in terms of clinical enlargement of lymphoid areas. The presence of BJP alone was also a major distinctive feature of patients with more aggressive disease. These preliminary data would suggest that the isolated urinary excretion of BJP may represent a parameter of clinical significance in evaluating patients with CLL.
Some characteristics of circulating erythrocytes in polycythaemia vera: Common features with normal young and foetal red blood cells
Authors: Streichman S. , Avdi N. , Joffe G. , Matathias L. , Tatarsky I.
Published: August 2010

Although it is well established that in polycythaemia vera (PV) both ‘normal’ and ‘abnormal’ erythroid progenitors proliferate, it is less known to what extent the circulating erythrocytes express normal characteristics. We found reduced erythrocyte densities, decreased MCHC, and increased lipid content. These properties, together with increased sialic acid, seem to explain the extremely low sedimentation rate and decreased deformability of polycythaemic blood samples. Other characteristics were high activity of glycolytic enzymes, increased in vitro production of lactate, and a concomitant decline in ATP and 2,3‐diphosphoglycerate. Some of these properties have been described in foetal erythrocytes and are features of normal young red cells. However, they seem to represent true features of PV and not a consequence of younger cell populations. The similarity between mature erythrocytes in PV and in foetal life supports the possibility that the proliferation process in this disease has a mechanism in common with foetal erythropoiesis.
Ifosfamide and etoposide as salvage therapy for non‐Hodgkin's lymphoma
Authors: Cavallin‐Stahl Eva , Lind Jack , Hagberg Hans
Published: August 2010

The result of a combination consisting of ifosfamide and etoposide was evaluated in 57 patients suffering from non‐Hodgkin's lymphoma (NHL) who had relapsed or were resistant to adriamycin‐containing combinations. Some of the patients (35%) also received methotrexate. 80% of the patients belonged to the histology group ‘high‐grade’ malignant NHL. 1times1 of the 57 patients (19%) obtained a remission, 5 of these were complete and 6 partial. The remission predominantly occurred in the group of patients with ‘high‐grade’ malignant histopathology who were treated at the first relapse after complete remission or when front‐line treatment induced a partial remission and ifosfamide and etoposide were added in direct connection with frontline treatment. It is concluded that the use of the combination of ifosfamide and etoposide should be limited to such patients.
Lack of correlation between iron stores and plasma lactoferrin concentration
Authors: Baynes R. D. , Bezwoda W. R. , Derman D. P. , Khan Q. , Mansoor N.
Published: August 2010

Plasma lactoferrin concentration and indices of iron nutrition including P‐Ferritin, P‐iron, total iron binding capacity and percentage saturation were measured in 75 otherwise normal females. This was done in order to evaluate previous reports that neutrophil lactoferrin content was altered by variations in iron storage status. Since P‐Lactoferrin is derived from and hence reflects neutrophil lactoferrin content it would seem reasonable to assume that P‐Lactoferrin would vary in accordance with iron stores. However, there was no statistically significant difference in P‐Lactoferrin concentration between iron deficient subjects and those who were iron replete. Furthermore, there was no statistically significant correlation between any iron related parameter and P‐Lactoferrin concentration.
Does the metabolite uracil arabinoside inhibit cytosine arabinoside (Ara‐C) penetration into the cerebrospinal fluid during high‐dose Ara‐C therapy?
Authors: van Prooijen Hendrik C. , Muus Petra , Roelofs Jan M. M. , Punt Kees
Published: August 2010

A patient with acute leukaemia was treated with i.v. 2‐h infusions of Ara‐C at a dose of 3.0 g/m2 every 12 h. During 6 d of therapy the concentrations of the metabolite Ara‐U in the CSF reached rather high levels of between 60 and 70 u.mol/1 from d 2–6 due to high levels of Ara‐U in the plasma. The concentration of Ara‐C in the CSF after the first infusion was 10.8 u.mol/1. After repetitive doses on d 2–6 the drug concentrations increased from about 3 nmol/1 just before infusion to about 8 umol/1 at the end of infusion, indicating inhibition of Ara‐C influx into the CSF during prolonged treatment. We suggest that the high levels of Ara‐U in the plasma interfere with Ara‐C transport across the blood‐brain barrier.
Halitosis among racially diverse populations: an update
Authors: Rayman S , Almas K
Published: August 2010

Abstract:  The aim of this paper is to highlight the cultural perceptions of halitosis to dental professionals. Halitosis (oral malodour or bad breath) is caused mainly by tongue coating and periodontal disease. Bacterial metabolism of amino acids leads to metabolites including many compounds, such as indole, skatole and volatile sulphur compounds (VSC), hydrogen sulphide, methyl mercaptan and dimethyl sulphide. They are claimed to be the main aetiological agents for halitosis. Gastrointestinal diseases are also generally believed to cause halitosis. In general, physicians and dentists are poorly informed about the causes and treatments for halitosis. The paper reviews the prevalence and distribution of halitosis, oral malodour, its aetiology, concepts of general and oral health and diseases and their perception among racially diverse population. Eating, smoking and drinking habits and understanding of halitosis as a social norm among different people has been highlighted. The treatment options have also been presented very briefly. A brief discussion about general importance within existing healthcare services has been highlighted. Oral malodour may rank only behind dental caries and periodontal disease as the cause of patient’s visits to the dentist. It is a public social health problem. The perception of halitosis is different in culturally diverse populations. So the dental professionals should be aware of the cultural perceptions of halitosis among racially and culturally diverse populations. There is a need to integrate the cultural awareness and knowledge about halitosis among the dental professional for better understanding of halitosis to treat patients with the social dilemma of halitosis to improve the quality of life and well‐being of individuals with the problem. It is concluded that dental professionals (especially dental hygienists) should be prepared to practice in a culturally diverse environment in a sensitive and appropriate manner, to deliver optimal oral health and hygiene care.