bluuebunny/crossref_metadata_2025 huggingface dataset:
Maturation changes in relapsing acute myeloid leukaemia
Authors: van der Weide M. , van Rhenen D. J. , Langenhuijsen M. M. A. C.
Published: August 2010

Smears of peripheral blood and bone marrow from 17 patients with acute myeloid leukaemia in relapse were compared with smears made at presentation. We found a change of more than 10 in Maturation Index (MI) in the bone marrow smears of 14 out of 17 patients (82%). Decreases and increases were equally represented. In 5 cases the changes caused assignment to another FAB‐group.
Significance of multiple types of antibodies on red blood cells of patients with positive direct antiglobulin test: A study of monospecific antiglobulin reactions in 85 patients
Authors: Ben‐Izhak Cfer , Shechter Yael , Tatarsky Ilana
Published: August 2010

Blood samples from 85 patients with a positive direct antiglobulin test were tested with monospecific antiglobulin reagents: anti‐IgG, anti‐IgM, anti‐IgA, and anti‐C3. No typical pattern of antiglobulin reaction could be correlated with specific diseases except for the patients with methyldopa‐induced positive direct antiglobulin test, all of whom had only IgG on their red blood cells. The presence of more than 1 type of antibody on red blood cells was associated with severe haemolysis. These patients responded less frequently to steroids, and in most of them no underlying disease could be found. Most patients with complement alone on red blood cells had no evidence of haemolysis, and when present it was never severe.
Non‐Hodgkin's lymphomas in leukaemic phase: Incidence, prognosis and therapeutic implications
Authors: Tura Sante , Mazza Patrizio , Lauria Francesco , Fiacchini Mauro , Pileri Stefano , Poletti Giovanni , Gherlinzoni Filippo , Cantore Maurizio , Bandini Giuseppe
Published: August 2010

248 patients with non‐Hodgkin lymphomas (NHL) were retrospectively analysed in an attempt to elucidate the risk factors, the prognostic importance and the therapeutic implications of blood involvement. Bone marrow involvement and large spleen were significantly correlated to leukaemic manifestations (P <0.0001 and P <0.0005, respectively); conversely no correlations were seen with bulky disease and symptoms. Among low‐grade malignant lymphomas (LGML) centroblastic‐centrocytic follicular and diffuse or diffuse and “CLL” subtypes were mostly associated with blood involvement (31% and 55%, respectively). Among high‐grade malignant lymphomas (HGML) lymphoblastic type is more frequently associated with blood involvement (42%) than the other subtypes. Blood involvement was not clearly correlated with the prognosis either in LGML (median survival 39 and 36 months for leukaemic and non‐leukaemic patients, respectively) or HGML (median survival 12 and 18 months, respectively), although a shorter survival of leukaemic than non‐leukaemic lymphoblastic lymphoma was observed (median survival 8 months versus 14 months, respectively). The poorer response rate to therapy of leukaemic patients (median duration of CR 22 and 5 months in LGML and HGML, respectively) as opposed to non‐leukaemic patients (median duration of CR 29 and 23 months, respectively) led us to consider an alternative treatment in such patients.
Immunologic subsets in B cell lymphomas defined by surface immunoglobulin isotype and complement receptor — Their relationship to survival
Authors: Kvaløy Stein , Langholm Ruth , Kaalhus Olav , Marton Per F. , Høst Herman , Godal Tore
Published: August 2010

Cell surface marker profiles were studied on cell suspensions from monoclonal B cell lymphomas. Surface immunoglobulin (sIg) was examined in 178 cases, whereas combined data with sIg and receptors for complement (C3) were available in 99/178 cases. The results showed that B cell lymphomas can be divided into distinct immunological subsets according to surface marker expression. Whereas some histologic subgroups (diffuse centroblastic/centrocytic, centrocytic, immunocytoma) (Kiel classification) consisted of few immunological subtypes, others were more heterogeneous (follicular centroblastic/centrocytic, centroblastic, lymphocytic). By combining immunological and histological subgroups, more than 40 phenotypes could be identified; this diversity most likely reflects, the heterogeneity of the B cell compartment.

As part of the same study the prognostic significance of cell marker phenotypes was examined. Survival analysis undertaken on 149/178 patients did not uncover any significant relationship between type of heavy or light chain expression, C3 receptor expression and clinical outcome. Our data do not confirm recent findings that the type of immunological phenotype may be of prognostic significance.
Diamond‐Blackfan syndrome: A possible role of cellular factors for erythropoietic suppression
Authors: Sawada Ken‐ichi , Koyanagawa Yoshinori , Sakurama Shoki , Nakagawa Shoichi , Konno Takahiko
Published: August 2010

CFU‐E growth from fractionated bone marrow cells of 5 children with Diamond‐Blackfan syndrome was studied. In all patients, CFU‐E growth was reduced in mononuclear cell‐rich fraction. In 2 of the 5 patients, CFU‐E growth was returned to normal by the depletion of E‐rosette forming cells or monocytes from mononuclear cell‐rich fraction. In the patient whose CFU‐E growth returned to normal by the depletion of E‐rosette forming cells, co‐cultivation between bone marrow buffy coat cells and autologous bone marrow E‐rosette forming cells resulted in a significant decrease of CFU‐E growth, and there was a significant increase in CFU‐E growth by treatment with monoclonal antiserum to OKT 4. We concluded that immunologic causes such as cellular factors may play a role, at least in part, in the pathogenesis of Diamond‐Blackfan syndrome.
The in vitro effect of leucocyte α‐interferon on human myeloma cells in a semisolid agar culture system
Authors: Brenning Gunilla
Published: August 2010

73 bone marrow samples from a total of 50 patients with multiple myeloma (MM) were tested for interferon (IFN) sensitivity in the human tumour colony assay (HTCA). 16 evaluable samples were obtained from untreated patients, 10 from patients during melphalan and prednisone treatment and 6 from patients on IFN treatment or after withdrawal of IFN. The sensitivity to IFN was individually distributed between the bone marrow samples from patients of alle 3 groups and a tendency towards IFN resistance could be found in serial assays from patients during MP‐treatment and after IFN withdrawal. Stimulation of growth was found in 31% of the cultures, usually at lower doses of IFN (10–100 U/ml) while higher doses (400–4000 U/ml) inhibited growth in 75% of the assays.
Zinc deficiency and blood lymphocyte function with sickle cell disease
Authors: Daeschner Charles W. , Carpentieri Ugo , Goldman Armond S. , Haggard Mary E.
Published: August 2010

A relationship between zinc deficiency and lymphocyte function in children with sickle cell disease (SCD) has been suggested. Number and function of B and T lymphocytes were assessed in 3 matched groups of children: normal subjects with Hb A and normal zinc; patients with SCD; and normal zinc (SCD‐N); and patients with SCD and decreased zinc (SCD‐D). Percentages of B and T cells, response to cutaneous antigens and increases in tetanus antibody titres were similar among all groups. Absolute numbers of WBC, lymphocytes and B and T cells were markedly increased in SCD‐N (p < 0.001) and to a lesser degree in SCD‐D (p < 0.01). Controls and SCD‐N had a normal response to all mitogens, which was not inhibited by SCD‐D sera. SCD‐D had a depressed response to PHA (p < 0.001), which was not corrected by zinc addition in vitro. These findings indicate that B cell function and T cell‐dependent delayed hypersensitivity are normal in children with SCD and are independent of body zinc status. They also suggest some abnormality of T helper cells in the presence of zinc deficiency, and in the absence of a demonstrable serum inhibitor.
Splenic function in children with sickle cell disease: Two different patterns in Saudi Arabia
Authors: Babiker M.A. , El‐Hazmi M.A.F. , Al‐Jobori A.M. , Obeid H. , Bahakim H.M.
Published: August 2010

Splenic function in 35 Saudi children homozygous for sickle cell disease (age range 3–9 years) was studied using radioactive colloid scans. Two different patterns emerged. Splenic dysfunction was demonstrated in more than 80% of children who were originally from the south‐western part of the country. They were found to have low HbF levels. In contrast normal or nearly normal splenic function was found in all patients from the Eastern Province in whom HbF levels were high. These different patterns of splenic function may contribute to the severe and mild forms of sickle cell disease seen in Saudi Arabia.
Development of chronic lymphocytic leukaemia in a case of Sjögren's syndrome with systemic lupus erythematosus
Authors: Ho Chao‐Hung , Chiang Yu‐Ming , Chong Lai‐Lu , Lin Hsiao‐Yi , Hwang Tien‐Szu
Published: August 2010

Persistent lymphocytosis and intermittent fever were found in a 68‐yr‐old Chinese woman 5 yr after the diagnosis of Sjögren's syndrome with systemic lupus erythematosus (SLE). A series of examinations ‐ including virology, bone marrow aspiration and surface markers of lymphocytes ‐ was made to evaluate the nature of the lymphocytosis which had not been found previously. All of the results were consistent with the diagnosis of B‐cell chronic lymphocytic leukaemia (CLL). Development of CLL in Sjögren's syndrome has seldom been described before and mey be added to other malignancies associated with Sjögren's syndrome.
Limited value of serum ferritin in evaluating iron status in children with protein‐energy malnutrition
Authors: Wickramasinghe S.N. , Gill D.S. , Broom G.N. , Akinyanju O.O. , Grange Adenike
Published: August 2010

26 of 46 Nigerian children with protein‐energy malnutrition (PEM) had elevated S‐Ferritin levels; the geometric mean value in the entire group of children with PEM was 146 μg/l and the observed range 11–7000 μg/l. There was no statistically significant correlation between the logarithm of the S‐Ferritin level and the amount of stainable iron in marrow fragments (r=0.23; p > 0.2). A stepwise multiple linear regression analysis in which the logarithm of the S‐Ferritin level was used as the dependent variable and a total of 17 clinical, biochemical and haematological parameters were used as the independent variables showed that 34.8% of the variability in S‐Ferritin could be accounted for by variations in the percentage of lymphocytes in the bone marrow. We speculate that the latter parameter may be an index of previous infection and that the elevated S‐Ferritin levels may, therefore, be at least partly caused by infections. Increased serum aspartate transaminase activities were also encountered in PEM, suggesting that hepatocellular damage may contribute to the high S‐Ferritin levels in this condition. Our data indicate that S‐Ferritin has a limited value as an indicator of iron status in subjects with PEM, presumably because of the frequency of infections and of hepatocellular damage in such subjects.
T‐cell prolymphocyte leukaemia: A clinical and immunological study
Authors: Lauria F. , Foa R. , Raspadori D. , Motta M. R. , Tazzari P. L. , Biagini G. , Preda P. , Algeri R. , Tura S.
Published: August 2010

2 cases of T‐cell prolymphocytic leukaemia (T‐PLL) were investigated for their reactivity with a series of monoclonal antibodies (MoAbs) as well as for the cytochemical expression and functional activity of the pathological cells. Both patients showed morphological (large cells with abundant cytoplasm and eccentric and irregularly shaped nucleous with large and prominent nucleoli) and clinical (high leucocyte count and splenomegaly) features typical of T‐PLL. The cells from 1 patient expressed a helper/inducer phenotype (T4+, T8‐) and were reactive with the anti‐Tac (interleukin‐2 receptor) MoAb, while the other case co‐expressed both the T4 and the T8 antigens. The response to phytohaemagglutinin and the natural killer activity (assessed by 51chromium release) were significantly reduced in both cases, while the helper capacity, tested in a pokeweed mitogen‐driven system, was maintained only in the 1st case. This latter case which expressed a more mature phenotype (T4+, T8‐) responded well to chemotherapy.
Quantification of the distribution of the marginating granulocyte pool in man
Authors: Peters A. M. , Saverymuttu S. H. , Bell R. N. , Lavender J. P.
Published: August 2010

The kinetics of autologous granulocytes, separated from whole blood and labelled with 111In‐tropolonate with continuous maintenance in plasma, have been studied in man, using a gamma camera and computer, with the aim of quantifying the distribution of the marginating granulocyte pool (MGP). We have used 3 approaches: dynamic gamma camera imaging immediately following i.v. injection of labelled cells, comparison of the activity signal from 111In‐granulocytes with that from previously injected 111In‐labelled red cells and absolute quantification of 111In present in liver, spleen and blood.

Deconvolution analysis of the hepatic and peripheral blood time activity curves indicated that hepatic granulocyte transit time was 2.5± 0.14 min. By comparison with 111In red cells, hepatic transit time was calculated to be 7.4± 0.82 that of red cells, which, assuming an hepatic red cell content of 6% that of the total red cell mass, is equivalent to a transit time of 1.8 min. By comparison with 111In red cells, lung granulocyte transit time as a factor of red cel transit time was 5.4± 0.7 at 5 min and 2.5± 0.13 at 40 min after granulocyte injection. Using these kinetic data, in combination with previously published values for splenic granulocyte transit time, it was calculated that, 5 min after injection, the MGP accounted for 54% of the total blood granulocyte pool (TBGP), was 90% filled, and was distributed between spleen (19%), liver (26%), lung (33%) and the remainder of the body (22%). At 40 min, the MGP accounted for 60% of the TBGP, had equilibrated with the circulating granulocyte pool (CGP), and was distributed between the spleen, liver, lung and remainder of the body according to the following respective percentages: 35, 25, 10 and 30.

The total granulocyte contents of the spleen and liver calculated on the basis of the kinetic data were 21% and 22% respectiely and in broad agreement with the values, 34 and 23% respectively, calculated from quantitative scanning.

It was concluded that about 70% of the body's MGP was present in the spleen, liver and lung. If the MGP is itself 60% of the TBGP then only about 18% of the TBGP marginates in extra‐hepatosplenopulmonary sites.
Iron absorption from red and white wines
Authors: Bezwoda W. R. , Torrance J. D. , Bothwell T. H. , Macphail A. P. , Graham B. , Mills W.
Published: August 2010

Iron (3 mg) was added as ferrous sulphate to 2 dl red wine, white wine and 7 % alcohol and its absorption was then measured in 38 fasting male subjects. (The original concentrations of iron in the two wines were low, being 1.01–1.08 mg/l (red wine) and 0.13–0.20 (white wine)). The geometric mean absorption from red wine was only 20 % of that from the alcohol solution whilst more than 4 times as much was absorbed from white wine as from the alcohol. Direct comparison showed greater absorption from white wine (10.4 %) than from red wine (4.4 %). Removal of about 80 % of the polyphenols in red wine increased the geometric mean iron absorption from 1.9 % to 3.6 %. In vitro experiments indicated that iron was less soluble and less dialysable in red wines than in white wines. This was possibly due to the binding of iron to polyphenols in red wines. Electrophoretic studies suggested that the iron in white wines was complexed to hydroxy‐carboxylic acids.
Procoagulant cellular activity and disseminated intravascular coagulation in acute non‐lymphoid leukaemia
Authors: Guarini Anna , Gugliotta Luigi , Timoncini Claudia , Chettai Leonarda , Catani Lucia , Russ Domenico , Tura Sante
Published: August 2010

The procoagulant cellular activity (PCA) of intact and lysed leukaemic cells was evaluated at diagnosis in 23 patients with acute non‐lymphoid leukaemia (ANLL). The leukaemic cells of all 13 patients having DIC feature (excess of fibrin monomers, serum FDP and plasma fibrino‐peptide A) showed a significant (P<0.0001) increase of PCA, while a pattern similar to that of normal granulocytes and lymphomonocytes was observed in the remaining 10 patients without evidence of DIC. When the patients were subdivided according to the FAB cytological classification, features of DIC and increased PCA were demonstrated in 3/3 M3 patients, 5/6 M5 patients and only in 5/14 remaining patients. These findings indicate that in ANLL patients: (1) the increased PCA of leukaemic cells is closely related to the occurrence of DIC; (2) the increased PCA seems related to the differentiation line and maturation level of the leukaemic cells.
How many plasma exchanges to cure thrombotic thrombocytopenic purpura?
Authors: Joneau Michele , Cordonnier Catherine , Vernant Jean‐Paul , Touzet Catherine , Sobel Alain
Published: August 2010

7 patients were treated by plasma exchanges and antiplatelet drugs for thrombotic thrombocytopenic purpura (TTP). The effectiveness of therapy was reviewed daily and retrospectively estimated on day 3, d6, d9, and d12. 6 of these 7 patients were cured. The low predictive value of the initial response to therapy is underlined. The clinical and biological status on d12 seems to have the best predictive value for the final outcome. These data encourage us to continue the plasma exchanges until d12, whatever the initial response to therapy in TTP.
Association of Gaucher's Disease and lymphoid malignancy in 2 children
Authors: Burstein Y. , Rechavi G. , Rausen A. R. , Frisch B. , Spirer Z.
Published: August 2010

2 cases of acute lymphoblastic leukaemia (ALL) of childhood in association with Gaucher's Disease (GD) are presented, which are the first to be described. One of them is a common non‐B non‐T cell ALL and the other is at T‐cell ALL. The association of GD with benign and malignant B‐cell proliferation has recently been described. It is suggested that chronic stimulation of the immune system by the accumulated glucocerebroside may predispose to B cell proliferation.
Assessment of the role of an immunofluorescent terminal deoxynucleotidyl transferase kit
Authors: Dunbar D. R. , Smith C. , Thornton J. A. , Waters H. M. , Hyde K. , Delamore I. W.
Published: August 2010

The laboratory use of a commercial kit for the immunofluorescent measurement of terminal deoxynucleotidyl transferase (BRL Terminal Deoxynucleotidyl Transferase Immunofluorescent Assay System) has been investigated. The precision of the assay system with stored patient material and its performance with regard to ease of microscopic examination were assessed. The intensity of the fluorescence obtained was found to vary. However, the precision of the assay system on fixed slides stored at ‐30°C was satisfactory; the method simple, and results obtainable in a relatively short space of time.
Meningeal involvement in non‐Hodgkin's lymphoma: Symptoms, incidence, risk factors and treatment
Authors: Ersbøll Jens , Schultz Henrik B. , Thomsen Birthe L. R. , Keiding Niels , Nissen Nis I.
Published: August 2010

Meningeal involvement (MI) by non‐Hodgkin's lymphoma (NHL) was seen in 38/602 patients (6.3%). In relation to histologic subtype the frequency of MI was: Follicular small cleaved and mixed cell 2/128 (1.6%), small lymphocytic and diffuse small cleaved cell 2/83 (2.4%), large cell and immunoblastic 13/295 (4.5%), small noncleaved cell 6/31 (19%), lymphoblastic 15/66 (23%). Risk factors that predict for MI were, besides histologic subtype, age under 40 yr, clinical stage IV, site of involvement (bone marrow, bone, skin gastrointestinal tract), and a poor response to initial therapy. In a Cox multivariate model encompassing the intermediate and high grade malignancy groups of the Working Formulation (WF), the 3 most important risk factors were histology, age, and stage. The estimated 1‐yr probability of MI for combinations of the 3 risk factors was: 3 risk factors (61 %), 2 risk factors (15–28%), 1 risk factor (4–8%), 0 risk factor (1.5%). At the diagnosis of MI, 84% of the patients had evidence of advanced systemic NHL, and the median survival after MI was 10 wk. CNS prophylaxis with whole‐brain irradiation and intrathecal chemotherapy can only be recommended in patients with 2 or 3 risk factors. Improvement of the systemic chemotherapy might be the most important factor for prevention of MI in NHL.
Serum ferritin and Hodgkin's disease
Authors: Bezwoda W. R. , Derman D. P. , Bothwell T. H. , Baynes R. , Hesdorffer C. , MacPhail A. P.
Published: August 2010

The haemoglobin, serum iron, transferrin saturation, serum ferritin, erythrocyte sedimentation rate (ESR), splenic weight and non‐haem iron concentration in the marrow, liver and spleen were measured prior to treatment in 35 patients with Hodgkin's disease who underwent staging laparatomy. The Hb, serum iron and transferrin saturation showed a significant decrease with increasing stage of the disease. In contrast, there was a significant increase in the serum ferritin, ESR, splenic weight and in all the tissue non‐haem iron concentrations. The calculated total iron content of the body remained relatively constant throughout at about 2 g but with increasing stage there was an internal redistribution of iron, with a progressive drop in Hb iron and a reciprocal rise in storage iron, especially in the liver. Serum ferritin concenrrations, which rose with progression of the disease, were inappropriately high in relation to the size of body stores at all stages but especially in patients with 4B disease and hepatic involvement. It was concluded that the serum ferritin concentrations are raised for several reasons in Hodgkin's disease. They reflect an increase in body iron stores, ferritin's role as an ‘acute phase’ protein in the inflammatory response and hepatic damage in patients with advanced disease.
Primary plasma cell leukaemia: immunological and ultrastructural studies in 6 cases
Authors: Parreira A. , Robinson D. S. F. , Melo J. V. , Ayliffe M. , Ball S. , Hegde U. , Baughan A. , Fairhead S. , Talavera J. G. , Katzmann J. A. , Catovsky D.
Published: August 2010

The clinical and laboratory studies of 6 patients with primary plasma cell leukaemia are described. The leukaemic cells had a variable morphology, ranging from lymphoplasmacytic and mature plasma cells to poorly differentiated blasts. The neoplastic plasma cells had a characteristic phenotype: they were positive for CyIg and the McAb OKT10 and Ri‐3, and did not express the B‐cell antigens Ia, B1 and B4. Ultrastructural studies confirmed the plasma cell nature of the leukaemic cells and showed the presence of a meshwork of cytoplasmic fibrils in 50 to 90% of the neoplastic cells from all cases. The distinct ultrastructural and immunological features of PCL described in this study will help the diagnosis and further characterisation of this disease entity.
Cerebellar dysfunction during high dose cytosine arabinoside therapy in a case of acute myelogenous leukaemia
Authors: Cold Søren
Published: August 2010

A case of persistent cerebellar toxicity following systemic high dose cytosine arabinoside (HDCA) treatment of acute myelogenous leukaemia is reported. The symptoms, which developed at a cumulative dose of 32 g/m2, subsided to some extent following discontinuation of the drug, but left signs of cerebellar dysfunction 10 months later. A review of previously published reports indicates that the cerebellar toxicity of HDCA, which has been ascribed to loss of Purkinje cells, usually occurs when the accumulated dose exceeds 36 g/m2. That it may occur at even lower doses is supported by the present case. In addition, available evidence of re‐appearance or worsening of previously induced signs of toxicity following its repeated administration stresses the importance of immediate and permanent cessation of cytosine arabinoside therapy, when signs of cerebellar dysfunction develop.
Therapy‐induced myelodysplasia and secondary leukaemia
Authors: Frisch B. , Bartl R. , Chaichik S.
Published: August 2010

A study was performed on bone marrow histology in patients before, during and at various time intervals after chemotherapy and/or radiotherapy for solid tumours, Hodgkin's disease, multiple myeloma and acute leukaemias. Alterations in both haematopoietic and stromal compartments (myelodysplasia) were found in varying degrees of severity depending on the type and the time elapsed from the treatment.

The tumour‐induced and post‐therapy myelodysplasia, restitution phase, pre‐leukaemic phase, and therapy‐induced leukaemia and aplasia are described. The findings are discussed in the light of current theories of the mechanisms of therapy‐induced myelodysplasia and acute leukaemia.
Cytogenetics of the myelodysplastic syndromes
Authors: Borgström Georg H.
Published: August 2010

Chromosome aberrations occur in 30–50 % of patients with myelodysplastic syndromes (MDS). Chromosome aberrations found in MDS are generally found in acute non‐lymphocytic leukaemia (ANLL), while certain aberrations of ANLL do not occur in MDS. The clinical significance of these aberrations has been evaluated on the basis of several series from literature and a large series from Helsinki University Central Hospital. The presence of an aberrant clone with major karyotypic aberration (MAKA) or monosomy 7 in a patient with MDS indicates a higher risk of transformation to acute leukaemia and a shorter survival.
Primary myelodysplastic syndrome: Treatment of 6 patients with 13‐cis retinoic acid
Authors: Kerndrup Gitte , Bendix‐Hansen Knud , Pedersen Bent , Ellegaard Jørgen , Hokland Peter
Published: August 2010

6 patients with primary MDS were treated with 20 mg/m2 13‐cis retinoic acid (13‐RA) (RoacutaneR, Roche, Basel) daily for 6 weeks and with an additional 100 mg/m2 daily for 4 weeks (3 patients). 4 patients responded either with an increase in peripheral blood neutrophil count (2 patients) or with a decrease in MPO‐deficient neutrophils (2 patients). These effects were not seen until 3 weeks after the onset of the treatment and they were accompanied by an increase in the growth of day 11 granulocytemonocyte colonies, and, in the patients showing clonal cytogenetic abnormalities, by a disappearance of minor clonal abnormalities during the treatment. These findings may be the result of a stimulation of the growth of normal or only slightly defective bone marrow progenitor cells in combination with a differentiational effect.

The clinical side effects were limited to hyperkeratosis and dryness of mucous membranes in 5 patients. I patient developed a dermatitis. None showed signs of hepatotoxicity. Because of the pronounced subjective discomfort experienced by the patients receiving 100 mg/m2, it seems recommendable to treat patients with MDS with low dosages of 13‐RA (10 to 20 mg/m2) for longer periods (3–6 months) in order to evaluate the effect of 13‐RA on the long term basis.
Non‐Hodgkin malignant lymphomas and Hodgkin's disease in first‐degree relatives
Authors: Bjerrum Ole W. , Hasselbalch Hans C. , Drivsholm Aage , Nissen Nis. I.
Published: August 2010

The occurrence of malignant lymphomas in 3 siblings is described. The cases include the first report of Hodgkin's disease and non‐Hodgkin malignant lymphoma in dizygotic twins. Another remarkable feature was the development of malignant lymphoma, when the siblings were at the same age. Although a transmissible agent or environmental factors cannot be excluded in this family, our observations may suggest a mutual genetic predisposition in Hodgkin's disease and non‐Hodgkin malignant lymphoma.
Twin pregnancy and parturition in a patient with the Bernard Soulier Syndrome
Authors: Heslop H. E. , Hickton C. M. , Laird E. , Tait J. D. , Doig J. R. , Beard M. E. J.
Published: August 2010

A patient with the Bernard Soulier Syndrome was successfully delivered of twins. The pregnancy was complicated by slight bleeding from the uterus during the first 8 weeks, by cord prolapse necessitating emergency caesarean section, and by moderate post partum haemorrhage. The management of pregnancy in the Bernard Soulier Syndrome is discussed.
Accessory spleen imaging
Authors: Hansen Sören , Järhult Johannes
Published: August 2010

A 42‐yr‐old man presented with thrombocytopenia 25 yr after splenectomy for idiopathic thrombocytopenic purpura (ITP). Preoperative investigations with 99Tc‐colloid scan, ultrasound and computed tomography (CT) revealed 2 accessory spleens. The patient recovered completely after splenunculectomy. CT was superior to radionuclide and ultrasonography in describing the exact location of the 2 spleens, thereby facilitating the operative procedure.
Effects of a hemoregulatory peptide (HP5b) on erythroid and myelopoietic colony formation in vitro
Authors: Kreja Ludwika , Hågå Per , Muller‐Bérat Nicole , Laerum Ole Didrik , Sletvold Olav , Paukovits Walter R.
Published: August 2010

A hemoregulatory peptide (HP5b) associated with mature human granulocytes has been investigated for inhibitory effects on murine hemopoietic stem cells in vitro. Both highly purified peptide from natural sources and a synthetic analog peptide have been investigated in parallel. Strong inhibitory effects were found on myelopoietic colony formation in the dose range 10−13‐10−5 mol/l. On exceeding this dose, the inhibitory effect disappeared. Moderate to slight inhibitory effects on erythroid colony formation (BFU‐E and CFU‐E) from adult animals were only seen in 1000 x the optimal doses for myelopoiesis. No effect was found on CFU‐E from fetal liver. The peptide thus has a selective effect on myelopoiesis in a certain dose range. A regulatory mechanism for the peptide on hemopoiesis is proposed.
Effect of splenectomy on circulating haematopoietic progenitors in myelofibrosis
Authors: Partanen Seija , Ruutu Tapani , Juvonen Eeva , Pantzar Pekka
Published: August 2010

The effect of splenectomy on peripheral blood CFU‐GM and BFU‐E numbers was studied in a patient with myelofibrosis. The numbers of CFU‐GM and BFU‐E were twice as high in the splenic venous blood as in the peripheral blood. The number of CFU‐GM in peripheral blood decreased to 20% and that of BFU‐E to 7% of the presplenectomy level at 1 wk after the operation. The progenitor numbers recovered to reach the presplenectomy level within 4–6 w after splenectomy. This effect of splenectomy was different from that seen in 3 patients with Hodgkin's disease, in whom increased numbers of progenitors were observed after a small initial notch.
A new form of coagulation factor VII in plasma
Authors: Dalaker Knut , Skartlien Anne Helen , Prydz Hans
Published: August 2010

We have reported the existence of a novel form of coagulation factor VII ‐ probably a factor VII‐phospholipid complex ‐ in plasma from pregnant women and men at risk for cardiovascular disease. We report here further observations on the presence and characteristics of this complex. Some apparently healthy individuals who, on testing by standard methods, have normal levels of factor VII activity achieve such levels by means of a phospholipase C‐sensitive modification of (some of) their factor VII molecules. Their residual factor VII activity after phospholipase C treatment of plasma may be as low as 10–20 U/ml. Antiserum to the protein component of thromboplastin (apoprotein III) had no effect on the factor VII activity, whereas antiserum to factor VII effectively blocked both the total factor VII activity and the residual activity of factor VII after treatment of plasma with phospholipase C. These factor VII complexes precipitate with the VLDL/LDL fraction in lipoprotein precipitations.
Correlation between donor cytomegalovirus immunity and chronic graft‐versus‐host disease after allogeneic bone marrow transplantation
Authors: Jacobsen Niels , Andersen H. Kerzel , Skinhøj Peter , Ryder Lars P. , Platz Per , Jerne Donald , Faber Viggo ,   For the Copenhagen Bone Marrow Transplantation Group
Published: August 2010

Chronic graft‐versus‐host disease (GvHD) in bone marrow allograft recipients is frequently preceded by a cytomegalovirus (CMV) infection. To elucidate whether an immune reaction of transplanted donor cells against CMV was involved in the pathogenesis of chronic GvHD, the effect of donor pretransplant CMV immune status on chronic GvHD incidence was analyzed. In 43 bone marrow recipients at risk, the 2‐yr cumulative chronic GvHD probability was 55% when the donor was immune, in contrast to 16.5% when the donor was non‐immune (p < 0.002). No correlation between recipient pretransplant CMV immunity and chronic GvHD was observed. Donor CMV immunity did not correlate with acute GvHD or posttransplant CMV infection and seemed to predispose for chronic GvHD regardless of donor and recipient age. However, the proportion of CMV immune donors increased with increasing donor and recipient age. This may account for the higher incidence of chronic GvHD in older patients. The present study suggests that chronic GvHD may be mediated by a reaction of immune donor cells against CMV infected recipient cells.
Glycolytic activity in human red cell populations separated by a combination of density and counterflow centrifugation. Evidence for an improved separation of red cells according to age
Authors: Jansen G. , Hepkema B. G. , van der Vegt S. G. L. , Staal G. E. J.
Published: August 2010

Human red blood cells were separated by a discontinuous Percoll density gradient into fractions of increasing density. Red cells comprising the lowest and highest density fractions, respectively, were subsequently separated according to mean cell volume (MCV) by means of counterflow centrifugation. The activities of 4 red cell age‐dependent enzymes (hexokinase (HK), pyruvate kinase (PK), glucose‐6‐phosphate dehydrogenase (G6PD) and aspartate aminotransferase (ASAT)) were highest in the red cell fraction with low density/large MCV, although the difference from red cell enzyme activities in the total low density fraction was not significant. These 4 enzyme activities were lowest in the fraction of red cells with high density/small MCV. The relative activities of the enzymes in the high density/small MCV fraction, as compared to the unseparated cell population, were: HK (58%), PK (49%), G6PD (53%) and ASAT (28%). These activities were all significantly lower than those measured in the total high density red cell fraction. The rates of lactate production in the low density/large MCV cells (0.89 ± 0.15 μmol.min1.10−1 cells) is approximately 3‐fold higher than in high density/small MCV cells (0.33 ± 0.03 μmol.min−1 .10−11 cells). This latter value is 1.8‐fold lower than the rate of lactate production in the total high density red cell fraction (0.59 ± 0.14 μmol.min−1.10−11 cells) and is, in contrast to lactate production in other density/size fractions, insensitive to phosphate as a metabolic stimulus. It is argued that the combination of density gradient and counter‐flow centrifugation offers a greater potential for obtaining an old red cell population than classical red cell density centrifugation alone.
Factor‐specific deactivation of leucocyte Chemotaxis in vivo
Authors: Briheim Gunnar , Stendahl Olle , Dahlgren Claes
Published: August 2010

This report analyses the locomotory capacity of polymorphonuclear leucocytes (PMNL) isolated from 7 patients with bacterial meningitis. 7 healthy control subjects were also investigated in parallel. It was found that PMNL from the patients suffering from meningitis, isolated both from peripheral blood and from the cerebrospinal fluid, had lost their ability to respond chemotactically to activated serum but not to the chemotactic peptide f‐Met‐Leu‐Phe. The normal chemotactic responsiveness of blood PMNL was restored once the patients recovered from infection.
Evidence that 111In‐labelled platelets pool in the spleen, but not in the liver of normal humans and baboons
Authors: Kotzé H. F. , Heyns A. du P. , Wessels P. , Pieters H. , Badenhorst P. N. , Lötter M. G.
Published: August 2010

The kinetics of pooling of platelets in the spleen may be used to measure spleen function. However, pooling of platelets in other organs, especially the liver, may affect these kinetic measurements. We have therefore compared, in man and baboon, the kinetics of 111In‐labelled platelets and 111In‐labelled red cells in the liver and spleen during the first 60 min after reinjection. This was determined in vivo with a scintillation camera and computer‐assisted image analysis. Organ radioactivity was expressed as a percentage of that of the whole body. In both humans and baboons, the spleen accumulated many more platelets than red cells. Also, the red cells equilibrated more rapidly. The presence of a splenic platelet pool was thus confirmed. In contrast, the percentages of labelled platelets and labelled red cells in the liver were similar in both species. In both species, the sum total of labelled platelets in the circulation, i.e. recovery, and that quantified in the spleen at equilibrium, were equal to approximately 100%. These results confirm the presence of the exchangeable splenic platelet pool, and indicate that there are no significant exchangeable platelet pools in either the liver or any other organs.
Activation of malignant B‐lymphocytes: pathophysiologic and clinical importance
Authors: Robèrt Karl‐Henrik , Juliusson Gunnar , Einhorn Stefan , Biberfeld Peter , Gahrton Gösta
Published: August 2010

Recent advances in clarifying the activation mechanisms of the normal immune system have provided the basis for successful experiments concerning the activation of malignant lymphocytes. Such studies with malignant cells, freshly sampled from patients with lymphoproliferative diseases, can be used for pathophysiological considerations, analysis of tumor evolution, classification of malignant subsets, and karyotyping. Of particular interest is the possibility of abrogating the maturation arrest in some clonally‐restricted cells. This might lead to therapeutical implications, since the differentiation blockage plays a fundamental role in the clonal expansion and pathogenesis of tumors. Recently, it has been shown that interferon (IFN) can be a potent inducer of various degrees of transformation, differentiation and even proliferation in different subsets of normal and malignant B cells. This may be important in explaining the divergent results of IFN treatment in various malignant B‐cell disorders.
Some parameters of haem synthesis in dialysed and non‐dialysed uraemic patients
Authors: Yalouris A. G. , Lyberatos C. , Chalevelakis G. , Theodosiadou E. , Billis A. , Raptis S.
Published: August 2010

Some parameters of haem synthesis were estimated in 60 uraemic patients (30 nondialysed, 30 dialysed) and in 30 matched controls. Serum δ‐aminolaevulinic acid and erythrocyte coproporphyrin and protoprophyrin were found significantly higher in the non‐dialysed uraemics than in the controls. Erythrocyte δ‐aminolaevulinic acid dehydrase (ALA‐D) activity was 498 ± 174 μmol/h.l in the non‐dialysed patients, 321 ± 146 in the dialysed (just before haemodialysis) and 833 ± 281 in the healthy controls, the differences between these groups all being statistically significant (p < 0,001). After haemodialysis the enzymic activity in the dialysed group increased significantly (380 ± 167, p < 0.001), but remained lower than normal (p < 0,001). A similar pattern ‐ although with less statistical significance of the differences between groups ‐was observed concerning erythrocyte uroporphyrinogen I synthase activity. Incubation of normal erythrocytes with uraemic plasma resulted in a considerable decrease of their ALA‐D activity (from 830 ± 263 to 616 ± 126) while incubation of uraemic erythrocytes with normal plasma increased their ALA‐D (from 384 ± 139 to 494 ± 77). Addition of zinc in the haemolysate caused a similar induction of ALA‐D in both controls and uraemics. The zinc‐induced uraemic ALA‐D practically reached normal levels. The mechanism of enzymic depression and the possible role of elevated δ‐aminolaevulinic acid concentrations (to which depressed ALA‐D activity considerably contributes) in the pathogenesis of the neurologic manifestations of uraemia, are discussed.
Two allotypes of factor IX present in haemophilia B
Authors: Ljung R. , Wallmark A. , Nilsson I. M.
Published: August 2010

Factor IX antigen (IX:Ag) was measured with three different immunoradiometric assays (IRMAs) in 30 healthy people and 43 patients with haemophilia B of varying severity. Two of the IRMAs were based on monoclonal antibodies capable of differentiating between two genetically determined molecular variants of normal factor IX. Most patients with severe hemophilia B lacked demonstrable IX:Ag. The factor IX variant that is undetectable with one of the monoclonal antibodies used was present in 2 out of 6 families with moderate haemophilia B and in 1 out of 6 families with mild haemophilia B. The existence of allotypes of factor IX in hemophilia B may have practical implications for carrier detection and prenatal diagnosis.
Adult acute non‐lymphoblastic leukaemia: reliability and prognostic significance of pretreatment bone marrow S‐phase size determined by flow cytofluorometry
Authors: Riccardi Alberto , Danova Marco , Montecucco Carlomaurizio , Ucci Giovanni , Cassano Enrico , Giordano Monica , Mazzini Giuliano , Giordano Paolo
Published: August 2010

46 adult patients with non‐lymphoblastic acute leukaemia (AnLL) had pretreatment proliferative activity of bone marrow (BM) blasts determined simultaneously with propidium iodide DNA flow cytofluorometry (as the percentage of cells with DNA content intermediate between the diploid and the tetraploid values, 2n‐4n cell %) and in vitro tritiated thymidine cytoautoradiography (as the labelling index, LI). They were then treated with the same chemotherapy regimen, including 2–3 courses of sequential vincristine, arabinosylcytosine and adriamycin, for response induction, and monthly courses of different cytostatics for maintenance. Median 2n‐4n cell % was 9.9 and median LI was 5.9. A close linear relationship (r = 0.913, p < 0.001) between the two parameters was found. Patients having a 2n‐4n cell % greater than 15.6 responded more often to chemotherapy than patietns having a lower percentage (p < 0.05) but also experienced a shorter duration of first response (p < 0.05). Overall survival was longer in patients with 2n‐4n cell % greater than 15.6 (p < 0.02). Multiple regression analysis indicates that 2n‐4n cell % is a statistically significant (p < 0.05) independent factor correlating with duration of response in adult AnLL.
A defect of platelet release reaction in a patient with SLE: Impaired platelet aggregation induced by phorbol ester with a normal phosphorylation of 40K protein
Authors: Fuse Ichiro , Hattori Akira , Higashihara Masaaki , Takizawa Shinichiro , Takeshige Tomio , Hanano Masaharu , Nagayama Reizo , Koike Tadashi , Takahashi Hoyu , Shibata Akira
Published: August 2010

A 37‐year‐old female who suffered from SLE had a bleeding disorder. At the time of initial evaluation, the main disease demonstrated was a8‐storage pool deficiency. After this improved, a marked decrease of aggregation still remained, when induced by either ADP, epinephrine, collagen, A23187, thrombin, or PAF‐acether. Although arachidonate‐induced aggregation was slightly decreased, thromboxane B2 was produced normally in response to exogenous arachidonate. The patient's endoperox‐ides and/or thromboxane A2 aggregated aspirin‐treated platelets, though her platelets were themselves unresponsive. Impaired aggregability induced by TPA (12‐0‐tetra‐decanoylphorbol‐13‐acetate) or OAG (l‐oleoyl‐2‐acetyl‐glycerol) was also found. However, the phosphorylation of P43 and P20 induced by several stimulators including Ca+ * ionophore was normal, using 32P‐labelled platelets. It is suggested that TPA or OAG‐induced platelet aggregation requires not only the phosphorylation of those proteins, but also another unknown mechanism after the phosphorylation, and that the platelet dysfunction of this patient was due to a defect of some mechanism involving Ca+ + uptake or mobilization of cytoplasmic Ca+ + from intracellular storage sites.
Monocyte ferritin in idiopathic haemochromatosis, thalassaemia and liver disease
Authors: Saab Ghaleb , Green Ralph , Jurjus Abdo , Sarrou Elias
Published: August 2010

Monocyte ferritin (MF) content was measured in normal subjects and patients with a variety of disorders of iron storage. MF was above the normal range in 4 patients with idiopathic haemochromatosis (IHC). However, in 4 patients with transfusion siderosis (TS), secondary to aplastic anaemia, who had similar elevations in serum ferritin, MF was highly elevated. 10 patients with thalassaemia intermedia and thalassaemia major with no previous history of transfusions, but with elevated serum ferritin, also had significantly elevated MF. Disproportionately low MF in IHC could reflect defective ferritin metabolism in reticuloendothelial cells in this disorder. Finally, in 3 patients with acute rises in serum ferritin caused by acute hepatitis, MF was not increased. This suggests that MF is not directly affected by high circulating levels of serum ferritin raised acutely, but rather reflects iron storage status in conditions not associated with primary disorders of iron metabolism.
Inhibition of proliferation of human leukaemic cell populations by deferoxamine
Authors: Foa P. , Maiolo A. T. , Lombard L. , Villa L. , Polli E. E.
Published: August 2010

Deferoxamine is a hydroxylamine which binds ferric ions to form a highly stable complex. Since iron is thought to be required at a critical stage for cell proliferation, we investigated the effect of deferoxamine on the proliferative activity of human leukaemic cell populations in vitro by means of 3 permanent cell lines, HL60, U937 and 8402. We found deferoxamine to be a potent inhibitor of DNA synthesis and proliferation of leukaemic cells, acting by accumulating treated cells at the early S phase of the cell cycle. Suppression of leukaemic proliferation was obtained at deferoxamine concentrations in the range usually achieved in the treatment of patients for iron overload. Deferoxamine might therefore warrant further investigation as a potentially useful agent for leukaemia chemotherapy.
Unilateral ovarian absence in two capuchin monkeys
Authors: Domingues Sheyla F.S. , Almeida Débora V.C. , Sealercio Sarah R.R.A. , Kastelic John P. , Santos Regiane R.
Published: August 2010

Background Ovarian absence is a rare condition with congenital or traumatic origin.

Methods Gross and microscopic examination of two capuchin monkeys were performed.

Results Case 1 had absence of the right ovary and distal uterine tube, bilateral absence of fimbriae, and a rudimentary uterus. Case 2 had unilateral ovarian absence, but a functional contralateral ovary, and a normal uterus.

Conclusions Unilateral ovarian absence was confirmed in two capuchin monkeys for the first time.
Bonding of fractured permanent central incisor crown following radiographic localization of the tooth fragment in the lower lip: a case report
Authors: da Silva Schwengber Gabriela Ferrari , Cardoso Mariane , de Souza Vieira Ricardo
Published: August 2010

Abstract –  This study describes the case of an 8‐year‐old boy who fractured his left maxillary central incisor, exposing the pulp. The trauma also resulted in a cut on his mentum. Radiographic examination revealed the fractured tooth fragment embedded in the lower lip. The fractured tooth, with incomplete root formation, was treated endodontically and received a temporary restoration. After 15 days, the temporary restoration was removed and the fractured tooth fragment was etched with 37% phosphoric acid. A bonding system was then applied to the etched‐fractured tooth surface without polymerization. The same bonding procedure was carried out on the tooth fragment. A layer of flowable resin composite was applied to the fragment, which was positioned on the remaining tooth. The resin composite was then polymerized, finished, and polished.