bluuebunny/crossref_metadata_2025 huggingface dataset:
Haemoglobin Concentration and Serum Erythropoietin in Renal Dialysis and Transplant Patients
Authors: Summerfield G. P. , Gyde O. H. B. , Forbes A. M. W. , Goldsmith H. J. , Bellingham A. J.
Published: August 2010

In patients with chronic renal failure the use of the relatively new dialysis technique of continuous ambulatory peritoneal dialysis (CAPD), unlike other forms of dialysis, is consistently associated with an increase in Hb concentration, but the mechanism remains obscure.

We measured Hb, haematocrit, S‐erythropoietin and Hb‐oxygen affinity in 3 groups of patients with chronic renal failure. (1) Untreated patients starting on haemodialysis. (2) Patients on intermittent peritoneal dialysis changing to CAPD. (3) Patients from the above 2 groups receiving renal transplants. In addition, red cell mass, plasma volume and red cell survival were measured in (2), before starting CAPD and at 6 months.

There were distinctly different patterns of change in Hb concentration, Hb‐oxygen affinity and S‐erythropoietin in the 3 groups of patients.

The increase in Hb concentration with CAPD is due to both a fall in plasma volume and an increase in red cell mass, with an increase in red cell survival. There was no change in Hb‐oxygen affinity or serum erythropoietin concentration. The improvement in red cell mass and survival may be related to increased clearances of substances with mol. wt.s between 500 and 5000 daltons which accumulate in renal failure (uraemic middle molecules).
Granulocyte Function in Malignant Monoclonal Gammopathy
Authors: Hopen G. , Glette J. , Halstensen A. , Kalager T. , Schreiner A. , Solberg C. O.
Published: August 2010

Granulocyte function was studied in 22 patients with untreated myelomatosis or macro‐globulinaemia. Granulocyte adhesiveness (GA) and migration in capillary tubes (Tm) were, except for light chain disease, significantly decreased in patients of all gammopathy classes especially IgG myelomatosis. A plasma factor inhibited GA. The impairment of Tm was due to an inhibiting factor as well as lack of a stimulating plasma factor. Migration of granulocytes to skin chambers was decreased in patients with IgG myelomatosis. Chemiluminescence production during phagocytosis of opsonized zymosan was decreased. Compared to control sera, the opsonic activity of patient sera was strongly decreased. The impaired granulocyte functions in patients with malignant monoclonal gammopathy may contribute to the enhanced susceptibility to infections in these patients.
Nuclear Hypersegmentation in the Myeloma Cells of a Patient with Multiple Myeloma
Authors: Djaldetti Meir , Lewinski Uri H.
Published: August 2010

A patient with multiple myeloma in whom the bone marrow plasmablasts showed extensive lobulation is described. In addition to numerous lobes, some of the nuclei had a cerebriform appearance, similar to that observed in Sezary cells. The possible relation of this phenomenon to the expression of the altered function of the malignant cells is discussed.
Cellular Origin of the Chemokinetic Inhibitor of Polymorphononuclear Leucocytes Found in Sera from Patients with Chronic Lymphocytic Leukaemia
Authors: Siegbahn Agneta , Venge Per , Nilsson Kenneth
Published: August 2010

In a previous study, we demonstrated the existence in serum from patients with chronic lymphocytic leukaemia (CLL) of a cell‐directed, heat‐labile chemokinetic inhibitor of polymorphonuclear leucocytes (PMN). The aim of this investigation was to study the cellular origin of this inhibitor. Supernatants from short term cultured lymphocytes from 3 healthy persons and 5 patients with CLL, and from 16 different established human non‐haematopoietic and haematopoietic cell lines were analyzed. Only the leukaemic B‐lymphocytes from the CLL patients produced the inhibitor. The presence of the inhibitor was also demonstrated in supernatants from the fresh CLL cells from one CLL patient and a leukaemic cell line established from these cells using Epstein‐Barr virus as the immortalizing agent. A lymphoblastoid cell line derived from the normal B‐cells of the same CLL patient did not produce the chemokinetic inhibitor. We therefore conclude that the cellular origin of the heat‐labile, cell‐directed chemokinetic inhibitor of PMN migration is the CLL cell.
The Association between Autoimmunity and Agnogenic Myeloid Metaplasia
Authors: Ben‐Chetrit Eldad , Gross David J. , Okon Elimelech , Levo Yoram
Published: August 2010

A patient with agnogenic myeloid metaplasia (AMM) associated with an ill‐defined autoimmune disease is described and the literature on autoimmunity in AMM is reviewed. In view of the available data, it seems that the association between AMM and autoimmunity observed in our patient is not fortuitous.
Simplified Assay for Antithrombin III Activity Using Chromogenic Peptide Substrate
Authors: Handeland Grete Frantzen , Abildgaard Ulrich , Aasen Ansgar O.
Published: August 2010

A simplified method for the assay of antithrombin III (AT) with the highly reactive thrombin substrate 2AcOH H‐D‐CHG‐Ala‐Arg‐pNA (substrate Th‐1) is described. The assay may be performed at either 30°C or 37°C, and alternatively with the substrate H‐D‐Phe‐Pip‐Arg‐pNA (S‐2238). The standard curve is linear in the 12.5–150% range. For routine assays, 3 standard dilutions of plasma are sufficient, and these may be stored at ‐20°C for 3 weeks. As only the test plasma must be diluted prior to the assay procedure, the test is more rapidly performed than previous manual assays.

In 80 patients plasma samples, with AT in the 19–108% range, there was a high correlation with the results of immunoquantification (r = 0.96). There was also a high correlation betweeen the results obtained with the manual method and the automated version described using the Cobas‐Bio® Centrifugal Analyser and substrate Th‐1 (r = 0.96). Low AT levels in hereditary deficiency (particularly during heparin treatment), in liver cirrhosis, in disseminated intravascular coagulation (DIC), and heparin‐treated thrombosis were confirmed.
An Improved Method of Harvesting Haemoglobin‐Free Human Erythrocyte Ghosts
Authors: Bhattacharya Syama P.
Published: August 2010

The ghosts (stromas) of human erythrocytes bearing Hb of different phenotypes were separately isolated by incubating the erythrocytes in hypotonic (20 imOsm) phosphate buffer saline (PBS) for 1 h at pH 7.4. All of these washed ghosts were found to react with benzidine indicating the presence of Hb. In the preparation of Hb free ghosts, the pH 8.2 of the phosphate buffer, incubation time (4 h) and extra oxygen supply to the medium were noticed to be very effective. The results shows that certain ghosts bound to SS, SF, and S‐Thal type of Hb were more resistant to the clearing procedure than the ghosts bound to AA, AF, ASF and AC types of Hb. The combined effect of above variables for the optimum isolation of clear ghosts with minimum or no loss of membrane components has been described.
Treatment of Adult Autoimmune Thrombocytopenic Purpura with High‐Dose Intravenous Plasmin‐Cleaved Gammaglobulins
Authors: Solal‐Celigny P. , Bernard J. F. , Herrera A. , Boivin P.
Published: August 2010

We present the results of the treatment of 15 adult patients with chronic autoimmune thrombocytopenic purpura with high dose i.v. gammaglobulins. In 11 patients, this treatment produced a remission, complete (platelet counts above 100 times 109/1) in 5, partial (platelet counts between 50 and 100 times 109/1) in 6. In all these patients, the response was transient lasting 2–4 weeks. In 4 patients, no response was observed. We used plasmin‐cleaved gammaglobulins which caused rare and mild side effects and gave results similar to those reported with intact human gammaglobulins. The indications of high dose i.v. gamma‐globulins in the management of adult patients with autoimmune thrombocytopenic purpura are discussed.
N2O and Urine Methylmalonic Acid in Man
Authors: Rask Henrik , Olesen Anders Schou , Mortensen Jens Zobbe , Freund Leif Gade
Published: August 2010

The excretion of methylmalonic acid was measured and deoxyuridine suppression test (dU‐test) was performed in 5 patients before and after 24 h of ventilation with nitrous oxide. The mean urinary 2‐methylmalonic acid was increased from 90 to 320 μmol/24 h and the dU‐tests became abnormal. These findings indicate that the 2 vitamin B‐12 dependent enzymes, methylmalonyl‐CoA‐mutase and methione synthetase, are depressed by nitrous oxide.
Lymphocyte Subpopulations in Benign Monoclonal Gammopathy
Authors: Silvian I. , Carter A. , Tatarsky I. , Spira G.
Published: August 2010

Peripheral blood samples from normal individuals and from patients with benign monoclonal gammopathy or multiple myeloma were separated and assayed by immunofluorescence and rosette formation for T, B, TG and TM subpopulations. When compared with normal individuals and multiple myeloma patients, the benign monoclonal gammopathy patients could be divided into 2 groups. The 1st group demonstrated a T/B ratio similar to normal individuals, whereas in the 2nd group the ratio resembled that of the myeloma patients, with a decrease in the fraction of T lymphocytes, accompanied by an increased number of B lymphocytes. An analysis of the monoclonal Ig fraction levels indicated that the 2 groups differ in this respect as well. In the 1st group, the level of the monoclonal immunoglobulin was stable, with small fluctuations. The 2nd group demonstrated a general increasing M‐component, especially in the 4–6 months preceding the study. The 2 benign monoclonal gammopathy groups exhibited a trend to a lower TM/TG ratio compared to normals; this change is more prominent in the 2nd group. Analysis of the T lymphocyte subpopulations indicated an overall decrease in the fraction of TM multiple myeloma. The above‐mentioned parameters might thus aid in discriminating among BMG patients with regard to their tendency towards a malignant transformation.
The Cellular Immune Response after Splenectomy in Humans
Authors: Nielsen Johan Lanng , Ellegaard Jørgen , Tauris Palle , Johnsen Hans Erik
Published: August 2010

The in vitro immune functions of peripheral blood lymphocytes have been studied in 70 splenectomized patients. 45 patients were splenectomized due to traumatic rupture of the spleen; in 22 of these patients residual splenic tissue was detected, employing a selective spleen scintigraphy. 14 patients were splenectomized due to hereditary spherocytosis and 11 patients due to immune thrombocytopenia or autoimmune haemolytic anaema; they were all without ectopic splenic tissue. The study revealed that splenectomized patients have (i) an elevated number of blood lymphocytes, (ii) an elevated relative number of EA‐RFC, but normal %s of E, EAC‐RFC and SmIg positive cells, (iii) normal T‐cell mitogenic responses induced by PHA, but enhanced responses induced by ConA and PWM, (iv) normal cell‐mediated enhancement of the PWM‐induced proliferative B‐cell response, (v) no cell‐mediated inhibition of the T‐cell dependent and PWM‐induced proliferative B‐cell response and (vi) an impaired number of PFC after stimulation with PWM. The findings were unrelated to the cause of the splenectomies or to the presence of residual splenec tissue. It is possible that the impaired B‐cell response as shown by the reduced number of PFC after stimulation with PWM may be of significance for the in vivo resistance to infections in splenectomized patients.
Congenital Spherocytosis unmasked by Lymph Node Tuberculosis
Authors: WISLØFF F. , GODAL H.C.
Published: August 2010

A case of previously undiagnosed congenital spherocytosis that became manifest during lymph node tubercolosis is described. It is suggested that mild forms of congenital spherocytosis are common, but may pass unnoticed unless revealed by an intercurrent infection.
Normalization of fibrin induced platelet aggregation in uraemic patients undergoing haemodialysis
Authors: RYGH M. , BROSSTAD F. , BJØRNSON J.
Published: August 2010

Platelet aggregation in response to ADP, collagen, ristocetin and polymerizing des‐AA fibrin monomers was studied in six patients in chronic, stable, renal failure on maintenance haemodialysis. Fibrin induced aggregation was markedly inhibited prior to but normalized after dialysis. ADP, collagen and ristocetin induced a normal aggregation response both before and after dialysis. The Ivy bleeding time remained prolonged after dialysis.
Fibrinolytic capacity after venous stasis in patients undergoing aorto‐coronary by‐pass surgery. Relation to shunt occlusion
Authors: ARNESEN HARALD , SEMB GUDMUND , HOL RAGNAR , KARLSEN HARALD
Published: August 2010

In a prospective study 99 consecutive patients admitted for elective aorto‐coronary by‐pass surgery were subjected to estimation of their fibrinolytic capacity.

Reevaluation after 16 months included shunt angiography and repeated testing of the fibrinolytic capacity.

In the total material consisting of 244 grafts (2.7 per patient) 76 (31%) were occluded. In the subgroup of 24 patients with reduced fibrinolytic capacity, 25 (38%) of the 66 grafts were occluded as compared to 53 (30%) of the 178 grafts in patients with normal fibrinolytic capacity (p = 0.2). The number of grafts per patient and the values of the peroperatively measured blood flow in the grafts were similar in the 2 groups.

Looking into the subgroup of grafts with “poor flow values” as measured peroperatively (≤ 35 ml/min), 14 (78%) of 18 grafts were occluded in patients with reduced fibrinolytic capacity, whereas 28 (53%) of 53 grafts were occluded in patients with normal fibrinolytic capacity (p = 0.06).

In conclusion, the present study is thought to indicate that reduced fibrinolytic capacity might be an additional risk factor for graft occlusion in patients undergoing aorto‐coronary by‐pass surgery, particularly so in grafts where low blood flow values are obtained.
FSF‐stabilized soluble fibrinogen/fibrin intermediates in “Ca++‐plasma” at 37 °C
Authors: KIERULF P. , ANDERSEN Å‐B. , BROSSTAD F.
Published: August 2010

Fibrinogen fibrin complexes exist in “Ca+ +‐plasma” (prothrombin‐depleted, Ca+ + boosted, 2.5 mmol/l) adsorbed with Bentonite and enriched with I‐131‐fibrinogen (1.5 mg/ml) and I‐125‐fibrin monomer Des AA (30 μg/ml) at 37 °C. Gel chromatography, in the presence of urea/EDTA, of plasma containing such complexes, which had been enzyme‐linked through the action of 2.5 u/ml plasma XIIIa, demonstrated that fibrin Des AA monomers largely tended to oligomerize (80 per cent of the molecules eluting in front of the 340.000 m.w. species), whereas fibrinogen tended to dimerize and to a smaller extent oligomerize. Furthermore, fibrin Des AA oligomers were partly α‐oligomerized in addition to γ – γ dimerized, as opposed to fibrinogen which became mainly γ – γ dimerized.
A New Glucose 6‐Phosphate Dehydrogenase Variant (G‐6‐PD Verona) in a Patient with Myelodysplastic Syndrome
Authors: Perona Giuseppe , Guidi Gian Cesare , Tummarello Diego , Mareni Cristina , Battistuzzi Giorgio , Luzzato Lucio
Published: August 2010

A 67‐year‐old woman investigated because of ‘myelodysplastic syndrome’ was found to have a 4‐fold increase in G‐6‐PD activity in her erythrocytes. The enzyme was partially purified and characterized. On grounds of (a) reduced electrophoretic mobility, (b) abnormal cathodic band(s) in isoelectrofucusing, (c) increased Michaelis constant for glucose 6‐phosphate, (d) abnormal thermostability, and (e) abnormal interaction with the ligand NADPH, we conclude that this is a new structural variant which we designate G‐6‐PD Verona. G‐6‐PD Verona was the sole apparent source of G‐6‐PD activity in the patient's erythrocytes; by contrast, the patient's fibroblasts had only normal G‐6‐PH (type B). The patient's haematological course terminated into acute myeloid leukaemia. We believe G‐6‐PD Verona was the result of a somatic mutation in an X‐chromosome which took place in a haemopoietic cell clone which subsequently underwent neoplastic transformation.
Variation with Age of Reference Values for P‐Cobalamins
Authors: Nexø Ebba
Published: August 2010

P‐cobalamins were recorded on plasma from 279 persons (age interval 20–80 years) who participated in a population survey. The values obtained showed a decrease in the mean and a broadening of the reference interval with age. The justification of employing one reference interval, 200–650 pmol/1, is discussed.
Cytochemistry of Normal Lymphocyte Subsets Defined by Monoclonal Antibodies and Immunocolloidal Gold
Authors: Crockard Alistair , Catovsky Daniel
Published: August 2010

The cytochemical reactivities of 3 acid hydrolases, α‐naphthyl acetate esterase (ANAE), acid phosphatase and β‐glucuronidase were investigated in normal peripheral blood lymphocyte subsets defined by monoclonal antibodies OKT3, 4, 8 and FuC4 (anti‐Ia). A combined monoclonal antibody‐immunocolloidal gold/cytochemical staining procedure was used to determine enzyme activities and distributions of reaction product in each subset. Cytochemical profiles for each lymphocyte subset were defined. The majority (> 85 %) of T cells (OKT3+) were positive for all 3 enzymes whereas a minority (<40%) of B cells (F1MC4+) displayed reactivity. The cytochemical profiles of T helper/inducer (OKT4+) and T suppressor/cytotoxic (OKT8+) cells were not significantly different and corresponded to that observed for OKT3+ cells; thus none of these enzymes can be used to distinguish normal lymphocyte subsets cytochemically. ANAE reactions were further analysed, in the respective subsets, on the basis of dot‐like or scattered/diffuse reactivity. The ratios of cells displaying dot‐like: scattered/diffuse reactivity, in the respective subsets, were OKT3+, 5.4:1; OKT4+, 8.1:1; OKT8+, 2.4:1; FMC4+, 0.4:1. The cytochemical profiles and ANAE reactivities of T cell subsets identified by monoclonal antibodies differ from those displayed by T cell subsets defined by Fc receptors and confirms that there is little correlation between subsets defined by these two methods.
Hereditary Ring Sideroblastic Anaemia and Christmas Disease in a Swedish Family
Authors: Hast R. , Miale T. , Westin J. , Birgegård G. , Möller E. , Reizenstein P. , Teger‐Nilsson A.‐C. , Wetterberg L.
Published: August 2010

The association of hereditary ring sideroblastic anaemia with Christmas disease in a Swedish family is described. We have studied the transmission of the sideroblastic trait, in relation to HLA groups and Christmas disease, and also evaluated the erythrocyte morphology, uroporphyrinogen‐I‐synthetase activity and S‐ferritin for the detection of latent cases of ring sideroblastic anaemia.

The proband had ring sideroblastic anaemia, Christmas disease and haemochromatosis. 3 cases of ring sideroblastic anaemia were found among the 12 family members studied. Using the factor IX deficiency as a marker of the X chromosome, it appeared that autosomal transmission of the sideroblastic trait was most likely. The sideroblastic trait did not seem to be linked to HLA‐A3‐alloantigen.

Erythrocyte morphology was normal in all non‐anaemic subjects. S‐ferritin was found to be increased in all 3 cases of sideroblastic anaemia as well as in 1 non‐anaemic relative. Erythrocyte uroporphyrinogen‐I‐synthetase was elevated in 10 of the 12 family members; those with sideroblastic anaemia had the highest values indicating that uroporphyrinogen‐I‐synthetase is of importance in the disturbed haem‐synthesis of ring sideroblastic anaemia. This interpretation is supported by the positive correlation between S‐ferritin values and the uroporphyrinogen‐I‐synthetase activity.
Chronic Myelocytic Leukaemia: Cytogenetical Studies on Haemopoietic Colonies and Diffusion Chamber Cultures
Authors: Carbonell F. , Hoelzer D. , Grilli G. , Issaragrisil S. , Harriss E. B. , Fliedner T. M.
Published: August 2010

In 20 patients with chronic myelocytic leukaemia in the chronic phase or in blast crisis, several in vitro (CFU‐C, BFU‐E) and in vivo (diffusion chamber) culture techniques were used to demonstrate the existence of a still diploid cell clone. In 4 out of 12 patients in the chronic phase, Ph1‐negative metaphases were found in the various cultures for haemopoietic progenitor cells but not in the standard suspension cultures used for cytogenetics. However, all the 8 patients investigated during blast crisis had only Phl‐positive and other abnormal clones, indicating the loss of the diploid clone during progression of the disease.
Diabetes insipidus in preleukaemic phase of acute myeloid leukaemia in 2 patients with empty della turcica: A report of 2 cases
Authors: Puolakka Kari , Korhonen Timo , Lahtinen Reino
Published: August 2010

2 patients with diabetes insipidus established in the preleukaemic phase of acute myeloid leukaemia are described. Both patients had probably coincidentally an empty sella turcica showed by a computerized tomography. In one of them, leukaemia developed secondary to a cytostatic therapy for a vesical cancer. In this patient, blast cells appeared in cerebrospinal fluid after the patients had become polyuric and later on autopsy findings showed a peripituitary leukaemic infiltrate. In the other patient, diabetes insipidus and the acute phase of leukaemia developed after a long period with a dysmyelopoietic syndrome. Hormonal tests suggested a hypothalamic injury in both patients due to a leukaemic infiltrate at least in the former of them. It is concluded that diabetes insipidus as a consequence of hypothalamic lesion may develop in the preleukaemic phase of acute myeloid leukaemia and become evident even before any other symptoms of leukaemia have manifested.
Chronic myeloid leukaemia presenting as femoral head necrosis
Authors: Gibson John , Joshua Douglas E. , Collis Donomic , Kronenberg Harry
Published: August 2010

A 17‐year‐old male whose initial presentation of chronic myeloid leukaemia (CML) was necrosis of the right femoral head is reported. This is the first case report to our knowledge of CML presenting with such a lesion and is of further interest in that almost all reported cases of destructive bony lesions in this disease have been associated with blast transformation. Our patient in contrast has remained in stable chronic phase for over 30 months.
Chronic T lymphocytic leukaemia: antigenic, morphological and functional properties of the neoplastic lymphocytes
Authors: de Paoli P. , Nimis R. , Molaro G. L. , Battistin S. , Reitano M. , Castiglia C. , de Filippo I. , Santini G.
Published: August 2010

We describe 2 cases of T cell leukaemia. Both patients react with OKT 4 monoclonal antibody, while their clinical course, surface markers and in vitro immunological reactivity present opposite behaviour. The cells of 1 patient react with all pan T cell markers (OKT 3, OKT 11, Leu 1), from E and Ea rosettes and respond well to mitogens. This patient does not present liver, spleen or lymph node enlargement; white cell count is stable and no other haematological alterations are present. The cells from the 2 patient are unreactive with OKT 3 and are unable to form Ea rosettes. The response to mitogens is almost abolished. The disease has an aggressive clinical course with progressively increasing blood leucocyte count and infiltration of liver and spleen. These data suggest that the study of antigenic and functional properties of T CLL may be useful to better define the biologic characteristics and the prognostic criteria of the disease.—
Red cell basic ferritin content of patients with megaloblastic anaemia due to vitamin B12 or folate deficiency
Authors: Van Der Weyden Martin B. , Fong Hubert
Published: August 2010

The basic ferritin content of red cells was measured in patients with untreated megaloblastic anaemia. The red cell ferritin of 10 patients with anaemia and vitamin B12 deficiency (mean 579, range 68–2616 attogram (ag)/cell); and of 8 patients with folate deficiency (mean 792, range 141–2373 ag/cell) were significantly elevated (P < 0.001) compared with normal subjects (mean 10.7, range 4–47 ag/cell) and showed a significant correlation with pre‐treatment levels of plasma ferritin and less so with percent transferrin saturation. Following vitamin replacement elevated red cell ferritin levels decreased during the period of reticulocytosis and was normal in 9 patients evaluated after 6 months.

The magnitude of increase in red cell basic ferritin levels observed in untreated megaloblastic anaemia is comparable to that of subjects with idiopathic haemochro‐matosis and suggests that interpretation of this index for iron overload should take into consideration concomitant body folate or vitamin B12 status.—
A study of certain functional parameters of monocytes from patients with multiple myeloma: Comparison with monocytes from healthy individuals
Authors: Blom Jens , Nielsen Henrik , Larsen Severin Olesen , Mansa Bendt
Published: August 2010

Selected functions (i.e. phagocytosis and chemotaxis of circulating blood monocytes were studied on cells obtained from 20 patients with untreated multiple myeloma (MM) and the results were compared with those obtained on cells from 60 healthy persons. The mean number of circulating monocytes was only slightly increased over the normal value although a marked monocytosis was demonstrated in all of the 4 patients with M‐components of the lambda light chain type. Plasma cells isolated from peripheral blood and from bone marrow of patients with MM were found to possess a strong a‐naphthyl acetate esterase activity. No differences were observed in the phagocytic activity of monocytes from patients compared with those of the controls, whereas the chemotactic responsiveness of the monocytes from the patients was slightly increased.
Isolation of ferritin from human hepatocellular carcinoma
Authors: Whittaker D. , Torrance J. D. , Kew M. C.
Published: August 2010

Ferritin was extracted from human hepatocellular carcinoma tissue and purified using column chromatography, gradient gel electrophoresis and cadmium sulphate crystallization. DEAE cellulose chromatography showed a difference between hepatoma and normal liver ferritin, indicative of a more acidic isoferritin profile in the tumour. Column‐purified and crystalline ferritin and that remaining in the mother‐liquor after crystallization was subjected to isoelectric focusing. Hepatoma ferritin showed higher concentrations of acidic isoferritins than liver ferrtin. This was most obvious with mother‐liquor ferritin, as crystallization tended to select out more basic isoferritins. Subunit analysis of hepatoma and liver ferritin showed a higher proportion of heavy subunits in the tumour ferritin, in keeping with the presence of acidic isoferritins. An antibody against hepatoma mother‐liquor ferritin was raised in rabbits. However, hepatoma ferritin proved to be antigenically identical with normal liver ferritin, and we were thus unable to develop a specific radioimmunoassay for hepatoma ferritin.
Surface morphology of human platelets during in vitro aggregation
Authors: Zilla Peter , Groscurth Peter , Rhyner Kaspar , von Felten Arthur
Published: August 2010

The alterations of surface morphology of human platelets during the course of the aggregometer tracing were studied by scanning electron microscopy (SEM). Prior to activation the platelet rich plasma was pre‐incubated at 37°C for at least 30 min in order to obtain a sufficient number of discoid platelets. Immediately following the addition of collagen or ADP platelets showed slender pseudopods which thereafter were replaced by bulbous protrusions. The decrease in percent light transmission (%T) in the aggregometer tracing was characterized in SEM by a significant enlargement of bulbous protrusions and by the transformation of platelet shape from disc to sphere. During the increase in %T platelets forming primary aggregates displayed spiny pseudopods at their surface indicating that, during in vitro aggregation induced by collagen or ADP, two generations of pseudopods are formed. Using a low dose of ADP, the return of aggregometer tracing to the base line was regularly accompanied by dissociation of primary aggregates, but platelets remained spheroid and displayed pseudopods for a long time. Our study indicates that the course of aggregometer tracing is closely associated to the surface morphology of platelets. Single morphological changes, however, are not reflected by the aggregometer method.
Hydrops foetalis in 3 infants of a mother with acquired chronic pure red cell aplasia: Transitory red cell aplasia in 1 of the infants
Authors: Øie B. K. , Hertel J. , Seip M. , Friis‐Hansen B.
Published: August 2010

A young woman with acquired chronic pure red cell aplasia (PRCA) gave birth to 3 infants with foetal hydrops. The 1st infant died shortly after birth, while the 2nd was stillborn with severe anaemia. Both had signs of increased erythropoiesis at autopsy. The 3rd infant was saved by foetal intrauterine red cell transfusions into the hepatic part of the umbilical vein. This infant had red cell aplasia lasting for about 3 months after birth, but then recovered. We believe that antibodies to red cell progenitors in the bone marrow were transferred transplacentally from mother to foetus, giving rise to severe affection of these infants. To the best of our knowledge this has not been reported previously in PRCA.
Prognostic factors in IgD myeloma: A study of 21 cases
Authors: Fibbe Willem E. , Jansen Jan
Published: August 2010

A series of 21 patients with IgD myeloma was studied retrospectively, to assess which parameter present at the time of diagnosis was of prognostic importance for survival and whether the clinical staging system of Durie and Salmon had predictive value for the survival time of these patients. Survival time did not appear to be correlated with haemoglobin concentration, thrombocytopenia, initial level of M‐protein, amount of Bence‐Jones proteinuria, hypercalcaemia, serum creatinine level, presence of osteolytic lesions or hepatosplenomegaly. Neither did the staging system of Durie and Salmon predict the survival time. It is concluded that clinical staging is of limited value in the management and prediction of the survival time of IgD myeloma patients.
Proliferation and differentiation of normal and chronic myeloid leukaemia (CML) marrow cells in suspension cultures
Authors: NILSSON BO , OLOFSSON TOR
Published: August 2010

Immature myeloid cells highly enriched for colony‐forming cells, myeloblasts and promyelocytes were isolated in Percoll gradients (lighter than 1.065 g/ml) from patients with chronic myeloid leukaemia (CML) or normal healthy volunteers. The proliferation and differentiation of these cells were followed in suspension cultures in McCoy's medium (15% FCS) with or without the addition of 15% human placenta conditioned medium (HP‐CM) over a culture period of 12–16 d. Ion‐exchange chromatography and gel chromatography indicated that the proliferation‐stimulating activity in HP‐CM was colony‐stimulating activity (CSA). In unstimulated cultures (without HP‐CM), normal cells produced very few neutrophils; macrophage production dominated. CML cells, on the other hand, gave rise to neutrophils even in the absence of stimulatory factor(s). The effect of HP‐CM was dependent on the initial concentration: at a ‘low’ (1 times 105/ml) concentration, the addition of HP‐CM resulted in a great increase in neutrophils, but at a ‘high’ (5–8 times 105/ml) initial cell concentration, HP‐CM gave only minimal increase in neutrophil numbers in both normal and CML cultures. These observations suggest endogenous differences between normal and CML precursors in their requirements for or responses to growth stimulatory or inhibitory factors.
Acid phosphatase and acid esterase activity in neoplastic and non‐neoplastic lymphoid cells A semiquantitative evaluation related to immunological markers in 112 cases
Authors: BOESEN ANNE MARIE , HOKLAND PETER , JENSEN O. MYHRE
Published: August 2010

Acid phosphatase (AcP) and acid α‐naphthylacetate esterase (ANAE) were examined in lymphoid cells from 104 patients suffering from various lymphoproliferative disorders and from 8 healthy controls. Enzyme activities were evaluated by means of a scoring system. Scores of AcP and ANAE were higher in normal T cells than non‐T cells. In comparison, the activated T cells in infectious mononucleosis showed increased AcP and decreased ANAE reaction. Malignant T lymphoblasts had a distinct granular AcP positivity in contrast to the faint reactivity observed in cALL blasts, whereas ANAE showed negative or weak reaction in both subsets. High scores and distinct staining patterns for both enzymes were found in T CLL and T prolymphocytic leukaemia, clearly different from the weak activities seen in B CLL, B PLL and some B cell lymphomas. The latter, too, could be distinguished by mutual differences in enzyme reactions. High AcP and ANAE scores were also found in hairy cell leukaemia, and the staining patterns together with the tartrate resistance firmly established the diagnosis. Thus, simultaneous determinations of AcP and ANAE can be of great value in the diagnosis of lymphoid malignancies.
Factor XI deficiency in an arab moslem family in Israel
Authors: AGHAI E. , YANIV I. , DAVID M.
Published: August 2010

An arab moslem family with members affected by PTA deficiency is described. 3 children were found to have major deficiency, factor XI procoagulant activity being 3, 3 and 4 units/dl. 8 members, including parents, paternal grandparents and 4 siblings, were found to have minor deficiency of factor XI (40 to 68 units/dl). Assays of immunoreactive material in 4 members corresponded to the level of procoagulant activity. In this family, gene expression is autosomal recessive. The only bleeding episode reported was haematuria in the propositus. No other spontaneous, post‐trauma or post‐operative bleeding was noted. The PTA deficiency was reported until now, mainly in ashkenazi jews. This family is the first case of PTA deficiency ever reported in arab moslems.
Spongy Lymphoid Myelofibrosis as a Predictor of Hairy Cell Leukaemia or a Variant of Hairy Cell Leukaemia without Hairy Cells?
Authors: Hasselbalch Hans , Berild Dag , Videbæk Aage , Lisse Ida
Published: August 2010

8 patients with clinical and haematological features suggestive of hairy cell leukaemia are described. During their initial illness morphologic and cytochemical evidence of hairy cell leukaemia were lacking in all but 2 patients. A common highly characteristic histopathologic finding was reticulin fibrosis in the bone marrow, present in all patients at first presentation. The term spongy lymphoid myelofibrosis is proposed for such cases, which may represent a variant type of hairy cell leukaemia or an early stage of the disease.
Mechanism of Aprindine Induced Agranulocytosis: Direct Toxicity on CFU‐C and CFU‐GEMM
Authors: Boogaerts M. A. , Peters M. , Peersman C. , Verwilghen R. L. , Kesteloot H.
Published: August 2010

The in vitro bone marrow growth of 2 patients with aprindine‐induced agranulocytosis was studied. Granulocyte‐macrophage committed stem cell (CFU‐C) growth is inhibited by aprindine in a dose dependent manner. 50% inhibition of CFU‐colony growth (TD50) was seen at 5.1 and 3.4 μg aprindine/ml medium respectively. The TD50 of control marrow CFU‐C was 3.2 μg/ml. 100% inhibition was seen at 16 μg aprindine/ml, both in patients and controls.

Pluripotential stem cell growth (CFU‐GEMM) in control marrow was equally inhibited in a dose dependent manner by aprindine, though to a lesser extent (TD50: 9.1 μg/ml) and with relative sparing of pure megakaryocyte and erythroid colonies. Co‐culturing of patients marrows with their respective acute phase serum did not inhibit CFU‐C growth.
Heterogeneity of T‐Lymphocyte Chronic Lymphatic Leukaemia (CLL)
Authors: Singh A. K. , Lewis P. , Wetherley‐Mein G.
Published: August 2010

Lymphocyte surface markers (E‐SRBC, EAC, EAγ and SmIg) and monoclonal antibodies (OKT3, OKT4, OKT8 and OKIa) were used to characterise the blood and bone marrow lymphocytes of T‐cell CLL (8 patients). The diagnosis of T‐cell CLL was made primarily as the majority of blood lymphocytes formed E‐SRBC in ecah patient. Other markers – EAC, EAγ and SmIg – showed different patterns of association with E‐SRBC. These findings considered together described 4 different phenotypes amongst these patients: (a) E+ (3 patients), (b) E+, EAC+ (1 patient), (c) E+, EAγ+ (2 patients), and (d) E+, SmIg+ (2 patients). Similarly, 4 different groups were defined with the help of monoclonal antibodies. Helper T‐cell (3 patients) and suppressor T‐cell (1 patient) CLL showed predominantly helper T‐ and suppressor T‐lymphocytes respectively. Mixed T‐cell CLL (1 patient) comprised an equal proportion of both subpopulations, while the remaining 3 patients, with excess of one or other subpopulations along with a considerable proportion of Ia antigen‐bearing lymphocytes, formed the indeterminate cell type CLL.
Classification of patients with myelodysplastic syndromes according to the FAB co‐operative group's proposals Proposals for a redefinition of blast cell type II
Authors: Nielsen B. , Thiede T. , Sundström C. , Hagberg H.
Published: August 2010

53 patients with the myelodysplastic syndromes (MDS) were classified according to the proposals of the FAB cooperative group 1982. 29 patients had refractory anaemia (RA), 10 refractory anaemia with excess of blasts (RAEB), 5 RAEB in transformation, 7 RA with ringed sideroblasts and 2 chronic myelomonocytic leukaemia (CMML). Counting blast cells type I and II involved no difficulties. 4 of 15 patients who developed acute myeloid leukaemia (AML) according to the FAB classification of 1976 did not fulfill the new 1982 criteria for AML. A redefinitioon of the blast cell type II to include a more granulated blast cells, without the characteristics of promyelocytes, would solve this problem. We conclude that a redefinition of the blast cell type II might turn out to be useful.
Immunohistochemical identification of lymphocyte subsets and accessory cells in human hyperplastic lymph nodes The functional significance of the compartmentalization of lymphoid tissue
Authors: Ralfkiær Elisabeth , Plesner Torben , Wantzin Gunhild Lange , Thomsen Kristian , Nissen Nis I. , Hou‐Jensen Klaus
Published: August 2010

Biopsies from 21 lymph nodes with benign hyperplasia were examined by immuno‐enzymatic labelling of frozen sections with a panel of monoclonal antibodies. B‐cells (B1+, HLA‐DR+, C3b receptor+/‐) localized in primary follicles, secondary follicles, and areas adjacent to the subcapsular sinus. The B‐cells in primary follicles and mantle zones of secondary follicles were indistinguishable (SmIgD+, SmIgM+, Cylg−, T10−, CALLA−). B‐cells adjacent to the subcapsular sinus demonstrated a higher density of SmIgM, and a weaker expression of SmIgD. The germinal centre cells showed a more differentiated phenotype (SmIgD−, SmIgM+, CyIgM+/‐), and also expressed T10 and CALLA. T‐cells (Lyt3+Lyt2+, Leu4+, OKT6−, OKT10−) localized in paracortial and interfollicular areas, and demonstrated a relative predominance of T‐helper/inducer cells (Leu3+). T‐helper/inducer cells were also identified in secondary follicles. The B‐cell areas contained dendritic reticulum cells (R4/23+, C3b‐receptor+). Interdigitating reticulum cells (HLA‐DR+, OKT6+/‐) localized in T‐cell regions. The cells in sinuses demonstrated monocyte/macrophage properties (MO2+, Ig+, C3b‐receptor+, HLA‐DR+/‐).
Alterations in T‐lymphocyte subsets among Danish haemophiliacs: Relation to source of factor VIII preparations and high dose factor VIII treatment
Authors: Gerstoft J. , Bentsen K. , Scheibel E. , Dalsgård‐Nielsen J. , Gormsen J. , Dickmeiss E.
Published: August 2010

Screening of 43 healthy Danish haemophiliacs revealed a significantly lower helper/suppressor (H/S) ratio than in controls. 8 of the haemophiliacs had an H/S ratio ≤ 1.0. A significant negative correlation occurred between the total lifetime factor VIII treatment and the H/S ratio. However, high‐dose factor VIII treatment given to patients with antibodies against factor VIII was not associated with immunological abnormalities. Children had a significantly higher H/S ratio than the adult haemophiliacs.

Patients exclusively treated with Danish cryoprecipitate during the last year had a significantly higher H/S ratio than patients receiving preparations from other sources. This difference might, however, be explained by lower age and lower total lifetime dose in the group receiving Danish preparations. Haemophiliacs treated with American preparations did not differ immunologically from those treated with preparations of other origin. Total serum IgG was increased in 23% of the patients. This parameter was negatively correlated with the H/S ratio. The possible relation of the observed immunological alterations among otherwise healthy haemophiliacs to the acquired immune deficiency syndrome warrants further attention.
Staging and survival in multiple myeloma
Authors: Wahlin Anders
Published: August 2010

The records of 91 patients with multiple myeloma were reviewed and staging was performed according to Durie & Salmon (1). Patients with progressive disease were re‐staged. Patients first diagnosed in stage I were younger than patients first diagnosed in stage III and survived longer than patients in stages II or III. The duration of stage I was longer than the duration of stages II and III. When patients in stage I‐II progressed into stage III, their age and remaining survival time were similar to the age and survival of patients first diagnosed in stage III. These findings are compatible with the hypothesis that myeloma stage I prededes myeloma stage III and is often diagnosed by chance. It is suggested that patients with myeloma stage I should be omitted from comparisons between different myeloma populations.
Malignant (acute) myelosclerosis ‐ a clinical and pathological study in 6 patients
Authors: Thiele J. , Krech R. , Vykoupil K. F. , Georgii A. , Georgii Med. A.
Published: August 2010

Clinical and morphological findings are described in 6 patients with malignant (acute) myelosclerosis/fibrosis (MMS). Haematological data are characterized by severe anaemia and thrombocytopenia, frequently accompanied by a leucopenia with an increase in myeloblasts and promyelocytes in the peripheral blood count. There is an absence of, or a minimal hepatosplenomegaly and the survival times after onset of clinical symptoms to death range from 4–12 months. The histopathology of the bone marrow shows a conspicuous proliferation of blasts (myeloblasts, promyelocytes and megakaryoblasts) in a variable amount, besides a fibrosclerosis consisting of reticulin and collagen fibrils. A comparison of MMS with ordinary myelofibrosis/osteomyelosclerosis (MF/OMS) of a chronic course implicates two important facts: (a) evolution of fibrosclerosis takes a considerable period of time for manifestation, which ranges between 20–30 months; (b) the histopathology of MMS is identical with those features observable in the rare event of a terminal stage, i.e. blastic transformation of chronic MF/OMS. Consequently MMS should be designated as an accelerated variant of MF/OMS with a rather early occurrence of a blastic crisis. The insidious onset with the dominant clinical finding of anaemia is probably responsible for the relatively late appearance of symptoms, while the progressive course prevents an overt myeloid metaplasia with a massive hepatosplenomegaly.
Suppressor T cell chronic lymphocytic leukaemia associated with red cell hypoplasia
Authors: Shionoya Shigeru , Amano Masamichi , Imamura Yukio , Nakahara Kazuhiko , Okawa Hiroji
Published: August 2010

A case of T cell chronic lymphocytic leukaemia (CLL) with red cell hypoplasia and neutropenia is reported. WBC was 10.0 times 109/1 with 78% being T lymphocytes. These T lymphocytes were positive for Fcγ receptor and had OKT8 and Leu 2a antigens on the cell surfaces. They suppressed both erythroid and granulocytemacrophage colony formation in normal bone marrow cultures and suppressed immunoglobulin production by normal B lymphocytes in vitro. Though the myeloid cells were preserved in the bone marrow and the values of serum immunoglobulins were within normal limits, it can be estimated from these results that red cell hypoplasia and neutropenia were partially due to the suppression of haemopoietic precursor cells by leukaemic T lymphocytes.
Iron overload and lysosomal stability in β°‐thalassaemia intermedia and trait: Correlation between serum ferritin and serum N‐acetyl‐β‐D‐glucosaminidase levels
Authors: Frigerio R. , Mela Q. , Passiu G. , Cacace E. , La Nasa G. , Perpignano G. , Carcassi U. E. F.
Published: August 2010

Increased iron storage represents a characteristic condition in patients with β‐thalassaemia intermedia. Iron overload is an important factor in cellular damage. Recent studies have shown an enhanced lysosomal fragility due to increased iron storage. 13 patients with β°‐thalassaemia intermedia, aged 17–44 years, were studied. Both serum ferritin and serum N‐acetyl‐β‐D‐glusosaminidase levels were evaluated in all subjects studied. A significant linear correlation (P < 0.05) between serum ferritin and serum N‐acetyl‐β‐D‐glucosaminidase levels were found.
Vitamin A and/or high‐dose Ara‐C in the maintenance of remission in acute myelogenous leukaemia in children?
Authors: Lie Sverre O. , Slördahl Sofie H.
Published: August 2010

8 children with acute myelogenous leukaemia were brought into remission with a induction regimen containing cytosin arabinoside (Ara‐C), 6‐thioguanine and adriamycin given as the DNA complex. After remissions were obtained, the children were consolidated with high‐dose Ara‐C (2 g/m2 every 12 h for 6 doses) and were given 50000 IU of vitamin A daily as an inducer of differentiation. All children are in continuous relapse‐free survival for periods of 5 to 29 months (mean 14 months).
The Hb F composition in a Moroccan Family with β°‐thalassaemia and Hb O‐Arab
Authors: Morlé François , Morlé Laurette , Baklouti Faouzi , Dorléac Evelyne , Baudonnet Claire , Delaunay Jean , Godet Jacqueline
Published: August 2010

We report on a Moroccan family in which the proposita displays a picture of β‐thalassaemia intermedia, associated with heterozygous Hb O‐Arab (β121 Glu → Lys) and a β°‐thalassaemia trait. Hb O‐Arab was ascertained by the disappearance of the Eco RI restriction site that, normally overlaps the β‐globin gene codon 121. The proposita further presents high proportions of Hb F (12.1%) and of Gγ chains (68.6%). The transmission of the proband's haemoglobin markers was analyzed (the proband's husband displaying normal haemoglobin). The β°‐thalassaemia and O‐Arab genes underwent mutual exclusion. A high Hb F (9.28%) level was found in one child, in association with the β°‐thalassaemia trait, while another child carrying the latter trait displayed normal levels of Hb F. This situation suggests that a hetero‐cellular HPFH determinant is involved. However, there was no means to establish whether the high Hb F proportion in the mother results solely from the β°‐thalassae‐mia – Hb O‐Arab association or whether an additional HPFH determinant is present. No DNA deletion was detectably associated with the high proportion of Hb F. In this family, the Gγ percentage was high whenever the β°‐thalassaemia gene was present, regardless of total Hb F percentage. This observation is consistent with the view that the control of the Gγ percentage in the adult is linked to the β‐locus.
Age, sex and late relapse in childhood lymphoblastic leukaemia
Authors: Woodcock B. E. , Anderson L. M. , Lilleyman J. S.
Published: August 2010

Of 53 children with lymphoblastic leukaemia in their first remission who reached the fourth year after stopping treatment, 4 have subsequently relapsed and their cases are described. All are boys and 3 were over 10 at diagnosis despite there being only 6 such patients in the group overall. This suggests that teenage males are at special risk of late relapse and should not be considered cured on criteria applicable to younger females.