bluuebunny/crossref_metadata_2025 huggingface dataset:
Interferon Production, Cellular and Humoral Immunity in Splenectomized Patients
Authors: Nielsen Johan Lanng , Haahr Sven
Published: August 2010

Undetectable levels of circulating interferon were found in 35 splenectomized patients and in 14 controls. In addition, no differences in virus, purified protein derivative (PPD), and phytohaemagglutinin (PHA) induced interferon production were observed between the patients and the controls. Virus induced lymphocyte transformation tended to be reduced after splenectomy, but only the herpes simplex virus (HSV) induced transformation index was significantly reduced in splenectomized patients without residual splenic tissue (P < 0.05). In contrast, the PHA lymphocyte transformation was unchanged in patients and controls. The number of natural viral antibodies in splenectomized patients did not differ from that of controls. Furthermore, there were no major differences in the magnitude of the titers of the sero‐positive cases between the various groups. Thus, splenectomy does not interfere with interferon production in vitro, and as seen from natural viral antibodies, no impairment of humoral immunity was observed. The reduced HSV lymphocyte transformation may indicate an impaired lymphocyte function after splenectomy.
Detection of Normal and Malignant Megakaryocytes by Anti β‐Thromboglobulin Serum
Authors: Pizzolo G. , Chilosi M. , Schiavon R. , Ambrosetti A. , Cetto G. L. , Sabbioni R. , Caramaschi G. , Perona G.
Published: August 2010

An antiserum to β‐thromboglobulin was used in immunofluorescence to detect normal and malignant megakaryocytes. In normal peripheral blood and bone marrow smears, only platelets and megakaryocytes were specifically stained by this antiserum. Among 25 cases of acute lymphoid and non‐lymphoid leukaemia, only 2 exhibited a clear positivity in a % of blasts, thus proving their megakaryocytic origin. This method is proposed as a simple and useful tool to detect normal and malignant megakaryocytes on smears.
Identification of a Cold Agglutinin with Anti‐Pr3 Specificity
Authors: Birgens H. S. , Dybkjaer E. , Roelcke D.
Published: August 2010

An apparent monoclonal IgM, x‐cold agglutinin with anti‐Pr specificity was detected in the serum of a 38‐year old woman. Haemagglutination inhibition studies disclosed the subspecificity anti‐Pr3, since the cold agglutinin was strongly inhibited by carbodiimide‐treated erythrocyte glycoproteins. Studies with animal cells showed that the corresponding Pr3 determinant was found only on human red cells. This is in contrast to earlier findings and indicates that distinct fine specificities within anti‐Pr3 antibodies might well exist.
Multiple Myeloma with a Sarcoidosis‐Like Reaction
Authors: Falini Brunangelo , Tabilio Antonio , Velardi Andrea , Cernetti Cristina , Aversa Franco , Martelli Massimo F.
Published: August 2010

An unusual case of multiple myeloma with extramedullary dissemination and co‐existing systemic non‐necrotic granulomas is described. Immunoperoxidase staining revealed that both lymph node and bone marrow infiltrating plasma cells contained the same IgG‐Λ type paraprotein, and that the granuloma epithelioid histiocytes contained intracytoplasmic lysozyme. As there was no evidence of either systemic infection or sarcoidosis, the nonnecrotic granulomas may represent the host's response to the myeloma, not unlike the reaction observed in Hodgkin's disease or solid tumours. A causal association cannot, however, be entirely excluded.
Expression of la‐Like Antigen in Lymphatic Leukaemias and Non‐Hodgkin Lymphomas in Correlation with Other Surface Markers
Authors: Bergmann Lothar , Mitrou Paris S. , Cherdron Thomas , Holloway Karen , Jost Jutta , Timm Viola , Zerth Gonther
Published: August 2010

Ia‐like antigen, surface Ig, E‐rosettes, mouse red blood cell rosettes and further surface markers were investigated in 88 specimens from 69 patients with various lymphocytic leukaemias and non‐Hodgkin lymphomas.

Ia‐like antigen was detectable in all cases of sIg+ leukaemias and lymphomas. E‐la+sIg‐ leukaemias, especially CLL and lymphomas were interpreted as ß‐cell neoplasias, which either did not produce sIg or expressed it in undetectable amounts. E+Ia+sIg+ malignant lymphocytes represented a ß‐cell population with antibody activity of sIg to SRBC. In the present series 9.5 % of all ß‐cell leukaemias and lymphomas demonstrated this phenotype. Whereas ALL may possess Ia‐like antigen, the vast majority of mature T‐cell lymphomas are lacking it. In the present series 2 T‐cell lymphomas expressed Ia‐like antigen and did not contain TdT (E+Ia+sIg‐TdT‐). These lymphomas are probably derived from a stimulated T‐cell clone synthesizing Ia‐like antigen.
Effect of Granulocytic Chalone on the Growth Rate of Continuous Cell Lines Propagated in Vitro A New Assay System
Authors: Foa P. , Maiolo A. T. , Lombardi L. , Rytomaa T. , Polli E. E.
Published: August 2010

The progress of research on granulocytic chalone has been hampered by a lack of reliable assay systems. This paper reports the use of continuous cell lines growing in liquid suspension culture as a new method for assessing the effect of granulocytic chalone on cell proliferation. Partially purified granulocytic chalone (PPGC) was added to cultures of a rat myeloid cell line (W25), a human myeloid cell line (HL60), a human B lymphocytic cell line (8392), and a human T lymphocytic cell line (8402). The growth rate of the target cell populations was directly assessed by serial cell counts. PPGC pretreatment resulted in a significant inhibition of cell proliferation of the 2 myeloid permanent cell lines, while the growth rate of the lymphocytic cell lines was not affected. Moreover, inhibition of cell proliferation was spontaneously reversed when PPGC treatment was discontinued. Since PPGC from ox leucocytes displayed an inhibitory effect on cell proliferation which was specific to the myeloid cells, spontaneously reversible and not species‐specific, it appears to have all the essential properties of a granulocytic chalone.
Influence of Autologous Monocytes on the Pokeweed Mitogen‐Induced Generation of Immunoglobulin‐Secreting Cells in Man
Authors: Petersen JØrgen , Kieffer Marianne , Lilic Desa , Rathlev Niels , Andersen Vagn
Published: August 2010

The regulatory influence of autologous blood monocytes on the PWM‐induced generation of Ig‐secreting cells was assessed using a reverse haemolytic plaque forming cell (PFC) assay. The PWM‐induced PFC responses of monocyte‐depleted cells were low or absent in most cases. Addition of 12–42% freshly isolated monocytes fully reconstituted the IgM‐, IgG‐ and IgA‐PFC responses. With more monocytes added, the PFC responses declined. Monocytes precultured for 48 h supported the PFC responses of monocyte‐depleted cells less well and addition of monocytes stimulated with phorbol myristate acetate (PMA) did not increase the response. The low responses of monocyte‐depleted cells co‐cultured with precultured monocytes were not increased by addition of supernatant from monocyte cultures.

The PFC responses of mononuclear cells induced by PWM were significantly inhibited by unstimulated precultured monocytes, and to a larger degree by PMA‐treated monocytes, indicating the presence of suppressor cells among the precultured monocytes. The PWM‐induced thymidine incorporation by monocyte‐depleted cells with precultured monocytes added was only slightly lower than that obtained with freshly isolated monocytes added, suggesting that the suppressive role of precultured monocytes was not due to cytotoxicity.
Malignant Centroblastic‐Histiocytic Lymphoma of the Skin
Authors: JØNSSON V. , HIPPE E. , CLEMMENSEN O. J. , BADSBERG E. , VIDEBÆK AA.
Published: August 2010

A mixed B‐cell type centroblastic lymphoma with extraordinarily many histiocytes in a 68‐year‐old man is reported. Multiple skin tumours were the only clinical manifestation during the first 5 months. The disease then progressed to the lymph nodes, spleen, and parenchymal organs, but the bone marrow remained unaffected until death, 11 months after the onset of signs. C3d receptors were the only surface markers of the centroblasts. The histiocytes were normal with respect to morphology, muramidase staining, and Fc and C3b receptors. This highly unusual spread from skin to lymphoid and parenchymal organs is discussed in the light of lymphoid cell kinetics.
Two Different in Vitro Growth Patterns for Erythroid Precursors in 18 Patients with Pure Erythrocytosis
Authors: Clement S. , Eberlin A. , Najean Y. , Chedeville A.
Published: August 2010

Growth patterns of marrow and blood erythroid progenitors were studied in 18 cases of pure erythrocytosis using different doses of erythropoietin. 8 cases demonstrated ‘spontaneous’ growth of CFU‐E and blood BFU‐E as observed in myeloproliferative disorders, but without an excess of circulating CFU‐GM. 3 of these patients also had other symptoms of a pan‐myelopathy. All these cases showed good sensitivity to 32P myelo‐suppression. 10 cases demonstrated growth patterns of erythroid progenitors similar to those observed in normal subjects, except for an excess of blood BFU‐E, which suggests an abnormality of homeostatic regulation. In 5 of these cases, myelo‐suppression was not effective.

It is suggested that a stem cell study could differentiate patients with pure erythrocytosis due to ‘autonomous’ abnormal stem cell growth from cases due to abnormal regulation factors, and that such a discrimination might be useful for the choice of therapy.
Effect of Blood Derived Monocytes on the Promotion of in Vitro Erythropoietic Colony Growth in Human Bone Marrow Cultures
Authors: GRILLI GUALTIERO , CARBONELL FELIX
Published: August 2010

The effect of monocytes on the promotion of erythropoietic burst formation in human bone marrow methylcellulose cultures was studied in a burst‐promoting activity (BPA)‐poor system. Irradiated Ficoll‐Isopaque separated blood mononuclear cells stimulated BFU‐E growth proportionally to the number of irradiated cells added. Monocyte‐depleted mononuclear cells stimulated as well as total mononuclear cells. Monocyte‐concentrates stimulated when added at low concentrations. Monocyte‐depleted mononuclear cells, although stimulatory at higher concentrations, did not stimulate at the concentrations used for monocyte‐concentrates.
The Effect of Storage of Whole Blood on the Association of Factor VIII‐Related Antigen and Factor VIII‐Coagulant Antigen
Authors: Hellings Jan A. , Over Jan , van Mourik Jan A.
Published: August 2010

To evaluate the extent of denaturation of factor VIII‐coagulant activity (VIII:C) during production of factor VIII concentrates, the factor VIII‐coagulant antigen (VIII:CAg)/VIII:C ratio was measured in plasma, cryoprecipitate and cryosupernatant from fresh and stored blood. This ratio was close to unity for both cryoprecipitate and other concentrates, suggesting that VIII:CAg is lost concurrently with VIII:C during cryoprecipitation and further fractionation.

Storage of blood (18 h, 22°C) before processing resulted in a 30% loss of VIII:C from the separated plasma; however, VIII:CAg was not affected. In cryoprecipitate prepared from this plasma, VIII:C and VIII:CAg both were 30% lower than when prepared from fresh plasma. In the corresponding cryosupernatant, however, more VIII:CAg but less VIII:C was present compared with fresh material.

Gel chromatography revealed that the rise of VIII:CAg in cryosupernatant prepared from stored blood, was due to an increased amount of VIII:CAg of low molecular weight, not being associated with factor VIII‐related antigen. Such an increase in dissociated VIII:CAg was not detected in the plasma prior to cryoprecipitation.

It is concluded that during storage of blood, molecular changes are induced in the factor VIII‐VWF complex, possibly by limited proteolysis, which make the complex more liable to dissociation during subsequent cryoprecipitation.
Two Additional Cases of Coexisting Polycythaemia Vera and Chronic Lymphocytic Leukaemia
Authors: Jacobsen Niels , Theilade Karen , Videbæk Aage
Published: August 2010

2 patients with coexistent polycythaemia vera and chronic lymphocytic leukaemia are described. A 61‐year‐old man presented with an increased packed cell volume (PCV) and a leucoerythroblastic blood picture, which was reversible upon treatment, neutrophil leucocytosis, bone marrow lymphocyte infiltration and splenomegaly, and subsequently developed lymphomas, blood lymphocytosis and thrombocytosis. The second case was a 58‐year‐old female presenting with increased PCV and leucocyte alkaline phosphatase score. She later had neutrophil leucocytosis, thrombocytosis, lymphocytosis, lymphomas and splenomegaly. These cases, together with 6 cases published by others, suggest that an association exists between the two diseases.
Platelet Function and Fibrinolytic Activity Following Distance Running
Authors: Knudsen J. Bjerre , Brodthagen U. , Gormsen J. , Jordal R. , Nørregaard‐Hansen K. , Paulev P.‐E.
Published: August 2010

6 long distance runners from the Danish marathon elite and 6 non‐runners completed test runs of 28 and 12 km, respectively. Distance runners and non‐runners showed the same responses in platelet function. We found a significant decrease in ADP induced platelet aggregability, a decreased serotonin release induced by ADP and collagen and an increase in platelet factor 4 immediately following the run. The antithrombin III levels remained constant. Euglobulin lysis time was shortened (by approximately 50 %) and the plasminogen levels significantly increased. The last 2 findings indicate an equal increase in fibrinolytic activity during distance running in both groups.

While short term, strenuous exercise induces platelet hyperaggregation, long term distance running induces a state of exhaustion of platelet aggregation capacity.
Kinetics of Heat Damaged Autologous Red Blood Cells
Authors: Peters A. M. , Ryan P. F. J. , Klonizakis I. , Elkon K. B. , Lewis S. M. , Hughes G. R. V. , Lavender J. P.
Published: August 2010

The kinetics of radiolabelled heat damaged red cell (HDRBC) distribution have been studied in humans using a gamma camera, and compared with the kinetics of other blood cells. Liver uptake of 111In labelled HDRBC was completed within about 10 min of injection; splenic uptake was biphasic with a half time of about 5 min over the first 20 min following injection, and a later half time much longer than this. Activity initially present in the lung fields cleared within 24 h. The rate constants of liver uptake of 99mTc labelled HDRBC and of 111In labelled platelets were very similar; the rate constants of splenic uptake of these 2 particles were also very similar up to about 20 min following injection when the splenic platelet levels became constant and the HDRBC level continued to slowly rise. Splenic uptake and blood clearance of red cells coated with IgG (IgG‐RBC), in contrast to HDRBC, were monoexponential.

It was concluded that: (1) the blood clearance of HDRBC was due to pooling within, and to irreversible extraction by, the spleen; (2) liver uptake of HDRBC, which was irreversible, was completed within 10 min of injection; (3) IgG‐RBC clearance was due to irreversible extraction by the spleen; (4) HDRBC uptake in the lung was unrelated to reticuloendothelial function, and represented prolonged transit through the lung microvasculature.
A Scanning Electron Microscopic Investigation of Effects of Prostacyclin on Platelet Retention in Glass Bead Columns
Authors: Ts'ao C. , Krajewski D. , Ng A.
Published: August 2010

As whole blood passes through a column of glass beads, platelets are activated and all 3 basic platelet properties (adhesion, release reaction and aggregation) displayed, an aspect similar to platelets being involved in a hemostatic process. For this reason, we chose the platelet retention test as the tool to study the effect of synthetic prostacyclin (PGI2) on platelet function and behavior. PGI2 inhibited platelet retention with its major impact on platelet aggregation. The extent of inhibition was not proportional to the concentration of PGI2. It appears that 2 levels of platelet aggregation take place inside the column. One, triggered by a weak stimulus, is readily inhibitable by low concentrations of PGI2. The other, brought about by a strong stimulus, requires higher concentrations of PGI2 to achieve complete inhibition. Even with as much as 100 ng PGI2/ml blood, a 15% retention remained which probably represented platelet adhesion to glass beads. Scanning electron microscopic examination of glass beads showed massive platelet aggregation and adhesion in control blood samples. No platelet aggregate was observed on beads filtered with blood containing 20 ng/ml or more PGI2. Individual platelets were found on glass beads filtered with samples containing any concentration of PGI2; these platelets exhibited extensive morphologic changes.
Aplastic Anaemia with Marrow Defective in Formation of Fibroblastoid Cell Colonies in Vitro
Authors: Wiktor‐Jedrzejczak W. , Siekierzynski M. , Szczylik C. , Gornas P. , Dryjanski T.
Published: August 2010

The formation of fibroblastoid colonies by marrow cells in vitro has been used as a putative assay for a stem cell of haemopoietic stroma. Bone marrow from one patient with aplastic anaemia did not form any of these colonies, while its growth in diffusion chambers as an indirect measure of a haemopoietic stem cell was even better than normal. On the other hand, marrow from the other aplastic anaemia patient showed only quantitative decrease in the formation of fibroblastoid colonies and simultaneously grew very poorly in diffusion chambers. These patients were indistinguishable by the cytological examination of their marrow, however, the peripheral blood abnormalities were expressed less severely in the first patient. These results suggest the contribution of the defect in marrow cells, which form fibroblastoid colonies in vitro to the development of aplastic anaemia in these patients.
Blastic Phase of Myeloproliferative Syndrome Coexisting with a Malignant Teratoma
Authors: Hagberg H. , Gustavson K. H. , SundstrÖm C. , Gerdes U.
Published: August 2010

A myeloproliferative condition in blastic phase is described in an 18‐year‐old male who was also found to have a mediastinal malignant teratoma. Myeloid metaplasia was found in the lymph nodes and spleen, and an infiltration of granulocytic blast cells was observed in the bone marrow and the lymph nodes. Aneuploidy with an extra chromosome (trisomy 8) was present in bone marrow cells. To our knowledge the combination of a myeloproliferative disorder and a malignant teratoma has not been earlier described.
Lithium Therapy for Cyclic Neutropenia in Children
Authors: Ishii Eiichi , Hara Toshiro , Miyazaki Sumio , Fujiwara Takashi , Goya Nagahide
Published: August 2010

2 children with cyclic neutropenia were treated with lithium carbonate. In one patient, fever and stomatitis were ameliorated, but the duration of neutropenia was increased and parotitis occurred. In another patient, aphthous stomatitis disappeared but fever persisted. The duration of neutropenia was prolonged and submaxillaritis and sinusitis occurred.

Thus, lithium administration may lead to complications when the duration of neutropenia is prolonged. We recommend discontinuation of the drug, even in the presence of amelioration of symptoms.
Plasma Lactoferrin Measured by an Enzyme‐Linked Immunosorbent Assay (ELISA)
Authors: Otnaess Anne‐Brit Kolstø , Meberg Alf , Sande Hans Andreas
Published: August 2010

The lactoferrin content of human plasma has been measured by an enzyme‐linked immunosorbent assay. In cord blood the level was 0.02‐0.3 mg/l, corresponding to 3–44 × 10‐10 mol/l lactoferrin; in plasma 5 d post partum the level had not changed. In adults the level was 0.02‐0.2 in 29 out of 30 plasma samples and above 1 mg/l in 1 sample. Similar results were obtained with EDTA, citrate or heparin as anti‐coagulant.
Influence of Human Serum Components on Measurement of Erythropoietin Biological Activity in Vitro
Authors: Firkin Frank C. , Russell Sue H.
Published: August 2010

The influence of human serum components during in vitro determination of erythropoietin biological activity has been examined in a modified in vitro bioassay procedure utilising rabbit bone marrow as the erythropoietin‐responsive tissue. The number of erythroblasts present after 5 d in suspension culture provided a measure of erythropoietin biological activity with a degree of sensitivity equivalent to that provided by estimation of 59Fe incorporation into haem, but possessed the advantage of lacking the susceptibility of the latter method to the level of human serum transferrin saturation. Erythropoietic stimulation was blocked by 10% human serum unless all sera incorporated into the bioassay was previously heated at 56°C. Human serum under the latter conditions was shown to contain material which stimulated erythropoiesis, but which differed in its mode of action from erythropoietin in that it operated by amplifying the response to erythropoietin. Correction for these effects substantially altered interpretation of erythropoietic stimulatory activity of human serum to yield values in keeping with those reported for in vivo bioassay. These findings illustrate the need to clarify the role of human serum components which modify the action of erythropoietin under culture conditions, in view of their potential contribution to discrepancies between values obtained by different bioassay procedures.
Severe Iron Deficiency Causing Loss of Ring Sideroblasts
Authors: Stavem Per , Rørvik Tore O. , Rootwelt Kjell , Josefsen Johan O.
Published: August 2010

A patient with primary sideroblastic anaemia was observed for more than 10 years with 50% ring sideroblasts in the bone marrow and a Hb of 84–109 g/l. Iron deficiency then developed with loss of stainable iron in marrow particles (reticuloendothelial iron) and loss of all ring sideroblasts. When iron tablets had brought the iron stores in the bone marrow up to subnormal levels, the Hb was over 110 g/l, somewhat higher than it had been when the iron stores were increased. The ring sideroblasts were again raised to more than 50%.
Transition of Myelofibrosis to Polycythaemia Vera
Authors: Hasselbalch Hans , Berild Dag
Published: August 2010

A case of typical myelofibrosis with a huge spleen is described in a 62‐year‐old man. During the subsequent 2‐year follow‐up, a clinical picture of polycythaemia vera with pancytosis and disappearance of the marrow fibrosis was observed. The pancytosis necessitated treatment with busulphan and frequent phlebotomies. The transformation was associated with prednisone treatment for a suspected haemolytic state. During this treatment, the spleen no longer became enlarged on clinical palpation, although it was still enlarged at post mortem examination, but much less than 2 years earlier.
The Value of Immunoperoxidase Studies in the Diagnosis of 2 Cases of Acute Leukaemia
Authors: Mirchandani Ila , Palutke Margarita , Dutcher Thomas F. , Bishop Carter R.
Published: August 2010

2 cases of acute leukaemia in which the precise diagnosis was established using the immunoperoxidase technique on particle sections, a method not usually employed for acute leukaemias, are reported. Morphologially and cytochemically these cases were initially diagnosed as acute megakaryoblastic (case 1) and acute monocytic (case 2) leukaemia. Based on the immunoperoxidase studies, these diagnoses were corrected to DiGuglielmo's disease (case 1) and plasma cell leukaemia (case 2).
Release of Growth Promotors from Mononuclear Human Blood Cells by LPS, Lectins and Lithium, as Tested in the Mouse CFU‐C Assay
Authors: Gjerde Bjørn , Bøyum Arne , Løvhaug Dagfinn , Melby Tone
Published: August 2010

Medium (MCM, 20 % human serum), conditioned for 24 h by mononuclear human blood cells, had no colony‐forming ability when tested in the mouse CFU‐C assay. However, when combined with L‐CSF, which predominantly generates macrophage colonies, MCM increased the colony number and size. Even more significant was the increased cellularity with a striking shift towards granulocyte production. Some human sera induced GIF formation without additives, but mostly LPS was required. After heat inactivation, all sera became dependent on LPS to yield an active MCM. Lithium, PHA, Con A and PMW also stimulated GIF formation, which itself depended upon protein synthesis.

Heat inactivation of LPS and serum did not reduce their ability, in combination, to yield an active MCM. However, when serum and LPS were mixed before heat treatment, the ability to induce GIF production was abolished, but could be restored by adding intact LPS. This may indicate that LPS exerts its effect by combining with a serum factor yielding a heat‐sensitive complex. However, even in the absence of serum, LPS had a stimulatory effect when used in large concentrations, but still the MCM was less active than MCM with serum.
The Peripheral Platelet Count and ADP‐Induced Platelet Aggregation in Response to Metoprolol and Propranolol as Studied in Young Healthy Male Volunteers
Authors: Vilén Lars , Kutti Jack , Fredén Krister , Lundborg Per , Cronberg Stig
Published: August 2010

In previous studies, we have demonstrated that the oral administration of one single dose of metoprolol as well as of propranolol causes a significant increase in the venous platelet concentration. In the present study, we investigated the effect of metoprolol, administered twice daily over a period of 1 week, on the venous platelet count in a group of healthy volunteers. The results showed that the drug significantly raised the venous platelet concentration over the whole study period. Further, the effect of 1 week's metoprolol and propranolol medication on ADP‐induced platelet aggregation was investigated in another group of healthy subjects; the results were compared to those obtained during 1 week of placebo medication. It was demonstrated that neither of the two β‐blocking drugs affected ADP‐induced platelet aggregability; this was true on the first as well as on the last day of study.
Circulating Immune Complexes in Haemophilia and von Willebrand's Disease
Authors: Passaleva A. , Massai G. , Morfini M. , Longo G. , Ferrini P. L. Rossi , Ricci M.
Published: August 2010

Sera from 63 patients with haemophilia A, 21 with haemophilia B and 29 with von Willebrand's disease were screened for the presence of circulating immune complexes (CICs), serological markers of hepatitis A and B virus, autoantibodies and factor VIII or factor IX inhibitors. CICs were detected by the 125J Clq binding assay (ClqBA), the solid phase conglutinin assay (KgBSP) and the solid phase Clq assay (ClqSP).

The incidence of CICs detected by the ClqBA and the ClqSP methods in haemophiliacs and in von Willebrand patients was higher than that observed in normal subjects, while the prevalence of CICs detected by the KgBSP method was not. The presence of CICs was not correlated with patient age, severity of disease, presence of hepatitis B virus serological markers, abnormal liver function tests or factor VIII inhibitors. A significant connection was demonstrated between CICs detected by the ClqBA method and replacement therapy when the dose administered over 1 year was over 20000 U of factor VIII or IX concentrates. The high proportion of CICs in von Willebrand's disease, not connected with the replacement therapy or the presence of serological markers of hepatitis virus, is in agreement with the possibility that immune complexes may be related to the disease itself and independent, at least in part, of exogenous agents.
Immunoglobulin Synthesis in Hairy Cell Leukaemia
Authors: Reynes M. , Tricottet V. , Capron F. , Diebold J.
Published: August 2010

3 cases of hairy cell leukaemia were studied with ultrastructural immunocytochemical methods using an anti human Ig HRPO‐Fab fragment. Ig were detected on the cell surface, in the perinuclear cisterna and endoplasmic reticulum of hairy cells. Evidence of Ig in these sites demonstrates a B‐lymphoid differentiation of the leukaemic cells.
Dominant familial erythrocytosis with low plasma erythropoitin activity. Studies on four cases
Authors: LY BERNT , MEBERG ALF , KANNELØNNING KJELL , REFSUM HARALD E. , BERG KÅRE
Published: August 2010

Within the same family erythrocytosis occurred in the mother, in three of five children and possibly in one child of an affected daughter. The occurrence of the anomaly in successive generations as well as the distribution of affected and healthy persons in the offspring of affected individuals are in excellent agreement with autosomal dominant inheritance.

Hemoglobin function (P50) and red cell function (2,3‐diphosphoglycerate) were normal. Plasma erythropoitin activity in plasma of affected individuals was undetectable when measured by cell culture assay. Only low levels of activity, comparable to that occurring in normal plasma, were detected using 59Fe incorporation in erythrocytes of exhypoxic, polycythemic mice and this activity did not increase upon phlebotomy of 15–20% of the blood volume. Thus, erythrocytosis was not caused by increased erythropoitin production but some other, unknown mechanism.
Soluble I131fibrin form high molecular weight complexes in prothrombin‐depleted, recalcified plasma at +37 °C
Authors: BROSSTAD FRANK , KIERULF PETER
Published: August 2010

Gel filtration of plasma samples containing I125fibrinogen and either I131des‐AA soluble fibrin or I131des‐AABB soluble fibrin, were performed on Sepharose 6B C1 columns at + 37 °C, equilibrated and developed with prothrombin‐depleted plasma, either recalcified or containing EDTA. With EDTA, des‐AA fibrin eluted in a homogenous, monomeric form, while des‐AABB fibrin had an elution profile indicating high molecular weight derivatives. In the presence of calcium ions, both des‐AA and des‐AABB soluble fibrin eluted mainly as high molecular weight derivatives. It could not, however, be decided whether the high molecular weight derivatives consisted of fibrin molecules alone, or of co‐polymers of fibrin‐fibrinogen. It is concluded that soluble des‐AA fibrin and soluble des‐AABB fibrin in plasma at + 37 °C and in the presence of calcium ions, participate in the formation of high molecular weight derivatives of fibrin.
Comparative Studies of the in Vivo Kinetics of Simultaneously Injected 111In‐ and 51Cr‐Labelled Human Platelets
Authors: Schmidt Kai Gjerløff , Rasmussen Jens Wæver , Rasmussen Anker Dahl , Arendrup Henrik
Published: August 2010

The kinetics of simultaneously injected 111In‐ and 51Cr‐labelled platelets have been assessed in 40 subjects, 13 of them thrombocytopenic. 4 platelet survival models were applied. The mean life‐time (MLT) of 51Cr‐platelets from non‐thrombocytopenic individuals was found to be slightly, but significantly, longer than that of 111In‐platelets by applying linear and exponential models for data fitting. The in vivo recovery (IVR) of 111In‐platelets was significantly higher than that of 51Cr‐platelets in this patient group when using all 4 models. In the group of thrombocytopenic patients no statistically significant differences in MLT or IVR were found between 111In‐ and 51Cr‐platelets. However, for each of the 11 51Cr‐labelled platelet suspensions with the shortest MLT, a longer MLT was observed in the corresponding 111In‐platelets, a finding probably related to antibody‐induced elution of 51Cr‐activity. The same mechanism might be responsible for an increasing 111In‐/51Cr‐recovery ratio in the early post‐injection period. The efficiency of platelet isolation from blood prior to labelling seemed to influence the IVR, inasmuch as the difference in IVR between 111In‐ and 51Cr‐platelets was eliminated in the group where the yield of 111In‐platelets surpassed that of the 51Cr‐platelets by more than 15 %.
Polycythaemia Following Splenectomy in Myelofibrosis with Myeloid Metaplasia. A Reorganization of Erythropoiesis
Authors: Barosi G. , Baraldi A. , Cazzola M. , Fortunato A. , Palestra P. , Polino G. , Ramella S. , Spriano P.
Published: August 2010

3 patients with myelofibrosis with myeloid metaplasia were splenectomized because of anaemia and disturbing splenomegaly. In the course of the 6 months following splenectomy, a polycythaemia developed. Erythrokinetic studies demonstrated that in all cases a reduction in plasma volume and an increase in red cell volume was obtained. Total erythropoiesis decreased along with normalization of ineffective erythropoiesis and peripheral haemolysis. The reappearance of an erythropoietic activity measured over the sacrum was a constant finding, while in 1 patient, a depression of activity over the liver was observed. The new distribution and organization of erythropoiesis in the splenectomized patients is hypothesized as being due to the removal of the influence of an enlarged spleen on erythropoietic organs.
Differential Effect of the Serine Protease Inhibitor Phenyl Methyl Sulfonyl Fluoride on Cytochemically Detectable Esterases in Human Leucocytes and Platelets
Authors: Dufer Jean , Trentesaux Chantal , Desplaces Andrée
Published: August 2010

Esterases of human leucocytes and platelets were studied by cytochemical methods. The aim of the study was to clarify the cellular distribution and possible nature of esterases types differing in their substrate specificity and/or their inhibitor sensitivity. 3 substrates (α‐naphthyl acetate: ANA; naphthol AS‐D chloroacetate: NASDCA; and N‐acetyl DL‐alanine α‐naphthyl ester: NACALA) were used and the effects of 2 inhibitors (sodium fluoride and the serine protease inhibitor phenyl methyl sulfonyl fluoride: PMSF) were evaluated. 4 enzyme types were described: Type I, present in granulocytes, was detected using NASDCA and NACALA and was resistant to fluoride but sensitive to PMSF. Other types were detected using ANA as substrate. Type II, present in monocytes, was inhibited by both fluoride and PMSF. Type III, present in platelets and plasma cells, was inhibited by fluoride but resistant to PMSF. Type IV, present in lymphocytes, was resistant to both fluoride and PMSF. The specific aims and possible areas for application of these results are discussed.
Treatment of Advanced Bone Marrow Neoplasms with Ifosfamide Combinations
Authors: Norberg Bo , Holm Jan , Winqvist Eric , Wahlin Anders
Published: August 2010

Ifosfamide, in combination with other cytostatics, was given to 15 patients with advanced neoplasia derived from bone marrow cells. A response to therapy was obtained in 8 patients. It is suggested that ifosfamide, in combination with other drugs, deserves larger trials in the treatment of advanced bone marrow neoplasia.
The effect of acetylsalicylic acid in 3 different formulations on in vitro and in vivo platelet function tests: An experimental study on healthy male volunteers
Authors: Kutti Jack , Safai‐Kutti Soodabeh , Sigvaldason Andres , Edgar Boo , Lundborg Per
Published: August 2010

The template bleeding time (TBT), ADP‐induced platelet aggregation, and serum production of TXB2 were measured in healthy young male subjects immediately before, and on days 1, 4 and 6 after the ingestion of 1 single dose of 500 mg acetylsalicylic acid (ASA) in 3 different formulations: Aspirin® (Bayer), and the 2 enteric‐coated formulations Reumyl® (Hässle) and Premaspin® (Lääke). The ingestion of Aspirin® resulted in a significant prolongation of the TBT over a period of 6 d. However, after the ingestion of the same amount of ASA in the 2 enteric coated formulations, the TBT as measured on day 6 had become normalized. After the ingestion of Aspirin®, there was no reappearance of the second wave of ADP‐induced platelet aggregation during the study period; however, after the ingestion of the 2 enteric‐coated formulations, secondary platelet aggregation occasionally returned on day 6. In response to the intake of each of the 3 ASA formulations, the serum TXB2 production as measured 24 h later was almost completely inhibited. In each of the 3 study groups, the TXB2 formation as measured on day 6 was still significantly impaired.
Haemolysis in a G6PD‐deficient child induced by eating unripe peaches
Authors: Globerman Hadas , Navok Tikva , Chevion Mordechai
Published: August 2010

A child suffering from G6PD deficiency developed a severe haemolytic crisis without an apparent trigger. The possible pathogenetic role of the ingestion of unripe peaches was studied biochemically in this anaemia. We show that an extract from the unripe peach exerts an oxidative challenge on normal as well as on asymptomatic G6PD‐deficient erythrocytes. This effect is analogous to that of the favism‐inducing agents. The effect of the extract on the patient's red blood cells was more pronounced than on other asymptomatic G6PD‐deficient erythrocytes, particularly during his haemolytic crisis. The chemical nature of the deleterious component was not identified. It is suggested that unripe peaches be added to the list of hazards for G6PD‐deficient subjects in combination with other factors.
Histocompatibility antigen frequencies in patients with chronic lymphocytic leukaemia: Possible identification of a subgroup with relatively benign disease
Authors: Kilpatrick D. C. , Dewar A. E. , Stockdill G. , Maginnis M. , Jordan A. , Neill G. W. , Gelsthorpe K. , Yap P. L.
Published: August 2010

A series of 57 patients with chronic lymphocytic leukaemia was typed for HLA‐A, B and DR antigens and compared with a control population of equal size. There was an increased frequency of HLA‐A1 and B8 in the patient group, although it was not statistically significant after allowance for the number of specificities tested. A subgroup of patients lacking both Al and B8 differed from the others in sex ratio, degree of lymphocytosis and incidence of hypogammaglobulinaemia at presentation. This subgroup also had less need for treatment within 6 months of presentation. While these findings must be considered preliminary, it is possible that tissue typing may help to identify patients with a relatively benign form of the disease.—
Different forms of AT‐III congenital defect: A study by crossed immunoelectrofocusing
Authors: Bizzi Bruno , Leone Giuseppe , Cotumaccio Roberto , de Stefano Valerio , Zanetti Letizia
Published: August 2010

Antithrombin III (AT‐III) deficiency may be due to quantitative or qualitative AT‐III reduction. The diagnosis of qualitative disorder is suspected when a discrepancy is found between immunological and functional levels of AT‐III. Heterogeneity has been hypothesized in both quantitative and qualitative deficiency of AT‐III. A technique based on crossed immunoelectrofocusing (CIEF) was applied to investigate molecular variants of AT‐III. 3 families with low functional and immunological levels of AT‐III and 1 family with only a low functional AT‐III level were investigated. An abnormal AT‐III pattern was found with CIEF in the family with suspected qualitative disorder and in 1 of the families with quantitative disorder. The 2 abnormal patterns were different. Thus the use of CIEF AT‐III patterns could help to define congenital AT‐III deficiencies and could serve as a basis for classification of different forms of AT‐III deficiency.
Ovarian function in young women in long‐term remission after treatment for Hodgkin's disease stage I or II
Authors: SPECHT LENA , HANSEN MOGENS MøRK , GEISLER CHRISTIAN
Published: August 2010

16 young women in long‐term remission after first‐line treatment for the early stages of Hodgkin's disease were examined for ovarian function 48 to 125 months after termination of therapy. The patients had received mantle field irradiation, plus either irradiation of infradiaphragmatic lymph nodes or 6 cycles of MOPP. 4 patients showed signs of ovarian failure judged by menopausal symptoms, menstrual pattern and/or hormone values. 12 patients had functioning ovaries; 8 of these had become pregnant after treatment, 2 had had an induced abortion, and 7 had given birth to a total of 9 healthy babies after treatment. The patients with signs of ovarian failure were older than the others, but the difference was not statistically significant. No difference between the patients who had received different treatments was established, nor does the study confirm the proposed protective effect of oral contraceptives. For women under 35 years of age, the long‐term chances of preserving ovarian function after standard treatment for the lower stages of Hodgkin's disease seem to be much better than hitherto assumed.
Prognostic implications of in vitro colony studies and clonal chromosomal aberrations in adults with acute leukaemia
Authors: HöRNSTEN PER , LINDQUIST RAGNHILD , GAHRTON GöSTA
Published: August 2010

A comparison was made between in vitro colony culture parameters and chromosomal abnormalities in 44 adult patients with acute leukaemia, with special reference to prognosis. The presence of clonal chromosomal aberrations in some (AN) or all (AA) metaphases of bone marrow cells was a fairly strong indication of poor prognosis, while the absence of abnormal metaphases (NN) was a favourable prognostic sign. 6 of 7 patients in the NN group entered complete remission (CR), as did 8 of 20 in the AN + AA group and 8 of 17 in the group with random loss of chromosomes (NNA). The median survival time for AN + AA patients was 4 months, while for patients without clonal aberrations (NN + NNA) it was 9 months. Of the cultural parameters studied, colony‐stimulating activity (CSA) of peripheral leucocytes had the strongest correlation to prognosis. CSA > 1 was related to remission (P< 0.01) and longer survival (P< 0.05 at 4 months), while 8 patients with CSA ≤ 1 were all dead at 8 months. However, very high CSA values (> 20) indicate a poor prognosis. Although both chromosome analysis and CSA were of prognostic value, combining the 2 parameters did not improve the prognostic information, mainly because of covariation of NN and CSA > 1.
Exercise‐Induced Changes in the in Vivo Distribution of 111In‐Labelled Platelets
Authors: Schmidt Kai Gjerløff , Rasmussen Jens Wæver
Published: August 2010

In order to throw some light on the mechanisms underlying exercise‐induced thrombocytosis, we investigated 15 healthy persons subjected to short‐term vigorous pedalling on a bicycle ergometer 1 d after injection of autologous 111In‐labelled platelets. Scintigraphic studies during the post‐exercise period showed the spleen to be the major platelet‐releasing organ. There was, however, a considerable interindividual variability manifested as signs of a contributing platelet release from the lungs in some cases and of deposition of a surplus of released platelets in the liver of others. Our results also seem to be compatible with the existence of an intravascular marginal platelet pool.
Hairy‐cell leukaemia simulating connective tissue disease
Authors: HASSELBALCH HANS
Published: August 2010

A patient is reported, in whom clinical and biochemical evidence of connective tissue disease preceded the diagnosis of hairy‐cell leukaemia by at least 2 years. The pathogenetic mechanism(s) responsible for the coexistence of hairy‐cell leukaemia with vasculitis and rheumatic disease is discussed. It is proposed that the 2 diseases may share a common predisposing factor, or that the hairy cells may elicit disturbances in immune homeostasis, implying a dysfunction of T suppressor cells. Hairy‐cell leukaemia should be considered in the differential diagnosis in cases of unexplained febrile illness associated with pancytopenia and signs of connective tissue disease.
Dyskeratosis congenita Haematologic, cytogenetic, and dermatologic studies
Authors: JACOBS PETER , SAXE NORMA , GORDON WALTER , NELSON MOLLY
Published: August 2010

In a family of 5 boys and 6 girls, 3 brothers have clinical dyskeratosis congenita. Teeth from 2 of the patients were taurodent, and mineral density of the enamel was significantly different from normal. The haematopoietic marrow was hypocellular and there was striking prominence of plasma cells having normal morphology; no granulomata were demonstrated. The decreased erythroid precursors in the marrow correlated with quantitatively reduced erythropoiesis demonstrated on ferrokinetic studies. Recurrent infections occurred but could not be related to neutropenia, and granulocytes and monocytes retained normal function. No abnormality was demonstrated in humoral or cellular immune mechanisms.

While superficially similar, dyskeratosis congenita and Fanconi's anaemia are genetically distinct, being X‐linked in the former and inherited as an autosomal recessive in the latter.
Interpretation of measured red cell mass and plasma volume in males with elevated venous PCV values
Authors: Pearson T. C. , Botterill C. A. , Glass U. H. , Wetherley‐Mein G.
Published: August 2010

A method of interpretation of red cell mass (RCM) and plasma volume (PV) data is described. The results in 188 males with PCV's over 0.50 places the patients in 4 groups: true (absolute) polycythaemias, relative (low plasma volume) polycythaemias, high normal red cell mass (HNRCM) and ‘physiological variant’. Absolute polycythaemias were increasingly frequent at higher PCV levels but only reached 100% at a PCV of 0.60. They showed an 18% incidence in the lower PCV range of 0.500‐0.519. Relative (low PV) polycythaemia was found in 18% of the patients with PCV values in the range 0.500 to 0.599. Although the HNRCM and ‘physiological variant’ types found mainly in the lower PCV ranges they occurred at the 0.54 level. While this method of interpretation of RCM and PV data is perhaps arbitrary, it does provide a basis for the proper study of the common group of patients with raised PCV levels in which classification, course and treatment are uncertain. In addition the findings suggest that both RCM and PV should be measured at all levels of PCV over 0.50; that relative (low PV) polycythaemia is a real entity but less common than sometimes believed; that diuretics do not have a notable part in its causation and that the commom HNRCM, ‘physiological variant’ groups are incompletely understood and require further study.
Myeloperoxidase‐deficient polymorphonuclear leucocytes (III): Relation to incidence of infection in acute myeloid leukaemia
Authors: Nielsen Henning Kaspersen , Bendix‐Hansen Knud
Published: August 2010

In 74 cases of acute myeloid leukaemia (AML) the relation between pretreatment myeloperoxidase (MPO) activity in polymorphonuclear leucocytes (PMN) and the incidence of infection in the preremission phase of the disease was investigated retrospectively. 36 patients had abnormal numbers (>4%) of MPO‐deficient PMN and 38 had normal numbers. In the first group more patients experienced fever attacks, more showed an infectious focus and an aetiological cause was demonstrated more frequently than among patients in the second group. This difference was statistically significant (P < 0.01). Furthermore, the patients in the first group experienced more fever attacks, showed more infectious focus and had infectious microorganisms demonstrated in more febrile episodes than patients in the second group (P < 0.01). The differences were not explained by differences in the incidences of neutropenia or other parameters investigated. It is concluded that decreased MPO activity in PMN from AML patients may contribute to the increased susceptibility to infections and that in the preremission phase of the disease it may account for approximately 15% of the infections.
Changes in the character of Factor VIII inhibitors following Factor VIII transfusion
Authors: Ghosh S. , Rizza C. R.
Published: August 2010

Factor VIII inhibitors from 4 haemophiliacs have been studied at intervals following transfusion of a factor VIII preparation with an aim to correlate their reaction kinetic and avidity with their immuno‐chemical character. Post‐treatment antibodies are more avid than pre‐treatment antibodies and show a second order reaction while pre‐treatment antibodies produce a graph of complex nature for the time course inactivation of Factor VIII. It seems that IgG3 anti VIII:C that forms a significant part of post‐treatment antibody is responsible for these discrepancies.