bluuebunny/crossref_metadata_2025 huggingface dataset:
Diurnal Variations in Cobalamin Binding Plasma Proteins
Authors: Brynskov Jørn , Gimsing Peter
Published: August 2010

The diurnal variations in cobalamin‐binding plasma proteins were studied in 10 healthy volunteers. The unsaturated cobalamin‐binding capacities of transcobalamins (TC‐II and R‐binders), P‐cobalamin and P‐protein were measured every third hour during 24 h. Only small variations within an interval of ± 20% were found. Highly significant decrements during the night were a uniform finding. After correction for variations of total plasma protein, the changes became less prominent, but still significant for P‐cobalamin and P‐R‐binders. Hence the mean values of P‐R‐binders and P‐cobalamin were 113 and 533 pmol/1 at 9:00 h, and decreased 12 and 8%, respectively, at 3:00 h.

When using cobalamin binding plasma proteins in the monitoring of various malignant, haematological and autoimmune disorders, the specimens should, therefore, be collected at defined hours and in a uniform posture.
A New Case of γ‐Heavy Chain Disease
Authors: Cozzolino Federico , Vercelli Donata , Castigli Emanuela , Becucci Aureliano , Guglielmo Renato Di
Published: August 2010

The detection of a γ‐heavy chain disease protein in the serum and urine of a patient with a history of Hodgkin's disease in complete remission is reported. The protein has been characterized as a γ1 dimer, MW 66000, beginning at Asp 221 residue in the hinge region. No clinical or laboratory findings of malignancy have been observed in the patient since the abnormal protein was detected. The pathogenesis of this association, unreported to date, is discussed on the basis of previously administered high‐dose anti‐neoplastic treatments and of immunological impairment.
A Megakaryocytic Thrombocytopenia Associated with an Excess of Leu 2a+ Suppressor Cells
Authors: Worman C. P. , Mills K. H. G. , Linch D. C. , Beverley P. C. L. , Goldstone A. H. , Cawley J. C.
Published: August 2010

8 patients with acute myeloid leukaemia (AML), either at presentation or in remission, had normal T‐cell subsets as defined by Fc receptors and monoclonal antisera. In contrast, a persistant excess of T lymphocytes staining with Leu 2a (defining suppressor cells) was noted in a case of AML in which persistant amegakaryocytic thrombocytopenia occurred after other haemopoietic lines had recovered from ablative chemotherapy. It is suggested that the 2a+ cells were involved in the suppression of megakaryocytopoiesis, and the phenotype of the lymphoid cells is compared and contrasted with previously described cases of other cytopenias associated with an excess of suppressor cells.
Foetal Type Acetylcholinesterase in an Anaemic Patient with high HbF
Authors: Garrè C. , Ravazzolo R. , Strada P. , Cappellini M. D. , Ajmar F.
Published: August 2010

A patient with sideroblastic anaemia and hereditary persistence of foetal Hb was examined for acetylcholinesterase (Ache) type and i antigen. Ache electrophoresis showed a mobility intermediate between adult and foetal blood; i antigen was of foetal type.
E Receptor Expression of T Lymphocytes in T Lymphoproliferative Diseases
Authors: Jønsson V. , Andersen B. Lasthein , Brandrup F. , Christensen B. E. , R.Othenborg H. W. , Thomsen K.
Published: August 2010

The lymphoid component of skin infiltrates from 6 cases of mycosis fungoides and 2 cases of T cell chronic lymphatic leukaemia consisted almost exclusively of T lymphocytes with abnormally high E receptor expression. Such T cells were also found to dominate the skin infiltrates from 10 patients with severe atopic dermatitis. Blood T lymphocyte counts from all types of patients were within the normal range, but even the blood T lymphocytes of the patients had a strong E receptor expression. The bone marrow in atopic dermatitis and in mycosis fungoides showed normal T cell counts with normal E receptor expression. Supported by basic lymphocyte‐kinetic considerations it is likely that the skin in T lymphoproliferative diseases is characterized by an abnormal ability to home and/or to trap T lymphocytes with strong E receptor expression.
Collagen and Bleeding Diathesis in Ehlers‐Danlos Syndrome
Authors: Udén Alf
Published: August 2010

Financial support was obtained from the Herman Järnhardt's Foundation.

There are at least 7 variants of Ehlers‐Danlos syndrome (EDS). One family with type 1, one with type 3 and one isolated case with an undefined type of EDS are described. They all had bleeding symptoms, but surgery had been performed without complications. Collagen is believed to be defective in EDS. However, in our cases the platelet aggregating power of the collagen was normal. The bleeding time (Ivy) was slightly prolonged. Extensive examination of the haemostatic process revealed a decreased platelet adhesiveness in only 2 of the patients, a finding believed to be coincidental. The history of former operations on the patients and on affected relatives is considered to be the best guide in determining the risk of an operation, but preoperative estimation of the bleeding time is recommended.
Does Preincubation of the Red Blood Cells Contribute to the Capability of the Osmotic Fragility Test to Detect very Mild Forms of Hereditary Spherocytosis?
Authors: Godal H. C. , Gjønnes G. , Ruyter R.
Published: August 2010

During incubation for 24 h at 37°C, erythrocytes from patients with hereditary spherocytosis (HS) undergo a greater increase in osmotic fragility than do normal cells, and this procedure has been recommended for differentiating more clearly between patients with very mild HS and normal subjects. The greater effect of preincubation on erythrocytes from patients with HS was confirmed, but, except in cases demonstrating a markedly increased osmotic fragility before incubation, this effect was outweighed by a simultaneous loss of test precision. It therefore seems that preincubation does not significantly contribute to the capability of the osmotic fragility test to detect very mild forms of HS.
High Dose Cytosine Arabinoside in the Management of Refractory Acute Leukaemia
Authors: Willemze R. , Zwaan F. E. , Colpin G. , Keuning J. J.
Published: August 2010

17 patients (age 15–58 years) with refractory acute leukaemia (14 AML, 3 ALL) were treated with high dose cytosine arabinoside (AraC) at a dose of 3000 mg/m2, twice daily for 6 d (13 patients with AraC alone, 4 patients with AraC and doxorubicin). 9 patients achieved complete remission (53 %) and 2 a partial remission. Although sophisticated isolation and blood banking facilities are required during the pancytopenic period, the toxicity of this treatment was acceptable. Vomiting, headache, somnolence, fever, conjunctivitis and cardiac arrhythmias were found most frequently. The unexpected pulmonary failure in 3 patients was worrisome. The duration of remissions was from 1 to 12 months.

Results obtained with high dose AraC are satisfactory and hold promise for the treatment of patients with previously untreated AML.
Generalized Monocyte Deficiency in Leukaemic Reticuloendotheliosis
Authors: Janckila Anthony J. , Wallace John H. , Yam Lung T.
Published: August 2010

Examination of the blood of 40 patients with leukaemic reticuloendotheliosis (LRE) revealed a drastic reduction in monocyte numbers (mean 74/μl) when compared to a normal group of 33 (mean 442/μl). Repeated blood studies in 8 LRE patients were made over periods of 2 months to 5 years and monocytopenia was observed to be a persistent phenomenon. Similarly, tissues from 29 patients showed an overall decrease in numbers of monocytes/histiocytes when compared with those of 73 control subjects. Skin window examinations in all 11 patients so studied showed marked reduction or absence of monocyte response to inflammation. 2 exceptional cases to this trend are noted. In one, circulating monocyte levels remained normal over a 14‐month period while examinations of blood and marrow in this case showed few hairy cells. In another case, severe and persistent monocyte deficiency was noted during a 2‐year period of unsuccessful treatment. When he eventually responded to low dose chlorambucil and his Hb rose from 40–140 g/1 and WBC became normal, the number of circulating monocytes also became normal.
Enhanced in Vivo Platelet Activation in Diabetes Mellitus
Authors: Ek I. , Thunell S. , Blombäck M.
Published: August 2010

Plasma levels of platelet factor 4 (PF4) determined in 58 apparently healthy subjects were found to increase with age. In 66 insulin dependent diabetics, PF4 plasma levels were increased but unrelated to the age of subject. Mean fibrinopeptide A levels were elevated in the diabetics but not correlated to PF4 levels. Activation of the coagulation system with thrombin generation thus seems to be operative in diabetes, but the enhanced platelet activation also observed in this disease is probably not thrombin mediated. No correlation was found between PF4 plasma concentration and the degree of vascular complication, HbA1c, blood glucose, 24‐h urinary glucose, serum cholesterol or serum triglycerides.
Inhibitory Effect on Monocyte‐Mediated Cytotoxicity of Sera from Patients with Multiple Myeloma and Malignant Lymphoma
Authors: Eggen Bjørn Magne , Lamvik Jon O. , Unsgaard Geirmund
Published: August 2010

There was no difference in monocyte‐mediated cytotoxicity between monocytes from patients with multiple myeloma or malignant lymphomas and monocytes from control persons after in vitro culture for 5 d. Cytostatic and cytolytic ability of lymphokine‐activated monocytes cultured in medium with patient serum was significantly depressed compared to the ability of monocytes cultured with normal serum. A similar depression of cytostasis was found with non‐activated monocytes of both patient and control origin. Sera from myeloma and lymphoma patients impaired the monocyte‐mediated cytotoxicity equally.
Hereditary Myeloperoxidase Deficiency: Study of 12 Cases
Authors: Larrocha Carmen , de Castro Mercedes Fernández , Fontan Gumersindo , Viloria Ana , Fernández‐Chacón José Luis , Jiménez Carmen
Published: August 2010

12 cases of hereditary myeloperoxidase (MPO) deficiency are reported. Histochemical stainings, lysosomal enzyme determinations, electron microscopic study of MPO and granulocytic function were performed. Family studies on 2 generations were carried out in 5 patients and histochemical stainings and biochemical lysosomal enzyme determinations were done. MPO deficiency was found to follow autosomal recessive inheritance and only rarely to have clinical effects.
Prognostic Significance of Cytogenetical Studies in Chronic Granulocytic Leukaemia
Authors: Cervantes F. , Rozman C. , Ballesta F. , Mila M.
Published: August 2010

In a series of 121 patients with chronic granulocytic leukaemia (CGL) the frequence and prognostic significance of two cytogenetical features (absence of ph1‐chromosome and presence of ph1‐mosaicism) were studied. 13% of the patients were ph1‐negative, these patients showing a shorter survival than the ph1‐positive ones (P < 0.005). 23 instances of ph1‐mosaicism were found (22.5%). This feature was observed either at diagnosis or after treatment, and irrespective of the patient's being in chronic or blastic phase of CGL, but in no case later than 2 years from diagnosis of the disease. ph1‐mosaicism patients vs the remainder of the ph1‐positive group were compared for survival, and no difference was found. The lack of prognostic significance of ph1‐mosaicism was evident regardless of the latter feature being detected at diagnosis or after treatment.
Cold Haemagglutinin Disease with Severe Anaemia, Reticulocytopenia and Erythroid Bone Marrow
Authors: Cazzola Mario , Barosi Giovanni , Ascari Edoardo
Published: August 2010

A quantitative assessment of total, effective and ineffective erythropoiesis, and mean red cell life‐span was performed in 2 patients with idiopathic cold‐haemagglutinin disease (CHAD) who had severe anaemia, reticulocytopenia and erythroid bone marrow. In both cases, ineffective erythropoiesis represented the major factor in the pathogenesis of the anaemia. The most likely explanation of this was that cold antibodies had some effect on the maturing red‐cell precursors. Ineffective erythropoiesis has already been shown in patients affected by auto‐immune haemolytic anaemia due to warm antibodies with reticulocytopenia and erythroid bone marrow. Therefore, it is apparent that both warm and cold antibodies may cause not only peripheral destruction of mature red cells but also intramedullary death of red‐cell precursors.
In Situ Labelling of Bone Marrow Lymphocytes with Fluorescein Isothiocyanate for Lymphocyte Migration Studies in Pigs
Authors: Pabst R. , Kaatz M. , Westermann J.
Published: August 2010

In normal young pigs, the femoral artery and vein were cannulated and after occluding other vessels to one hind leg they were connected to an extracorporeal perfusion system. Fluorescein isothiocyanate (FITC) was added to the perfusate to selectively label cells in the bone marrow.

Large numbers (˜0.9 × 109) of labelled lymphocytes left the bone marrow of one leg within 1 d and migrated via the blood to the bone marrow in other bones, lymph nodes, spleen, Peyer's patches and even into the thymus. On average 1.7% of the lymphocytes in the blood and about 1 % within the spleen were labelled. Peyer's patches and the thymus showed very low indices. Thus the bone marrow is an integral part of the migratory route of lymphocytes. Selective labelling of bone marrow cells in their normal microenvironment with FITC is a suitable method for studies of cell migration from the bone marrow.
Latent Period of 9 Years in the Presentation of a Myeloproliferative Disorder
Authors: Watkins Sylvia M. , Williams J. R. B. , Turnbull A. L.
Published: August 2010

A woman developed a leukaemic infiltrate at the site of a smallpox vaccination; the lesion subsequently regressed spontaneously. 9 years later she developed a generalised myeloproliferative disorder.
Density Distribution and Size of Megakaryocytes in Inflammatory Reactions of the Bone Marrow (Myelitis) and Chronic Myeloproliferative Diseases
Authors: Thiele J. , Holgado S. , Choritz H. , Georgii A.
Published: August 2010

Morphometric evaluation was performed on semi‐thin sections of core biopsies of the bone marrow and included 20 cases of each group of diseases besides control specimens. (i) Hyperergic myelitis of rheumatic origin. (ii) Chronic granulocytic leukaemia (CGL). (iii) Polycythaemia vera (P. vera). (iv) Chronic megakaryocytic‐granulocytic myelosis (CMGM). (v) Myelofibrosis or osteomyelosclerosis (MF/OMS). The following classification of megakaryopoiesis was applied: normal megakaryocytes; giant forms; microforms; intussusceptions; cytoplasmic fragments; naked nuclei. The density distribution shows an increase of megakaryocyte number in those 5 different marrow disorders, ranging from about 13/mm2 in the normal sample up to 65 cells/mm2 in MF/OMS. Microforms are most frequently encountered in CGL, whereas giant megakaryocytes, intussusceptions and many cytoplasmic fragments characterize P. vera, CMGM and MF/OMS. Our measurements suggests 3 distinct categories of bone marrow lesions with corresponding alterations of the megakaryopoiesis: (i) myelitis and CGL; (ii) P. vera; (iii) CMGM and MF/OMS.
Hereditary Factor VII Deficiency in a Chinese Family
Authors: Shih Lee‐Yung , Hung Iou‐Jih
Published: August 2010

Hereditary deficiency of factor VII is demonstrated in a Chinese family. The proposita was a 32‐year‐old female with bleeding diathesis consisting of spontaneous ecchymosis, menorrhagia and recurrent haemarthrosis. The prothrombin time was prolonged and the prothrombin and proconvertin test was 10% of normal. The activated partial thromboplastin time and the Stypven‐cephalin clotting time were normal. The prolonged prothrombin time could be corrected by the addition of normal serum, but not by adsorbed normal or coumadin plasma. The factor VII level was 3.6% of normal. One of her brothers had bleeding symptoms and died at age 25, suggesting that factor VII deficiency might have been present. 13 of her family members had partial deficiency of factor VII with plasma levels ranging from 24 to 50%. These results suggest an autosomal recessive inheritance with a homozygous state occurring in the proposita and possibly in her brother, and a heterozygous state occurring in 13 of her family members. Our study marks an extensive survey of factor VII deficiency in an Oriental family.
Influence of the Spleen on the Distribution of Blood Neutrophils
Authors: Rohrer C. , Arni U. , Deubelbeiss K. A.
Published: August 2010

The influence of the spleen on the distribution of blood neutrophils was studied in rats with varying spleen sizes following transfusion of isologous 3H‐dT‐labelled neutrophils. The weight of the spleen correlated with the splenic neutrophil pool (r = 0.87, P < 0.01). Alterations in spleen size did not significantly change the total blood neutrophil pool, but with increasing splenomegaly circulating and marginal neutrophils were shifted into the splenic pool. With massive splenomegaly, up to 50% of the total blood neutrophils were trapped in the spleen. These findings suggest splenic pooling of neutrophils and a shift of circulating and marginal neutrophils into the spleen proportionate to the splenic tissue present.
Haematopoietic Progenitors in Essential Thrombocythaemia
Authors: Partanen Seija , Ruutu Tapani , Vuopio Pekka
Published: August 2010

Colony formation by haematopoietic progenitors from the bone marrow and blood of 4 patients with essential thrombocythaemia was studied in vitro using the methyl cellulose assay. 3 patients had clearly elevated numbers of BFU‐E in bone marrow. 1 patient also had markedly increased numbers of CFU‐E and CFU‐GM, whereas the other patients had only marginally increased or normal numbers of these progenitors in the marrow. 3 patients showed markedly increased numbers of all progenitors in peripheral blood. All 4 patients showed spontaneous erythroid colony formation by progenitors from the bone marrow and 2 had spontaneous colony formation by progenitors from the blood. We conclude that essential thrombocythaemia shows abnormal colony formation in line with other myeloproliferative syndromes.
Surface Features of Leukaemic Megakaryocytic Precursors
Authors: Polliack Aaron , Leizerowitz Rachel , Berrebi Allan , Gurfel Dorit , Gamliel Haim
Published: August 2010

Megakaryoblasts and maturing megakaryocytic precursor cells from 5 patients with megakaryoblastic leukaemia were studied by scanning electron microscopy (SEM). The diagnosis in all cases had been established by ultrastructural cytochemistry on the basis of a positive platelet peroxidase reaction with negative staining for myeloperoxidase. 1 case presented as acute myelofibrosis and 4 as acute megakaryoblastic transformation of chronic granulocytic leukaemia. Under the SEM, megakaryoblasts and maturing megakaryocytic precursors showed typical surface features including the presence of rounded and irregular blebs, broad folds and pseudopodia. The nature of these surface blebs is still unclear but they probably represent surface membrane alterations relating to imminent platelet shedding at least in the more mature precursors. These surface microprojections are distinctly different from those encountered on leukaemic lymphoblasts, myeloblasts and monoblasts. It is suggested that SEM may be used in conjunction with the PPO reaction as in aid in the diagnosis of megakaryocytic leukaemias.
The Nature of Hairy Cells ‐ A Study with a Panel of Monoclonal Antibodies
Authors: Worman C. P. , Brooks D. A. , Hogg N. , Zola H. , Beverley P. C. L. , Cawley J. C.
Published: August 2010

In all 11 patients with hairy‐cell leukaemia (HCL) tested, a high percentage of peripheral blood and splenic (3 cases) hairy cells (HCs) reacted with 2 B‐cell‐specific monoclonal antibodies (FMC1 and FMC7), but failed to stain with 5 monocyte/macrophage‐specific (FMC17, UCHM1, SM1, SMΦ and OKM1) and with 4 T‐cell‐specific antibodies (UCHT1, Leu1, 2a and 3a). The data provide further evidence in support of the B‐cell nature of the HCs of HCL.
Plasma Fibronectin Deficiency during Chemotherapy of Acute Myeloid Leukaemia
Authors: Brodin Bengt , Liedén Gudrun , Malm Claes , Vikrot Olle
Published: August 2010

Plasma fibronectin was determined using a laser nephelometric method in 10 patients with acute myeloid leukaemia undergoing chemotherapy. There was a continous fall during the first 3 weeks to about 50% of the normal level. The decrease of fibronectin may contribute to the lowered resistence against infection characteristic of these patients.
An Assessment of the Sensitivity of 3 Bleeding Time Techniques
Authors: Bain Barbara , Forster T. , Baker Anne
Published: August 2010

The sensitivity of 3 bleeding time techniques to an aspirin‐induced defect of platelet function has been assessed in 35 normal volunteers. A carefully standardised Ivy technique was compared with 2 commercial devices for a template bleeding time, the Simplate II and the Thrombolette. The standardized Ivy and the Simplate II were found to have a similar sensitivity; the Thrombolette tended to be less sensitive but the difference was not significant.
Kinetics of Indium‐111‐Labelled T Lymphocytes in a Patient with Adult T‐Cell Leukaemia
Authors: Yamauchi Kunihiko , Nagao Tadami , Sugihara Masami , Suzuki Yutaka
Published: August 2010

Clinical studies using indium‐111 oxine labelling of leukaemic T lymphocytes from a patient with adult T‐cell leukaemia are presented. Leukaemic T lymphocytes did not take less than 48 h to traverse the spleen. This result was different from that of normal subjects, in which T lymphocytes continue to fall up to 44 h after the initial rise. Liver activity was seen to accumulate progressively up to 68 h. This could be explained by the infiltration of the liver by T lymphocytes or phagocytosis of damaged T lymphocytes due to Kupffer cells. Leukaemic T lymphocytes entered the enlarged, inguinal lymph nodes at 3 h and left after 68 h, but few T lymphocytes crossed the axillary lymph nodes, which were enlarged as much as the inguinal lymph nodes. This observation suggests that the degree of migration of leukaemic T lymphocytes to each lymph node differs.
A Case of L1 Acute Lymphoblastic Leukaemia Relapsing in L3 (Burkitt's Type) with Non‐T, Non‐B Cells
Authors: Efira A. , Haardt R. , Feremans W. W. , Demaertelaere E.
Published: August 2010

A case of acute lymphoblastic leukaemia with L1 morphology relapsed in L3 (Burkitt's type). These L3 cells disclosed neither T nor B markers and were CALLA positive. This morphology change occurred simultaneously with a rising antibody titer to the Epstein‐Barr virus.
Generation of Procoagulant Activity (PCA) by Macrophage‐Like Cells Derived from Acute and Chronic Myeloid Leukaemia Cells in Response to Phorbol Esters
Authors: Kornberg Abraham , Treves Aliza , Rachmilewitz Eliezer A. , Fibach Eitan
Published: August 2010

Normal human monocytes and macrophages, as well as in vitro human leukaemic promyelocytic cell line (HL‐60) transformed into macrophage‐like cells by 12‐0‐tetradecanoyl‐phorbol‐13‐acetate (TPA) generate potent procoagulant activity (PCA) similar to tissue thromboplastin. In the present study, only mild PCA was detected in primary cultures of cells from the peripheral blood of patients with acute lymphatic leukaemia (ALL), acute myeloid leukaemia (AML) and chronic myeloid leukaemia (CML). After exposure to TPA, AML and CML cells assumed characteristics specific to monocytes and macrophages. Differentiation was associated with the generation of PCA. PCA was not found in ALL cells exposed to TPA. The PCA of TPA‐induced macrophages derived from AML and CML cells resembled tissue thromboplastin and normal monocyte and macrophage PCA in several aspects: (a) accelerated clotting via the extrinsic coagulation pathway, (b) inhibition by concanavalin A and protection against lectin inhibition by methyl‐α‐D‐mannopyranoside, (c) localization in the cell membrane. The capacity for PCA generation is additional evidence for the similarity between TPA‐induced macrophages derived from AML and CML cells and normal human monocytes and macrophages.
Zinc, Copper, Magnesium, and Calcium in Blood and Plasma after Phlebotomy
Authors: Qvist Irene , Abdulla Mohammed , Mathur Arun , Robertson Bertil , Svensson Sven
Published: August 2010

Zinc levels in plasma and whole blood were investigated in 2 groups of blood donors. The first group consisted of 19 men and 11 women who donated blood for the first time and were followed during 3 phlebotomies. The second group comprised 44 men with 9 or more previous blood donations, their blood being analysed on a single occasion. Plasma concentrations of copper, magnesium, calcium and ferritin were also analysed. The zinc levels in whole blood and plasma in the second group were significantly lower as compared to the first group (P < 0.001). Furthermore, the second group had significantly higher levels of copper and copper:zinc quotient in plasma as compared to those of the new blood donors (P < 0.001). Our results indicate that besides the earlier known effect of blood donation on iron stores, there are also signs of zinc depletion when judged from the zinc levels in plasma and in erythrocytes. No significant changes in calcium and magnesium concentrations were seen during phlebotomy.
Tropical Anamnesis Disguising Malignant Lymphoma
Authors: Loft Steffen H. , Willumsen Lars
Published: August 2010

The fatal history of 2 young men returning from the tropics is reported. The 1st case was characterized by recurrent fever and cholestatic jaundice, and the diagnostic and therapeutic efforts were directed towards exotic infection. Finally, indications of Hodgkin's disease were found and cytostatic treatment was tried unsuccessfully. Autopsy confirmed the diagnosis. The 2nd case was characterized by continuous fever and haemolysis requiring blood transfusion and prednisone. Tropical infection, among other things, was constantly suspected, but finally a liver biopsy demonstrated non‐Hodgkin lymphoma. The diagnosis of malignant lymphoma in 2 previously healthy men was in both cases delayed by their stay in the tropics.
Graft‐Versus‐Host Reaction in 3 Adult Leukaemia Patients after Transfusion of Blood Cell Products
Authors: Nikoskelainen J. , Söderström K.‐O. , Rajamäki A. , Meurman L. , Korvenranta H. , Kalliomäki J. L. , Toivanen A.
Published: August 2010

We describe 3 cases of acute graft‐versus‐host (GVH) disease in patients with acute myeloid leukaemia following transfusions taken from non‐HLA‐identical healthy donors. The leucocyte transfusions were given because of severe bone marrow aplasia and granulocytopenia following leukaemia induction treatment. The first patient had an acute GVH reaction with an erythrodermia‐like skin reaction all over and associated with severe abdominal cramping, enlarged liver and pathological liver function tests. The second patient had a relatively mild skin reaction and enlarged liver. Both died of severe pulmonary infection. The third patient also had a mild skin reaction and enlarged liver. He died of pulmonary embolism. The diagnosis of GVH of the latter 2 cases was made on skin biopsy. The autopsy samples revealed in all cases a heavy lymphocytic infiltration of the kidneys and liver portal area. Until more precise guidelines can be established, irradiation of blood cell products given to patients with neutropenia due to leukaemia induction treatment should be considered.
Terminal Deoxynucleotidyl Transferase (TdT) in Human Foetuses
Authors: Bonati Antonio , Casoli Claudio , Starcich Bruno , Buscaglia Mauro
Published: August 2010

The liver, bone marrow and cord blood of 30 human foetuses between the 15th and 21st weeks of gestational age were examined. Liver suspensions were investigated both by immunofluorescent and biochemical assay; cord blood suspensions and bone marrow touches with immunofluorescent assay. The results indicate the existence of a few TdT+ cells in the liver, cord blood and bone marrow of human foetuses in the ages studied.

The peaks for each organ were: 15 + 1.60 (mean + SD) TdT+ cells in the liver, 12 + 2 in the bone marrow and 18 + 2.83 in the cord blood. In all the organs TdT+ cells decrease in the later weeks. In the liver, the results of the immunofluorescent assay were confirmed by the biochemical activity.
Antigenic Expression and Proliferative Status of Multilineage Myeloid Progenitor Cells (CFU‐GEMM) in Normal Individuals and Patients with Chronic Granulocytic Leukaemia
Authors: Hibbin Jill A. , McCarthy Donald M. , Goldman John M.
Published: August 2010

We assayed the number of multilineage myeloid progenitor cells (CFU‐GEMM) in the blood and marrow of patients with newly diagnosed chronic granulocytic leukaemia (CGL). The mean number of CFU‐GEMM in the blood was increased 600‐fold and CFU‐GEMM in the marrow was doubled in the CGL patients compared with normal. A complement‐fixing monoclonal antibody with HLA‐DR specificity inhibited the proliferation of CFU‐GEMM from CGL blood to a greater extent than that of comparable cells in normal marrow. Using a hydroxyurea ‘suicide’ method we found that the proportion of CFU‐GEMM in proliferative cycle was higher in CGL blood than in normal marrow. We conclude that (1) CFU‐GEMM numbers are greatly increased in the blood of patients with CGL, (2) CFU‐GEMM express HLA‐DR antigens on their surface, and (3) the apparently increased expression of the antigen on CFU‐GEMM from CGL blood in comparison with CFU‐GEMM from normal marrow may parallel the relatively higher proportion of CGL CFU‐GEMM in cell cycle.
Influence of Plasma Proteins on Erythrocyte Morphology and Sedimentation
Authors: Talstad I. , Scheie P. , Dalen H. , Röli J.
Published: August 2010

The influence of plasma proteins on erythrocytes was studied by interference microscopy, scanning electron microscopy (SEM), and by Westergren erythrocyte sedimentation rate (ESR). Albumin kept erythrocytes dispersed as discoid spheres. Fibrinogen seemed responsible for the rouleaux phenomenon, but needed the co‐influence of an immunoglobulin to induce rouleaux type of aggregates and high ESR. IgG, IgA and IgM caused immunologic type of aggregates. Albumin acted synergistically with fibrinogen and immunoglobulins. Normal blood contained a network of rouleaux, which probably explained the low normal ESR. High ESR was either due to rouleaux type aggregates where fibrinogen was dominant, or immunologic type aggregates where IgG, IgA or IgM were dominant proteins. Cold agglutinin disease showed normal blood morphology and normal ESR at 37°C and immunologic type aggregates and high ESR at 25°C.
High‐Dose Intravenous Immunoglobulin for Post‐Transfusion Purpura
Authors: Glud Torben K. , Rosthøj Steen , Jensen Mogens Krogh , Laursen Benedicte , Grunnet Niels , Jersild Casper
Published: August 2010

Successful treatment of a case of post‐transfusion purpura with high‐dose i.v. human immunoglobulin is reported. A 62‐year‐old multiparous woman developed severe thrombocytopenia in the wake of transfusions given during and after vascular surgery. A platelet alloantibody with anti‐Zwa‐specificity was demonstrable by an indirect immunofluorescence technique. A single infusion of Sandoglobulin® 1 g/kg body weight caused an immediate platelet response with cessation of haemorrhagic manifestations within 12 h, and the platelet count became normal within 3 d. Thrombocytopenia recurred 8 d after the infusion, but one further dose of Sandoglobulin 0.5 g/kg body weight caused definitive reversal of the thrombocytopenia.
Successful treatment with selective intracoronary streptokinase in impending myocardial infarction
Authors: ARNESEN H. , EYJOLFSSON O. , HOL R.
Published: August 2010

A case report is presented concerning a 39 years old man with 8 days history of impending myocardial infarction with transitory ECG changes, but no rise in serum enzymes.

Selective coronary angiography revealed single vessel coronary artery disease with occlusion of the LAD in the middle 1/3.

Streptokinase was then given selectively into the left coronary artery by continuous infusion for 140 min. At the end of the infusion continuous flow was noted in a narrowed LAD.

Clinically he was later fully normalized including a normal ergometer bicycle exercise test.

Control angiography after 7 weeks revealed an almost fully normalized LAD, significantly improved from just after the streptokinase treatment.

In the discussion of this successful case, special attention is paid to the duration of the streptokinase infusion, as well as to the improvement achieved in the post‐infusion period.
Reduced Fibrinolytic Capacity Associated with Low Ratio of Serum Testosterone to Oestradiol in Healthy Coronary High‐risk Men
Authors: ANDERSEN P. , NORMAN N. , HJERMANN I.
Published: August 2010

In a study of 42 healthy, middle‐aged men with high risk of coronary heart disease (CHD), we found a highly significant correlation between low ratio of serum testosterone to oestradiol and delayed clot lysis after venous stasis as measured with the euglobulin clot lysis time (ECLT). The upper normal limit of ECLT was set at 60 min. Half of the examined specimen, i.e. from 21 individuals, lysed as normal; 4 specimen lysed between 60 and 90 min, whereas the remaining 17 specimen did not lyse within 2 hours. Sixteen of these 17 individuals with the most defective fibrinolytic capacity belonged to the group of individuals with the lowest ratio of serum testosterone to oestradiol. The association was highly significant (p < 0.001). In comparison, the correlation between serum triglyceride concentration and the ratio of serum testosterone to oestradiol was significant at the 2% level, whereas serum cholesterol and this ratio were not associated. The significance of the findings remains obscure, but may be important for the incidence of CHD in men.
Peripheral Blood Lymphocyte Subpopulations in Polycythaemia and Thrombocythaemia
Authors: Eridani Sandro , Fiorini Gian Francesco , Batten Elaine
Published: August 2010

Lymphocyte subpopulations from peripheral blood of normal subjects and patients with primary proliferative polycythaemia (PPP), idiopathic erythrocytosis (IE) and essential thrombocythaemia (ET) were separated using antihuman immunoglobulin antiserum for B lymphocytes and the following monoclonal antibodies: OKT3, directed against the general T‐lymphocyte subpopulation, OKT4 amd OKT8, detecting respectively T‐helper and T‐suppressor lymphocyte subpopulations, OKM1 reacting mainly with monocytes. A decrease in the number of OKT3+ cells was observed both in PPP and IE, with a particular fall of the OKT8+ (suppressor) cells, so that the T4/T8 ratio was significantly increased (P < 0.03 in PPP and P < 0.0005 in IE). The ratio remained normal in samples from ET. OKM1+ cells were significantly increased in PPP (P < 0.04), but not in IE, while in ET there was a rise in a few cases only. The present data point out some definite changes in the circulating lymphomonocytic cell subsets, which may be of interest in the study of this group of myeloproliferative disorders.
Monoclonal B Cells in Peripheral Blood in Non‐Hodgkin's Lymphoma. Correlation with Clinical Features and DNA Content
Authors: Lindh Jack , Lenner Per , Roos Göran
Published: August 2010

Peripheral blood from 69 patients with non‐Hodgkin's lymphoma was examined with respect to B and T cell markers. Evidence for monoclonal B cell was found in 29 cases, 8 of ‘high grade’ and 21 of ‘low grade’ malignancy according to the Kiel classification. 17 out of the 29 patients had a normal lymphocyte count. Using conventional staging methods 4 cases of the 29 were in stages II and III, all others in stage IV. The proportion of S‐phase cells in peripheral blood, determined by flow cytometry, was found to be elevated in cases with a monoclonal cell population. It is concluded that surface marker analysis of blood cells may be valuable as a diagnostic tool, as an indicator of prognosis and perhaps for the staging procedure of malignant lymphomas.
Serum Thymidine Kinase in Vitamin B12Deficiency
Authors: Hagberg H. , Gronowitz S. , Killander A. , Källander C.
Published: August 2010

In DNA synthesis deoxythymidine kinase (TK) catalyses the conversion of deoxythymidine to deoxythymidine monophosphate (dTMP) via the ‘salvage pathway’. Serum deoxythymidine kinase (S‐TK) was measured in this study in 75 patients with vitamin B12deficiency by a new, very sensitive method, using125I‐deoxyuridine as substrate. Elevated S‐TK levels were found in those patients who had developed haemolysis and anaemia and the more advanced the disease the higher the S‐TK value. Thus there was a highly significant correlation between S‐TK, haemoglobin level and lactic dehydrogenase activity. These findings are consistent with the theory that elevated levels of S‐TK are due to release from unstable proliferating tissue.
Acute monoblastic leukaemia: Clinical, biological data and survival in 45 cases
Authors: Janvier M. , Tobelem G. , Daniel M. T. , Bernheim A. , Marty M. , Boiron M.
Published: August 2010

Between 1978 and 1980, 45 cases of acute monoblastic leukaemia have been diagnosed, treated and followed in our institute. Morphological diagnosis was performed according to the French‐American‐British classification. Tumoral syndrome (particularly extra‐medullary) and hyperleucocytosis were the most striking findings at the time of diagnosis. Cytogenetic analysis performed in 31 cases before treatment has showed that abnormality of the long arm of chromosome 11 seemed to be more frequently associated with the poorly differentiated cytological subtype M5 (a). Intensive chemotherapy with zorubicine and cytosine arabinoside led to complete remission in 75 % of the cases. Central nervous system prophylaxis appeared definitively useful in preventing meningeal relapse. Despite a prolongation of the median duration of complete remission which now reaches 12 months, the prognostic is still poor.
Bleeding on patients with autoimmune thrombocytopenic purpura and normal platelet count
Authors: Cortelazzo S. , Viero P. , Casarotto C. , D'Emilio A. , Dini E. , Barbui T.
Published: August 2010

80% of patients with chronic autoimmune thrombocytopenic purpura (ATP) may reach normalization of platelet count after steroids or splenectomy. In some of these cases a bleeding tendency may still persist and this has been attributed to abnormalities of platelet function resulting from the effect of platelet‐bound antibodies. We have studied 49 ATP patients in order to assess the frequency and the pattern of this immune thrombopathia, and the correlation with the levels of platelet associated IgG (PAIgG). Only 3 patients (6%) had prolonged bleeding time and persistence of mucocutaneous haemorrhages, whereas 36% presented an altered platelet aggregation pattern. Platelet serotonin content, platelet and plasma β‐thromboglobulin concentration, and malondialdehyde generation after thrombin stimulus were found also altered, but the findings were not clearly correlated with the concentration of PAIgG.
Depressed NK cell activity of peripheral blood mononuclear cells in untreated Hodgkin's disease: Enhancing effect of interferon in vitro
Authors: Levy S. , Tempe JL. , Aleksijevic A. , Giron C. , Oberling F. , Mayer S. , Lang JM.
Published: August 2010

Natural killer (NK) cell activity of unseparated peripheral blood mononuclear cells from 30 untreated patients with Hodgkin's disease and 22 age‐ and sex‐matched normal controls was evaluated using the classical K 562 cells as targets. A significant defect was demonstrated in the patients with stage I‐II and seemed to be more profound in patients with advanced disease (stage III‐IV) and in those with B symptoms. The differences between subgroups of patients, however, were not statistically significant, mostly because of the wide dispersion of individual data. Pre‐incubation of effector cells with alpha A leucocyte recombinant interferon led to a clear increase in NK cell activity in 4 of 6 patients tested, showing that depressed NK activity in Hodgkin's disease is still susceptible to the enhancing effect of interferon, at least in some patients.—
Decreased red cell enolase activity in a 40‐year‐old woman with compensated haemolysis
Authors: Boulard‐Heitzmann P. , Boulard M. , Tallineau C. , Boivin P. , Tanzer J. , Bois M. , Barriere M.
Published: August 2010

A 40‐year‐old woman splenectomized 17 years previously for hereditary haemolytic anaemia was investigated in our laboratory because of persistent conjunctival subic‐terus associated with compensated haemolysis. The results of the autohaemolysis and osmotic fragility tests were similar to those usually observed in hereditary spherocytosis. Red cell enzyme assays indicated a decreased amount of kinetically normal enolase. The genetic transmission of this defect could not be established since the only other affected member of the family was the proposita's father who died several years ago after splenectomy for an undefined haemolytic disorder.
Diagnosis of Hodgkin's disease hampered by an Epstein‐Barr virus infection treated with acyclovir
Authors: Berild Dag , Bendtzen Klaus , Badsberg Edith
Published: August 2010

We report a case of Hodgkin's disease associated with Epstein‐Barr virus (EBV) infection in a 75‐year‐old male. The patient was given a 5‐day course of acyclovir to which he responded well, with partial resolution of the lymphoma and return to well‐being. A biopsy from the lymphoma after acyclovir treatment showed a change in the histological picture with a reduction in the number of giant cells. Relationships between EBV infection and Hodgkin's lymphoma are discussed.
Changes in T‐lymphocytes and response to high‐dose γ‐ globulin therapy in idiopathic thrombocytopenic purpura
Authors: Brearley R. L. , Rowbotham Beverley , Collins R. J.
Published: August 2010

Peripheral blood lymphocytes were studied using OKT3, OKT4 and OKT8 monoclonal antibodies in patients with idiopathic thrombocytopenic purpura ITP) before and after high dose gammaglobulin therapy. Total lymphocyte counts increased in 8 of 10 patients studied within 16 weeks of presentation (acute ITP) but decreased in all 6 with chronic (6 months‐14 years) thrombocytopenia (P = 0.003). A relative increase in the proportion of inducer‐helper (OKT4) cells was seen in 5 of 6 patients with sustained responses whereas there was no clear cut change or a relative increase in suppressor‐cytotoxic (OKT8) cells in 7 or 9 with transient responses. The data suggest that short term gammaglobulin therapy may induce sustained responses by immunoregulation of T‐lymphocyte subsets.
The myelodysplastic syndromes ‐ A study of haemostatic function and platelet ultrastructure
Authors: Pamphilon D. H. , Aparicio S. R. , Roberts B. E. , Menys V. C. , Tate G. , Davies J. A.
Published: August 2010

The myelodysplastic syndromes (MDS) are characterised by dysplastic marrow and cytopenia. Clinically detectable bleeding is uncommon and usually attributed to thrombocytopenia. We have investigated some aspects of haemostatic function in 17 patients with MDS and compared the results with findings from 17 control patients matched for age and sex. No specific disorder of blood coagulation or fibrinolysis was identified. The main abnormalities observed in the patients were: prolongation of the bleeding time which was greater than could be explained on the basis of thrombocytopenia in 13 patients; absent, or severely impaired platelet aggregation in response to collagen in 7 patients; impaired platelet production of malondialydehyde when stimulated with collagen and abnormal release of 14C‐5 hydroxytryptamine in 5 patients; and abnormalities of ultrastructure in all 5 patients whose platelets were viewed by electron microscopy.
Effects of polyamines on platelet aggregation
Authors: PALES JORGE L. , LOPEZ ARMANDO , VIDAL SANTIAGO
Published: August 2010

Polyamines, spermine and spermidine in the micromolar range (1–100 μmol/l) increase ADP and epinephrine‐induced platelet aggregation in vitro. The possible rôle of polyamines as regulating factors of platelet aggregation in physiological and pathological states is discussed.
Immune haemolytic anaemia associated with ampicillin dependent warm antibodies and high titre cold agglutinins in a patient with Mycoplasma pneumonia
Authors: MICKLEY HANS , SøRENSEN PAUL GADE
Published: August 2010

A case of severe immune haemolytic anaemia in a 54‐year‐old man suffering from Mycoplasma pneumonia is presented. A strongly positive direct Coombs test with erythrocyte bound IgG, C3d and C4 was demonstrated during the haemolytic process. Further, serologic investigations revealed ampicillin‐dependent ‘warm’ antibodies and a cold agglutinin titre > 2000. No firm conclusion can be drawn as to which antibody caused the haemolysis. Treatment including withdrawal of antibiotics, blood transfusions (washed red cells, concentrated erythrocyte suspension) and corticosteroids was successful.

It is suggested that tests should be carried out for erythrocyte bound drug‐induced antibodies in cases of haemolysis with a history of drug exposure.
Successful Treatment of Refractory Autoimmune Haemolytic Anaemia by Plasmapheresis
Authors: Kutti Jack , Wadenvik Hans , Safai‐Kutti Soodabeh , Björkander Janne , Westberg Gunnar , Johnsen Svend‐Aage , Larsson Bo , Hanson Lars Åke
Published: August 2010

A 16‐year‐old female was admitted because of rapidly progressive fatigue, severe anaemia and icterus. The S‐bilirubin was 190 μmol/l, COHb 7.3% and high amounts of free Hb in plasma were present. The Coombs' direct test was strongly positive with anti‐IgG but negative with anti‐IgM and anti‐C3. Conventional therapy with very high doses of hydrocortisone i.v., cyclophosphamide, azathioprine, and transfusions of washed packed red cells proved ineffective. During 5 consecutive days she also received i.v. infusions of gammaglobulin (25 g each day). Nevertheless, her condition deteriorated and 3 plasma exchanges were carried out with impressive clinical and laboratory effects. After the 3rd plasma exchange, the patient did not require further transfusions of packed red cells. Therapy with corticosteroids could be rapidly reduced and she was discharged after 5 weeks with a normal blood picture. Since then she has remained in excellent health.
Hexagonal crystals in the bone marrow in patients with myeloproliferative disease and preleukaemia
Authors: Nesland Jahn M. , Langholm Ruth , Marton Per F.
Published: August 2010

In epoxy resin‐embedded bone marrow biopsies from 238 cases of myeloproliferative disease and preleukaemia, crystalline inclusions were found in the bone marrow cells of 24 (10.1%), most often in patients with acute myeloproliferative disease (23%). By light microscopy the crystals were easily recognized after Giemsa staining, lying within the cytoplasm of bone marrow macrophages. By electron microscopy the hexagonal crystals were encountered chiefly within secondary lysosomes of the macrophages, but also within immature myeloid cells in one of the two cases studied. In the latter a tight‐fitting membrane around the crystals suggested that they were of lysosomal origin and represented a form of abnormal granulation. We suggest that the presence of crystals in myeloid cells represents a kind of abnormal granula formation in the neoplastic cells. In the macrophages the crystals lodge in secondary lysosomes, probably after phagocytosis of crystal‐bearing non‐viable myeloid cells. The accumulation of crystals in lysosomes indicates resistance to degradation.
An abnormal fibrinogen (Copenhagen II) with increased sialic acid content associated with thrombotic tendency and normal liver function
Authors: Hansen M. Sandbjerg , Schousboe I.
Published: August 2010

An increased sialic acid content of the fibrinogen molecule is found in foetal fibrinogen and as an acquired disorder in hepatic disease. A qualitatively abnormal fibrinogen was detected in the plasma of a 25‐year‐old man with a thrombotic tendency. The purified fibrinogen had a significantly increased content of sialic acid, an abnormal fibrinmonomer polymerization, and a changed mobility in crossed affino‐immunoelectrophoresis using immobilized helix pomatia lectin. The patient had no biochemical or clinical signs of liver disease. The occurrence of a thrombotic tendency and an increased fibrinogen sialic acid content without signs of liver disease may represent a new variant of congenital dysfibrinogenaemia.