bluuebunny/crossref_metadata_2025 huggingface dataset:
Heterogeneity of Hereditary Methaemoglobinaemia: A Study of 4 Cuban Families with NADH‐Methaemoglobin Reductase Deficiency Including a New Variant (Santiago de Cuba Variant)
Authors: González R. , Estrada M. , Wade M. , de la Torre E. , Svarch E. , Fernández O. , Ortiz R. , Guzmán E. , Colombo B.
Published: August 2010

NADH‐methaemoglobin reductase deficiency has been found in 4 Cuban families; 3 subjects carried the mild form of the deficiency while in 2 sibs of the fourth family the deficiency was associated with neurological involvement. The parents in this family were consanguinous and the sibs were shown to be homozygous for a new fast electrophoretic variant. It was named Diaphorase Santiago de Cuba.
Paroxysmal Cold Haemoglobinuria in Children: 3 Cases Encountered within a Period of 7 Months
Authors: Johnsen H. E. , Brostrøm K. , Madsen M.
Published: August 2010

3 children who fulfilled the criteria for the diagnosis of paroxysmal cold haemo‐globinuria of the classical Donath‐Landsteiner type are described. The benign clinical course of this illness is confirmed. A serological study of two of the Donath‐Landsteiner antibodies revealed the anti‐P specificity in one, but not in another serum.
Fletcher Factor Deficiency: Report of a New Family
Authors: Aznar J. A. , España F. , Aznar J. , Tascon A. , Jimenez C.
Published: August 2010

3 cases of Fletcher factor deficiency in a family not related to the 6 families already published (Hathaway et al 1965, Hattersley & Hayse 1970, Abildgaard & Harrison 1974) are studied. In the family described here, 3 of 4 siblings have a Fletcher factor level of less than 1 % and the fourth has a level of 46 %; the Fletcher factor level in the father is 48 % and in the mother 38 %. This suggests an autosomal recessive transmission.

Clinically they do not present spontaneous bleedings and only one of the siblings required a unit of blood after an amygdalectomy. It is also of interest to emphasize that 3 of the siblings suffered from congenital multiple arthrogryposis and that 2 of them presented the arthrogryposis together with the Fletcher factor deficiency, a circumstance which could have been favored by the consanguinity of the parents. The fact that the family described here is white and of Mediterranean origin contradicts the idea that there exists a special predisposition among members of the black race for this disease.
Lymphomatoid Granulomatosis of the Lung, Liver and Spleen
Authors: Schjølseth S. A. , Blom G. P.
Published: August 2010

A female patient with lymphomatoid granulomatosis (LYG) involving lung, liver and spleen is described. Our case presented with signs and laboratory data indicating severe hepatic failure.

It is not clear if this disorder represents a distinct pathological or clinical entity. In this report the differences between LYG and the two disease groups which it most resembles, Wegener's granulomatosis and malignant lymphoma are discussed.
Studies on Human Preleukaemia II In Vitro Colony Forming Capacity in Aregenerative Anaemia with Hypercellular Bone Marrow
Authors: Beran Miloslav , Hast Robert
Published: August 2010

The colony and cluster forming capacity of bone marrow and mononuclear leucocytes and colony:cluster ratio (CCIR) was studied in 23 patients with resistant anaemia and hypercellular bone marrow. Bone marrow controls included 28 patients with iron deficiency, haemolytic, megaloblastic and secondary anaemia as well as 15 haematologically normal subjects and 13 healthy volunteers. Colony formation by blood leucocytes was studied in aregenerative anaemias and in 13 healthy volun‐Colonies and clusters were grown in soft agar cultures in vitro from all control subjects. In normal volunteers the marrow grew between 24 and 201 colonies and 37 to 212 clusters/105 cells with the CCIR of 0.48–2.50. Blood leucocytes produced 6–184 colonies and 6–88 clusters/106 mononuclear cells with CCIR 0.43–2.64. Colony formation and CCIR were largely normal in haematologically intact subjects and patients with anaemia of known etiology.

Patients with aregenerative anaemia showed abnormal colony formation by bone marrow and blood cells more often (P < 0.01) than control groups. Only 7 of 23 patients had colony formation within the normal range. Blood leucocytes in 12 out of 18 aregenerative anaemias failed to grow colonies in vitro. The marrow CCIR was below the normal range in all aregenerative anaemias with defective and in 3 with normal colony formation and was significantly lower than in the controls (P < 0.01). Of 19 aregenerative anaemias with defect colony formation 11 died, 7 with acute leukaemia.

The in vitro colony formation appears useful for subclassification of anaemic conditions and for identification of patients who may be at higher risk of developing leukaemia.
Interpretation of Measured Red Cell Mass in the Diagnosis of Polycythaemia
Authors: Pearson T. C. , Glass U. H. , Wetherley‐Mein G.
Published: August 2010

Expression of predicted and measured red cell mass (RCM) in terms of ml/kg body wt. lacks precision. The use of formulae for prediction of normal mean red cell mass derived from the blood volume prediction (BV) of Nadler et al (1962) has been examined. It is proposed that a more accurate determination of mean normal predicted red cell mass (MNRCM) is obtained by using:

0.47 times 0.91 × BV = MNRCM for males

0.43 times 0.91 × BV = MNRCM for females

The MNRCMs given by these formulae agree with those given by predictions based on lean body mass and surface area.

Examination of the ‘95 % confidence limits’ of the ‘Nadler’ prediction indicates that males with measured RCMs greater than 25 % and females with measured RCMs greater than 30 % above their MNRCM may be regarded as having polycythaemia.
Postirradiation Erythropoietic Recovery in Splenectomized Mice
Authors: Aggio Marlo Carlos
Published: August 2010

The influence of splenectomy on erythropoietic recovery of lethally X‐iradiated mice injected with different doses of syngeneic bone marrow was studied. Splenectomized animals showed less activation than unoperated controls in the lower dose range; however, by increasing the number of injected cells the response obtained was similar in both groups. Providing an adequate number of stem cells is administered to the splenectomized recipients, an enhanced erythropoietic activity of the graft may compensate for the absence of the spleen, which is an important organ in postirradiation recovery. Suggested explanations for this observation are lack of an haematopoietic inhibitory effect of the irradiated spleen, or changes in the environment provided by the spleenless host.
A Case of Pure Monocytic Leukaemia in a Child — Characterization of Cellular Morphology, Membrane Markers, Surface Glycoproteins and Karyotype
Authors: Weber Theodor H. , Wegelius Ruth , Borgstrom Georg H. , Gahmberg Carl G. , Andersson Leif C.
Published: August 2010

A case of pure monocytic, ‘Schilling‐Type’ leukaemia in a 9‐year‐old boy is described. The leukaemic cells resembled monocytes and were abundantly present in blood and bone marrow. They were strongly αnaphthyl‐acetate‐esterase positive, contained fine PAS‐positive granules and did not stain by Sudan B. They were negative for surface‐bound and intracytoplasmic IgG, but had a high density of membrane Fc‐receptors and showed phagocytosis. The surface glycoprotein pattern resembled that of monocytes, but was clearly different from those of lymphocytic and myelocytic cells or those of acute lymphocytic leukaemia cells. The karyotype of bone marrow cells was 44, X, —15, —21, + mar, del(2)(pll), with the missing Y‐chromosome located in the marker chromosome. Especially the surface glycoprotein and membrane receptor analyses aided in the accurate classification of the monocytic origin of the leukaemic cells.
Haemolysis in Athletes due to Hereditary Spherocytosis
Authors: Godal H. C. , Refsum H. E.
Published: August 2010

Three athletes, one cross‐country skier and two middle distance runners, experiencing poor response to intensified training and decline in performance during prolonged periods of frequent competitions, associated with a tendency to develop anaemia, were found to have spherocytosis and increased osmotic fragility of the red blood cells. All had family members with the same blood abnormality, but without symptoms.

The observations suggest that the frequency of hereditary spherocytosis is higher than indicated by overtly affected cases and that mild cases are easily overlooked. Hereditary spherocytosis should be looked for in athletes with a tendency to decline in blood haemoglobin concentration during periods of intensive training and competition.
The Colony Forming Cell in the Myeloproliferative Disorders and Aplastic Anaemia
Authors: Rickard K. A. , Brown R. D. , Wilkinson T. , Kronenberg H.
Published: August 2010

Bone marrow colony forming cell (CFC) concentration and the proportion of CFC in DNA synthesis were studied in myeloproliferative disorders and aplastic anaemia. Growth patterns of bone marrow cells in agar cultures were able to supplement traditional morphological and clinical criteria in the diagnosis of these haematolog‐ical conditions. Bone marrow CFC concentration tended to be increased in chronic myeloid leukaemia (CML) and polycythaemia vera (PV), but decreased in myelo‐fibrosis, erythroleukaemia, paroxysmal nocturnal haemoglobinuria (PNH) and the aplastic phase of aplastic anaemia. The proportion of CFC in DNA synthesis was decreased in CML, myelofibrosis and aplastic anaemia, but increased in blastic transformation, PV, PNH and during regeneration from aplastic anaemia. The proportion of CFC in DNA synthesis in bone marrow from patients with CML in blastic transformation was directly related to the percentage of myeloblasts in the bone marrow. CFC kinetics in blastic transformation have been demonstrated to be different from those in acute leukaemia.
A Re‐Examination of the Alleged Monocytic Features of Hairy‐Cell Leukaemia
Authors: Burns Gordon F. , Cawley John C.
Published: August 2010

A number of the ‘monocytic’ features of the hairy cells (HCs) of hairy‐cell leukaemia (HCL) are studied in detail and discussed in relation to the now established B‐cell nature of the disease. By means of a rosette assay employing erythrocytes coated with IgG (EAG), a receptor for the Fc of IgG is shown to be strongly expressed on all HCs, whether in suspension or in monolayers. This receptor is shed and re‐expressed over a period of some 6 h. In EAG rosette formation the indicator erythrocytes are deformed and the receptor is not blocked by an antiserum to the la‐like antigen. By ultrastructural acid‐phosphatase cytochemistry, it is established that HCs phago‐cytose latex particles, but do not phagocytose a variety of other particles to a significant extent. These, and other features, are discussed in relation to the literature and it is shown that all are compatible with the current view that HCL is a form of B‐cell lymphoproliferation.
Antithrombin III and the Nephrotic Syndrome
Authors: Jørgensen Kaj Anker , Stoffersen Erik
Published: August 2010

Plasma and urinary antithrombin III (AT‐III) was measured in 15 cases of nephrotic syndrome. Plasma AT‐III correlated well with serum albumin, but poorly with pro‐teinuria, whereas urinary AT‐III correlated well to proteinuria. The plasma AT‐III level had a mean similar to 25 healthy controls, but the range was significantly wider. A case with nephrotic syndrome and left renal vein thrombosis is reported. The urinary output of AT‐III rose and the plasma level fell with the activity of the disease. Although AT‐III and albumin have similar molecule weight, their renal clearance was found to be different. It is suggested that urinary loss of AT‐III plays a role in the hypercoagulable state sometimes found in the nephrotic syndrome.
Effect of Pentoxifylline on Red Cell Flexibility and Cation Transport in Healthy Subjects and Patients with Hereditary Spherocytosis
Authors: Johnsson Riitta
Published: August 2010

The effect of pentoxifylline (3,7‐dimethyl‐1‐(5‐oxo‐hexyl)‐xanthine) on the flexibility of red cells was studied using a filtration method in which the red cells are forced, at a constant flow‐rate, through a porous pdycarbonate membrane. The filtration pressure reflects red cell rigidity and the amount of Hb released from the disrupted cells (‘free Hb’) red cell fragility. The advantage of this method is that it allows the two important determinants of red cell flexibility, rigidity and fragility, to be studied simultaneously. Pentoxifylline significantly improved normal red cell flexibility both in vivo and in vitro as judged by this method.

The impaired flexibility of red cells from patients with congenital or hereditary spherocytosis (HS) was aggravated by pentoxifylline in vitro. A similar effect of pentoxifylline was also observed on red cells from relatives of HS patients. The effect of pentoxifylline on Ca2+ transport and Ca2+Mg2+‐ATPase activity in red cells from normal and HS patients was investigated. Under in vitro conditions pentoxifylline did not affect the Ca2+Mg2+‐ATPase activity of or Ca2+ efflux from normal and HS red cells. Neither the influx of monovalent cations (Na+, Rb+) or the osmotic resistance of normal or HS red cells was affected by pentoxifylline.
The Effect of Thioguanine on a Combination of Daunorubicine, Cytarabine and Prednisone in the Treatment of Acute Leukaemia in Adults
Authors: Ikkala E. , Lahtinen R. , Niskanen E. , Ruutu T. , Vuopio P. , Hänninen Auli , Palva I. , Timonen T. , Lehtinen Marja , Levanto A. , Rajamäki A. , Vilpo J. , Selroos O. , Ahrenberg Pia , Rosengård S. , Waris E. , Wasastjerna C. , Wasastjerna C. , Sarna S. ,   The Finnish Leukaemia Group
Published: August 2010

194 adults with acute leukaemia were randomly allocated to be treated with a combination of daunorubicine, cytarabine and prednisone either with (RAP+T) or without (RAP) thioguanine. A remission was achieved in 37% of 101 patients treated with RAP and in 35% of 93 patients treated with RAP+T. The survival and length of remission were similar in both groups. Neither regimen was superior to the other in any type of leukaemia nor in any age group of patients. In 9 of the patients failing to remit with RAP treatment a remission was obtained with other chemotherapy, while none of the patients not responding to RAP+T achieved a remission with further chemotherapy.
Circulating Megakaryocytes in Patients with Pulmonary Inflammation and in Patients Subjected to Cholecystectomy
Authors: Hansen Mads , Pedersen Niels Tinggaard
Published: August 2010

30 patients with pulmonary inflammation and 17 patients subjected to cholecystectomia à froid were investigated for circulating megakaryocytes in a cubital vein using the saponin‐haemolysis leucoconcentration technique. The number of circulating megakaryocytes was significantly higher in patients with pulmonary inflammation than in healthy humans. In 15 patients with bronchitis, bronchopneumonia, and leucocytosis the arithmetic mean was 15.6 megakaryocytes per 1.5 ml blood (range 3 to 47). In 15 patients with bronchitis and a normal leucocyte count the arithmetic mean was similarly 14.1 megakaryocytes per 1.5 ml blood. After cholecystectomy a significant maximum increase in the number of circulating megakaryocytes to 3 times the preoperative value was found on the third postoperative day but not on the other postoperative days. Of the observed megakaryocytes 99% had only a narrow rim of cytoplasm or were naked nuclei.
Altered Erythrocyte Membrane Protein Phosphorylation in an Unusual Case of Hereditary Spherocytosis
Authors: Boivin Pierre , Delaunay Jean , Galand Colette
Published: August 2010

Membrane protein phosphorylation was examined in several members from a family with an unusual form of hereditary spherocytmis. After incubation of membrane ghosts with (γ‐32P) ATP, the phosphorylation of spectrin component II was diminished both in the absence and presence of CAMP. In the presence of this nucleotide, the phosphorylation of components IV5 and IV8 was also decreased. Along with a previously reported alteration of a membrane neutral phosphatase in this family, these abnormalities remove the present condition from the usual form of hereditary spherocytosis.
Characterization of Peripheral Blood, Spleen and Bone Marrow Cells from a Patient with Hairy Cell Leukaemia
Authors: Førre ØYstein , Ly Bernt , Thorsteinsson Leifur , Hovig Torstein
Published: August 2010

The peripheral blood cells, spleen cells and bone marrow cells from a patient with hairy cell leukaemia were studied by means of several immunological methods and by phase contrast and electron microscopy. Both by light and electron microscopy the cells had the morphology of hairy cells. 60% of all the peripheral blood cells, and 80% of the spleen cells had membrane‐bound IgGk immunoglobulin. 60% of the peripheral blood lymphocytes and 90% of the spleen cells were positive for Ia‐antigens, and 80% of the peripheral blood, and 70% of the spleen cells had receptors for complement factor C3. The percentages of cells with receptor for the Fc part of IgG and receptors for sheep red blood cells (SRBC) were low both in peripheral blood and in spleen. Reduced numbers of peroxidase positive cells and cytotoxic plaque‐forming cells were also observed as well as reduced lymphocyte responses after stimulation of peripheral blood lymphocytes with PHA, PWM, ConA, PPD and allogeneic cells. A normal antibody‐dependent cell cytotoxicity (ADCC) and PHA‐induced cytotoxicity was observed for the peripheral blood lymphocytes of the patients. Our results suggest that the hairy cells in our patient are derived from B lymphocytes and have a monoclonal origin.
Humoral and Cellular Immunity to Intrinsic Factor in Myasthenia Gravis
Authors: Zittoun J. , Tulliez M. , Estournet B. , Goulon M.
Published: August 2010

Myasthenia gravis (MG) is an autoimmune disease often associated with other autoimmune disorders. A case history of MG with a coexisting atypical megaloblastic anaemia with vitamin B12 deficiency and anti Intrinsic Factor (IF) antibodies, led to a study of humoral and cellular immunity to IF in 81 MG patients. Within this series, 3 other patients had a disturbed humoral and cellular immunity to IF. These 3 patients presented no other features of pernicious anaemia. The possible origins and significance of the anti IF antibodies in MG patients are discussed.
Hexokinase Deficiency in Erythrocytes: A New Variant in 5 Members of a Finnish Family
Authors: Siimes Martti A. , Rahiala Eeva‐Liisa , Leisti Jaakko
Published: August 2010

15 cases of congenital haemolytic anaemia have thus far been attributed to hexokinase (HK) deficiency in erythrocytes. We report some clinical, biochemical and genetic findings from 5 members of a Finnish family with this deficiency. The proband, a 1‐year‐old girl, was the only patient with anaemia. All subjects had either mild or marked reticulocytosis. Red cell ATP levels were at the lower range of normal in all subjects and 2,3‐DPG was abnormally low in one. The activities of red cell enzymes, other than HK, were within or above the normal range, respectively. The Km‐values for glucose and fructose were elevated (ATP normal) in the subjects with HK deficiency. We speculate that the family represents heterozygosity of a mutant allele and that there is phenotypic variation associated with the HK mutant. The locus might be subject to mutations which lead to a variety of HK variants and to a spectrum of diseases. This point of view is in accordance with the overwhelming variation of reaction kinetics and metabolic effects of this and other reported cases.
Retention in Glass Bead Columns as a Measure of Leucocyte Adhesiveness
Authors: Hopen Gunnar , Schreiner Aksel
Published: August 2010

Erythrocyte sedimentation significantly influenced test results when leucocyte retention in glass bead columns was used as a measure of leucocyte adhesiveness. In blood with elevated ESR, this influence was especially evident. Erythrocyte sedimentation highly increased leucocyte retention in vertical columns with a downward flow, whereas in slightly tilted columns with an upward flow, the retention was reduced. The error in measurements caused by erythrocyte sedimentation could be corrected by means of the haematocrit values in samples and eluates. Correction was better for results obtained with slightly elevated than with vertical columns. The granulocyte retention rates increased with increasing sample size, whereas lymphocyte retention remained constant.
Fatal Iron Intoxication with Multiple Coagulation Defects and Degradation of Factor VIII and Factor XIII
Authors: Henriksson P. , Nilsson L. , Nilsson I. M. , Stenberg P.
Published: August 2010

A severe iron intoxication in a 15‐year‐old girl resulted in profound damage to vital organs and multiple clotting defects, but no haemorrhagic diathesis. Investigation revealed not only impaired synthesis of coagulation factors but also abnormal proteo‐lysis by plasmin as well as by other proteolytic enzymes liberated from leucocytes and damaged tissue cells affecting especially factor VIII and factor XIII.
The Proliferative States of Circulating Granulopoietic Stem Cells in Man
Authors: Liu Yong K. , Stallard Stephen S. , Koo Victor , Dannaher Charles L.
Published: August 2010

The fraction of granulocyte‐macrophage colony‐forming cells (CFC) in DNA synthetic phase in blood from 25 normal adults and those in blood and bone marrow from 8 haematologically normal subjects were evaluated by in vitro culture of cells with and without prior exposure to 3H‐thymidine (12.5 μCi) for 1 h at 37° C. The exposure of blood cells from normal adults to 3H‐thymidine resulted in 26 ± 10 % reduction in colony formation and in 14 ± 10 % reduction in cluster formation. There was no difference in the magnitude of reduction in colony formation following exposure to 3H‐thymidine by cells in blood and those in bone marrow in 6 of the 8 haematologically normal subjects. These findings indicated that about one fourth of the circulating CFC in normal adults are in proliferative state and that significant difference in proliferative states beween CFC in blood and those in bone marrow probably does not exist in the majority of haematologically normal subjects.
Endotoxin‐Induced Colony Stimulating Activity in Normal and Myeloid Leukaemic Subjects
Authors: Hinterberger Wolfgang , Paukovits Walter R. , Mittermayer Karl
Published: August 2010

Low doses of endotoxin were administered to normal and acute and chronic myeloid leukaemic subjects. Temperature, leucocyte count, blood colony stimulating activity and the number of circulating colony forming units were monitored. All subjects acquired fever while the leucocyte count remained unaltered.

Blood CSA rose sharply in 6 normal individuals, but failed to increase in 4 acute leukaemic patients and in 2 patients with CML in blast crisis. 2 patients with CML in the chronic stage increased their blood CSA comparable to normal individuals. The number of circulating colony forming units rose following endotoxin with a peak at 30 min and declined subsequently. 4 patients with acute leukaemia showed no increase of circulating colony formers.
Rough Endoplasmic Reticulum‐Associated Tubular Structures Occurring in Acute Promyelucytic Leukaemia
Authors: Tange Tsuyoshi , Chiba Shyozo
Published: August 2010

Intracytoplasmic tubular structures (TS) which have not yet been described were found electron microscopically in a large percentage (26.5%) of leukaemic cells from a 25‐year‐old female with acute promyelocytic leukaemia. The TS were bundles of tubules measuring about 90 nm in diameter and continuous with rough endoplasmic reticulum, the maximum of the TS attaining 30 μm in length and 0.53 μm in width. The TS were mostly solitary, occasionally 2 or 3 within a cell in a plane of sectioning and were usually located around the Golgi region. By three‐dimensional observation with a goniometer stage, it was confirmed that the TS showed latticework of rings in cross sections and fascicular tubular arrays in longitudinal sections. In the latter, some tubules exhibited meshworks of polygonal vesicles measuring about 20 nm and cross striations of filamentous bristles with the periodicity of about 22 nm. The origin and nature of the TS are discussed.
Acquired Stomatocytosis in Alcoholic Liver Disease
Authors: Wislöff F. , Boman D.
Published: August 2010

The occurrence of transient stomatocytosis observed on peripheral blood smears and by scanning electron microscopy, was studied in 100 alcoholic patients admitted to a general medical ward. 15% of the patients manifested marked stomatocytosis, i.e. 10% or more of the cells being stomatocytes and a further 29% had slight stomatocytosis (3–9%). All patients with marked stomatocytosis had a daily average ethanol ingestion of 100 g or more, and had also more pronounced histological and biochemical evidence of alcoholic liver disease than those lacking stomatocytosis. MCV was significantly higher in these patients, but there were no differences in Hb, serum B12, folate, cholesterol or triglyceride levels. The stomatocytosis subsided gradually during 4–6 weeks of abstinence. Triconcave cells (knizocytes) were observed in 2 patients with terminal alcoholic liver disease.

Along with an increased MCV, the presence of stomatocytosis may be a useful erythrocyte indicator of alcoholic liver disease.
The Osmotic Fragility of Red Blood Cells: A Re‐Evaluation of Technical Conditions
Authors: Godal H. C. , Nyvold N. , Rustad A.
Published: August 2010

The osmotic fragility of red blood cells is influenced by even modest environmental changes. Consequently, the technical procedure must be strictly standardized. This implies that temperature equilibrium of the buffered salt solutions should be reached prior to the addition of blood. Furthermore, since erroneous statements concerning the composition of phosphate buffers regularly used to secure correct pH of the salt solutions have repeatedly appeared in the literature, pH control of such solutions prior to use becomes essential.
Fanconi's Anaemia Developing Erythroleukaemia
Authors: Prindull Gregor , Jentsch Eberhard , Hansmann Ingo
Published: August 2010

A 15‐year‐old boy with Fanconi's anaemia (FA) for 10 years developed acute erythroleukaemia. During the leukaemic phase, granulopoietic stem cells (CFUc) were absent from his bone marrow and blood, but proliferation and differentiation of bone marrow cells could be seen in semipermeable diffusion chambers in vivo, and globin synthesis of erythroblasts had become imbalanced. Chromosomal lesions of peripheral blood lymphocytes differed in the leukaemic phase from those in the pancytopenic phase. These data indicate that erythro‐, myelo‐, thrombo‐, and lymphocytic cell lines all were involved in both the leukaemic and the pancytopenic process. It is suggested that terminal myeloproliferative disease developed as part of the natural history of FA.
Acquired, Transient Factor X (Stuart Factor) Deficiency in a Patient with Mycoplasma Pneumonial Infection
Authors: Peuscher F. W. , Van Aken W. G. , Van Mourik J. A. , Swaak A. J. G. , Sie L. H. , van Eps L.W. Statius
Published: August 2010

A case of severe haemorrhagic diathesis due to acquired deficiency of factor X (both immunologically and in procoagulant activity) is presented. The clinical and serological features of this case indicated mycoplasma pneumonial infection. Factor X in the peripheral blood did not appear to be influenced by administration of vitamin K, prothrombin‐complex concentrate, fresh plasma or fresh whole blood. Circulating inhibitors of blood coagulation were absent and systemic amyloidosis could not be demonstrated. After 20 d, factor X spontaneously returned to normal. In view of the absence of other known causes of factor X deficiency, a possible relationship with mycoplasma pneumonial infection is suggested.
Acid Alpha‐Naphthyl Acetate Esterase (ANAE) Activity and DNA Synthesis of Lymph Node Cells in Hodgkin's Disease
Authors: Herva E. , Ryhanen P. , Blanco G. , Järvenpää K. , Helin H. , Alavaikko M.
Published: August 2010

Lymph node and peripheral blood lymphocytes in a case of Hodgkin's disease (mixed cellularity) were studied using May‐Grünwald‐Giemsa (MGG), acid naphthyl acetate esterase (ANAE), immunoperoxidase staining and lymphocyte surface markers, auto radiographic, and lymphocyte stimulation techniques.

According to MGG staining and auto radiographic studies of lymph, node cells, small lymphocytes, intermediate lymphoid cells, and large mononuclear cells resembling in vitro stimulated immunoblasts, Hodgkin's cells and Reed‐Sternberg (RS) cells formed a morphologically continuous DNA synthetizing series.

A large majority of small lymphocytes from a lymph node were ANAE positive, thus being T‐lymphocytes, and formed rosettes around large mononuclear cells and RS cells. Most RS and large mononuclear cells had ANAE positive spots in the cytoplasm, thus resembling T‐lymphixytes more than diffusely staining monocytes. These cells did not contain cytoplasmic immunoglobulin and were muramidase negative. Both lymph node and peripheral blood lymphocytes responded strongly to PHA. The role of T‐lymphocytes in Hodgkin's disease and the origin of RS cells are discussed on the basis of the findings.
Turnover of Human Lactoferrin in the Rabbit
Authors: Karle Hans , Hansen Niels Ebbe , Malmquist Jörgen , Karle Agnete K. , Larsson Ingvar
Published: August 2010

Lactoferrin turnover was studied in the rabbit with 125I‐ and 131I‐labelled human lactoferrin. Plasma lactoferrin activity showed a rapid decrease during the first 24 h, followed by a ‘final slope’ with a T1/2 of about 25 h. Turnover studies after transfer of plasma from one rabbit (A) 3 h after injection to another rabbit (B), showed a recovery of 100 % compared to 30 % in A rabbits but otherwise a similar disappearance curve. The rapid turnover was confirmed in whole body studies. Concomitantly with the initial disappearance from the plasma, there was a marked accumulation of protein bound activity only in the liver in both A and B rabbits. From these results, the rate of synthesis in normal man can be estimated at around 25 mg per d. The disappearance pattern and hepatic uptake are discussed in relation to knowledge about lactoferrin receptors in macrophages, asialo elimination, and fucose group recognition.

Concerning the significance of lactoferrin turnover for iron metabollism it is concluded that the plasma turnover results are insufficient to explain the disturbance in iron kinetics seen in the anaemia of chronic disorders; however, it is likely that lactoferrin plays a role in iron metabolism within the extravascular space.
Expression of DR Antigens on Early Human Progenitors of Neutrophilic and Eosinophilic Colonies in Diffusion Chambers (CFUd)
Authors: Jacobsen Niels , Broxmeyer Hal E. , Winchester Robert J. , Moore Malcolm A. S.
Published: August 2010

Incubation of the non‐adherent low density cells from normal human bone marrow with an antiserum to DR antigens markedly inhibited their capacity to form granulocytic colonies in fibrin clot diffusion chambers implanted intraperitoneally into irradiated mice. The inhibitory effect of antiserum was abrogated after absorption of antiserum with B cells. Inhibition was obtained both with and without complement treatment. Neutrophilic colony formation exhibited a higher sensitivity to diluted antiserum to DR antigens as compared with eosinophilic colony formation. These results suggest that DR determinants are expressed on the neutrophilic and eosinophilic progenitor cells (CFUd) which give rise to colonies in diffusion chambers.
FACTOR ASSAY (VIII AND IX) RESULTS IN THE COLLEGE OF AMERICAN PATHOLOGISTS SURVEY PROGRAM (1976–1979)
Authors: Triplett D.A. , Kocoshis T.A. , Harms C.S.
Published: August 2010

Data from the 1976–1979 CAP Surveys for Factor VIII assays are reviewed. Beginning in 1979, Factor IX assays were also performed by survey participants. Data was obtained from approximately 2500 laboratories and the different instrument‐reagents are ranked according to precision and sensitivity. Also, the results of questionnaires on specific assay techniques (1977 through 1979) are analyzed. A trend was noted indicating an increasing number of laboratories are now performing factor assays. There is poor correlation between precision and sensitivity of most systems (i.e., instrument and reagent combination). For any given system, there was considerable variation in precision from a normal Factor VIII levels specimen to a low Factor VIII specimen. This was also the case with respect to Factor IX assays. With respect to the technical aspects of factor assays there was marked interlaboratory variation (Le., dilution of normal plasma used to construct standard curve, etc.). The need for standardization of factor assays is evident from the survey data.
Familial Functional Antithrombin III Deficiency
Authors: Sørensen P. J. , Dyerberg J. , Stoffersen E. , Jensen M. Krogh
Published: August 2010

A family with a tendency to thrombosis and decreased antithrombin III (AT III) activity in plasma, but normal immunoreactive AT III is reported. 7 members of the family had the AT III defect, 4 of whom have had thrombotic episodes. The importance of biological determination of AT III when studying patients with recurrent thrombotic episodes is emphasized.
Clot Retraction in a Factor XIII Free System
Authors: Rao K. Murali Krishna , Newcomb Thomas F.
Published: August 2010

The role of Factor XIII in clot retraction was studied using the plasma from Factor XIII‐deficient patients. Time course experiments revealed no significant difference in clot retraction between a plasma deficient in Factor XIII and one to which purified Factor XIII had been added. Using Factor XIII‐free fibrinogen and Factor XIII‐deficient platelets, it is shown that there is no significant difference in clot retraction with or without added Factor XIII.
Lineation of the Osmotic Fragility Curve of Erythrocytes
Authors: Try K.
Published: August 2010

Lineation of the osmotic fragility curve by the method of Detraglia et al (1974) may be performed in most samples from a hospital population. Using the lineation procedure, the osmotic fragility may be tested by only 2 solutions of known osmolarity without any great loss of precision or accuracy. The osmotic fragility curve may be described by 2 values: C50 = the concentration at which 50% of the erythrocytes are haemolyzed and C50 ‐ C80 = the decrease in concentration raising the fraction of haemolysis from 0.50 to 0.80.
Human Lymphocyte Populations in Blood after Tetanus Vaccination
Authors: Enk Claes , Jønsson Viggo , Christensen Bjarne Egelund
Published: August 2010

The content of circulating T and B lymphocytes in 9 healthy volunteers was estimated before and after secondary vaccination with tetanus toxoid. E, Fc, and C3 receptors were demonstrated by rosette techniques and surface Ig by fluorescence technique. The absolute number of T and non‐T (B) lymphocytes was simultaneously increased during the first 48 h and at 144 h after vaccination. The correlation between paired T and non‐T (B) lymphocyte counts was enhanced after vaccination, prolbably reflecting mechanisms governing the traffic of lymphocytes.
Release and Quantitation of a Leucocyte Derived Protein (L1)
Authors: Fagerhol Magne K. , Dale Inge , Anderson Terje
Published: August 2010

Immunological methods have been developed for quantitation of a protein, provisionally called L1, which constitutes about 5% of the total protein in granulocytes. The protein has a mol wt of about 51000 daltons and an isoelectric point of about 6.3–6.5. It is readily released from leucocytes during storage and following immunological injury to the cell membrane. The concentration of L1 in normal plasma is about 300 ng/ml, but levels above the normal mean + 5 SD were found in 23 out of 30 patients with various malignant diseases and in 4 patients with septicaemia. It is suggested that plasma levels of L1 may reflect the rate of leucocyte turnover in vivo and that the L1 release phenomenon may be used for studies on leucocyte reactions in vitro.
In Vitro Colony and Cluster Growth in Haemopoietic Dysplasia (the Preleukaemic Syndrome)
Authors: Lidbeck Jan
Published: August 2010

Bone marrows from 23 patients with haemopoietic dysplasia were cultured in agar‐gel and the patients were followed for at least 6 months after culture. The incidence of colonies, microclusters and macroclusters and the colony to cluster and microcluster to macrocluster ratios were compared to those of a control group. In the control patients a normal wide range of colonies and clusters was observed and colony growth correlated with cluster growth. About half of the dysplastic patients showed a normal growth pattern and ran a stable and benign course. The other dysplastic patients had defective colony growth, a low colony to cluster ratio, an increased microcluster to macrocluster ratio and a deficient macrocluster growth early in culture. These patients more often presented with cytopenia and had a malignant course with increasing bone marrow dysplasia and a frequent need of blood transfusions. Some patients died of cytopenia or transformed into acute leukaemia.

The bone marrow cultures of haemopoietic dysplasia did not predict the short term (within 6 months) risk of leukaemia transformation. However, the results indicate that examination of culture growth can be used to differentiate stable and benign patients from those in whom clonal abnormalities may suggest a progressive and malignant course.
Prolonged Bleeding Time with Adequate Platelet Count in Hospital Patients
Authors: Wisløff F. , Godal H. C.
Published: August 2010

100 patients with a modified Ivy bleeding time longer than 10 min in the presence of more than 80 times 109/1 blood platelets were studied retrospectively. 72 patients had repeated bleeding times between 10 and 20 min or one or more measurements exceeding 20 min, and were considered to have an unquestionable platelet dysfunction. 39 (54 %) of these 72 patients were receiving large doses of antibiotics. Nearly one half of the patients on antibiotics had a bleeding tendency, but most of these patients had additional features that may have interfered with platelet function, or a coagulation defect.

In the remaining 33 patients, the prolonged bleeding time was associated with von Wille‐brand's disease, liver disease, leukaemia/myeloproliferative disease, paraproteinaemia or aspirin ingestion. High doses of penicillin seem to be the most common cause of qualitative platelet disorders in general hospital practice.
Monocyte Function in Normal Adults
Authors: Lawton J. W. M. , Gardner I. D.
Published: August 2010

Human blood monocytes were separated by centrifugation over modified Ficoll‐Hypaque and by this one‐step procedure relatively high proportions (mean 54%) and yields of monocytes were obtained. Yields ranged from 0.22 times 109 to 0.98 times 109/1 of blood and averaged 2 1/2 times the theoretical maximum yield calculated from the blood leucocyte and differential counts. A range of tests of monocyte function are described, namely, adhesion to glass, spreading on glass, chemotaxis under agarose and phagocytosis and killing of Candida albicans. These tests are generally simpler to perform than similar tests previously described and give very reproducible results which compare well with those reported by others. Normal values from 35 healthy Chinese adults are recorded.
Tissue and Plasma Lysozyme in Hodgkin's Disease
Authors: Hansen Niels Ebbe , Clausen Per Prætorius , Karle Hans , Christoffersen Per
Published: August 2010

Lysozyme positivity in lymph nodes from 33 patients with Hodgkin's disease was examined with an immunoperoxidase technique. Positive reactive histiocytes were found in 18 cases; in 7 cases lysozyme was found also in mononuclear Hodgkin cells and in 3 cases in both reactive histiocytes, mononuclear Hodgkin cells and Reed‐Sternberg cells. There was no clear‐cut correlation between cellular lysozyme positivity and such feature as histological type, clinical stage and plasma lysozyme. The findings support the theory that the malignant cell in Hodgkin's disease is derived from a lysozyme producing macrophage. The finding that lysozyme positivity was an inconstant feature may reflect both varying functional status of the macrophages and varying differentiation of Hodgkin cells. Increased plasma lysozyme in Hodgkin's disease may stem from both reactive histiocytes, mononuclear Hodgkin cells and Reed‐Sternberg cells, but the major part is probably contributed by reactive histiocytes.
Giant Lymph Node Hyperplasia (Castleman's Disease) Associated with Temporal Arteritis
Authors: Lenner Per , Lundgren Erik
Published: August 2010

Giant lymph node hyperplasia is a lesion of lymphoid tissue, which may be accompanied by systemic manifestations like fever, anaemia and hyperglobulinaemia. A case is described with temporal arteritis, an association hitherto not described in the literature.
Monoclonal Blood Lymphocytes in Benign Monoclonal Gammopathy and Multiple Myeloma in Relation to Clinical Stage
Authors: Pettersson Dagny , Mellstedt Håkan , Holm Göran , Björkholm Magnus
Published: August 2010

20 patients with benign monoclonal gammopathy (BMG) have been studied for blood lymphocyte and subpopulations. 4 patients had slightly decreased T‐lymphocyte values. Total B‐lymphocytes were within the normal range. In 3 BMG patients an abnormal ratio between χ‐ and Λ‐bearing lymphocytes was detected indicating circulating monoclonal cells. 41 patients with untreated multiple myeloma have also been analysed for blood monoclonal lymphoid cells using the χ:Λ‐ratio. 54% of the patients had monoclonal blood cells at diagnosis. The incidence and numbers of such cells increased with advanced clinical stage. Thus, it seems as if tumor volume is the main factor responsible for the appearance of monoclonal blood cells in multiple myeloma.
Immune‐Complexes (IC) in Idiopathic Neutropenia
Authors: Cappio Federico Caligaris , Camussi Giovanni , Novarino Anna , Campana Dario , Masera Clotilde , Infelise Vittorio , Gavosto Felice
Published: August 2010

The presence of immune‐complexes (IC) and antipolymorphonuclear neutrophil (PMN) autoantibodies was investigated in 28 patients with chronic idiopathic neutropenia and normal or hypercellular bone marrow, 19 with a metamyelocyte arrest and 9 with more dysplastic features. The in vivo interaction between IC and PMN membrane receptors was evaluated by means of the PMN immunohistological technique. Circulating IC was evaluated with the C1q and rheumatoid factor agglutination inhibition techniques. An anti‐PM auto‐antibody activity was investigated by challenging Fab obtained from the sera of 22 patients with PMN from normal donors. IC were detected in a high percentage of patients; in no case could an anti‐PM autoantibody activity be seen. Most patients with a metamyelocyte arrest, but only 1 with more dysplastic features, were IC+. During a follow‐up period of 12–52 months, none of the patients with a metamyelocyte arrest (IC+) developed anaemia, thrombocytopenia or leukaemia, while anaemia and thrombocytopenia were almost the rule in the clinical course of dysplastic bone marrow IC‐ patients: 2 of them developed acute myeloblastic leukaemia.
Evidence for Imbalanced Furosemide‐Sensitive Na+, K+ Cotransport in Hereditary Stomatocytosis
Authors: Chailley Bernadette , Feo Claude , Garay Ricardo , Dagher Georges , Bruckdorfer Richard , Fischer Siegmund , Piau Jean‐Pierre , Delaunay Jean
Published: August 2010

The red cells from 5 related patients with hereditary stomatocytosis were investigated. Maximal rate constant of Na+ passive permeability was increased while that of K+ passive permeability was nearly normal. Ouabain‐sensitive Na+ efflux was elevated. The Na+ component of furosemide‐sensitive Na+, K+ cotransport was also increased. However, its K+ component, determined in 2 patients, remained within normal limits, thus departing from the strict 1:1 stoichiometry of the Na+, K+ cotransport system. Yet, intracellular Na+ and K+ concentrations displayed limited and inconstant changes. A variety of abnormally‐shaped cells, including stomatocytes, were observed in scanning electron micrographs. Upon differential centrifugation, reticulocytes usually concentrated in the most dense region of the gradient. Red cell deformability, as studied by ektacytometry, was reduced. Membrane phosphatidylcholines and sphingomyelins were increased and decreased, respectively, whereas fatty acid distribution was unchanged. Membrane microviscosity was normal.
Megakaryocytes in the Foetal Circulation and in Cubital Venous Blood in the Mother before and after Delivery
Authors: Pedersen Niels Tinggaard , Petersen Sten , Greulich Lone
Published: August 2010

22 newborn infants delivered by Caesarean section were investigated in order to ascertain the number and morphology of megakaryocytes in the umbilical artery and vein. Averages of 65.5 and 47 MK per ml blood were found, respectively. 25% of MK in the artery and 8% in the vein had copious cytoplasm. Thus there was a significantly higher total number of MK and number of MK with copious cytoplasm in blood from the artery than from the vein (P < 0.05). In blood from the aorta in 16 newborn infants we found an average of 28 MK per ml blood, 8% had copious cytoplasm. There was a significantly smaller number of MK in the aorta than in both the artery and the vein of the umbilical cord (P < 0.05). There was the same percentage of MK with copious cytoplasm in the aorta as in the umbilical vein.

The number of MK in cubital venous blood from the mother prior to delivery was 17.5 MK per ml and was significantly higher than about 3 months after delivery when it was 5 MK per ml blood, which is a normal value (P < 0.05). In both series, 1.5% of MK had copious cytoplasm, as in a normal material.
Blood Lymphocyte Functions in Relation to Splenic Weight and Tumor Involvement in Untreated Hodgkin's Disease
Authors: Björkholm Magnus , Askergren Jutta , Wedelin Christina , Holm Göran , Mellstedt Håkan
Published: August 2010

Spontaneous and mitogen induced blood lymphocyte DNA synthesis was studied in relation to weight and tumor involvement of the spleen in 33 untreated patients with Hodgkin's Disease. Splenic tumor involvement was found in 14 patients. In this group there was a strong inverse correlation between spleen weight and lymphocyte DNA synthesis induced by PWM, ConA and PPD. In patients with uninvolved spleens the spontaneous blood lymphocyte DNA synthesis increased with spleen weight. No correlation with the mitogen response was observed in this group.
Antibody‐Dependent Monocyte‐Mediated Cytotoxicity.
Authors: Kragballe Knud , Ellegaard Jørgen , Herlin Troels
Published: August 2010

Monocyte mediated lysis of human type B‐erythrocytes coated with heat‐inactivated anti‐B serum was measured as 51Cr‐release. Preincubation of monocytes with heat‐inactivated normal AB serum inhibited cytolysis while a strong stimulation of cytolysis was seen with fresh AB serum. These effects of serum were reversible upon cell washing. Preincubation of monocytes with hyperimmune human serum against type B erythrocytes did not alter the cytolysis. After incubation at 37° C of monocytes with immune complexes formed of human albumin and rabbit anti‐human albumin a dose‐related inhibition of cytolysis was observed. This inhibition was equally expressed at different albumin to anti‐albumin ratios. Pretreatment with immune complexes at 4°C had the same effect on cytolysis as pretreatment at 37° C. The effect of immune complexes on monocyte‐mediated cytolysis was resistant to repeated cell washing. After incubation of pretreated monocytes at 37°C for up to 18 h some of the inhibition disappeared, but without normalization. Platelet depletion of monocyte suspensions resulted in a significant increase of cytolysis, and addition of washed platelets reinduced a decrease of cytolysis, which correlated with the number of platelets added.