bluuebunny/crossref_metadata_2025_split huggingface dataset:
Thalassemia in the West Indies
Authors: WENT L. N. , MACIVER J. E. 
Published: October 2019

Abstract
Data are presented on seven cases of thalassemia major (six of whom were negroes) and 32 cases of thalassemia minor of whom eight had high levels (20-26 per cent) of fetal hemoglobin.
Two families with hemoglobin H disease are presented, in whom the presence of thalassemia minor could be demonstrated. The mode of inheritance of hemoglobin H disease is discussed.
Two families with an inherited double A2 fraction of hemoglobin are presented. In one of these thalassemia was also found, in the other elliptocytosis occurred concurrently.
On the basis of these findings the existence of at least five different types of thalassemia is postulated.
A study on 93 healthy students suggests a high incidence (above 3 per cent) of thalassemia minor in the West Indies.
Neoplastic Lymphoid Reticulum Cells in the Peripheral Blood: A Histochemical Study
Authors: MITUS W. J. , MEDNICOFF I. B. , WITTELS B. , DAMESHEK W. 
Published: October 2019

Abstract
Abnormal appearing cells having the superficial appearance of large lymphocytes were found in some cases of generalized malignant proliferations af the reticulum cell system and were studied by cytochemical methods and by phase microscopy. They were found to be very similar in their reactions to the "fixed" or tissue reticulum cells and in many respects to monocytes, but differed markedly from the cells of the lymphocytic system with which they are often confused. The term neoplastic lymphoid reticulum cell is applied to these cells since it indicates that they are malignant in nature and fundamentally of the reticulum cell type, although in fixed and stain material they may show some of the morphologic characteristics of the lymphocytic cells.
Polycythemia Vera in Childhood. Studies of Iron Kinetics with Fe59 and Blood Clotting Factors
Authors: AGGELER P. M. , POLLYCOVE M. , HOAG S. , DONALD W. G. , LAWRENCE J. H. 
Published: October 2019

Abstract
A case of polycythemia vera, first diagnosed in a nine year old girl, is presented. The increase in red cell count, hematocrit and hemoglobin values was accompanied by an increase in total blood volume and true red cell volume. No other condition reported to be accompanied by polycythemia was found. Specific, mild deficiencies in certain coagulation factors were found. Iron kinetic study with Fe59 showed increased hemoglobin formation within normal iron pathways which is characteristic of polycythemia vera.
Blood Group Antibody Levels in Hiroshima
Authors: HOLLINGSWORTH JAMES W. , HAMILTON HOWARD B. , BEEBE GILBERT W. , YAMASAKI MITSURU , Ueda Noboru 
Published: October 2019

Abstract
Blood group antibody levels were measured in 526 irradiated survivors of the 1945 atomic bombing of Hiroshima and in 516 nonirradiated subjects. The study was undertaken in order to determine the age changes in antibody levels in irradiated and nonirradiated subjects, as well as to investigate the pattern of blood agglutinin levels in the Japanese population for comparison with that of Caucasians. The following observations were made:
1. In 475 people of group A, 228 of group B, and 339 of group O, the mean serum levels of anti-A and anti-B antibodies were virtually identical irrespective of the blood group of the subjects.
2. In individual group O subjects, the titers of anti-A and anti-B antibodies were very highly correlated (correlation coefficient of +.92).
3. Females exhibited higher antibody levels, although the magnitude of the difference was small.
4. Peak antibody titers were reached at age 20-30, with progressive linear regression in levels with advancing age. Peak titers in early adulthood were 5-10 times higher than those of the very elderly.
5. No correlation between blood group antibody levels and atomic irradiation was detected, whether irradiation was represented by (a) presence or absence of acute radiation symptoms in 1945, (b) distance from the hypocenter, or (c) numerical dosage estimate.
6. As one of a battery of tests of physiologic aging designed for detection of irradiation induced nonspecific aging acceleration, blood group antibody levels seem of modest value. After age 30, the linear correlation with chronologic age is -.72.
7. Differences in blood group antibody levels in Japanese and Caucasians indicate that (a) Caucasians have higher anti-A antibody levels as compared to anti-B levels than do Japanese, and (b) the peak antibody level occurs at an earlier age in Caucasians than in Japanese. It is not clear whether these differences are related to race and heredity or to external antigenic stimulation with A and B antigenic materials, but studies of blood group antibody levels in different racial groups should help elucidate the nature of these differences.
pH Dependent Hemolytic Systems. II. Serum Factors Involved in Red Cell Lysis
Authors: YACHNIN STANLEY , GARDNER FRANK H. 
Published: October 2019

Abstract
An analysis of the serum factors involved in the lysis and agglutination of artificially altered red cells in compatible human serum has been presented. Human sera have been found to vary widely in their capacity to hemolyze a panel of artificially altered red cells.
These differences have been shown to be the result of a multiplicity of more or less specific serum factors for each altered red cell type. These factors have many of the properties commonly associated with classical antibody.
These serum factors are different from complement and properdin.
The range of specificity and the degree of cross reaction for these various serum factors have been analyzed.
These serum factors are important in the consideration of the use of enzyme treated red cells for the detection of incomplete antibodies, and of certain acquired hemolytic states in man.
Pyridoxine-Responsive Anemia: Report of 2 Cases in Brothers and a Review of the Literature
Authors: MEDAL LUIS SANCHEZ , ELIZONDO JORGE , GALLARDO JESUS TORRES , GITTLER CARLOS 
Published: October 2019

Abstract
The clinical and laboratory findings in two brothers with severe anemia are presented. These findings were very similar in both cases. Evidence that at least one of them suffered from a pyridoxine-responsive anemia is presented. It was assumed that the other had the same disorder since, in addition to the striking similarity in the clinical and laboratory abnormalities, the latter’s anemia disappeared completely with the parenteral administration of vitamin B complex, which provided him with 2 mg. of pyridoxine daily. These patients have not relapsed 1.5 and 6 years after stopping the therapy.
A review of the reported cases with anemia responding to vitamin B6 administration is presented. Common factors observed in some of these cases are: (1) severe anemia, microcytic and hypochromic in type, chiefly due to a striking maturation erythroblastic arrest at the basophilic stage with no defect in leukocytes or thrombocytes; (2) hyperferremia and hemosiderosis; (3) an abnormal tryptophan-loading test; and (4) complete or almost complete correction of all abnormalities with administration of vitamin B6, even at small doses. These data were considered to suggest that the patients had a true deficiency in pyridoxine.
In other patients, no increased excretion of xanthurenic acid has been observed after a tryptophan-loading dose, and pyridoxine administration has improved the anemia only partially and has not reversed the serum iron parameters to normality. All of these other patients suffered from a familial hypochromic anemia not due to iron deficiency or from a "sidero-achrestic" or "refractory normoblastic" anemia.
The cause of the disorder in patients in the first group is unknown, but, by exclusion, increased requirement of vitamin B6 seems to be the most likely possibility.
The Hemolytic Anemia of Human Bartonellosis
Authors: REYNAFARJE CÉSAR , RAMOS JOSÉ 
Published: October 2019

Abstract
A study of the processes of formation and destruction of blood has been carried out, in addition to other investigations of the physiopathology of the anemia of human bartonellosis. From the results obtained the following conclusions may be drawn:
1. The life-span of the red cells parasitized by Bartonella bacilliformis is greatly shortened. However, not all the parasitized red cells are prematurely destroyed.
2. Red cells from normal subjects are partially destroyed when they are injected into infected patients. More than 50 per cent of them survive normally.
3. The mechanical fragility of the red cells is increased in the majority of the cases.
4. The index of sequestration of red cells by the liver and spleen was increased in the three patients studied. Also, the products of catabolism of hemoglobin were increased in all the patients studied.
5. The increased production of red cells as a response to the great destruction was prevented at first, but later it reached its peak, being in some cases five times greater than normal.
6. The search for agglutinins and hemolysins was negative.
7. The amount of free protoporphyrins in the red cells was increased, indicating that there was some interference to the synthesis of hemoglobin that would also explain the hypochromia of the red cells.
8. The increase in the diameter of the red cells was independent of the actual amount of reticulocytes.
Sickle Cell Trait as a Tag for Bone Marrow Transplantation
Authors: MILLER DANIEL G. 
Published: October 2019

Abstract
The sickle cell trait appears to offer a reliable and replicable tag for bone marrow transplantation. Red cell survival curves using sickle cell and nonagglutinable cell counts show good agreement. The recipients exhibited no untoward effects as a result of receiving marrow from a donor with sickle cell trait.
Decreased Erythrocyte Survival in Hemoglobin H Disease As a Result of the Abnormal Properties of Hemoglobin H: The Benefit of Splenectomy
Authors: RIGAS DEMETRIOS A. RIGAS , KOLER ROBERT D. , Cummings George , Duerst Marie L. , Malm Donald R. , Swisher Katherine , Vanbellinghen Patricia 
Published: October 2019

Abstract
Two of three siblings with hemoglobin H disease were splenectomized. Both were benefited as judged by improved feeling of well-being, exercise tolerance and improvement in hemoglobin level and in erythrocyte mean lifespan. However, they were still susceptible to hemolytic crises, and their erythrocyte mean lifespan was below the normal range. Erythrocyte survival studies by the Cr51 method indicated a finite lifespan of 40-45 days before splenectomy, which increased to normal following splenectomy. In addition, there was random destruction of the erythrocytes, which was slightly reduced following splenectomy. The erythrocyte survival of a member of this family who had the hereditary leptocytosis trait, but no hemoglobin H, was normal.
Evidence is presented indicating that hemoglobin H denatures and precipitates irreversibly at a cell age of 40-45 days, forming intraerythrocytic inclusions which lead to shortening of the erythrocyte finite lifespan due to rapid removal of such erythrocytes by the spleen. In addition, hemoglobin H has a lower solubility in the deoxygenated state and precipitates reversibly, regardless of cell age, in the capillary bed, causing random erythrocyte destruction. Splenectomy is beneficial because of survival of erythrocytes with inclusions whose hemoglobin A presumably retains its function in oxygen transport. Methemoglobin-forming chemicals such as amyl nitrite and drugs such as sulfisoxazole were found to denature hemoglobin H and produce in vitro inclusions in every erythrocyte of these patients but not of normal subjects. It is suggested that the shortening of the erythrocyte survival and the degree of postsplenectomy improvement is greater the higher the amount of hemoglobin H present in the erythrocytes.
The apparent aging of the hemoglobin H molecule which leads to shortened erythroctye finite lifespan is discussed, and a concept of aging proteins determining the lifespan and/or the function of cells is advanced.
Glucose-6-Phosphate Dehydrogenase Deficiency in Greece
Authors: ZANNOS-MARIOLEA LEDA , KATTAMIS CHRISTOS 
Published: October 2019

Abstract
The glutathione stability of red cells was estimated in 40 patients during acute hemolysis induced by fava beans. There were wide individual differences but in all cases except one (Case 18) the post-incubation GSH fell to levels below 40 mg. per cent packed RBC which is the lower normal limit.
The GSH stability on 44 mothers and 37 fathers gave results consistent with the genetic hypothesis that in male patients the mother is the carrier of the biochemical defect, while in female patients both parents are carriers, since, as a rule, only female homozygotes suffer from hemolytic episodes. However, in only 77.7 per cent of the mothers could the biochemical defect be proved by this method.
The Motulsky test was performed in 30 of the 40 patients. It gave abnormal decolorization times in 25 or 83 per cent of the cases. This test is therefore valuable for diagnosing "sensitivity" during a hemolytic episode; it is, nevertheless, less sensitive than the GSH stability method.
The Motulsky test was also performed on 31 mothers, 18 fathers and 8 siblings. It proved to be unreliable in the detection of female heterozygotes.
G-6-PD deficiency is widely disseminated in Greece; it is, however, not evenly distributed throughout the country.
The highest frequency of G-6-PD deficiency found so far in males was about 3 per cent; the lowest was 0.7 per cent.
The Mode of Inheritance of PTA Deficiency: Evidence for the Existence of Major PTA Deficiency and Minor PTA Deficiency
Authors: RAPAPORT SAMUEL I. , PROCTOR ROBERT R. , PATCH MARY JANE , YETTRA MAURICE 
Published: October 2019

Abstract
Plasma thromboplastin antecedent (PTA) activity was measured with a quantitative assay in the available members of the families of eight propositi with severe PTA deficiency. PTA deficiency was found to exist in two forms: major PTA deficiency, characterized by PTA levels of up to 20 per cent of our standard reference plasma and by the potential for serious surgical bleeding, and minor PTA deficiency, characterized by PTA levels between 30 and 65 per cent of our standard reference plasma and by the absence of significant surgical bleeding. Minor PTA deficiency was found in parents and children of subjects with major PTA deficiency.
It would appear that the gene for PTA deficiency is an incompletely recessive or "intermediate" gene which produces major PTA deficiency in the homozygote and minor PTA deficiency in the heterozygote.
In Vivo Cultivation of Leukocytes in Diffusion Chambers: Requirement of Ascorbic Acid for Differentiation of Mononuclear Leukocytes to Fibroblasts
Authors: PETRAKIS NICHOLAS L. 
Published: October 2019

Abstract
Studies were made to evaluate the influence of ascorbic acid upon the differentiation of mononuclear leukocytes to fibroblasts when cultivated in diffusion chambers, in vivo. Ascorbic acid-depleted leukocytes grown in ascorbic acid-deficient host guinea pigs developed into abnormal cellular forms characterized by nuclear enlargement, multipolar mitoses, and giant forms These changes could be reversed by treatment of the host guinea pigs with ascorbic acid. The findings indicate a direct cellular role of ascorbic acid in the differentiation of mononuclear leukocytes to fibroblasts.
Hydroxocobalamin. II. Absorption from the Site of Injection and Uptake by the Liver and Calf Muscle in Man
Authors: GLASS GEORGE B. JERZY , LEE DUK HO , HARDY WILLIAM W. 
Published: October 2019

Abstract
Labeled hydroxocobalamin, administered intramuscularly in man, is absorbed more slowly from the site of injection and deposited in the liver and calf muscle to a degree equal to or higher than that of cyanocobalamin at an equivalent dose. These findings, together with those reported in Part I of this paper, suggest the applicability of hydroxocobalamin as a long-acting vitamin B12 in man. These conclusions, however, require the support to be obtained from long-range observations on patients with vitamin B12 depletion.
Differences Between Lymphocytes of Leukemic and Non-Leukemic Patients with Respect to Morphologic Features, Motility, and Sensitivity to Guinea Pig Serum
Authors: SCHREK ROBERT , DONNELLY WILLIAM J. 
Published: October 2019

Abstract
Observations were made on viable lymphocytes in 22 specimens of blood from 20 patients to test whether leukemic lymphocytes have distinctive characteristics. Special slide-chambers were used in this study. The features suggestive of leukemic lymphocytes were: large chromatin masses, prominent nucleoli, abnormal size of cells, flagella-like structures, reduced motility, and an increase in the sensitivity to inactivated guinea pig sera. An index summarizing these abnormal features selected correctly 9 blood specimens from 7 patients with chronic lymphocytic or lymphosarcoma-cell leukemia. Distinctive abnormalities of the living lymphocytes were not found in the blood of 3 patients who were diagnosed lymphosarcoma and had normal lymphocyte counts and some lymphosarcoma-cell lymphocytes in stained blood films. Blood lymphocytes from 8 patients with infectious mononucleosis, diabetes, hemochromatosis, and other diseases were correctly identified as non-leukemic. The findings obtained in this study indicate that viable leukemic lymphocytes are characterized by abnormal morphologic features, reduced motility, and increased sensitivity to heat-inactivated guinea pig sera.
Variations in Catalase Activity in Human Leukocytes
Authors: KIDSON CHEV 
Published: October 2019

Abstract
The enzyme, catalase, has been studied in normal leukocytes, and in leukocytes from patients with leukemia and infective states. Examination of both granulocyte-rich and lymphocyte-rich fractions prepared from normal leukocyte populations showed the catalase activity of normal granulocytes to be higher than that of normal lymphocytes.
In acute and chronic myeloid leukemia, catalase levels were found to be higher than the range for normal granulocytes, whereas in acute and chronic lymphatic leukemia, the levels were of the same order as those for normal lymphocytes.
Low catalase values were found in infective leukocytosis, suggesting that young granulocytes may have lower activities than the mature cells. The significance of the changes in leukocyte catalase activity in myeloid leukemia are discussed in relation to cell age.
Pyruvate Kinase (PK) Deficiency Hereditary Nonspherocytic Hemolytic Anemia
Authors: TANAKA KOUICHI R. , VALENTINE WILLIAM N. , MIWA SHIRO 
Published: October 2019

Abstract
1. The erythrocytes of seven patients conforming to the criteria of Type II congenital nonspherocytic hemolytic anemia have been demonstrated to have a specific deficiency in the glycolytic enzyme pyruvate kinase. Other glycolytic enzymes, glucose-6-phosphate and 6-phosphogluconic dehydrogenases, and certain non-glycolytic erythrocyte enzymes are normally active. The leukocytes of these patients possess normal pyruvate kinase activity.
2. Although no inhibitors were detected, the exact nature of the enzymatic defect remains to be elucidated.
3. Family studies provide strong evidence for a genetically determined disorder and are consistent with an autosomal recessive transmission of the defect. A partial enzyme deficiency, not reflected in clinical disease, is present in heterozygotes. The symptomatic disease, though variable in severity, appears to be due to homozygosity for the defect.
4. It is suggested that the enzyme deficiency is pathogenetically related to the premature demise of the red cells in vivo.
5. The name "pyruvate kinase (PK) deficiency hereditary nonspherocytic hemolytic anemia" is proposed for these patients.
Hereditary Benign Erythroreticulosis
Authors: BERGSTRÖM INGMAR , JACOBSSON LENNART 
Published: October 2019

Abstract
In the province of Västerbotten, Northern Sweden, we have observed a blood disease formerly unknown in Europe. It is characterized by:
1. A non-hemolytic normochromic anemia with a low or normal reticulocyte count and without indications of reduced or increased activity of either granulocytopoiesis or thrombocytopoiesis.
2. A characteristic bone marrow picture corresponding to the di Guglielmo disorder.
3. An abnormal hemoglobin.
4. A favorable clinical course, but refractory to therapy.
5. An hereditary component.
The Unitary Nature of "Complete" and "Incomplete" Pathologic Cold Hemagglutinins
Authors: LEDDY JOHN P. , TRABOLD NORMA C. , VAUGHAN JOHN H. , SWISHER SCOTT N. 
Published: October 2019

Abstract
Several human pathologic sera containing high titered cold agglutinins were studied to determine whether the serologic activity ascribed to an "incomplete cold antibody" could be separated from the "complete" cold agglutinin activity. Separation was not achieved by physicochemical methods, including zone electrophoresis, density gradient ultracentrifugation, and anion exchange chromatography. Both activities were susceptible to destruction by mercaptans. Neither activity could be differentially absorbed from the sera. Using "Bombay" and I-negative ("i") red cells, a difference in specificity of the two activities for the H or I antigen of human erythrocytes could not be demonstrated. The simplest interpretation of these findings is that there is only one antibody involved, the cold agglutinin, and that the serologic manifestation usually attributed to an additional "incomplete cold antibody", i.e. the production of a positive antiglobulin reaction of the "non-γ-globulin" type, results from an interaction of complement components with the cold agglutinin-erythrocyte complex.
Three of these cold agglutinating sera were unreactive with I-negative erythrocytes, in keeping with the reported anti-I specificity of these antibodies. A fourth serum retained moderate, though greatly reduced, activity against these cells, and the interpretation of this finding is discussed.
The anti-H specificity of the incomplete cold antibodies in normal human sera was confirmed by their failure to sensitize "Bombay" erythrocytes. This was in sharp contrast to the excellent reactivity of the pathologic sera with these cells, demonstrating that pathologic cold agglutinins are unrelated to the incomplete cold antibodies present in most normal sera.
Spontaneous Hemorrhagic Diathesis in Inbred Mice Due to Single or Multiple "Prothrombin-Complex" Deficiencies
Authors: MEIER HANS , ALLEN ROBERT C. , HOAG WARREN G. 
Published: October 2019

Abstract
Spontaneous hemorrhagic diathesis had been observed in certain inbred strains of mice. Characteristically, bilateral hemothorax with or without multiple bleedings occurred elsewhere in the body; the mice were anemic and jaundiced.
The major histopathologic findings were myocarditis and various degrees of hepatomegaly. Associated with myocarditis was elevation of lactic dehydrogenase; anemia and icterus were thought to be consequent to extensive hemorrhage rather than to hepatic dysfunction since serum phosphatase and transaminase were, except for lowered serum glutamic pyruvic transaminase, within normal range or only slightly altered.
The mice were found to suffer from single and multiple prothrombin-complex deficiencies, specifically affecting Stuart-Prower factor (X), PTC (IX), SPCA (VII), and prothrombin. Activities of the first three factors were less than three per cent of normal and the last was less than 10; Ac-G (V), Hageman factor and fibrinogen were normal. Details of component assays and substitution analyses were given. The disease was acquired, but affected various strains differentially, and involved only males. No explanation was offered for this peculiar strain and sex susceptibility. Evidence was presented for implicating ethylene glycol and higher polymers produced by ethylene oxide sterilization of mouse cage bedding.