bluuebunny/crossref_metadata_2025 huggingface dataset:
Murine recovery from busulfan‐induced hematopoietic toxicity as assessed by three assays for colony‐forming cells
Authors: Boggs Dane R. , Boggs Sallie S. , Chervenick Paul A. , Patrene Kenneth D.
Published: February 2007

These studies were designed to explore further the interrelationships between various stem cell and other hematopoietic compartments of the mouse. Busulfan (BU) has been shown by others to have a profound effect on the transplantable pluripotent stem cells capable of forming colonies on spleens (CFU) of lethally irradiated recipients – more so than on cells of differentiated compartments. Another presumed assay of pluripotent stem cells consists of counting colonies on spleens of nontransplanted sublethally irradiated mice as a quantitation of endogenous colony‐forming units (E‐CFU). We examined and compared BU‐induced damage and recovery as measured by E‐CFU, CFU, CFU‐C, total cellularity of marrow, peroxidase‐positive cells per marrow, spleen weight, radioactive iron uptake into spleen and marrow, and hematocrit. E‐CFU were severely damaged by BU, but recovery was significantly more rapid than that measured for CFU. These results were compatible with other data suggesting that cycling stem cells are more likely to survive radiation and less likely to survive removal and transplantation. Thus, the actual number of pluripotent stem cells lies between the CFU and E‐CFU values. Damage to more mature granulocytic and erythrocytic compartments lagged behind damage to E‐CFU and CFU. Abortive rises in these more mature compartments were observed, which suggested limited but distinct self‐replication activity of “stem cells” more differentiated than the CFU, E‐CFU systems. Time of final recovery of the damaged hematopoietic system was compatible with a concept of recovery of pluripotent stem cell system(s) preceding recovery of more mature compartments.
A survey of the effectiveness of cyclophosphamide in patients with severe aplastic anemia
Authors: Griner Paul F.
Published: February 2007

The members of the American Society of Hematology were surveyed to determine their experience in the treatment of patients with severe aplastic anemia (AA) with cyclophosphamide (CYT). Among the 401 members responding, 50 had treated AA patients, and three remissions were reported. These remissions can be explained either by response to CYT in a subgroup of patients with AA due to an immune mechanism or to spontaneous remission. Clinical trials of the efficacy of cytotoxic drugs in the treatment of AA patients will not likely permit a distinction between drug effect and spontaneous remission until the subgroup of patients with immune‐mediated AA is identified through the development of more sensitive and specific in vitro tests.
Mitochondrial metabolism in normal, myeloid, and erythroid hyperplastic rabbit bone marrow: Effect of chloramphenicol
Authors: Abou‐Khalil Samir , Salem Ziad , Yunis Adel A.
Published: February 2007

Protein synthesis, respiration, and oxidative phosphorylation were studied in mitochondria isolated from normal, myeloid, and erythroid hyperplastic bone marrow of rabbits. Mitochondria from normal and myeloid hyperplastic marrow showed a similar degree of 14C‐leucine incorporation while a threefold higher activity was demonstrated in mitochondria from erythroid marrow. The incorporation was mitochondrial‐concentration‐dependent and in all cases was inhibited by low concentrations of chloramphenicol (CAP). The respiratory activity of mitochondria isolated from either myeloid or erythroid marrow was twice as high as the normal, but in all cases respiration and phosphorylation were unaffected by concentrations of CAP which totally inhibited protein synthesis.
Detection of accessory spleens with indium 111‐labeled autologous platelets
Authors: Davis Harmon H. , Varki Ajit , Heaton W. Andrew , Siegel Barry A.
Published: February 2007

In two patients with recurrent immune thrombocytopenia, accessory splenic tissue was demonstrated by radionuclide imaging following administration of indium 111‐labeled autologous platelets. In one of these patients, no accessory splenic tissue was seen on images obtained with technetium 99m sulfur colloid. This new technique provides a simple means for demonstrating accessory spleens and simultaneously evaluating the life‐span of autologous platelets.
Essential thrombocythemia in a child: Platelet ultrastructure and function
Authors: Barnhart Marion I. , Kim Tae H. , Evatt Bruce L. , Ragab Abdelsalam H. , Lui Victor K. , Herman Jay , Lusher Jeanne M.
Published: February 2007

A nine‐year‐old black girl with essential thrombocythemia developed hemoptysis. Only two other cases in the English literature have been described. Ultrastructure and functional characteristics of this patient's platelets were studied. Twenty‐six percent of the patient's platelets were very large (megathrombocytes). Spontaneous aggregates from the patient's platelets were not compact, and the pseudopods did not interdigitate. Both qualitative and quantitative defects in platelet organelles were detected. The microtubular system was faulty in organization. Furthermore, the number of granules (especially alpha granules) was reduced. Platelet aggregation studies demonstrated subnormal aggregation in response to ADP, epinephrine, and collagen, but aggregation with ristocetin was normal. It is postulated that a platelet membrane abnormality may be the cause of their defective platelet aggregation.
Splenic artery embolization prior to splenectomy in end‐stage polycythemia vera
Authors: Hocking William G. , Machleder Herbert I. , Golde David W.
Published: February 2007

A patient with polycythemia rubra vera and myeloid metaplasia required splenectomy because of local symptoms attributable to a massively enlarged spleen. Preoperative embolization of the splenic circulation with inert materials was performed to reduce splenic size and vascularity in order to permit safe splenectomy. This case illustrates the utility of preoperative splenic embolization when routine splenectomy is contraindicated for technical reasons. The hazards of this technique and guidelines for its clinical use are outlined.
Transfusion‐induced malaria in a splenectomized β‐thalassemia major patient and review of blood donor screening methods
Authors: Joishy Suresh K. , Lopez Cynthia G.
Published: February 2007

Transfusion‐induced malaria may be a serious threat to the life of a group of special patients: those who require many transfusions, those who have been treated with immunosuppressants, and those who have undergone splenectomy. We describe transfusion‐induced malaria in a β‐thalassemia major patient who had undergone splenectomy. Existing guidelines for screening and excluding donors who have or have had malaria are inadequate, and serologic screenings are too costly. We suggest some guidelines that may help prevent transfusion‐induced malaria and supply an index of suspect signs and symptoms as a key to diagnosis.
On the methods calculating mean survival time in 51Cr‐platelet survival study
Authors: Tsukada Toshiyasu , Tango Toshiro
Published: February 2007

The International Committee for Standardization in Hematology recommended three methods for calculating objectively the mean platelet survival time, ie, weighted mean of linear estimate and logarithmic estimate, truncated exponential model, and gamma model. For determining which method is better, data of the survival study were fitted to each of three methods, as well as the Paulus polynomial and exponential polynomial models, and the mean survival time and the mean square error were compared.

Mean survival obtained by each method was always longest in healthy controls and shortest in ITP. In 3rd degree of polynomial estimate and 2nd and 3rd degrees of logarithmic estimate, several cases showed practically outlying mean survival time of more than 12 days. In healthy controls, highest mean square error was observed in 1st degree logarithmic estimate, and in cases with short mean survival time the highest one was obtained in 1st degree polynomial estimate. Mean square error in ITP pre‐ and postsplenectomy and in aplastic anemia was smallest in the gamma model.

Among nine methods recommended by the International Committee for Standardization in Hematology and by Dr. Paulus, the gamma model so far provides the best method for calculating the mean survival time of 51Cr‐platelets.
Oxygen affinity and stability of hemoglobin dunn (α6(A4)Asp→Asn): Use of isoelectric focusing in recognition of a new abnormal hemoglobin
Authors: Charache S. , Brimhall B. , Zaatari G. , Hathaway P. J. , Steiman I.
Published: February 2007

A new slow‐moving hemoglobin was found in low proportion in an asymptomatic black woman. Isoelectric focusing helped to distinguish it from other hemoglobins with similar electrophoretic mobility, and amino acid analysis showed that aspartic acid α6 (A4) had been replaced by asparagine. Oxygen affinity was increased, but the Bohr and DPG effects were normal. Stability of the purified hemoglobin was decreased, but that of hemolysates was normal. Abnormal oxygen affinity of this variant, and that of hemoglobin Sawara (α6(A4)Asp→Ala), may reflect loss of a salt bridge between Asp α6 and Lys‐α127(H10) which would tend to favor the high‐affinity R conformation of the molecule.
Disappearance of Hb F and i antigen during the first year of life
Authors: Henri Annie , Testa Ugo , Tonthat Hoi , Riou Jean , Titeux Monique , Vainchenker William , Feuilhade Franck , Galacteros Frédéric , Rochant Henri
Published: February 2007

In order to investigate whether a common control mechanism is involved in the diminution of i antigen expression and that of Hb F content in human erythrocytes during the postnatal period, we compared changes in 72 normal infants aged from 0 to 12 months. The proportion of hemoglobins (Hb F, Hb A, Hb A2) and the quantitation of „i”︁ antigen were determined on the total population of red blood cells. In addition, the percentage of individual cells containing Hb F or „i”︁ antigen or both (F cells, „i”︁ cells, and F + „i”︁ cells) were evaluated by using a rhodamine‐conjugated anti‐Hb F and a fluorescein conjugated anti‐i system on the same smear preparation, The results provided by the two most sensitive techniques (F cell counting and „i”︁ agglutinability) indicated that the curves of disappearance of Hb F and „i”︁ antigen along the 12 first months after birth were identical. A strong correlation (r = 0.97, P < 0.0001) existed between the percentage of F cells and „i”︁ antigen expression. In addition, the progressive increase in Hb A2 concentration was inversely correlated firstly with the proportion of Hb F and second with the expression of the „i”︁ antigen.

These results suggest that the switch from fetal to adult hemoglobin and the transformation of „i”︁ antigen expression occurring during the first year following birth are governed by a common control mechanism.
Mechanisms of levamisole‐induced granulocytopenia in breast cancer patients
Authors: Vogel Charles L. , Silverman Michael A. , Mansell Peter W. , Miller Alan M. , Thompson John S. , Herbick John M. , Brunskill Dennis E. , Padgett Darlene C. , McKinney E. Churchill , Sugarbaker Everett V.
Published: February 2007

Five of 39 (13%) women treated with adjuvant combination chemotherapy plus levamisole immunotherapy after mastectomy for Stage II or III breast cancer developed levamisole‐induced granulocytopenia. This complication occurred in each of the women between six and ten weeks after the completion of six months of combination chemoimmunotherapy when they were taking levamisole alone. Although none of the patients had an HLA B‐27 locus and leukoagglutinins could not be demonstrated, complement‐dependent, IgM mediated, peripheral destruction of granulocytes was documented using a microgranulocytotoxicity assay. In addition, a factor(s) present in serum from patients developing levamisole‐induced granulocytopenia caused suppression of bone marrow granulocyte progenitor cells (CFU‐C). The possible relationships between levamisole‐induced peripheral granulocyte destruction and bone marrow CFU‐C suppression are discussed.
Pulmonary manifestations of waldenstrom macroglobulinemia
Authors: Rausch P. Gregory , Herion John C.
Published: February 2007

Waldenstrom macroglobulinemia (WMG), a proliferation of malignant monoclonal IgM secreting plasmacytoid lymphocytes in lymph nodes, spleen, and marrow, usually pursues a chronic clinical course. A patient with WMG for five years who developed pulmonary tumors consisting of plasmacytoid lymphocytes prompted a review of the literature for pulmonary manifestations of WMG.

Twenty‐six males and 18 females, ranging in age from 33 to 84 years, have been reported with histologically proven pulmonary involvement by WMG. The x‐ray findings, evident in most patients when first seen, consisted of masses (22 patients), infiltrates (31 patients), and pleural effusions (19 patients). Most patients (24) had two or more of these manifestations but only five, in addition to our patient, had isolated pulmonary nodules. Isolated pulmonary infiltrates were found in ten patients and isolated pleural effusions in only four. Symptoms at the onset of pulmonary involvement included dyspnea (54%), nonproductive cough (33%), and chest pain (7%); 15% were asymptomatic. Pulmonary manifestations, like other features of WMG, respond to alkylating agents or irradiation and do not appear to affect prognosis adversely. Pulmonary involvement should be suspected in any patient with WMG who develops an abnormal chest x‐ray.
Lymphoblastic leukemic transformation (lymphoblastic crisis) in myelofibrosis and myeloid metaplasia
Authors: Polliack Aaron , Prokocimer Miron , Matzner Yaacov
Published: February 2007

Acute lymphoblastic leukemia (ALL) developing in myelofibrosis (MF) and myeloid metaplasia (MM) is reported in two patients. In both cases, the clinical course of the „blastic crisis”︁ was rapidly progressive with little response to chemotherapy. The circulating cells were readily identified as lymphoblasts on the basis of cytology, cytochemistry, immunologic studies, and ultrastructure. In one of the cases, 40% of cells had T‐cell markers and all cells contained paranuclear acid phosphatase. In the second case, cells had a „Burkitt‐like”︁ appearance, contained multiple cytoplasmic vacuoles positive for oil red O, and one‐third of them had B‐cell markers. The development of lymphoblastic crisis in MF and MM occurs rarely, is analogous to blastic transformation in chronic granulocytic leukemia, and supports the hypothesis that myeloproliferative disorders originate from pluripotent hematopoietic stem cells.
Hemolytic anemia in wilson disease: Clinical findings and biochemical mechanisms
Authors: Forman Stephen J. , Kumar K. Sree , Redeker Allan G. , Hochstein Paul
Published: February 2007

Two patients with Wilson disease who presented with severe hemolytic anemia are described. One was noted to have unusually high serum copper levels (369 μg/100 ml). A review of similar such patients in the literature suggests that, rather than having a low serum copper, patients with hemolysis accompanying Wilson disease have very high serum copper levels. For this reason, in vitro studies of the toxic effects of copper on erythrocytes were undertaken. It was found that, although copper does not have a major direct inhibitory effect on glycolytic enzymes such as hexokinase, the metal does inhibit hexokinase as a consequence of its interaction with oxyhemoglobin. However, such inhibition does not appear to be a major factor in copper‐induced hemolysis. On the other hand, the addition of the lipid antioxidant butylated hydroxyanisole (BHA) suppresses hemolysis in copper‐treated cells. These experiments suggest that the primary toxic effect of copper is mediated through its oxidant actions on membrane phospholipids rather than through its potential inhibitory effects on intracellular enzymes.
Post‐transfusion purpura: An enigma of alloimmunization
Authors: Lau Peter , Sholtis Carol M. , Aster Richard H.
Published: February 2007

A patient with post‐transfusion purpura is reported. This female patient, sensitized by previous blood transfusion, developed symptomatic thrombocytopenia seven days after a second blood transfusion. A platelet antibody, which had anti‐P1 A1 specificity, was identified by 51Cr‐release assay and by indirect immunofluorescence. Hemorrhage stopped abruptly after plasma exchange therapy. Thrombocytopenia did not recur when the patient was further challenged with P1A1‐positive blood and plasma. Anti‐P1A1 antibody, detectable by immunofluorescence but not by complement‐dependent platelet cytoxicity, persisted in high titer for at least one year after recovery. The heterogeneity and pathogenesis of this clinical syndrome are discussed.
Intracranial extramedullary hematopoiesis in β°‐thalassemia/hemoglobin E disease
Authors: Fucharoen Suthat , Tunthanavatana Chalermchai , Sonakul Dhira , Wasi Prawase
Published: February 2007

A 27‐year‐old woman with β‐thalassemia/hemoglobin E disease presented with an intracranial mass of 10 × 9 × 1.2 cm firmly attached to the dura and the falx cerebri extending over the central part of both hemispheres, with pressure atrophy of the underlying brain. Histopathologic examination revealed that the mass consisted entirely of hematopoietic cells. We have seen 1,315 cases with β‐thalassemia/hemoglobin E, but this was the first case with intracranial extramedullary hematopoietic mass.
Polycythemia vera in identical twins
Authors: Friedland Michael L. , Wittels Edward G. , Robinson Rita J.
Published: February 2007

The occurrence of polycythemia vera in family members has been a rare event, with little or no documentation in the literature to suggest a common genetic defect. We report here on the first case of polycythemia vera documented in identical twins. No chromosomal abnormalities have yet been detected in these patients. The criteria for the diagnosis of polycythemia vera are reviewed, and the possible implications of our findings are discussed.
Terminal deoxynucleotidyl transferase‐positive acute myeloblastic leukemia
Authors: McGraw T. P. , Folds J. D. , Bollum F. J. , Stass S. A.
Published: February 2007

Terminal deoxynucleotidyl transferase (TdT) is a biochemical marker for acute lymphoblastic leukemia (ALL). In studies of ALL at diagnosis, there are usually greater than 40% TdT‐positive cells by indirect immunofluorescence, whereas acute myeloblastic leukemia (AML) shows less than 1% TdT‐positive cells. Rare cases of TdT‐positive AML have been reported. We present here three AML patients with TdT in 15%, 45%, and 90% of the leukemic blasts. The diagnosis of AML was established on the basis of morphology and cytochemistry, and the cases included one patient with Auer rods. Myeloperoxidase was present respectively in 20%, 90%, and 5% of the blasts. There was no Philadelphia chromosome present in the three cases. These results may indicate the simultaneous presence of lymphoid and myeloid populations, or the presence of a blast cell with both lymphoid and myeloid markers.
Juvenile and adult types of chronic granulocytic leukemia of childhood: Growth patterns and characteristics of granulocyte‐macrophage colony forming cells
Authors: Barak Y. , Levin S. , Vogel R. , Cohen I. J. , Wallach B. , Nir E. , Zaizov R.
Published: February 2007

Peripheral blood and bone marrow cells from three children with the juvenile (Ph1 negative) type of chronic granulocytic leukemia and from one with the adult (Ph1 positive) type were cultured in soft agar, and their specific growth patterns were evaluated. Greatly increased numbers of colonies were obtained in all cases, particularly from peripheral blood cells. By morphologic, cytochemical and ultrastructural criteria, colonies from one juvenile type and from the single adult type patients were found to be almost exclusively granulocytic, whereas in the other two juvenile type leukemia patients colonies were either granulocytic or macrophage. Moreover, both growth patterns were obtained in the same patients on different occasions. It appears that the leukemic cell populations of the juvenile and the adult forms of chronic granulocytic leukemia do not arise from different cell lines. Rather, both are the progeny of the common monocyte‐granulocyte progenitor cell, whose abnormal proliferation and differentiation along either the granulocytic or the monocytic pathway is probably directed by fluctuations in humoral and/or microenvironmental factors.
Human platelet factor 4: Preparation from outdated platelet concentrates and application in cerebral vascular disease
Authors: Levine Peter H. , Fisher Marc , Fullerton Albert L. , Duffy Celeste P. , Hoogasian James J.
Published: February 2007

Platelet factor 4 (PF4), the platelet antiheparin protein, was isolated from both the supernatant and the cells of recently outdated platelet concentrates. Following purification by affinity chromatography, a competitive binding radioimmunoassay was developed to detect this protein in human plasma. The normal range was determined to be 9.4 ± 4.7 ng/ml (mean ± SD for 52 healthy adults). In order to determine whether individuals with transient ischemic attack (TIA) or stroke had measurable increments of PF4 in their plasma, radioimmunoassay studies were performed on 11 patients with well‐documented TIA, 10 patients with well‐documented stroke and on 16 age‐matched controls hospitalized on a neurology service with disorders unrelated to arterial thrombosis. The 16 hospitalized controls had PF4 levels of 10.3 ± 9.1 ng/ml, a value not significantly different from the 52 normals (P > 0.50). Patients with TIA had PF4 levels of 24.6 ± 12.1 ng/ml, a value significantly higher than both the 52 normals (P < 0.001) and the 16 hospitalized control patients (P < 0.005). Patients with stroke had PF4 levels of 35.4 ± 29.2 ng/ml, a value significantly higher than both the 52 normals (P < 0.001) and the 16 hospitalized control patients (P < 0.005).

Outdated platelet concentrates facilitate the development of a reproducible radioimmunoassay for PF4. The elevation of this platelet‐derived protein in the plasma of patients with stroke and TIA provides evidence for recent or ongoing platelet activation in the cerebral vascular disease population.
Thrombotic thrombocytopenic purpura: Prolonged coma with recovery of neurologic function with intensive plasma exchange
Authors: Frankel Arthur E. , Rubenstein Martin D. , Wall Robert T.
Published: February 2007

A 45‐year‐old‐woman was presented with fever, microangiopathic hemolytic anemia, thrombocytopenia, purpura, and mental status changes. She was diagnosed as having thrombotic thrombocytopenic purpura (TPP). She was treated with daily plasma exchange and antiplatelet drugs, steroids, and vincristine. This patient had a remarkable course with 18 days of coma on full therapy followed by essentially complete recovery coincident with an increase of the plasma exchange dose to two plasma volumes processed per procedure. The patient has remained well with discontinuation of plasma exchange. We conclude that prolonged coma without evidence of major central nervous system structural lesions in TTP should be treated vigorously and continuously with plasma exchange.
Acute lymphoblastic leukemia – hand mirror variant – viral immune interrelationship as demonstrated by ultrastructural studies
Authors: Schumacher Harold R. , Thomas William J. , Strong Michael , Creegan William J. , Rowden Geoffrey , Phillips Terry M. , More Newton S. , Perlin Elliott , Stass Sanford A.
Published: February 2007

Acute lymphoblastic leukemia – hand mirror variant – was extensively restudied in a 22‐year‐old white female who survived for 22 months without therapy. Immune complexes to the baboon endogenous virus (BaEV) were found in the bone marrow plasma of the relapse specimen in 1977, but not in the bone marrow plasma from the terminal state in 1979. Immunoperoxidasetagged IgM antibody prepared from the patient's bone marrow plasma revealed BaEV antigen on the tip of the uropod of the HMC at the time immune complexes were found in the marrow. Absence of immune complexes in the bone marrow in the terminal state suggested a failure of the patient's immune surveillance system and/or possible immune suppression by chemotherapy.
Treatment of graft‐versus‐host disease in human allogeneic marrow graft recipients: A randomized trial comparing antithymocyte globulin and corticosteroids
Authors: Doney K. C. , Weiden P. L. , Storb R. , Thomas E. D.
Published: February 2007

This prospective randomized trial compared the effect of antihuman thymocyte globulin (ATG) versus corticosteroids as treatment of graft‐versus‐host disease (GVHD) in recipients of HLA‐identical allogeneic bone marrow transplants. Patients undergoing transplantation as therapy for either hematologic malignancies or aplastic anemia were given methotrexate as postgrafting immunosuppression. Patients who nevertheless developed acute GVHD of moderate severity were randomized to receive either corticosteroid therapy or ATG therapy.

Thirty‐seven patients were randomized: 20 patients received corticosteroids, and 17 received ATG. Both ATG and corticosteroids were in general well tolerated, although all patients receiving ATG developed fever and chills. Both treatment modalities were associated with a mild decrease in severity of GVHD after therapy. There was, however, no significant difference between treatment groups, whether assessed by improvement in specific organ involvement, improvement in the overall grade of GVHD, need for additional therapy for acute GVHD, or the proportion of patients who developed chronic GVHD. Infectious complications and survival were also not different between treatment groups. Thus, corticosteroids were as effective as ATG for the treatment of acute GVHD in recipients of HLA‐identical marrow transplants and, therefore, appear to be a reasonable choice as primary therapy for acute GVHD.
Activation and partial characterization of a human reticulocyte heme‐dependent elF‐Z α kinase
Authors: Franco R. S. , Hogg J. W. , Martelo O. J.
Published: February 2007

An inhibitor of globin translation initiation that is activated under heme‐deficient conditions and whose activity is associated with phosphorylation of the 38,000 dalton (α) subunit of the initiation factor eIF‐2 has been previously described in rabbit reticulocytes. We describe here an analogous enzyme in human reticulocytes. The human enzyme can be activated in intact reticulocytes under conditions of heme synthesis inhibition or by incubation of lysates in the absence of hemin. Addition of hemin to lysates prevents activation of the eIF‐2 a kinase. The partially purified kinase is associated with inhibition of globin synthesis in a rabbit cell‐free system and phosphorylates the small subunit of highly purified rabbit eIF‐2. We conclude that human reticulocytes contain a protein kinase that is analogous to the rabbit HCR and that may play a role in clinical heme deficiency states.
Evidence that platelet buoyant density, but not size, correlates with platelet age in man
Authors: Mezzano Diego , Hwang Kai‐Lun , Catalano Patricia , Aster Richard H.
Published: February 2007

Following infusion of 51Cr‐labeled autologous platelets into normal subjects, high‐density (HD) and low‐density (LD) platelet cohorts were isolated by prolonged centrifugation in isosmotic arabino‐galactan (Stractan). Specific radioactivity of LD platelets declined rapidly post‐infusion (T1/2 = 1.5 days), but specific radioactivity of HD platelets remained constant or increased over a 3–4‐day period and gradually declined for 6–7 days thereafter. These differences were exaggerated when platelet cohorts enriched in LD or HD cells by slow centrifugation in high‐density albumin were labeled and transfused. Mean survival of a platelet cohort enriched with HD cells was significantly (P < 0.02) shorter (7.73 days) than that of a cohort enriched with LD cells (9.33 days). In normal subjects treated with aspirin, capacity for thromboxane synthesis was regained more rapidly (P < 0.05) in LD than in HD platelets. HD and LD platelets differed only slightly in mean volume (HD platelets = 7.57 μ3, LD platelets = 6.87 μ3, 0.05 < P < 0.01).

We believe the most logical interpretation of these findings is that under normal conditions in man, newly formed platelets are less dense on the average than total platelets and become more dense as they age in the circulation. Thus, specific radioactivity of LD platelets declines rapidly as these platelets move into a more dense compartment and are replaced by newly formed, un‐labelled cells; specific radioactivity of HD platelets remains constant or increases as labelled platelets enter this compartment in numbers equal to or greater than the number leaving it at the end of their life span. The similarity in mean volumes of LD and HD platelets suggests that platelet size is unrelated to platelet age under normal conditions.
Human lgG antigranulocyte antibodies: Comparison of detection by quantitative antiglobulin consumption and by binding of 125I staph protein A
Authors: Blumfelder Theresa , Logue Gerald
Published: February 2007

The amount of IgG in the serum of patients with suspected immune neutropenia that binds to normal paraformaldehyde‐fixed human granulocytes was measured simultaneously by a quantitative antiglobulin consumption assay and by binding of 125I‐staphylococcal protein A (SPA). There was a significant linear relationship between the results of these two assays for the sera of 42 different patients. However, SPA binding appeared more sensitive than the quantitative antiglobulin assay for determining IgG antigranulocyte antibodies in serum. In a patient with Felty's syndrome who underwent splenectomy, the results of both assays on sequential serum samples correlated with clinical improvement. Thus, SPA binding appears to be a sensitive and reliable technique for measuring antigranulocyte antibodies, and there is a close correlation between antibody measured by antiglobulin consumption and those detected by SPA binding.
Bone marrow transplantation followed by normal pregnancy
Authors: Jacobs Peter , Dubovsky D. W.
Published: February 2007

A full‐term normal male child was delivered using forceps fifteen months after successful allogeneic bone marrow transplantation in a woman who presented with severe acute aplastic anemia. The uneventful course of this pregnancy demonstrates that the high‐dose cylcophosphamide administration used to prepare the patient for the procedure need not irreversibly suppress ovarian or endocrine function. This observation has relevance for the increasing numbers of young patients with aplastic anemia or acute leukemia undergoing bone marrow transplantation.
Prothrombin gainesville. A dysprothrombinemia in a pair of identical twins
Authors: Smith Laura G. , Coone Leah A. H. , Kitchens Craig S.
Published: February 2007

A pair of identical twins was evaluated for menometrorrhagia and mild post‐operative bleeding. Each had a slightly prolonged partial thromboplastin time and prothrombin time. Full hemostatic evaluation was normal, except that plasma prothrombin activity was 23% and 25% of normal with a Russell's viper venom assay. Each twin had 70% of normal plasma prothrombin antigen. Prothrombin kinetic data were obtained before and during hysterectomy. In these twins, infused prothrombin (Konyne®) was found to have a volume of distribution of approximately one and one‐half times the plasma volume and a half‐life of 34 hours. The previously reported dysprothrombins are briefly reviewed. Prothrombin Gainesville is the twelfth such disorder reported.
Evaluation of the platelet storage pool deficiency in the feline counterpart of the chediak‐higashi syndrome
Authors: Meyers K. M. , Seachord C. L. , Holmsen H. , Prieur D. J.
Published: February 2007

Cats with the Chediak‐Higashi (CH) syndrome have abnormal hemostasis with prolonged bleeding times and normal coagulation times. Platelet aggregation induced by serotonin, ADP, and collagen was impaired. Platelets from normal and CH cats were incubated with 14C‐adenine and then gel‐filtered. Gel‐filtered platelets (GFP) from CH cats contained 63% of the ATP, 38% of the ADP, 100% of the Ca2+, and 75% of the Mg25 of normal platelets. Serotonin could not be detected in CH platelets. Acid hydrolase and total platelet protein of CH platelets was similar to normal platelets. Gel‐filtered platelets were treated with thrombin to induce maximal secretion. Secretion of ATP, Ca2+, and Mg2+ was 1.9%, 12.4%, and 16% respectively of normal platelets. ADP secretion by CH platelets was not detectable. The ATP/ADP ratio in the 14C‐labeled metabolic pool of normal platelets was similar to that of total measured nucleotide pool of CH platelets. These findings suggest that in feline CH platelets, as in platelets from CH mink and cattle, there is storage pool deficiency that is virtually complete, and the virtual absence of ADP and 5HT may in part account for the abnormal hemostasis.

Aggregation of platelets from CH cats was impaired, but these platelets did aggregate to arachidonate, serotonin‐induced biphasic aggregation, and the aggregation response to ADP and collagen varied according to the amount of serotonin‐induced TxB2 formed. These findings support a major role for arachidonate in platelet activation.
Extramedullary blast transformation in the central nervous system in idiopathic myelofibrosis
Authors: Barnes Harry M. , Prchal Josef T. , Scott C. W.
Published: February 2007

Idiopathic myelofibrosis (IM) is a myeloproliferative syndrome characterized by anemia, fibrosis of the bone marrow, variable proliferation of megakaryocytes, myelocytic and erythrocytic precursors, and splenomegaly. Extramedullary hematopoiesis can develop in diverse areas. Unlike in chronic granulocytic leukemia, transformation to acute leukemia is uncommon in IM. Transformation to acute leukemia is usually accompanied by the findings of progressive increase in blasts in peripheral blood or bone marrow [1]. We have recently treated a patient with an unusual transition from IM to acute leukemia in whom leukemic transformation began in the central nervous system.
Alternative pathway of complement in multiple myeloma
Authors: Kraut Eric H. , Sagone Arthur L.
Published: February 2007

The alternative pathway of complement was studied in 16 newly diagnosed and 2 previously treated patients with multiple myeloma. Functional activity of this pathway was abnormal in 10 of these 18 patients (55%). These abnormalities correlated with depressed levels of alternative pathway components in 3 of 10 patients, but in the remaining 7, no etiology was demonstrated. There was an inverse correlation between activity and concentration of the monoclonal protein. This defect is an additional factor that may predispose patients with myeloma to overwhelming infection.
Differing platelet aggregating effects by two tumor cell lines: Absence of role for platelet‐derived ADP
Authors: Bastida Eva , Ordinas Antonio , Jamieson G. A.
Published: February 2007

Two different mechanisms of aggregation of heparinized human platelet‐rich plasma have been identified with two tumor cell lines: In neither case are these mechanisms dependent on platelet‐derived ADP. U87MG cells from a glioblastoma line of human origin caused a single irreversible wave of aggregation simultaneously with the onset of platelet secretion, and this was inhibited by heparin and hirudin but not by apyrase or phospholipase D. In contrast, Hut 20 cells from an undifferentiated tumor cell line of murine origin gave an initial reversible wave followed by a second irreversible wave, which then led to secretion. The first wave of platelet aggregation was unaffected by heparin or hirudin but was inhibited by apyrase, and the second wave was inhibited by phospholipase D. Citrate caused irreversible inhibition with either cell line, and aggregation did not occur with gel filtered platelets. These results suggest that platelet aggregation by the Hut 20 line is initially dependent on ADP released from the tumor cells, whereas aggregation induced by the U87MG line is dependent on a procoagulant activity of the tumor cell surface.
Identification and quantitation of embryonic and three types of fetal hemoglobin produced on induction of the human pluripotent leukemia cell line K‐562 with hemin
Authors: Fuhr J. E. , Bamberger E. , Lozzio C. B. , Lozzio B. B. , Felice A. E. , Altay G. , Webber B. B. , Reese A. L. , Mayson S. M. , Huisman T. H. J.
Published: February 2007

The hemoglobins synthesized by the pluripotent K‐562 leukemia cell line of human origin after induction with hemin have been isolated by DEAE‐cellulose chromatography and characterized by electrophoresis, high pressure liquid chromatography, and a radio‐immunological assay. Six hemoglobin zones have been observed with the following likely compositions. Zone 1: α2ϵ2, or Hb Gower‐2; zone 2: ζ 2ϵ2, or Hb Gower‐1; zone 3: ζ2γ2, or Hb Portland‐I; zone 4: Hb F, or α2γ2; zone 5: a mixture of acetylated Hb Portland‐I and Hb F; zone 6: Hb Bart's, or γ4. The embryonic Hbs (zones 1, 2, and 3) constituted 50%–75% of the total Hb present; the quantities varied from one experiment to the other. Both Hb Gower‐1 and Hb Gower‐2 were present. The γ chain was heterogeneous and contained the Gγ,AγI, and AγT types in a ratio of about 4:2:1, indicating a heterozygosity for the Ile → Thr substitution at position γ75. The methodology used can be applied for additional studies evaluating quantitative changes in Hb types due to in vitro manipulations.
Immunologic evaluation of long‐term effects of childhood all chemotherapy: Analysis of in vitro NK‐ and K‐cell activities of peripheral blood lymphocytes
Authors: McGeorge M. B. , Russell E. C. , Mohanakumar T.
Published: February 2007

Twenty‐five children with acute lymphoblastic leukemia (ALL) were tested for natural killer (NK) and K‐cell activity in vitro using the nonsensitized myeloid/erythroid cell line K562 and the K562 sensitized with rabbit antithymocyte globulin, respectively. The patients consisted of two groups: 1) 13 patients in continuous first remission undergoing maintenance chemotherapy and 2) 12 patients in remission for at least five years in whom chemotherapy had been discontinued at least six months before this study. The first group consistently demonstrated a marked depression in their NK activity and antibody‐dependent cell‐mediated cytotoxicity (K‐cell activity), as compared with normal controls. In contrast, normal levels of cytotoxicity were found in the second group of patients off of all chemotherapy. One patient studied while on chemotherapy and on two occasions following discontinuation of maintenance medications demonstrated that while NK and K‐cell activity was depressed during therapy, normal activity returned within days when immunosuppressive therapy was stopped. Thus, present modes of chemotherapy clearly had a profound effect on the in vitro NK and K‐cell activity; however, no long‐term effect on these functions was noted in our studies.
Acute monocytic leukemia: A surface marker study
Authors: Mirchandani Ila , Tabaczka Pamela , Palutke Margarita
Published: February 2007

Thirteen cases of acute monocytic leukemia (AMoL) were studied morphologically, cytochemically, and immunologically in an attempt to determine similarities and/or differences between acute monocytic and lymphocytic leukemias. Single class surface immunoglobulins (IgG kappa) were seen in six cases. These markers are thought to represent serum immunoglobulins attached to the leukemic monocytes via the receptors for the Fc portion of immunoglobulin and are not synthesized by the cells as is the case with B lymphocytes. Receptors for complement were also noted. It appears that surface immunoglobulins and receptors for the Fc portion of the immunoglobulin and complement, which are found on several classes of normal lymphocytes and monocytes, may also be found on leukemic monocytes. Antisera to B lymphocytes, detecting HLA‐DRw antigens, also reacted with some of the leukemic monocytes.
Agranulocytosis associated with chronic oral administration of cloxacillin for suppression of staphylococcal osteomyelitis
Authors: Shah I. , Kumar K. S. , Lerner A. M.
Published: February 2007

Oral cloxacillin was used for chronic suppression of a 59‐year‐old woman with staphylococcal osteomyelitis. She received 380.5 gm of cloxacillin over 263 consecutive days. Severe agranulocytosis followed, reverting rapidly to normal with cessation of drug. The absence of signs of an allergic reaction such as rash, fever, or eosinophilia suggests that cloxacillin, like other semisynthetic penicillins, may exert a direct bone marrow toxicity.
Severe fletcher factor (plasma prekallikrein) deficiency with partial deficiency of hageman factor (factor xii): Report of a case with observation on in vivo and in vitro leukocyte chemotaxis
Authors: Poon Man‐Chiu , Moore Michael R. , Castleberry Robert P. , Lurie Aubrey , Huang Shu Tsong , Lehmeyer Joyce
Published: February 2007

A case of cross‐reacting material‐negative Fletcher trait with additional partial deficiency of Hageman factor (HF, Factor XII) is described. Although the patient presented with a recent history of frequent epistaxis, he had no other personal or family history of a tendency toward bleeding or infection. Similar to other cases of Fletcher trait, his plasma showed a markedly prolonged partial thromboplastin time which could be corrected by prolonged incubation with the surface‐activator kaolin. Surface‐induced fibrinolysis, amidolysis of α‐N‐benzoyl‐proline‐L‐phenylalanine‐L‐arginine‐p‐nitro‐ anilide, and cold‐promoted enhancement of factor VII activity, reactions requiring the presence in the plasma of Fletcher factor (pre‐kallikrein), in addition to Hageman factor and Fitzgerald factor (high‐molecular weight kininogen), were also defective. In vivo chemotaxis of polymorphonuclear leukocytes and monocytes (Rebuck's skin window technique) in response to skin abrasions was defective, but was normal when diphtheria‐tetanus toxoid was also applied. In vitro leukocyte chemotaxis (Boyden chamber technique) in response to normal or patient's own serum activated with zymosan was normal. Together with previous observations that kallikrein generated chemotactic activity, possibly via activation of C5, the present observations suggest that prekallikrein activation may be important for in vivo leukocyte chemotactic response to skin abrasion. The inheritance of Fletcher trait in this patient is unclear. Although the father was an apparent heterozygote, the mother was completely normal for Fletcher factor procoagulant activity and antigen. The mild Hageman factor deficiency in the patient did not contribute significantly to the plasma defects described and was likely inherited from the father who had a low HF procoagulant activity.
Computed tomography in two cases of hemophilic pseudotumors
Authors: Kitchens Craig S.
Published: February 2007

Thigh swelling developed in each of two hemophiliacs, one having factor XIII deficiency and one having factor VIII deficiency. Computed tomography demonstrated the extent of the cystic and destructive nature of these pseudotumors and assisted in therapeutic formulation.
Diminished autologous mixed lymphocyte reaction in patients with hodgkin disease: Evidence for non‐t cell dysfunction
Authors: Zamkoff Kenneth W. , Dock Nancy L , Kurec Anthony S. , Davey Frederick R.
Published: February 2007

In the autologous mixed lymphocyte reaction (AMLR), T lymphocytes are stimulated to proliferate by autologous non‐T mononuclear cells. In five untreated patients with Hodgkin disease, the AMLR was diminished. In addition, in the same five patients, T cell response to PHA was inhibited by a cell in the non‐T cell fraction, the response of non‐T cells to PWM was diminished, and there was a diminished ability of the non‐T cell population to stimulate in allogeneic MLR. However, the response of T cells from patients with Hodgkin disease to allogeneic antigen was normal. The AMLR and allogeneic MLR were then studied in an additional five untreated patients before and after monocyte depletion of the stimulating non‐T mononuclear cell population. In this second group of Hodgkin disease patients, the AMLR was again diminished when T cells were incubated either with non‐T cells or non‐T cells depleted of monocytes. In the Hodgkin patients, monocyte depletion did not alter the T cell response in the AMLR. In the controls, monocyte depletion greatly diminished the proliferative response. The diminished AMLR in untreated Hodgkin disease patients may be the result of a failure of adequate monocyte stimulation of autologous T cells.
Detection of igg sensitization of red cells with 125I staphylococcal protein A
Authors: Yam Priscilla , Petz Lawrence D. , Spath Peter
Published: February 2007

Most cases of immune hemolytic anemia are associated with a positive direct antiglobulin test. However, in some cases, the antiglobulin test is not sensitive enough to detect low levels of red‐cell bound antibodies. This report describes a method using radiolabelled purified staphylococcal protein A which is capable of detecting IgG sensitization of red cells beyond the threshold of serologic techniques. It is less cumbersome than previously described methods and does not require antibody purification procedures. Its effectiveness was demonstrated for the detection of red‐cell alloantibodies and in evaluation of patients with acquired hemolytic anemias associated with a negative direct antiglobulin test.
Hemoglobin E diseases: Hematological, analytical, and biosynthetic studies in homozygotes and double heterozygotes for α‐thalassemia
Authors: Wong S. C. , Ali M. A. M.
Published: February 2007

Two families with Hb E diseases are described. In the Laotian family S, three homozygous Hb E were found. In the Vietnamese family H, double heterozygous Hb E‐α‐thalassemia‐2 and Hb E‐Hb H diseases were found. Anemia or hemolysis was absent in Hb E carriers, unless complicated by iron deficiency, the presence of severe α‐thalassemia gene (Hb H disease), or oxidative drug (paraaminosalicylic acid). Moreover, iron deficiency or concurrent α‐thalassemia genes resulted in a decreased amount of Hb E in its heterozygous carriers. Mild microcytosis and hypochromia were observed in Hb E heterozygotes, whereas the microcytosis and hypochromia were more pronounced in Hb E homozygotes. Globin chain synthesis studies yielded unbalanced α/non‐α ratios in both heterozygotes and homozygotes (average ratios were 1.13 and 1.56, respectively). The unbalanced biosynthetic ratios with microcytosis and hypochromia in Hb E carriers represented a β‐thalassemia phenotype, which could be a result of reduced synthesis of βE‐globin mRNA, as suggested by recent hybridization studies.
Clinical, hematological, and biochemical features of Hb SC disease
Authors: Ballas Samir K. , Lewis Cynthia N. , Noone Ann Marie , Krasnow Steven H. , Kamarulzaman Embi , Burka Edward R.
Published: February 2007

Twenty‐seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.