bluuebunny/crossref_metadata_2025 huggingface dataset:
Serum ferritin in hematologic malignancies
Authors: Matzner Y. , Konijn A. M. , Hershko C.
Published: February 2007

Serum ferritin was measured in a variety of hematologic malignancies at presentation, in remission following therapy, and in relapse. Ferritin was strikingly increased in all acute leukemias at presentation and in relapse, in the blastic crisis of CML, and in smouldering leukemia. Remission in both ALL and ANLL was associated with a reduction of serum ferritin, and this normalization was a function of remission duration. In the malignant lymphomas serum ferritin was related to tumor histology. Highest levels were found in Hodgkin disease and histiocytic lymphoma, normal levels in lymphocytic lymphoma, and intermediate levels in mixed histiocytic‐lymphocytic lymphoma. In all cases, remission was associated with normalization of serum ferritin. These correlations suggest that serum ferritin measurements may be of clinical usefulness in the initial evaluation and in the assessment of response to therapy in patients with acute leukemia and malignant lymphoma.
Hexokinase “New Hyde Park”: A low activity erythrocyte isozyme in a chinese kindred
Authors: Paglia Donald E. , Shende Ashok , Lanzkowsky Philip , Valentine William N.
Published: February 2007

Chronic hemolytic anemia in a 7‐year‐old Chinese boy was found to be associated with decreased activity of erythrocyte hexokinase (approximately 20% of appropriate control values). Alterations in kinetics, stability, pH optimum, or electrophoretic patterns could not be demonstrated with proband hexokinase, but subtle differences between maternal and paternal hexokinase characteristics suggested that two separate mutant isozymes were involved and that the proband was heterozygous for each. Comparisons with previously reported cases of hexokinase deficiency demonstrate the broad genetic heterogeneity that characterizes this disorder.
Effect of zinc supplementation on serum testosterone level in adult male sickle cell anemia subjects
Authors: Prasad Ananda S. , Abbasi Ali A. , Rabbani Parviz , Dumouchelle Elizabeth
Published: February 2007

Previously, we have documented primary testicular failure in adult male subjects with sickle cell anemia. We have also reported the occurrence of zinc deficiency and suggested that androgen deficiency may be related to zinc deficiency in such patients. In this study, we present data with respect to the effect of oral zinc supplementation on serum testosterone levels in adult male patients with sickle cell anemia. An increase in serum testosterone, neutrophil zinc, and neutrophil alkaline phosphatase activity was observed in the zinc‐supplemented group in comparison with the group on placebo. Additionally, body weight increased and serum lactic dehydrogenase activity decreased in response to zinc supplementation. We conclude that androgen deficiency in adult male subjects with sickle cell anemia is correctable with zinc supplementation and that the determination of neutrophil zinc and alkaline phosphatase activity in the neutrophils may be utilized as good indicators of body zinc status in such subjects.
The aggregation of human platelets by ascitic fluid: A possible mechanism for disseminated intravascular coagulation complicating LeVeen shunts
Authors: Salem Hatem H. , Koutts Jerry , Handley Chris , Van Der Weyden Martin B. , Dudley Frank J. , Firkin Barry G.
Published: February 2007

To identifiy causative factors responsible for the disseminated intravascular coagulation complicating peritoneovenous (LeVeen) shunts, the ascitic fluid from 12 patients with alcoholic liver disease or peritoneal malignancy was examined for its effects on human platelets. In all patients, concentrated ascitic fluid caused irreversible platelet aggregation. Properties of the aggregating factor suggested that it is collagen, and subsequently, the presence of collagen in ascitic fluid was confirmed.

This finding, together with the known effects of collagen on platelets and contact clotting factors, would be sufficient to explain the development of disseminated intravascular coagulation following this procedure. Aspirin by inhibiting collagen‐induced aggregation may have a therapeutic role in the management of this problem.
Hematopoiesis in the human spleen
Authors: Freedman Melvin H. , Saunders E. Fred
Published: February 2007

To assess the capacity of the human spleen to function as a hematopoietic organ, spleen mononuclear cells from patients undergoing splenectomy were cultured to assay CFU‐E, BFU‐E, and CFU‐C. Although the peripheral blood from cases of hereditary spherocytosis, Gaucher disease, Thalassemia major, and idiopathic thrombocytopenic purpura yielded 3–20 BFU‐E/5 × 105 cells plated and 1–7 CFU‐C/5 × 105, suspensions of spleen cells failed to grow colonies. Histologically these spleens did not show extramedullary hematopoiesis. In contrast, peripheral blood from a patient with osteopetrosis (marble bone disease) yielded 130 BFU‐E/5 × 105, 40 CFU‐E/5 × 105, and 9 CFU‐C/5 × 105. The spleen had extensive extramedullary hematopoiesis on microscopy and grew 180 BFU‐E/105, 210 CFU‐E/105, and 25 CFU‐C/105. We conclude that spleens from patients without extramedullary hematopoiesis do not contain committed hematopoietic progenitors in spite of normal precursors in the blood. In osteopetrosis the spleen contained stem cells in a concentration comparable to bone marrow, and the embryonic role of the spleen in blood formation appeared to continue in postnatal life.
The myeloproliferative reaction in a child with down syndrome: Cytological and chromosomal evidence for a transient leukemia
Authors: Lazarus Kenneth H. , Heerema Nyla A. , Palmer Catherine G. , Baehner Robert L.
Published: February 2007

Infants with Down syndrome occasionally develop marked myeloproliferative reactions (MPR) indistinguishable from acute myelocytic leukemia but which spontaneously regress. Most authors have attributed MPR to immature or defective control on bone marrow development. In this report serial cytomorphologic and chromosomal findings in the bone marrow and peripheral blood of a child with the MPR suggests that MPR may be due to a spontaneously regressing clone of malignant cells. At the height of the myeloproliferative response, chromosomal studies demonstrated a clone of blast‐like cells with a 21‐22 translocation consistent with Down Syndrome and an X‐8 translocation which was present only in the initial peripheral blood analysis harvested at 24 hours but was not present in the 72 hours samples. This X‐8 translocation was not demonstrated in bone marrow or peripheral blood samples obtained at random times throughout the one year followup. During this period there was complete resolution of the MPR. In addition the chromosomes from one cell demonstrated several double minutes at the time of the MPR. Double minutes have previously been reported only in malignant diseases, especially tumors of neural origin and leukemia. These findings raise questions about the benign origin of the MPR and support the idea the MPR may be the result of a spontaneously regressing clone of malignant cells.
Coexistence of paroxysmal nocturnal hemoglobinuria and chronic lymphocytic leukemia
Authors: Omura George A.
Published: February 2007

A man with paroxysmal nocturnal hemoglobinuria and a lymphoproliferative disorder is described. The latter condition evolved into a typical picture of chronic lymphocytic leukemia. The significance of this association is obscure, but treatment of one or the other disease was clearly not an etiologic factor in this concurrence.
Surface immunoglobulins on hairy cells of 55 patients with hairy cell leukemia
Authors: Golomb Harvey M. , Davis Susan , Wilson Carol , Vardiman James
Published: February 2007

Surface immunoglobulins (SIg) were determined on peripheral blood samples from 55 patients with hairy cell leukemia (HCL) and on hairy cells from spleen preparations of 14 of these 55 patients. The patterns of SIg for HCL was compared to the patterns on peripheral blood leukemic cells from 39 patients with chronic lymphocytic leukemia (CLL) and 15 patients with poorly differentiated lymphocytic (PDL) lymphoma. Of the 55 HCL patients, 42 could be scored for individual heavy and light chains; 16 had only IgG, 14 had two or three heavy chains, 7 had only IgD, and 5 cases had no SIg and were E‐rosette negative. This pattern was different from the B‐cell pattern in CLL and PDL where there were few cases of IgG alone (5%) and many cases of IgM alone (50%). Surface marker profile did not correlate with survival in any of the sub‐groups tested. HCL appears to be a B‐cell lymphoproliferative disease in greater than 90% of cases; many combinations of heavy chains with only a single light chain can be demonstrated.
Increased adsorption of cytoplasmic proteins to the erythrocyte membrane in ATP‐depleted normal and pyruvate kinase‐deficient mature cells and reticulocytes
Authors: Allen David W. , Groat Jerome D. , Finkel Barbara , Rank Brian H. , Wood Patricia A. , Eaton John W.
Published: February 2007

How the metabolic defect of pyruvate kinase deficiency (PK(–)) accelerates red blood cell (RBC) destruction is not established, but may be related to RBC membrane abnormalities associated with altered cellular metabolism. Furthermore, it has been shown that PK(–) reticulocytes are especially sensitive to metabolic depletion. Therefore, we compared the membranes of reticulocyte‐rich PK(–) RBC, both fresh and ATP depleted, with membranes of fresh and ATP depleted normal mature RBC and reticulocytes. There was no difference between the specific gravity (SG) of the membranes of normal mature RBC (SG 1.152 ± 0.004) and membranes of reticulocyte‐rich RBC from several anemias (SG 1.150 ± 0.002). However, membranes from fresh, reticulocyte‐rich PK(–) RBC were dense with SG of 1.165 ± 0.004 which correlated with a corresponding increase of protein to lipid phosphorus ratio of 66 ± 8 μg protein/μg lipid phosphorus (normal 52 ± 6 μg/μg). The membrane density of PK(–) RBC was further increased when the PK(–) RBC ATP was depleted by anaerobic incubation (SG 1.188 ± 0.004) or cyanide inhibition (SG 1.182 ± 0.001). When ATP was depleted in normal RBC and in non‐PK(–) reticulocytes, corresponding increases in membrane SG occurred. A distinctive 50,000 MW peptide is adsorbed from the cytoplasm to the membranes of reticulocytes (both normal and PK(–) when these cells were depleted of ATP. The increased membrane adsorption of cytoplasmic proteins by PK(–) RBC was not associated with increased RBC calcium uptake, sulfhydryl oxidation, or altered membrane protein phosphorylation. All the observed abnormalities of PK(–) RBC membranes could by reproduced by ATP depletion of reticulocyte‐enriched non‐PK(–) RBC.
Monocytes with binding capacity to sheep red blood cells in a case of acute undifferentiated leukemia
Authors: Liso Vincenzo , Tursi Alfredo , Pavone Vincenzo , Specchia Giorgina , Loria Maria Paola , Bonomo Lorenzo
Published: February 2007

Monocytes with binding capacity to sheep red blood cells (SRBC) are described in a case of acute undifferentiated leukemia with blast cells showing no cytochemical or immunological markers. The monocyte nature of the rosetting cells was confirmed by intense reactions of naphthol AS esterase and α‐naphthyl esterase and sometimes by peroxidase positivity.
Severe thrombocytopenia following oral cholecystography with iocetamic acid
Authors: Insausti Carmen L. G. , Lechin Fuad , van der Dijs Bertha
Published: February 2007

We report the first case of severe thrombocytopenia occurring after ingestion of a widely used cholecystographic medium, iocetamic acid. The patient had not been given any treatment before he received the gallbladder contrast medium. Onset of symptoms was acute, and the clinical course was benign with complete recovery after one week. Bone marrow aspiration showed increased numbers of megakaryocytes, suggesting that platelets were rapidly removed from circulation. In vitro tests for antiplatelet antibodies were not performed because iocetamic acid is insoluble in aqueous solutions. Since only a few cases of thrombocytopenia following ingestion of iodine‐compounds have been reported, it seems highly unlikely that purpura will be, in the future, a major hazard in cholecystography. However, it would be wise to question patients as to any prior sensitivity to iodine before proceeding with oral cholecystography.
Unique sensitivity of Hb Zürich to oxidative injury by phenazopyridine: Reversal of the effects by elevating carboxyhemoglobin levels in vivo and in vitro
Authors: Virshup David M. , Zinkham William H. , Sirota Ronald L. , Caughey Winslow S.
Published: February 2007

Phenazopyridirie (PAP) causes a hemolytic anemia in normal individuals who receive an overdose or in patients with decreased renal function given therapeutic doses. There are no reports of PAP‐induced hemolysis in individuals with unstable hemoglobins. Therapeutic doses of PAP administered to a subject with Hb Zürich (His E7 (63) β→ Arg) caused a severe hemolytic anemia with many large Heinz bodies appearing in the red cells.

Incubation of whole blood from three asymptomatic Hb Zürich subjects with PAP at a molar ratio of PAP/Hb of 1.3/1 produced a moderate to marked increase in methemoglobin (MHb) and Heinz body formation accompanied by a slight to moderate decrease in levels of reduced glutathione. The rates of MHb formation were proportional to the concentration of PAP. In two of the subjects the rates of formation of Heinz bodies and MHb were substantially higher than in the third subject. Red cells from five normal adults were not affected. In Hb Zürich red cells the affinity of the abnormal β chain for carbon monoxide is much greater than that of the normal α and β chains. The two subjects with higher rates of MHb and Heinz body formation were nonsmokers with in vivo carboxyhemoglobin (HbCO) levels of 4–6%. The third subject with low rates of MHb and Heinz body formation was a smoker with in vivo HbCO levels of 15–18%. Increasing levels of HbCO from 8.2% to 14.3% by the in vitro addition of CO caused a marked reduction in the rate of Heinz body formation and a moderate decrease in MHb formation. Rates of MHb formation similar to those observed in normal red cells occurred at HbCO percentages of 89.2 and 99.2. Red cells containing Hb Zürich are extremely sensitive to oxidative injury by PAP, either in vivo or in vitro. The degree of oxidative injury diminishes as the level of HbCO increases, a phenomenon that is enhanced by preferential binding of CO to the abnormal β subunit of Hb Zürich.
Glycophorin a expression in malignant hematopoiesis
Authors: Liszka Kristof , Majdic Otto , Bettelheim Peter , Knapp Walter
Published: February 2007

Two hundred twenty‐nine patients with hematopoietic malignancies were tested for reactivity with a monoclonal anti‐human glycophorin A antibody. One hundred twenty‐three of these cases were classified as acute leukemias of either the myeloid, lymphoid, erythroid, or undifferentiated type. The monoclonal antibody we used (VIE‐G4) was obtained after immunization with a human thymocyte suspension. It selectively reacts with glycophorin A (GpA) and strongly binds to 40% of K‐562 cells and all morphologically recognizable erythroid precursor cells. Apart from two cases with acute erythroid leukemia, this antibody reacted with none of the malignant cells in the 229 tested hematopoietic malignancies, including the 121 nonerythroid acute leukemias. This finding seems to contradict the earlier observations by L. Andersson and colleagues that a considerable proportion of acute leukemias express GpA on their surface. One reason for this discrepancy might be the fact that VIE‐G4 detects only complete glycosylated GpA. If this is the sole explanation, this would mean that the poorly differentiated cells in these cases express incompletely glycosylated GpA.
High‐dose cytosine arabinoside for acute nonlymphocytic leukemia
Authors: Cantin Guy , Brennan James K.
Published: February 2007

Eighteen patients with acute nonlymphocytic leukemia (ANLL), aged 17–73 years, were treated with high‐dose cytosine arabinoside (HD‐Ara‐C)) using 3 g/m2 IV q 12 hours × 12 doses. Seven patients were treated for relapse and four (57%) obtained a complete remission with a median duration of 19.5 weeks. In nine patients, refractory to conventional chemotherapy, no complete responders were observed. Treatment failure was most commonly due to drug resistance. Two elderly patients with ANLL not previously exposed to chemotherapy died during the initial induction. Recent data on the HD‐Ara‐C experience in ANLL are presented and compared with this study.
Juvenile features in adult‐type chronic granulocytic leukemia
Authors: Gay James C. , Dessypris Emmanuel N. , Roloff James S. , Lukens John N.
Published: February 2007

Philadelphia chromosome (Ph1) positive, or “adult‐type,” chronic granulocytic leukemia (CGL) differs in many clinical and laboratory aspects from PH1‐negative, or “juvenile,” CGL. Because of these differences, the disorders have been considered two distinct disease entities. We present a child with Ph1‐positive CGL whose disease has several features more characteristic of the Ph1‐negative syndrome. This case demonstrates that the chromosomal anomaly in CGL is not always predictive of phenotypic expression in this disease.
In adult acute nonlymphoblastic leukaemia extended maintenance chemotherapy has no benefit
Authors: Jacobs Peter , Dubovsky D. W. , Wood Lucille
Published: February 2007

Fifty‐two consecutive and previously untreated adults with nonlymphoblastic leukaemia underwent remission induction chemotherapy with a combination of the epipodophyllotoxin VP16‐213, cytosine arabinoside, and doxorubicin (Adriamycin). Complete remission was achieved in 23 of the 52 patients (44%) by means of a single course of therapy in 20 individuals, two courses in two, and three courses in one; median duration of complete remission was 48 weeks. Failure to achieve remission status was due to primary drug resistance in 13 patients (25%), and adequate trial of therapy was not possible in 16 patients (31%) owing to their late referral and accounts for the low remission rate. Individuals achieving complete remission were randomly assigned to receive either 6 (n = 8) or 15 months (n = 13) of maintenance therapy; respective median survival was 95 and 78 weeks (P > 0.10). These data confirm previously reported results for complete remission induction with this three‐drug combination and fail to show any difference between short (6 months) and long (15 months) maintenance chemotherapy.
Variations in the hereditary patterns of factor VIII complex in a large kindred: Coexistence of hemophilia a with von willebrand
Authors: Tavori S. , Tatarsky I.
Published: February 2007

Coagulation studies and clinical observations were carried out in 47 members belonging to three generations in one kindred. Classical Hemophilia A, Von Willebrand (VWD) variants, and normal individuals were revealed in this study.

The coexistence of Hemophilia A and VWD in different siblings of the same progenitors indicates the difficulty to distinguish between these two major factor VIII abnormalities as two different traits.

A hypothesis based on these findings is elaborated.
Platelet factor 4 release during exercise in patients with coronary artery disease
Authors: Levine Shirley P. , Suarez Augustin J. , Sorenson Richard R. , Raymond Nancy M. , Knieriem Linda K.
Published: February 2007

Many recent studies provide evidence that increased platelet activation occurs in a significant number of patients with atherosclerotic coronary artery disease. The mechanisms responsible for this activation are unknown, although there have been studies suggesting a correlation with abnormal lipoproteinemia, acute myocardial infarction, unstable angina, and exercise‐induced myocardial ischemia. We studied 84 patients undergoing standardized treadmill exercise using either a Bruce [N = 63] or symptom‐limited Naughton protocol [N = 21]. In contrast to ten healthy volunteer subjects, the patient group demonstrated a significant increase in plasma concentrations of platelet factor 4 [PF4] between pre‐ and postexercise blood samples confirming earlier reports of exercise‐induced platelet activation and secretion. As with previous studies, however, only a subset of patients demonstrated this response. When the entire group was analyzed for the presence or absence of electrocardiographic ischemic changes and the presence of documented versus suspected coronary artery occlusions, there were no differences noted between groups that explained the variable responses measured. However, there was a significant difference between patient groups when analyzed by whether or not they were being treated with β‐blocking agents. Patients who were being treated with propranolol or one of the longer‐acting β‐blocking agents did not have a significant increase in plasma PF4 following exercise, in contrast to patients who were not β‐blocked. Plasma concentrations of epinephrine, norepinephrine, and lactic acid were measured in 49 patients and all normal subjects. There was no correlation between the changes in plasma PF4 concentrations and any of these three variables, suggesting that platelet activation was not occurring through direct platelet activation by circulating catecholamines. This study provides further evidence that there is a subset of CAD patients with platelet hyperactivity. This is the first time that β‐blockade has been demonstrated to modify this platelet response. The effectiveness of β‐blocking agents in CAD may be in part related to their antiplatelet effect.
Kinetics of indium‐111‐labeled leukemic cells in patients with acute nonlymphocytic leukemia
Authors: Yamauchi Kunihiko , Suzuki Yutaka , Sugihara Masami , Nagao Tadami , Arimori Shigeru
Published: February 2007

The distribution within the body of autologous leukemic cells labeled with indium‐111 oxine was studied in seven patients with acute nonlymphocytic leukemia. The leukemic blood cells initially entered the spleen and liver, and the major site of localization was the former rather than the latter. The majority of the leukemic cells had not left the spleen and liver within 48 hr. Liver radioactivity fell transitorily up to the third hr after the initial rise. The clearance curve of radioactivity from the blood showed a plateau or the appearance of a “hump” from 1 to 5 hr after injection of labeled leukemic cells. These results might reflect recirculation of a portion of the leukemic cells between these organs and the bloodstream. In a patient with acute monoblastic leukemia, OKMl monoclonal‐antibody‐treated monoblasts showed the lowest recovery into the blood and a greater increase of liver than splenic radioactivity at 30 min after injection. These results suggest the removal of damaged cells by the cytotoxic effects of antibody mediated by reticuloendothelial clearance mainly of the liver and others. In one patient with acute promyelocytic leukemia, leukemic cells accumulated in both kidneys, indicating the possible infiltration of these cells. Since indium‐111 oxine stays firmly attached to the cells in spite of the possibility of radiation damaged in a long‐term survey, it seems an ideal label for studying leukemic cell kinetics.
Burkitt cell leukemia following treatment of Hodgkin's disease: Report of a case and review of the literature
Authors: Krikler S. H. , Bain G. O. , Gordon P. A.
Published: February 2007

The development of Burkitt cell leukemia (FABL3) [1] in a patient 7 years after successful treatment of Hodgkin's disease (HD) by combined modality (chemotherapy and radiotherapy) therapy is described. The B‐cell nature of the leukemic blasts is shown by immunologic surface marker studies. While L3 leukemia is very rarely seen following therapy for HD, diffuse lymphoma of small noncleaved cells (LSNC)— resembling the Burkitt tumor—is more common in this setting.
An analysis of the mechanism by which cetiedil inhibits the gardos phenomenon
Authors: Berkowitz Lee R. , Orringer Eugene P.
Published: February 2007

Energy depletion in the human erythrocyte causes a rise in intracellular calcium. This in turn accelerates the transmembrane movement of potassium and chloride, resulting in cell dehydration. This process, known as the Gardos phenomenon, is inhibited by cetiedil. The present study examines the mechanism by which cetiedil inhibits the Gardos phenomenon. The ability of cetiedil to retard the initial step in the Gardos phenomenon, a rise in intracellular calcium, was first tested. Cetiedil did not prevent calcium accumulation. Cetiedil's ability to inhibit anion movement was next evaluated, as cetiedil could appear to be blocking K movement when in fact it was preventing the movement of its accompanying anion. No inhibitory effect on anion movement was seen. Since cetiedil prevented neither calcium accumulation nor anion movement, it must inhibit the Gardos phenomenon by preventing the opening of the K‐specific gate in the erythrocyte membrane. The fact that cetiedil's effect on the Gardos phenomenon could not be removed with repeated cell washing indicates that this effect is irreversible.
Selective loss of the expression of OKT‐4 or leu‐3A defined antigen by 12‐0‐tetradecanoylphorbol‐13‐acetate (TPA) in human lymphoid cells
Authors: Tatsumi Eiji , Piontek Catherine , Sugimoto Tohru , Minato Keisuke , Minowada Jun
Published: February 2007

In ten human malignant T‐cell lines (HPB‐ALL, HPB‐MLT, CCRF‐CEM, RPMI‐8402, MOLT‐4, JM, P12/Ichikawa, TALL‐1, SKW‐3, HUT‐102), the expression of the antigen defined by OKT‐4 or Leu‐3A monoclonal antibody was remarkably decreased after a short incubation (1 hr) with 12‐0‐tetradecanoylphorbol‐13‐acetate (TPA) (1.6 nM). This phenomenon was also observed in all types of cells tested, including two lymphoblastoid cell lines with B‐cell properties (RPMI‐6410 and RPMI‐8392), thumus cells from three children, peripheral blood lymphocytes from four normal individuals, and fresh neoplastic T cells from a case of T‐cell acute lympho‐blastic leukemia (ALL) and a case of T‐cell lymphoma. The presence of puromycin (10 μM), cycloheximide (35 μM), or retinoic acid (1.0 μM), had no effect on the TPA‐induced loss of OKT‐4 defined antigen. Incubation at 4°C or with lidocaine (4 mM) partially prevented this effect of TPA, and the presence of sodium azide (150 mM) did so totally. Recovery of antigen expression occurred 24 hours after cessation of TPA treatment. Comparative study using several phorbols other than TPA showed that this effect accorded with the tumor promotor activity of the compounds, but the phenomenon appears to be at the membrane level, not necessarily as a result of TPA‐induced cellular differentiation.
Comparison of the measurement of surface or total platelet‐associated IgG in the diagnosis of immune thrombocytopenia
Authors: Kelton John G. , Denomme Greg , Lucarelli Ann , Garvey Judy , Powers Peter , Carter Cedric
Published: February 2007

Platelet‐associated IgG (PAIgG) can be measured on intact platelets or following platelet lysis. Measurement of PAIgG following platelet lysis may provide different or additional information compared to PAIgG measured on intact platelets. The PAIgG of lysed platelets represents “total” PAIgG, ie, IgG on the surface of platelets plus any IgG that was inside the platelet. To investigate the clinical relevance of the two types of PAIgG assay we performed a prospective study on washed platelets collected from 47 patients with idiopathic thrombocytopenic purpura (ITP). The PAIgG was measured on intact and lysed platelets using an immunoradiometric assay. Platelet‐associated IgG was 2–3 times higher when measured on lysed platelets from healthy controls or patients with ITP compared to PAIgG measured on the same intact platelets. The higher level of PAIgG observed following platelet lysis was not due to the reactions not achieving equilibrium. Using lysed platelets, PAIgG was elevated on 29 of 47 samples from different ITP patients and elevated in 31 samples when measured on intact platelets.

The PAIgG is invariably higher when measured following platelet lysis compared measurements made on intact platelets. Neither technique offers a diagnostic advantage over the other.
Severe congenital neutropenia with unique features of dysgranulopoiesis
Authors: Lightsey Alton L. , Parmley Richard T. , Marsh William L. , Garg Aruna K. , Thomas William J. , Wolach Baruch , Boxer Laurence A.
Published: February 2007

Congenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3‐year‐old girl with neutropenia (<500/mm3) and recurrent severe infections in whom repeated marrow evaluations revealed large (30‐50 μm) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly. immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony‐forming cells, and normal colony‐stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B12, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously described cases.
Hairy‐cell leukemia associated with Hodgkin's disease: A case report
Authors: Vokes Everett E. , Bitter Mitchell A. , Prystowsky Michael B. , O'Reilly William , Golomb Harvey M.
Published: February 2007

A 56‐year‐old man who had easy bruising, an enlarged left supraclavicular lymph node, and splenomegaly was diagnosed as having hairy‐cell leukemia. Treatment consisted of splenectomy. Because of progressive lymphadenopathy in the following months, the patient required reevaluation. Examination of a lymph node biopsy specimen now revealed Hodgkin's disease, nodular sclerosis. This is the first pathologically confirmed case of hairy‐cell leukemia coexisting with Hodgkin's disease.
α Globin gene rearrangements in polynesians are not associated with malaria
Authors: Trent R. J. , Mickleson K. N. P. , Wilkinson T. , Yakas J. , Bluck R. , Dixon M. , Liley A. W. , Kronenberg H.
Published: February 2007

Using gene mapping, 16.3% of Polynesians were shown to have α thalassemia. These results are surprising since malaria is not found in Polynesia. Moreover, triplicated α gene rearrangements were identified in a further 7.7%, a frequency not seen in other populations.
Normal human blood density gradient lymphocyte subset analysis: I. An interlaboratory flow cytometric comparison of 85 normal adults
Authors: Marti G. E. , Magruder L. , Patrick K. , Vail M. , Schuette W. , Keller R. , Muirhead K. , Horan P. , Gralnick H. R.
Published: February 2007

An interlaboratory flow cytometric comparison of several commercially available human lymphocyte subset reagents was undertaken in three different laboratories. Fresh Hypaque‐Ficoll purified blood mononuclear cells were stained at 4°C or 22°C. Direct or indirect surface immunofluorescence was carried out at all sites using an EPICS V flow cytometer. Fullbright, 10‐μm fluorescent polystyrene microspheres were used for optical alignment and standardization. A log integral fluorescent histogram gated on forward and right angle scatter was collected on 1–2 × 104 cells for each reagent and the proportion, of positive cell determined for each reagent. With the exception of one reagent, anti‐B1, which showed an approximately twofold variation, all three laboratories showed remarkable agreement. Thus there was no significant difference noted for the following reagents: OKT4, CCT4, Leu 3a, Leu 2a, OKT8, or CCT8. We attribute these findings to the availability of quality reagents, precision instrumentation, and a standard lymphocyte preparation.
Prolonged survival in Richter syndrome with subsequent reemergence of CLL: A case report including serial cell‐surface phenotypic analysis
Authors: Baumann Michael A. , Libnoch Joseph A. , Patrick Catherine W. , Choi Hongyung , Keller Robert H.
Published: February 2007

The development of a large cell lymphoma in a patient with chronic lymphocytic leukemia (Richter syndrome) is associated with a poor prognosis. There is dispute regarding the clonal origin of the large cell component. We described a patient in whom prolonged remission of the large cell component was achieved with combination chemotherapy, followed by subsequent reemergence of the less aggressive CLL clone. Serial cell surface phenotypic data are presented suggesting origin of both histologies from a common B‐cell clone.
Dyskeratosis congenita with hypoplastic anemia: A stem cell defect
Authors: Friedland M. , Lutton J. D. , Spitzer R. , Levere R. D.
Published: February 2007

A case of dyskeratosis congenita (DC) with pancytopenia was studied in an attempt to elucidate the cause of the hypocellular bone marrow and pancytopenia. Levels of erythroid hemopoietic stem cells were estimated by clonal cell culture. Results showed a decrease in the number of precursor cells in both the bone marrow and the peripheral blood of this patient.
Concomitant inheritance of α‐thalassemia in β°‐thalassemia/hb e disease
Authors: Winichagoon Pranee , Fucharoen Suthat , Weatherall David , Wasi Prawase
Published: February 2007

Concomitant inheritance of α‐thalassemia in patients with β°‐thalassemia/hemoglobin (Hb) E disease was detected by restriction endonuclease DNA mapping. Among 42 patients with β°‐thalassemia/Hb E disease, seven were found to have an α‐thalassemia‐2 haplotype. Of these, five belonged to the rightward or 3.7‐kb type of α‐thalassemia‐2 and the remaining two the leftward or 4.2‐kb type. All the seven patients with α‐thalassemia‐2 haplotype had hemoglobin levels of 7.4 g/dl or above; those without detectable α‐thalassemia had hemoglobin levels both higher and lower than 7.4 g/dI. The latter attended the clinic regularly, the former did occasionally. These findings suggest that concomitant inheritance of α‐thalassemia can alleviate the severity of β°‐thalassemia/Hb E disease. Failure to find α‐thalassemia‐1 haplotype in these patients suggests that concomitant inheritance of α‐thalassemia‐1 with β°‐thalassemia/Hb E might lead to so mild a condition that the individuals do not present clinically. The fact that many patients without a detectable α‐thalassemia haplotype also had hemoglobin levels of 7.4 g/dl or higher suggests that there are additional factors responsible for the mildness of β°‐thalassemia/Hb E disease.
In vitro generation of procoagulant activity by leukemic promyelocytes in reponse to cytotoxic drugs
Authors: Fibach Eitan , Treves Aliza , Korenberg Avraham , Rachmilewitz Eliezer A.
Published: February 2007

Disseminated intravascular coagulation (DIC) is a frequent occurrence in acute promyelocytic leukemia (APL), especially after onset of chemotherapy. We have used a human promyelocytic leukemic established cell line (HL‐60) and various other human leukemic cells to investigate the effect of cytotoxic drugs on generation of procoagulant activity (PCA). The results indicate that, unlike normal human peripheral blood monocytes and certain other cell types where PCA induction requires active mRNA and protein synthesis, in HL‐60 cells, compounds such as actinomycin D, puromycin, and cytosine arabinoside and a variety of other cytotoxic agents, induced generation of a potent PCA.

Although different in its mechanism of induction, this HL‐60 cell PCA was similar, and may be identical, to mononuclear cell tissue factor. The PCA induction was rapid and preceded the lytic effect of the drugs. It was first detected on the outer cell surface but, following prolonged exposure to the drugs, upon lysis of the cells, it was also found in the extracellular medium.

This in vitro effect mimics the development of DIC in patients with APL. The system may, therefore, serve as a model for the study of the cellular and molecular events associated with PCA generation by malignant promyelocytes and DIC occurrence in patients with APL and other malignancies.
Chronic granulocytic leukemia: Correlation of elastic transformation type with karyotypic evolution
Authors: O'Malley F. M. , Garson O. Margaret
Published: February 2007

Over a 3‐year period, 26 patients with Philadelphia chromosome‐positive chronic granulocytic leukemia were studied cytogenetically in both the chronic and blastic transformation phases of the disease; a further three patients were studied only after blastic transformation. Sixteen were considered to have adequate evidence of the type of transformation and form the basis of this report, where chromosome changes have been correlated with the morphological type of blastic transformation.

Seven patients developed a myeloblastic transformation, seven a lymphoblastic transformation, and two an erythroblastic transformation. All patients in the myeloid group acquired one or more of the nonrandom changes associated with CGL blastic transformation, viz. +8,i(17q), + 19,+22q‐. Patients in the lymphoblastic group acquired structural abnormalities, apparently random in nature and usually in a small percentage of cells. The two patients with erythroblastic transformation developed markedly hyperdiploid cells (> 50 chromosomes) with both numerical and structural abnormalities. Patients in the lymphoblastic group appeared to have a slightly better prognosis than the myeloid group, whilst the patients with erythroblastic transformation had a very poor prognosis.
Hypertonic cryohemolysis of pathologic red blood cells
Authors: Streichman S. , Kahana E. , Tatarsky I.
Published: February 2007

Human erythrocytes suspended in hypertonic solutions undergo hemolysis when the temperature of the suspension is changed from 37°C toward 0–4°C. It has been suggested that the hypertonic environment causes some proteins of the skeletal network to be changed in such a way that their normal adaptation to temperature changes is prevented, thus resulting in cryohemolysis. In the present study, we compared the cryohemolysis of some pathologic red blood cells in hypertonic sucrose and NaCl to normal cells. Erythrocytes of hereditary spherocytosis (HS) were found to be significantly more fragile than all others in hypertonic sucrose, while they behaved normally in hypertonic NaCl. In contrast, erythrocytes of thalassemic patients showed decreased susceptibility to cryohemolysis, both in hypertonic sucrose and in NaCl. Autoimmune hemolytic anemia samples behaved like normal samples, both in NaCl and in sucrose. The erythrocytes of congenital dyserythropoietic anemia‐type II patients showed two types of cryohemolysis; one pattern was similar to that of HS, and the other one presented normal levels in sucrose and reduced levels in NaCl. The different patterns of cryohemolysis described for the pathologic cells are thought to reflect different lesions in the membranes of the erythrocytes of the various hemolytic disorders. It is hoped that studying the cryohemolysis of abnormal red cells may contribute some illumination as to molecular interactions in intact cells in health and in disease.
Marrow mass and distribution in murine skeletons cleaned by beetles as compared to cut up carcasses and a further simplification of the latter technique
Authors: Boggs Dane R. , Patrene Kenneth D.
Published: February 2007

Distribution of 59Fe into various bone groups of the complete murine skeleton was studied using two methods of dividing up the bones: 1) our previously reported technique of simply cutting up a skinned, eviscerated carcass and 2) separating bones from skeletons cleaned of overlying tissue by beetles, Dermestes species. The total percentage of injected 59Fe recovered in the sum of all skeletal parts, the percentage of total skeletal 59Fe found in each bone group, and the overall accuracy of determining these values were quite similar for the two techniques. The only statistically significant difference shown was a modest decrease in the percentage of total skeletal iron found in ribs plus sternum plus cervical and thoracic spine in beetle‐cleaned as compared to cut up groups and we would not consider this to be of biological significance. Cutting up carcasses is the simpler of the two techniques but there are circumstances in which beetle digestion would be advantageous.

In addition, we collected data on the reproducibility and precision of determining the percentage of 59Fe injected which is found in a „pulled off”︁ foreleg plus scapula and of the distribution of 59Fe within three cut up pieces from the leg and within the scapula. These data can be used as a measure of overall changes in marrow mass and/or distribution, or at least they can be used as a screening procedure to detect such. This simple procedure adds potentially useful values for fully interpreting hematopoietic changes in the mouse.
Erythrocyte incorporation of ingested stable isotope of iron (58Fe)
Authors: Janghorbani Morteza , Ting Bill T. G. , Fomon Samuel J.
Published: February 2007

Because of a possible hazard from the use of radioisotopes to determine iron absorption by infants, the use of stable isotopes for this purpose has much appeal. We have applied the method of inductively coupled plasma mass spectrometry (ICP/MS) to determine the mass ratio, 58Fe/57Fe, in blood before and after oral administration of 58Fe. From the increase in erythrocyte enrichment with 58Fe, we have calculated percentage absorption of iron. We have shown that the coefficient of variation of measured mass isotope ratio is 0.1‐1.0%, depending on the conditions of the measurement.

The method has been applied to a feasibility study involving four infants. Each infant was given 58Fe either as a single dose or as one dose on each of two consecutive days. Each dose provided 1.945 mg iron and 1.440 mg 58Fe. Samples of blood were obtained before isotope administration and at 14, 42, and 60 days thereafter. Isotopic analysis of the samples demonstrates that this approach results in a sufficiently large isotope enrichment to permit satisfactory measurement of iron availability.

It is concluded that this new method is highly promising for studies of iron availability in infants and children.
Determination of ectoenzyme activities in leukemic cells and in established hematopoietic cell lines
Authors: Morell Andreas , Losa Gabriele , Carrel Stephan , Heumann Didier , Von Fliedner Vladimir E.
Published: February 2007

Ectoenzyme activities were determined in peripheral blood cells from patients with acute leukemias, from normal controls, and in cells of hematopoietic cell lines. In common acute lymphoblastic leukemia, cell membrane‐associated 5′‐nucleotidase (5′‐N) activity was significantly higher than in acute T and unclassified lymphoblastic leukemias. In acute myeloblastic and myelomonocytic leukemias, cells contained significantly higher γ‐glutamyl transpeptidase (γ‐GT) activity than in lymphoblastic leukemias. Normal B lymphocytes differed from T cells and monocytes mainly in their 5′N activity, whereas in monocytes, γ‐GT activity was more pronounced than in other normal blood cells. Hematopoietic cell lines showed some distinct patterns of ectoenzyme activity. Most B cell lines had high 5′N and (Na‐K‐Mg) adenosine triphosphatase activities. In lines of myeloid origin, elevated γ‐GT values were found. In lymphoid stem cells and in T lymphoblast lines, most ectoenzyme activities were lower than in the other cell lines. In some cell lines, characteristic high‐activity marker enzymes were detected.
Acquired von willebrand syndrome due to an inhibitor specific for von willebrand factor antigens
Authors: Lazarchick John , Pappas Alexandros A. , Kizer Joette , Hall Sam A.
Published: February 2007

A patient with acquired von Willebrand syndrome associated with polycythemia rubra vera is described. Her plasma factor VIII procoagulant activity (67 U/dl) and factor VIII‐related antigen (117 U/dl) were normal but no von Willebrand factor activity could be detected. Factor VIII crossed immunoelectrophoresis revealed decreased levels of less anodic polymeric forms of factor VIII. Mixture of her plasma or immunoglobulin G (IgG) fraction with normal plasma resulted in complete recovery of factor VIII activity and related antigen but no measurable von Willebrand factor activity, confirming the presence of an unique inhibitor. The limited specificity of this inhibitor to antigenic sites solely on the von Willebrand portion of the factor VIII bimolecular complex is distinct from all previous reports of this syndrome. This unique inhibitor offers a molecular probe to examine the von Willebrand factor: platelet interaction.
Hemopoietic marrow function in chronic neutropenia of blacks: Cure of aplastic anemia by allogeneic marrow transplantation from a neutropenic sibling donor
Authors: Ash Robert C. , Mendelsohn Larry A. , Marshall M. Ernest
Published: February 2007

A black patient with severe aplastic anemia is described who underwent successful bone marrow transplantation from a sibling with chronic neutropenia. During an evaluation to identify a suitable donor, it was found that the majority of famiily members tested had neutropenia, with no familial history of significant infections or related hospitalizations. In vitro hemopoietic culture studies of marrow from the patient's HLA‐MLC‐matched siblings showed normal numbers of pluripotential and committed hemopoietic progenitors; in vitro hemopoietic colony formation from the patient was markedly subnormal, consistent with the clinical picture of severe aplastic anemia. Following appropriate conditioning therapy, marrow transplanted from one of these neutropenic sibs produced full hematopoietic reconstitution. Posttransplant marrow culture studies of the patient showed restoration of a normal pattern of in vitro hemopoiesis. The in vitro culture studies and clinical experience in this patient support the concept that chronic neutropenia of blacks is not primarily a marrow progenitor cell disorder but, more likely, a manifestation of a genetically determined alteration in granulocyte kinetics.
DDAVP in acquired von Willebrand syndrome associated with multiple myeloma
Authors: Takahashi Hoyu , Nagayama Reizo , Tanabe Yasuhiko , Satoh Ken , Hanano Masaharu , Mito Masao , Shibata Akira
Published: February 2007

The response to a single intravenous infusion of 1‐deamino‐8‐D‐arginine vasopressin (DDAVP, desmopressin) was studied in two patients with acquired von Willebrand syndrome associated with IgG‐k myeloma. Following infusion of DDAVP (0.3–0.4 μg/kg), prolonged bleeding time was normalized; plasma ristocetin cofactor activity, von Willebrand factor antigen, and factor VIII activity were remarkably increased; and high‐molecular‐weight forms of von Willebrand factor were demonstrated by crossed immunoelectrophoresis in both patients. Excellent hemostasis was achieved following adminstration of DDAVP in one patient when it was used for the treatment of gum bleeding and for the prophylaxis of bleeding during and after dental extractions. These observations suggest that DDAVP is an effective alternative to blood products for at least some patients with acquired von Willebrand syndrome in addition to patients with inherited von Willebrand disease, hemophilia A, and uremia.
Acute nonlymphocytic leukemia (M7 subtype) with erythrocytic internalization
Authors: Woessner S. , Lafuente R. , Giralt M. , Molina J. , Florensa L.
Published: February 2007

A case of acute nonlymphocytic leukemia of megakaryocytic lineage or M7 with a conspicuous phenomenon of erythrocytic internalization present in nearly 3% of the leukemic megakaryocytic precursors (LMP) is reported. The phagocytosis was detected at light microscopy and ultrastructural levels. The erythrocyte might have reached the interior of the LMP through cytoplasmic vacuoles that are connected with the extracellular space and that can be considered rudimentary equivalents of the demarcation membrane system (DMS). Erythrocytic internalization is not a specific feature of the mononuclear phagocytic system and can be misleading in the classification of undifferentiated blast cells.