bluuebunny/crossref_metadata_2025 huggingface dataset:
Deficiency of mature B and T lymphocyte subsets in the blood of non‐Hodgkin lymphoma patients
Authors: Janowska‐Wieczorek Anna , Andrews E. Joy , Khaliq Abdul , Pilarski Linda M.
Published: February 2007

The expression of mature B‐cell markers and T markers was determined in lymphocytes isolated from the peripheral blood (PBL) of 20 healthy adults and 51 patients with non‐Hodgkin malignant lymphoma (NHL). The disease was classified as newly diagnosed, in remission, or being treated with chemotherapy and of low‐, intermediate‐, or high‐grade malignancy. To avoid technical problems associated with artifacts involving cytophilic immunoglobulins (Ig), we defined mature B‐cells by means of three criteria: a) expression of high surface density of Ig sufficient to allow polar movement of receptors to form a cap in an indirect immunofluorescence (IF) assay, b) expression of high density of the human leukocyte antigens DR (HLA‐DR) under capped conditions, and c) expression of a 41H.16 marker exclusive to surface Ig+ B‐cells. Percentages of PBL able to cap surface Ig (sIg) (λ, K), HLA‐DR (7H.3), and 41H.16 markers were significantly reduced (p<0.001) in all of the patients, regardless of treatment status, and the numbers of sIg+‐capping cells were similarly reduced in the patients, regardless of the grade of malignancy. Studies with ring fluorescence showed mean percentages of cells expressing OKT3 and OKT4 determinants significantly reduced (P<0.001) but OKT8+ cells not significantly different from control. The OKT4/OKT8 ratio was reduced in all patients and did not differ significantly in relation to the degree of malignancy. We conclude that, in NHL, essentially all patients have severe abnormalities in the number of B‐ or T‐cells needed for normal immune responses.
Cryptic cold agglutinin activity of monoclonal macroglobulins
Authors: Pruzanski W. , Jacobs H. , Saito S. , Donnelly E. M. , Lui L. C.
Published: February 2007

One hundred and twenty sera with monoclonal immunoglobulin M's (IgM's) were tested for cryptic cold agglutinin activity against papain‐ and neuraminidase‐treated adult and cord red blood cells (RBC). Fifteen sera (12.5%) agglutinated papain‐treated RBC. Fourteen of these 15 sera also agglutinated neuraminidase‐treated RBC. Ten additional sera agglutinated neuraminidase‐treated but not papain‐treated cells. Three patterns were observed among agglutinators of papain‐treated RBC: either predominant agglutination of adult RBC, or of cord RBC or equal reaction, thus resembling I, i and non‐Ii patterns of conventional cold agglutinins. We propose to call these patterns, tentatively, Icr (cr for cryptic), icr, and non‐Iicr, respectively. Purified monoclonal IgM's retained the above patterns. Eluates from enzyme‐treated cells contained monoclonal IgM's. Investigation of neuraminidase‐treated RBC has shown that in distinction to T‐anti‐T system (Thomsen‐Friedenreich receptor system), the majority of sera with monoclonal IgM's agglutinated more strongly adult RBC, and some agglutinated more strongly cord RBC. There was no loss of agglutinating activity after repeat freezing and thawing of the sera. Thus, it seems that monoclonal IgM's which react with neuraminidase‐treated RBC detect antigenic determinants different from the T antigen. Cryptic CA activity of IgM's should be added to the list of antibody activities of these monoclonal immunoglobulins.
Cyclosporin‐a for the treatment of pure red cell aplasia in a patient with chronic lymphocytic leukemia
Authors: Chikkappa G. , Pasquale D. , Phillips P. G. , Mangan K. F. , Tsan M. F.
Published: February 2007

A 62‐year‐old man with B‐cell chronic lymphocytic leukemia had three separate episodes of pure red cell aplasia (PRCA). The last episode was treated with cyclosporin‐A (CyA) and prednisone. After the patient was on the therapy for 2 weeks, erythropoietic recovery was observed and with continued therapy the hematocrit (Hct) became normal. The PRCA remission was associated with a fall in the blood lymphocyte count, and a reduction in the spleen and lymph node size and bone marrow lymphocyte density. At diagnosis of PRCA the blood T‐cells bearing IgG Fc receptors (T gamma cells) were increased, and the marrow contained very few or no late‐stage erythroid progenitors. After remission of PRCA the T gamma cell fraction decreased, and the marrow erythroid progenitor's number became normal. We speculate that therapy with CyA and prednisone inhibited the production of interleukins‐1 and ‐2 from monocytes and T‐cells, respectively, and was responsible for the reduction of the T gamma cell fraction and B‐cell leukemic mass in this patient. Further, we believe that normalization of T gamma cells in association with the therapy was responsible for the PRCA remission.
Relationship of fluorescence polarization to cell lineage in lymphocytes from normal subjects and patients with chronic lymphocytic leukemia
Authors: Glotzer Taya V. , Liebes Leonard F. , Kanganis Denise , Silber Robert
Published: February 2007

Previous investigations have shown differences in fluorescence polarization between normal and chronic lymphocytic leukemia lymphocytes following incubation with the probe 1,6‐diphenyl‐1,3,5‐hexatriene. In the present study, we determined the fluorescence polarization of unseparated or enriched subpopulations of T and B lymphocytes from normal subjects and patients with chronic lymphocytic leukemia. As had been observed by others, the mean polarization (P) value at 25°C for unseparated chronic lymphocytic leukemia lymphocytes, .240 ± .007 (N = 22), was lower than that of unseparated normal lymphocytes, .248 ± .005 (N = 18), P < .001 (Student's t‐test). The difference was greater when B‐enriched populations were compared. The mean P value of B‐cell‐enriched chronic lymphocytic leukemia lymphocytes, .240 ± .007 (N = 5), was significantly lower than that of B‐cell‐enriched normal preparations, .256 ± .004 (N = 5), P < .001. In contrast, no significant difference was found between normal and chronic lymphocytic leukemia T cells. The anomalous fluorescence polarization manifested by chronic lymphocytic leukemia lymphocytes of B‐cell origin serves to distinguish this lineage from its normal counterpart and from T cells of either source.
Association in cis of β+‐thalassemia and hemoglobin S
Authors: Baklouti Faouzi , Francina Alain , Dorléac Evelyne , Richard Georges , Rosenberg Daniel , Godet Jacqueline , Delaunay Jean
Published: February 2007

A Moroccan woman was investigated because of a typical β‐thalassemia trait associated with a low‐percentage (11%) hemoglobin (Hb) variant. The β‐thalassemia trait was manifested by a microcytosis, a high HbA2 (above 6%), and an increase of the α/β biosynthetic ratio (1.31). The variant was identified to HbS by amino acid analysis of the abnormal peptide (β T1) and by DNA mapping with Sau I (Mst II) restriction endonuclease. No additional amino acid substitution was recorded in the βs‐chain. The reduction of β‐globin synthesis occurred exclusively at the expense of the βs‐chain. These results are consistent with the existence of a βs mutation and a β+‐thalassemia in cis.
Excess HLA antigens after transfusion of sickle cell patient with AIDS
Authors: Castro Oswaldo , Alarif Lamya , Saxinger Carl , Frederick Winston
Published: February 2007

A woman with sickle cell‐hemoglobin C disease developed the acquired immunodeficiency syndrome (AIDS), possibly as the result of blood transfusions administered 4 years earlier. Three months after subsequent transfusions, she had excess HLA‐A and DR antigens (triplets) on her circulating lymphocytes. Three of her first‐degree relatives were HLA‐typed, and none had excess antigens. Family studies also indicated that the patient had at least one HLA‐A antigen that was probably not inherited. These findings suggest the possibility that transient hemopoietic engraftment can occur in transfused sickle cell patients with AIDS.
Corticosteroid‐responsive pure red cell aplasia in chronic lymphatic leukemia
Authors: Vashi P. , Patel B. , Musson P. , Fahrner J. , Rosenbaum R.
Published: February 2007

Acquired pure red cell aplasia characterized by selective failure of erythropoiesis was seen in a 37‐yr‐old female patient with a diagnosis of chronic lymphatic leukemia (CLL). Though different immunosuppressive therapies have been tried in the past with variable response, there has been no case report of such favorable response to corticosteroids alone. We report this rare and unusual case of PRCA associated with CLL who responded to steroid therapy.
Relationship of HLA and platelet‐reactive antibodies in alloimmunized patients refractory to platelet therapy
Authors: Brubaker Daniel B. , Romine Maxine
Published: February 2007

Platelet crossmatching assays have been used to predict the outcome of platelet transfusions in alloimmunized patients by detecting antibodies against platelets. The transfusion failure of HLA‐matched platelets predicted by platelet crossmatching may be related to HLA antibodies undetected by lymphocytotoxicity but detected by platelet immunoglobulin‐binding assays or platelet‐specific antibodies (both antibodies defined here as platelet‐reactive antibodies). To differentiate platelet‐reactive antibodies from lymphocytotoxic HLA antibodies, we used HLA characterized lymphocytes in parallel with platelets from individuals to form separate frozen panels. Sera from 10 allosensitized patients were studied in the lymphocyte panel by lymphocytotoxicity and in the platelet panel by enzyme‐linked immunoassay (ELISA). By comparing pattern and percent wells reacting in each panel, lymphocytotoxic HLA antibodies and antibodies reactive with platelets in ELISA were detected separately. In all 10 allosensitized patients, platelet‐associated antibodies were present and 7 had additional lymphocytotoxic HLA antibodies. Using this double parallel panel technique, we found platelet‐reactive antibodies important in platelet alloimmunization, unrecognized by lymphocytotoxicity. These data indicate platelet‐crossmatching be solely used in the selection of platelets for allosensitized patients.
Erythrocyte pyruvate kinase (PK): The variable significance of “nucleotide specificity” in the characterization of mutant variants
Authors: Valentine W. N. , Paglia D. E. , Nakatani M. , Brockway R. A.
Published: February 2007

The half‐saturation constant (K0.5s) phosphoenolpyruvate (PEP) for red cell pyruvate kinase (PK) with co‐factors UDP and GDP is less than one‐half that with ADP with or without additions of the allosteric modifier, fructose‐1, 6‐dephosphate (F‐1, 6‐P2) to the assay. The Vmax is markedly greater with ADP than with UDP or GDP, but with (PEP) at 0.5 mM, activity with all co‐factors is about equal and at lower concentrations greater with UDP and GDP. With high K0.5s (PEP) mutant enzymes, and at the usual test concentration (1mM) for PEP when nucleotide specificity is assessed, the abnormally low saturation of variant enzymes may result in higher activity with UDP and GDP than with ADP–the opposite of the “normal situation.” The apparent aberration in nucleotide specificity may thus be illusory and secondary to the abnormal K0.5s (PEP) of the mutant. Example data are recorded. Variations in K0.5s (PEP) may also be introduced during enzyme preparation for assay, particularly when partial purification is employed.
Lack of evidence of circulating retroviral antibodies in patients with classic Hodgkin's disease
Authors: Chorba T. L. , Kalyanaraman V. S. , Lacher M. J. , Schulof R. S.
Published: February 2007

Because of the T‐cell abnormalities observed in Hodgkin's disease and the growing number of Hodgkin's disease cases observed in association with the acquired immunodeficiency syndrome (AIDS), concern has been expressed that a retrovirus may be the primary cause of Hodgkin's disease. We examined plasma specimens from 17 patients with Hodgkin's disease that were drawn in 1979. Because serum containing antibodies to either human T‐lymphotropic virus type I (HTLV‐I) or HTLV‐II precipitate the major core protein, p24, of HTLV‐I, lack of reactivity to HTLV‐I p24 in all the specimens demonstrated absence of antibodies to HTLV‐I or ‐II. None of the specimens was reactive to human immunodeficiency virus type 1 (HIV‐1) by ELISA. None of the specimens were reactive on Western blot assays for HTLV‐I or ‐II or HIV‐1. Lack of evidence of cross‐reacting antibodies to prototype strains of those retroviruses in specimens drawn before the AIDS epidemic suggests that classic Hodgkin's disease is not the result of infection with one of the known human lymphocytotropic retroviruses or a closely related agent.
Response in serum ferritin and haemoglobin to iron therapy in blood donors
Authors: Mackintosh Wayne , Jacobs Peter
Published: February 2007

Seven hundred seventeen healthy male blood donors regularly donating four or more units a year were surveyed for haemoglobin and serum ferritin levels. One hundred fiftyone (21%) had a haemoglobin less than 13.5 g/dl and were therefore disqualified from further blood donation, having a mean serum ferritin of 28 μg/liter. Of the remaining 566 donors with haemoglobin levels equal to or greater than 13.5 g/dl, the mean serum ferritin was 33 μg/liter, although in 299 (53%) the value was less than 28 μg/liter. To document response to iron therapy 46 donors with haemoglobin levels equal to or greater than 13.5 g/dl were stratified into those with the lowest iron stores (group 1; n = 23), defined as a serum ferritin less than 20 μg/liter, and controls (group 2; n = 23), with serum ferritin between 50 and 150 μg/liter. Within each stratum donors randomly received ferric polymaltose at a dose of 100 mg elemental iron twice daily for 56 days (groups 1a and 2a) or an identical iron‐free placebo tablet administered on the same schedule (groups 1b and 2b). Iron therapy in the iron‐deficient group (group 1a:n = 11) resulted in a significant rise in haemoglobin (p = .03) and iron stores reflected in serum ferritin (p = .002) compared to those receiving placebo (group 1b). In the control group iron therapy or placebo was without significant effect. Thus, ferric polymaltose preparation is bioavailable and is notable for the virtual absence of gastrointestinal tract side effects.
Intraarticular methylprednisolone therapy in hemophilic arthropathy
Authors: Shupak R. , Teitel J. , Garvey M. B. , Freedman J.
Published: February 2007

This small pilot study examined the use of intraarticular methylprednisolone in hemophilic synovitis. Nineteen joints in ten adult hemophiliacs were studied. There was subjective improvement at 24 hr following injection in 79% of joints injected, and the improvement persisted up to 8 wk in 58%. The number of hemarthroses decreased following intraarticular steroids (mean of 7.7 bleeds in the 8 wk prior to injection versus a mean of 1.9 bleeds in the 8 wk following injection). Similarly the amount of clotting factor used for the injected target joint decreased from a mean of 7,616 units to 2,315 units postinjection (p <.001). Improvement correlated with presence of synovitis but not with radiologic stage of the joint. Aspirated synovial fluids were analyzed and showed characteristics consistent with low‐grade inflammation. These preliminary observations suggest that intraarticular corticosteroid injection may be a useful therapeutic tool in the medical management of hemophilic arthropathy.
Genotypic analysis using a Y‐chromosome‐specific probe following bone marrow transplantation
Authors: Morisaki Hiroko , Morisaki Takayuki , Nakahori Yutaka , Ogura Hiromi , Kanno Hitoshi , Tani Kenzaburo , Kodo Hideki , Fujii Hisaichi , Asano Shigetaka , Miwa Shiro
Published: February 2007

To monitor successful engraftment after bone marrow transplantation, we performed Southern hybridization analysis or dot blot analysis of DNA in a set of sex‐mismatched cases using a Y‐chromosome‐specific DNA probe (pHY10). This method was extremely sensitive and rapid for checking which cells contain the Y‐chromosome. Using this probe, analysis of cells from peripheral blood and bone marrow after transplantation demonstrated the usefulness of confirming engraftment of donor cells and of detecting mixed lymphohematopoietic chimerism.
Acute myeloblastic leukemia with hyperleukocytosis: Risk factors for early mortality in induction
Authors: Ventura Gerard J. , Hester Jeane P. , Smith Terry L. , Keating Michael J.
Published: February 2007

Eighty‐five patients with acute myeloblastic leukemia (AML) presenting with hyperleukocytosis (HL) were analyzed to assess morbidity and mortality in early induction. Patients who failed to achieve remission were older and more often had pulmonary leukostasis (62% vs 23%, p = .01) and hepatomegaly (54% vs 31%, p = .06) at presentation. Thirty‐seven patients (44%) did not achieve complete remission (CR); 17 (54%) died early in induction therapy, 11 directly as a result of pulmonary hemorrhage with respiratory failure, while 5 had both pulmonary hemorrhage with respiratory failure and CNS hemorrhage. Early death patients were older and more often had pulmonary leukostasis (88% vs 29%, p < .0001), hepatomegaly (71% vs 34%, p = .01), hyperbilirubinemia (60% vs 16%, p = .01) and hypofibrinogenemia (47% vs 12%, p < .01) at presentation. Primarily for technical reasons, preinduction leukapheresis was not employed as often in this very‐high‐risk group as in other patients (56% vs 82%, respectively). Thus, sufficient heterogeneity exists in patients presenting with HL to define a subset of patients at particularly high risk for early mortality. Preinduction leukapheresis applied in a prospective controlled fashion should be evaluated to assess if such treatment may decrease early mortality in this group.
Oxygen‐induced marrow red cell hypoplasia leading to transfusion in sickle painful crisis
Authors: Lane Philip K. , Embury Stephen H. , Toy Pearl T. C. Y.
Published: February 2007

The benefit of oxygen (O2) therapy in non‐hypoxic sickle cell patients in painful crisis is uncertain. We report a case of a non‐hypoxic sickle cell patient in painful crisis who developed marrow red cell hypoplasia requiring transfusion support after O2 therapy. The uncertain benefits of O2 use in such cases must be weighed against the serious and underrecognized risks of transfusion. In patients who develop O2‐induced marrow red cell hypoplasia, cessation of O2 therapy may reverse the anemia and obviate the need for transfusion.
Effects of purified human native granulocyte colony‐stimulating factor (G‐CSF) on proliferation of blast progenitors in acute myeloblastic leukemia
Authors: Nara Nobuo , Murohashi Ikuo , Suzuki Toshiya , Yamashita Yoko , Maruyama Yasuo , Aoki Nobuo , Tanikawa Shu , Onozawa Yasusuke
Published: February 2007

Blast progenitors in acute myeloblastic leukemia (AML) grow in methylcellulose and suspension cultures. Blast colony formation in methylcellulose culture reflects the terminal divisions of blast progenitors, while secondary colony formation, by replating in methylcellulose and recovering clonogenic cells in suspension culture, reflects the self‐renewal of blast progenitors. To analyze the regulatory mechanisms of the proliferation of leukemic blast progenitors, the effects of highly purified native granulocyte colonystimulating factor (G‐CSF) obtained from human squamous cell carcinoma line (CHU‐2) on blast progenitors in AML patients were studied in methylcellulose and suspension cultures. Purified G‐CSF stimulated the growth of blast progenitors in both culture systems, although sensitivity to G‐CSF varied from patient to patient. No obvious maturation of leukemic blasts was noted in suspension culture in the presence of G‐CSF. The data suggest that a normal hemopoietic regulator may play a role in the growth of blast progenitors in AML patients.
Granulopoietic differentiation in long‐term bone marrow cultures from children with congenital neutropenia
Authors: Coulombel Laure , Morardet Nada , Veber Florence , Leroy Christine , Mielot Francoise , Fisher Alain , Teillet‐Thiebaud Françoise , Tchernia Gil , Griscelli Claude , Parmentier Claude
Published: February 2007

The capacity of granulopoietic precursor cells (CFU‐GM) to differentiate in vitro was evaluated in five children with congenital neutropenia using short‐term colony assays and long‐term marrow cultures. In all five children, methylcellulose assays revealed normal numbers of CFU‐GM, which displayed an appropriate response to various sources of GM‐CSF and differentiated up to the polymorphonuclear leukocyte state (PMN). In contrast, neutrophil PMN were not observed in long‐term bone marrow cultures from three patients, despite a normal production of CFU‐GM, myeloblasts, and promyelocytes during the 5‐6‐week culture period. Thus, in these patients, the characteristic “block” in granulocytic maturation observed in vivo was reproduced in vitro in long‐term cultures. Granulocytic differentiation proceeded normally in long‐term cultures from the two other patients, thus indicating heterogeneity in the expression of the defect. These results might indicate abnormal interactions between stromal and hematopoietic cells in long‐term marrow cultures from some patients with congenital neutropenia. Furthermore, our results showed some correlation between the granulocytic defect in vitro and the clinical outcome in vivo.
Prognostic implications of basophil differentiation in chronic myeloid leukemia
Authors: Denburg Judah A. , Browman George
Published: February 2007

Basophilia has been reported to indicate an accelerated phase of chronic myeloid leukemia (CML), heralding a poor prognosis. We have studied 47 patients with chronic‐phase CML by basophil growth and differentiation assays in vitro, demonstrating an association between basophil growth index (BGI) and clinical time to blast crisis as well as overall survival. In addition to confirming an association between positive BGI and phase of CML in a larger group of patients, a positive BGI predicted death or blast crisis within 2 years' study of chronic phase CML (p<0.01), with a sensitivity of 78%, specificity, 81%; the positive predictive value of a positive test, 64%; and a negative predictive value of a negative test, 89%. The survival experience of the 22 evaluable patients with chronic‐phase CML and a positive BGI was significantly worse than the survival of the 19 patients with a negative BGI (p<0.0001). At 1, 2, and 3 years the proportion of surviving patients with a positive BGI was 0.64, 0.32, and 0.23, respectively, compared with 1.00, 0.90, and 0.79 for those with a negative BGI. The median survival of chronic phase CML patients with a positive test at diagnosis (n = 14) was 27 months versus 54 months for those with a negative diagnosis (n = 14) (p<0.05). These findings emphasize the prognostic utility of basophil growth assays in CML and suggest a molecular relationship between leukemic transformation and basophil lineage expression.
Metastatic extramedullary plasmacytoma: A case report and review of the literature of a rare pseudocarcinoma
Authors: Akiyama Hideki , Krigel Robert L.
Published: February 2007

Extramedullary plasmacytomas are unusual plasma cell tumors arising outside the bone marrow. Most extramedullary plasmacytomas are solitary, but occasionally they disseminate, usually in conjunction with systemic myelomatosis. The authors report a case of disseminated extramedullary plasmacytoma with a picture suggestive of metastatic pancreatic carcinoma. This unusual presentation reconfirms the need for tissue diagnosis in all suspected cases of pancreatic carcinoma and demonstrates that extramedullary plasmacytoma can be an aggressive cancer without any sign of osseous or systemic disease.
Prolonged remission after life‐threatening gastrointestinal hemorrhage from coexistent angiodysplasia and acquired bleeding diathesis
Authors: Woodlock Timothy J. , Francis Charles W. , Rowe Jacob M. , Brown M. Julianne , Marder Victor J.
Published: February 2007

A patient with angiodysplasia of the stomach and duodenum developed exceptionally severe and protracted gastrointestinal hemorrhage. Bleeding was intensified by a coexistent acquired hemostatic disorder characterized by decreased platelet aggregation with adenosine diphosphate, collagen, epinephrine and ristocetin, and a decrease in both plasma ristocetin cofactor activity and high‐molecular‐weight von Willebrand factor multimers. Cryoprecipitate infusion corrected the von Willebrand factor defect but did not improve platelet aggregation. Bleeding stopped after prolonged aggressive combined medical and surgical therapy, and the patient had no recurrence of bleeding while followed for 27 months. Hemorrhage from the vascular lesions themselves dominated the clinical picture of this patient and other reported patients with coexistent angiodysplasia and congenital or acquired hemostatic abnormalities.
Increased production of superoxide anion by neonatal polymorphonuclear leukocytes stimulated with a chemotactic peptide
Authors: Yamazaki Munehiro , Matsuoka Takafumi , Yasui Kozo , Komiyama Atsushi , Akabane Taro
Published: February 2007

In order to assess the functional property of neonatal polymorphonuclear leukocytes (PMNs), we studied their chemoattractant‐stimulated superoxide anion (O2−) production using a soluble chemotactic peptide N‐formyl‐methionyl‐leucyl‐phenylalanine (FMLP) as a PMN stimulus. O2− production by neonatal PMNs was increased: when stimulated with 10−7M FMLP, O2− values (nanomoles; mean ± SE) produced by 106 PMNs were 16.5 ± 1.3 for neonatal PMNs and 12.6 ± 0.6 for adult PMNs (p < .02). When studied under various concentrations (10−9 to 10−6M) of FMLP, neonatal PMNs produced more O2− than adult cells under the stimulation of a lower concentration (10−8M) as well as a higher concentration (10−7M) of FMLP. The increased O2− production by neonatal PMNs was also observed under their stimulation by phorbol myristate acetate. Neonatal and adult PMNs produced equal amounts of O2− when stimulated with a particulate stimulus of opsonized zymosan. To analyze further the mechanism for the increase in FMLP‐induced O2− production by neonatal PMNs, we next studied whether there was some abnormality in their FMLP receptors. Neonatal PMNs had normal numbers of FMLP receptors; the receptor numbers (mean) per cell were 54,000 in neonatal PMNs and 56,000 in adult cells. Additionally, the FMLP receptors had normal affinity for the peptide. These results demonstrate that the increased production of O2− by FMLP‐stimulated neonatal PMNs is not due to the abnormality of its binding to the receptors but is due to that of the subsequent events.
Abnormal platelet von willebrand factor interaction in patients with TTP
Authors: Rock G. , Tittley P. , Taylor J. R.
Published: February 2007

Thrombotic thrombocytopenic purpura (TTP) is associated with abnormal platelet function and disturbances in coagulation; however, a specific causative factor is not defined. Plasma infusion or plasma exchange (PE) are thought to be of benefit in replacing a deficient plasma component or removing some toxic compound. In three patients with TTP, samples taken prior to initiation of PE showed high levels of vWF:Ag in the plasma (208, 264, and 321 U/dl), whereas the VIII:C levels were normal. The vWF:Ag multimer patterns of the plasma demonstrated a decrease in the amount of high molecular weight (HMW) forms. Analysis of the platelets from one patient also showed an increase in the HMW multimers. Platelets from all three patients showed a decreased ability to absorb vWF:Ag, with little or no absorption of the HMW forms. Following extensive PE and resolution of disease, the platelets regained their ability to absorb vWF:Ag in the one patient examined.
Hemolytic uremic syndrome in a patient with small cell lung cancer
Authors: Avvento Louis , Gordon Sandra , Silberberg Jeffrey M. , Zarrabi M. Hosein , Zucker Stanley
Published: February 2007

Hemolytic uremic syndrome is a rare entity in patients with carcinoma and presents with a triad of renal insufficiency, microangiopathic hemolytic anemia, and thrombocytopenia. We report this syndrome for the first time in a patient with small cell lung carcinoma. Spontaneous platelet aggregation of washed normal platelets was demonstrated using patient plasma. Circulating immune complex levels were not elevated. The entity completely resolved after treatment with plasma, vincristine, aspirin, and dipyridamole.
Occurrence of spontaneous malignant lymphoma in the course of idiopathic myelofibrosis
Authors: Bogliolo Giuseppe V. , Leza Roberto A. , Ruggieri Corrado , Pannacciulli Ivo M.
Published: February 2007

The spontaneous development of malignant non‐Hodgkin lymphoma in a patient with idiophatic myelofibrosis is described. The tumor appeared 3 years after clinical diagnosis of the latter. Implications of the association between myeloproliferative and lymphoproliferative disorders at a pathogenetic level are briefly discussed.
Restriction fragment length polymorphism in the interzeta hypervariable region for prenatal diagnosis of non‐deletion α thalassemia
Authors: Chan Vivian , Chan T. K. , Wong A. C. K. , Chan T. P. T. , Ghosh A. , Todd D.
Published: February 2007

A Bam HI restriction fragment length polymorphism in the interzeta hypervariable region (IZ HVR) of the ζ‐α gene cluster was used for the prenatal diagnosis of a pregnancy at risk for Hb H hydrops fetalis. The parents had ζ‐α thalassemia 1 and non‐deletion α thalassemia, respectively, and a previous hydrops was missed using the conventional method of gene detection. In this prenatal diagnosis, linkage to IZ HVR was used to exclude non‐deletion α thalassemia, and the numbers of ζ and α genes in the fetus were quantitated to predict the exact genotype. Confirmation was made by analysis of cord blood at delivery.
Altered platelet deformability in patients with type IIa and type IV hyperlipoproteinemia
Authors: Smith Clark M. , Burris Steven M. , Hunninghake Donald B. , White James G.
Published: February 2007

Platelets from subjects with hyperlipoproteinemia (HLP) differ from normal platelets in lipid composition and function depending upon the phenotypic classification of the HLP. The present study has evaluated the deformability of platelets from human subjects with type Ila and type IV HLP. Platelets suspended in autologous plasma diluted 30‐fold with buffer were aspirated into micropipettes 0.7‐0.8 microns in diameter by step‐wise increment in tension, and the resulting extension lengths were recorded. Platelets from type IIa subjects could not be aspirated as far into the micropipettes as normal platelets. However, less tension was required to reach maximum cell extension than with normal platelets, and the initial extension lengths and slopes of the stress responses were the same as the control. In contrast, platelets from subjects with type IV HLP showed a generalized increase in deformability. The initial cell extensions aspirated from type IV platelets were longer than normal, and larger maximum cell extensions were achieved at lower tensions than control platelets. The type IV platelets were also mechanically fragile and fragmented at lower tensions than control or type IIa platelets. The variance in platelet deformability between subjects of the same phenotype was not directly correlated to plasma lipid or lipoprotein concentrations. This study confirms alterations in the structural organization of platelets from subjects with type IIa and type IV HLP.
Asymptomatic association of hemoglobin Dunn (α6[A4]Asp→Asn) and hemoglobin O‐Arab (β121[GH4]Glu→Lys) in a Moroccan man
Authors: Baklouti Faouzi , Francina Alain , Dorléac Evelyne , Baudin‐Chich Véronique , Gombaud‐Saintonge Germaine , Plauchu Henri , Wajcman Henri , Delaunay Jean , Godet Jacqueline
Published: February 2007

We report on the association of Hb Dunn (α6[A4]Asp→Asn) and Hb O‐Arab (β121[GH4]Glu→Lys) in a healthy Moroccan man. Hb Dunn had the same electrophoretic properties as Hb G‐Philadelphia, but its percentage was lower. Its identification was based on sequence determination of the αT1 peptide. Bgl II and Eco RI mapping showed the presence of four α‐genes. Hb O‐Arab was easily recognized through its electrophoretic properties and was confirmed by the suppression of the Eco RI site located in exon 3 of the β‐gene. The percentages of the various hemoglobins showed that the doubly mutated hemoglobin Dunn/O‐Arab has a normal stability and suggested that the Dunn mutation is carried by the α1‐gene. In cord blood [propositus's son], the output of the αDunn gene was found equivalent to that existing in the adult.
Simultaneous study of karyotype and cell morphology in childhood erythroleukemia
Authors: Stamberg Judith , Zaslav Ann , Shende Ashok , Festa Robert
Published: February 2007

We followed a 2‐year‐old girl with erythroleukemia (EL) for 7 months, from the time of her initial diagnosis until her death. Immunophenotyping of bone marrow was negative for markers of myeloid and lymphoid lineages. Chromosome study of marrow at diagnosis revealed abnormalities in all mitotic cells, with a clonal karyotype of 48,XX,t(2;12)(p11.2; p13), + 6, + 21. Subsequent studies showed that the clone rapidly evolved and accumulated additional structural and numerical abnormalities, in spite of intensive chemotherapy during the final months.

Simultaneous study of the karyotype and cell morphology of dividing bone marrow cells after 24 hours of in vitro culture, using a technique that preserves cell structure, showed that cells with the chromosome abnormalities were PAS‐negative. The combined immunological, cytogenetic, and morphological data indicate that the original malignant cell type was neither myeloblast nor erythroblast but an early progenitor type that rapidly proliferated.
Factor XI deficiency: Genetic and clinical studies of a single kindred
Authors: Litz Craig E. , Swaim William R. , Dalmasso Agustin P.
Published: February 2007

A four‐generation 25‐member kindred with Factor XI:C deficiency is reported. Factor XI:C levels in heterorygotes varied from 15 to 58%, suggesting that Factor XI:C values for homozygote determination should be less than 15%. The frequency of bleeding was not correlated with Factor XI:C levels in this range. Individuals with joint pain had significantly lower Factor XI:C levels than members without joint pain and pain occurred more frequently in frequent bleeders. Lod scores showed no close genetic linkage of Factor XI:C deficiency with blood group MNSs (chromosome 4), complement components Bf and C4B (chromosome 6), or blood group P.
Erythrocytes in Hb SC disease are microcytic and hyperchromic
Authors: Ballas Samir K. , Kocher William
Published: February 2007

We have used the new Technicon H.1 Hematology Analyzer to determine the indices of erythrocytes obtained from 18 nonthalassemic (αα/αα genotype) adult patients with Hb SC disease. Controls were 14 normal black adults and 29 white adults. The data showed that SC erythrocytes are significantly smaller than normal RBC (P < .001) with a significantly higher MCHC value (P < .001) than controls. These features of SC RBC could not be reproduced by an Ortho ELT‐8 electonic counter. Hb SC erythrocytes have unique indices which are best demonstrated either by semimanual methods or by the H.1 system.
Acute promyelocytic leukemia presenting as sudden blindness and sinus vein thrombosis
Authors: Hazani Anna , Weidenfeld Yaakov , Tatarsky Llana , Bental Efraim
Published: February 2007

An 11‐year‐old boy was hospitalized with sudden blindness and sagittal sinus vein thrombosis, which were the presenting signs of acute promyelocytic leukemia (APL). The association of disseminated intravascular clotting (DIC) with APL is well‐known, and DIC usually affects the smaller blood vessels. The obliteration of a large blood vessel, as in our case, is an uncommon manifestation of DIC. This presentation of acute childhood leukemia is unusual and, to the best of our knowledge, has not been previously reported.
Regional and temporal variation in oscillatory blood flow in sickle cell disease
Authors: Kennedy Alan P. , Williams Barbara , Meydrech Edward F. , Steinberg Martin H.
Published: February 2007

We examined 55 patients with sickle cell anemia (HbSS) and 16 with hemoglobin SC disease (HbSC) to ascertain the presence of periodic microcirculatory flow (PMF) using laser‐Doppler velocimetry. Forty‐nine percent of patients with HbSS and 12.5% of HbSC patients had PMF on at least one occasion and in at least one site. The presence of PMF could be correlated with an increase in packed cell volume (PCV) and hemoglobin in individuals with HbSS and an increased white blood cell and platelet count in patients with HbSC. PMF may result from complex interactions among perfusion pressure, capillary tone, and the numbers and intrinsic properties of formed blood elements.
Simultaneous expression of lymphoid and myeloid phenotypes in acute leukemia arising from myelodysplastic syndrome
Authors: Komatsu Norio , Yoshida Minoru , Eguchi Mitsuoki , Akashi Makoto , Sasaki Ryuhei , Sakamoto Shinobu , Miura Yasusada
Published: February 2007

A 57‐year‐old female developed myelodysplastic syndrome (MDS) that terminated as a biphenotypic leukemia after exposure to chemoradiotherapy. Double staining of blast cells, using monoclonal antibodies specific for myeloid and lymphoid lineage, demonstrated that one‐third of the leukemic cells simultaneously expressed the E rosette‐associated antigen (OKT11) and myeloid‐associated antigen (MY7). This finding suggests the possibility that some cases of MDS are clonal disorders that arise in a pluripotent stem cell that can also differentiate to T cell lineage.
Plasma cell leukemia with iga paraproteinemia and hyperviscosity
Authors: Kosmo Michael A. , Gale Robert Peter
Published: February 2007

Primary IgA plasma cell leukemia is a rare disorder in which hyperviscosity has not been reported. We studied a patient with plasma cell leukemia and IgA lambda monoclonal gammopathy with an elevated relative serum viscosity and congestive heart failure. Plasmapheresis and chemotherapy were successful in controlling symptoms. We discuss this unusual association and review the literature.
Genetic analysis of adenosine deaminase expression in adult T‐cell leukemia
Authors: Morisaki Takayuki , Horiuchi Naomi , Tsutsumi Hisashi , Ogura Hiromi , Kanno Hitoshi , Tani Kenzaburo , Fujii Hisaichi , Miwa Shiro
Published: February 2007

We investigated the genetic basis for the increased adenosine deaminase (ADA) expression in adult T‐cell leukemia (ATL). We found a correlation between the levels of ADA‐specific mRNA and ADA immunoreactive protein in ATL. Southern blot analysis revealed no gene amplification or rearrangement of the ADA gene. These findings indicate that increased ADA expression in ATL cells reflects increased transcriptional activity for the ADA gene or increased stability of ADA mRNA.
Use of differentiation inducing agents in the myelodysplastic syndrome and acute non‐lymphocytic leukemia
Authors: Hoffman Stephen J. , Robinson William A.
Published: February 2007

The use of chemical agents that induce differentiation of malignant cells to normal cells has held great promise as an adjunct to standard chemotherapy. In vitro data has shown that 13‐cis‐retinoic acid can differentiate certain leukemia cell lines (e.g., HL‐60) into stable granulocyte cells. In this study, oral 13‐cis‐retinoic acid was administered to four patients with the myelodysplastic syndrome (MDS) and to four patients with acute nonlymphocytic leukemia (ANLL). None of the MDS patients showed an hematologic response to the drug, while three of four ANLL patients responded with normalized peripheral blood counts. The side effects of the drug at 80‐120 mg/d (dry skin, cheilitis, epistaxis) were self limiting.
Unmasking of cryptic natural killer (NK) cell recognition sites on chronic lymphocytic leukemia lymphocytes
Authors: Spitz Daniel L. , Zucker‐Franklin Dorothea , Nabi Zeenat F.
Published: February 2007

The sensitivity of chronic lymphocytic leukemia (CLL) lymphocytes to attack by natural killer (NK) cells has remained questionable. To clarify this issue, freshly isolated lymphocytes of 37 patients with B‐CLL, five with WDLL and two with HCL, were tested with a standard cytotoxicity assay with NK cells from normal donors. All these targets were resistant to cytolysis by the effectors. Freeze‐fracture analysis of CLL cell plasma membranes revealed that they have a larger number of intramembranous particles (IMP) associated with the external leaflet (E‐face) than have normal lymphocytes. Unlike other neoplastic cells, exposure of CLL lymphocytes to phorbol esters or treatment with neuraminidase did not render them vulnerable to attack by NK cells, nor did 5 days of culture have an effect. Incubation of CLL lymphocytes with anti‐lg‐μ (24–72 hr) or with 0.1% pepsin (15 min) resulted in 15% and 27% cytolysis, respectively. B‐lymphocytes from the blood of healthy donors were not killed when treated similarly: These data establish that freshly isolated B‐CLL lymphocytes are resistant to NK cytolysis but that in contrast to normal B‐cells, they possess cryptic NK‐recognition structures, which may be uncovered by surface modulation.
Spontaneous mutation in the male gamete as a cause of hemophilia A: Clarification of a case using DNA probes
Authors: Howard Phillip L. , Hoag Jennifer B. , Bovill Edwin G. , Heintz Nicholas H.
Published: February 2007

The carrier status of two sisters of the mother of a hemophilic boy was clarified by the use of DNA probes in a family with a single case of hemophilia A and no family history of the disease. The extragenic polymorphic site demonstrated by probe DX13 (locus DXS 15) and the intragenic polymorphic site demonstrated by Bgl I digestion and a factor VIII partial cDNA probe indicated that the mother of the index case carried a mutation in the X‐chromosome received from her nonhemophilic father rather than the X‐chromosome received from her mother. In spite of equivocal coagulation data, the mother's two sisters were shown not to be carriers of hemophilia A.
Hemoglobin J Guantanamo [α2β2 128 (H6) Ala→Asp] in association with hemoglobin C and α‐thalassemia in a family from Benin
Authors: Wajcman H. , Baudin‐Chich V. , Kister J. , Feo C. , Gombaud‐Saintonge G. , Bohn B. , Marden M. , Pagnier J. , Poyart C. , Dodé C. , Galacteros F. , Blouquit Y. , Cynober T. , Tchernia G.
Published: February 2007

Hemoglobin J (HbJ), Guantanamo, which had been described but once in the literature, was found in a tamily originating from Benin; this second case was found to be in association with hemoglobin C (HbC) and α‐thalassemia. High‐performance liquid chromatography (HPLC) procedures and microsequencing were used for characterization of the aminoacid substitution. The main hematological disorder, in relation with the instability of Hb J Guantanamo, seems to be a worsening of the rheological properties of the red blood cells (RBC), as demonstrated by ektacytometric studies. Oxygen‐binding properties of the RBC were almost normal, but a slight decrease in cooperativity and lowered Bohr and 2,3‐diphosphoglycerate (DPG) effects were observed for pure stripped Hb J Guantanamo. The expression of the electrophoretic charge difference was partly masked, as is often observed when the structural abnormality is situated in or near a contact area.
Two homozygous cases of erythrocyte pyruvate kinase (PK) deficiency in Japan: PK sendai and PK shinshu
Authors: Tani Kenzaburo , Fujii Hisaichi , Takahashi Keisuke , Ogura Hiromi , Kanno Hitoshi , Hayasaka Kiyoshi , Narisawa Kuniaki , Nakahata Tatsutoshi , Akabane Taro , Morisaki Takayuki , Tsutsumi Hisashi , Miwa Shiro
Published: February 2007

Two new erythrocyte pyruvate kinase (PK) variants with severe nonspherocytic hemolytic anemia are presented. These cases are both considered to be homozygous because of the consanguineous marriages in their parents. Their erythrocyte PK's were characterized by the recommended methods of the International Committee for Standardization in Haematology (ICSH). These two variants have been named PK Sendai and PK Shinshu. PK Sendai showed a high K0.5S(phosphoenolpyruvate), was remarkably inhibited by ATP, and was thermolabile, while PK Shinshu demonstrated remarkably low enzyme activity and required a high level of fructose 1,6‐diphsphate for activation.
Significance and limits of cerebrospinal fluid beta‐2‐microglobulin measurement in course of acute lymphoblastic leukemia
Authors: Musto Pellegrino , Tomasi Paolo , Cascavilla Nicola , Ladogana Saverio , La Sala Antonio , Melillo Lorella , Nobile Michele , Castoldi Gianluigi , Carotenuto Mario
Published: February 2007

Cerebrospinal fluid beta‐2‐microglobulin (CSF‐β2m) was measured longitudinally in 48 patients affected by acute lymphoblastic leukemia (ALL). Thirteen developed a central nervous system (CNS) involvement during the course of the disease; although moderately higher mean CSF‐β2m levels were found in these subjects, no significant statistical differences were observed in comparison with patients without this complication and compared with the control group. No correlations were found between β2m and other biochemical parameters in CSF. Furthermore, CSF‐β2m levels appeared to be influenced by previous combined chemoradiotherapeutic treatment for CNS prophylaxis, presence of meningeal non‐neoplastic infiltrates, patients' ages, amount of CSF blasts, and their immunological phenotype. In particular, only clearly B‐committed leukemic cells, when tested, showed a strong surface expression of β2m, as demonstrated by immunocytochemical detection of this protein on cell membrane. However, in specific cases, CSF β2m measurement and CSF/serum β2m ratio were helpful in diagnosing and monitoring isolated CNS disease. Such findings suggest that CSF‐β2m assay may be a useful tool in the management of CNS involvement in the course of ALL in only selected patients, as several factors can modify the outcome.
Successful outcome of severe aplastic anemia following epstein‐barr virus infection
Authors: Grishaber Judy E. , McClain Kenneth L. , Mahoney Donald H. , Fernbach Donald J.
Published: February 2007

Severe aplastic anemia has been reported in children following Epstein‐Barr virus (EBV) infection. The pathophysiology of disease is not known, but an immunologic mechanism is suspected because of the rapid response to steroids. A patient with severe aplastic anemia following EBV infection experienced bone marrow recovery after treatment with high‐dose prednisone. After reviewing the literature it is concluded that steroid therapy is indicated in this select group of patients.
CD5 positive immunoregulatory B cell subsets
Authors: Paglieroni Teresa , Caggiano Vincent , Mackenzie Malcolm
Published: February 2007

B‐chronic lymphocytic leukemia (B‐CLL) is a heterogeneous disease often expressed as a clonal expansion of CD5+ B cells. We report the characterization of CD5+ B cells from two unique B‐CLL patients. Cells from patient 1 coexpressed CD5 (leu‐1), CD19 (Leu‐12), CD20 (B1), and HLA‐DR; they were CD10 (J5), CD21 (B2), CD22 (Leu‐14), CD25 (IL2‐R1), PCA‐1, surface, and cytoplasmic lg negative. They suppressed normal peripheral blood lymphocyte (PBL) pokeweed mitogen (PWM) ‐stimulated Immunoglobulin (lg) synthesis >80%. Cells from patient 2 were CD5 (Leu‐1), CD19 (Leu‐12), CD20 (B1), CD21 (B2), CD22 (Leu‐14), HLA‐DR, IgM, and kappa positive. They were negative for CD10 (J5), CD25 (IL2‐R1), and PCA‐1. These cells did not suppress normal PBL PWM‐stimulated lg synthesis but produced a monoclonal IgM kappa protein with rheumatoid factor‐like activity. These observations suggest that there are different CD5+ B cell subsets, one immunosuppressive and the other autoreactive.