bluuebunny/crossref_metadata_2025_split huggingface dataset:
CD5‐Negative, CD10‐Negative small B‐cell leukemia: Variant of chronic lymphocytic leukemia or a distinct entity?
Authors: Sheikh Salwa S. , Kallakury Bhaskar V.S. , Al‐Kuraya Khawla A. , Meck Jeanne , Hartmann Dan P. , Bagg Adam 
Published: November 2002

AbstractCD5‐ and CD10‐negative chronic lymphocytic leukemias are quite uncommon as compared to the CD5‐positive CLL. We reviewed 250 sequential cases of peripheral blood lymphocytosis to characterize cases of small B‐cell lymphoproliferative disorders, submitted with a clinical diagnosis of chronic lymphocytic leukemia exhibiting a non‐classic immunophenotypic profile. Six cases of CD5‐, CD10‐negative chronic lymphocytic leukemias and no tissue involvement were identified that revealed high‐density surface‐membrane immunoglobulin and CD20 expression, with variable expression of CD11c, CD23, and CD25. Most had a profound leukocytosis (mean WBC 180 × 109/L) with proliferation of mature‐appearing lymphocytes. Subsequent bone marrow biopsies showed diffuse infiltration by neoplastic cells in all evaluated patients. The clinical course appeared indolent, with follow‐up revealing three patients alive (survival time 38–68 months), while two died of unrelated causes and one was lost to follow‐up soon after diagnosis. These cases may represent somewhat unusual chronic lymphoproliferative disorders, with morphologic features and immunophenotypic profile not readily classifiable, but which are certainly atypical for classic chronic lymphocytic leukemia. Some of these features are reminiscent of those seen in marginal‐zone lymphoma. However, it is most unusual for this known to be tissue‐based disease to present primarily as leukemia rather than lymphoma. Am. J. Hematol. 71:306–310, 2002. © 2002 Wiley‐Liss, Inc.
Prevalence of factor V G1691A (factor V‐Leiden) and prothrombin G20210A gene mutations in a recurrent miscarriage population
Authors: Finan Ramzi R. , Tamim Hala , Ameen Ghada , Sharida Huda E. , Rashid Mooza , Almawi Wassim Y. 
Published: November 2002

AbstractFactor V G1691A (FV‐Leiden) and prothrombin G20210A mutations are major inherited risk factors for venous thrombosis. Recently, it was suggested that both mutations, through stimulation of venous and placental thrombosis events, were strongly associated with recurrent idiopathic miscarriages, although other studies disputed such a link. The aim of this study was to determine the prevalence of prothrombin G20210A and factor V G1691A (R506Q, FV‐Leiden) mutations in women with recurrent idiopathic abortions and to recommend management for high‐risk mutation carriers. One hundred ten women with two or more consecutive unexplained first‐trimester miscarriages (mean age ± SD, 32.3 ± 5.3) were compared to 67 parous women with uncomplicated pregnancies (mean age ± SD, 33.9 ±7.3) (P = 0.134) from the same ethnic background. The presence or absence of the prothrombin G20210A and FV‐Leiden mutations was assessed by PCR and RFLP analysis, using HindIII and MnlI digestion, respectively. In women with primary habitual abortion, 45 (40.91%) carried the FV‐Leiden mutation, of whom 7 were in the homozygote and 38 were in the heterozygote states, and 15 (13.64%) carried the prothrombin G20210A mutation all as heterozygotes, compared to 16.42% and 2.99% carrier rates among controls, respectively, all of whom were heterozygote carriers. Of the other risk factors analyzed, smoking (OR 1.76; 95% CI = 0.79–3.94) was more prevalent in habitual aborters compared to controls. Both FV‐Leiden and factor II G20210A mutations are major inherited risk factor associated with primary recurrent miscarriages. Women with a family or personal history of thrombosis should be screened before or early in the pregnancy for FV‐Leiden and factor II G20210A mutations. Am. J. Hematol. 71:300–305, 2002. © 2002 Wiley‐Liss, Inc.
Factor XIII deficiency mistaken for battered child syndrome: Case of “correct” test ordering negated by a commonly accepted qualitative test with limited negative predictive value
Authors: Newman Richard S. , Jalili Mehrdad , Kolls Bradley J. , Dietrich Rosalind 
Published: November 2002

AbstractWe report herein a case of Factor XIII deficiency that remained undiagnosed until 2 years of age. Part of the delay in diagnosis was a consequence of testing that was performed on a blood sample obtained after plasma transfusion therapy for a life‐threatening bleeding episode. Due to insufficient family follow‐up after discharge from the hospital, the diagnosis was delayed 1 year until the child was rehospitalized and a pre‐transfusion plasma sample was tested. The commonly accepted approach of using only a qualitative test for the diagnosis of factor XIII deficiency is challenged by this case report. Am. J. Hematol. 71:328–330, 2002. © 2002 Wiley‐Liss, Inc.
Chronic lymphocytic leukemia presenting in association with aplastic anemia
Authors: Zonder Jeffrey A. , Keating Michael , Schiffer Charles A. 
Published: November 2002

AbstractAn unusual case of chronic lymphocytic leukemia presenting in association with aplastic anemia is discussed, along with a review of the few previously reported cases. Possible humoral and cellular autoimmune mechanisms are examined as possible causes of marrow aplasia in this setting. Am. J. Hematol. 71:323–327, 2002. © 2002 Wiley‐Liss, Inc.
Additional chromosomal abnormalities and variability of BCR breakpoints in Philadelphia chromosome/BCR‐ABL‐positive acute lymphoblastic leukemia in Taiwan
Authors: Ko Bor‐Sheng , Tang Jih‐Lu , Lee Fen‐Yu , Liu Ming‐Chi , Tsai Woei , Chen Yao‐Chang , Wang Chiu‐Hwa , Sheng Ming‐Chin , Lin Dong‐Tsam , Lin Kai‐Hsin , Tien Hwei‐Fang 
Published: November 2002

AbstractFrom 1986 to 1998, 26 (23%) of 114 adult acute lymphoblastic leukemia (ALL) patients and 11 (4%) of 328 pediatric patients were found to have Philadelphia (Ph) chromosome. In the 30 patients with available data at diagnosis, 18 (60%) had extra‐chromosomal abnormalities. They included 1q duplication (5/18, 28%), supernumerary Ph chromosome (4/18, 22%), 9p abnormalities (3/18, 17%), 7q deletion/monosomy 7 (3/18, 17%), trisomy 19 (1/18, 6%), and trisomy 8 (1/18, 6%). Excluding those with specific cytogenetic changes, only one patient had hyperdiploid karyotype with more than 50 chromosomes. The incidence of 1q duplication was higher and that of hyperdiploidy was lower in this study than has been previously reported. There was no prognostic implication of these additional cytogenetic abnormalities. With fluorescence in situ hybridization (FISH) and reverse transcription‐polymerase chain reaction (RT‐PCR), 14 (27%) of 53 unselected adult ALL patients and 2 (5%) of 38 unselected pediatric patients were BCR‐ABL‐positive, including one adult and two children without Ph chromosome. The BCR‐ABL fusion genes/transcripts were also present in all other 16 selected Ph‐positive ALL patients. The BCR‐ABL fusion subtypes were determined in all these 32 patients: 91% (11/12) childhood cases showed m‐type fusion gene while 45% (9/20) adult ones did so (P = 0.0083). The clinical outcome was similar between the two groups of patients with m‐type and M‐type BCR‐ABL. In conclusion, both cytogenetic and molecular studies are very helpful for identifying the subgroup of ALL patients with Ph/BCR‐ABL. The additional cytogenetic abnormalities and subtypes of BCR‐ABL fusion genes/transcripts had no significant implications in this group of patients. Am. J. Hematol. 71:291–299, 2002. © 2002 Wiley‐Liss, Inc.
Successful treatment of a young infant who developed high‐titer inhibitors against VWF‐cleaving protease (ADAMTS‐13): Important discrimination from Upshaw‐Schulman syndrome
Authors: Ashida Akira , Nakamura Hyogo , Yoden Atsushi , Tamai Hiroshi , Ishizashi Hiromichi , Yagi Hideo , Matsumoto Masanori , Fujimura Yoshihiro 
Published: November 2002

AbstractWe report herein the case of a 9‐month‐old female infant with acquired thrombotic thrombocytopenic purpura (TTP), which was initially suspected to be either Upshaw‐Schulman syndrome (USS or a congenital TTP) or hemolytic uremic syndrome (HUS) because of onset of clinical signs in infancy and accompanying diarrhea. She received combination therapy of plasma exchange, steroid pulse, and high‐dose intravenous immunoglobulin infusion that was initiated before the definitive diagnosis, which resulted in excellent clinical improvement. The retrograde analysis of plasma ADAMTS‐13 activity and its inhibitor showed a lack of this enzyme activity and the presence of a high‐titer IgG inhibitor (200–320 Bethesda units/mL) to this enzyme activity. From our experience, it was suggested that we should recognize the possibility of the patient with acquired TTP in infancy and the importance of plasma exchange therapy for management of its clinical symptoms. Am. J. Hematol. 71:318–322, 2002. © 2002 Wiley‐Liss, Inc.
Prospective study of the G20210A polymorphism in the prothrombin gene, plasma prothrombin concentration, and incidence of venous thromboembolism
Authors: Folsom Aaron R. , Cushman Mary , Tsai Michael Y. , Heckbert Susan R. , Aleksic Nena 
Published: November 2002

AbstractCase–control studies have indicated increased risk of venous thrombosis associated with the prothrombin gene G20210A polymorphism and with elevated plasma prothrombin levels. We sought to confirm these results in a prospective population‐based study of 21,690 persons. We measured G20210A and prothrombin antigen on pre‐event blood samples of 302 participants who developed venous thromboembolism (VTE) and 626 participants who remained free of VTE. Approximately 4.0% of cases and 2.4% of controls carried the G20210A polymorphism, but only one of 137 African Americans did. The odds ratio in whites was 1.87 (95% CI = 0.85, 4.11)—higher for those who reported a prior history of VTE (OR = 5.44) than those reporting no VTE history (OR = 1.41) and in those with idiopathic VTE (OR = 2.51) than those with secondary VTE (OR = 1.38). There was no association between venous thromboembolism and plasma prothrombin antigen level. We estimated that the G20210A polymorphism may account for approximately 2.5% of venous thromboembolism events in United States whites. Am. J. Hematol. 71:285–290, 2002. © 2002 Wiley‐Liss, Inc.
Novel nonsense mutation in the platelet glycoprotein Ibβ gene associated with Bernard‐Soulier syndrome
Authors: Kunishima Shinji , Matsushita Tadashi , Ito Takahiko , Kamiya Tadashi , Saito Hidehiko 
Published: November 2002

AbstractBernard‐Soulier syndrome (BSS) is an autosomal recessive bleeding disorder caused by quantitative or qualitative abnormalities in the glycoprotein (GP) Ib/IX/V complex, the platelet receptor for von Willebrand factor. This complex is composed of four subunits, GPIbα, GPIbβ, GPIX, and GPV, and the coordinated assembly of GPIbα, GPIbβ, and GPIX is required for the efficient surface expression of a functional complex. We report here a novel nonsense mutation of the GPIbβ gene associated with BSS. Flow cytometric analysis of the patient's platelets showed markedly reduced GPIbα and absent GPIX surface expression. Immunoblot analysis of solubilized platelets showed that a small amount of GPIbα was detected; however, GPIbβ and GPIX were undetectable. DNA sequencing analysis revealed a novel nonsense mutation of the GPIbβ gene that converts Trp (TGG) to a stop codon (TAG) at residue 123. Transient transfection studies revealed that the mutant GPIbβ polypeptide was not detected in the transfected 293T cells, suggesting that null expression of the mutant GPIbβ impairs expression of the GPIbα and GPIX subunits and results in a BSS phenotype in the patient. Am. J. Hematol. 71:279–284, 2002. © 2002 Wiley‐Liss, Inc.
Serum α‐fetoprotein level in Fanconi's anemia: Evaluation of 33 Turkish patients
Authors: Aslan Deniz , Gümrük Fatma , Alikaşifoğlu Mehmet , Altay Çiğdem 
Published: November 2002

AbstractRecently, measurement of serum α‐fetoprotein (sAFP) was introduced as a preliminary test for diagnosis of Fanconi's anemia (FA). In the present study, sAFP levels were measured in order to determine its sensitivity and specificity in 33 Turkish FA patients (17 males and 16 females) with a mean age of 11.6 ± 7.7 (1.0–28.0) (median 10.0). Complementation groups were available in 12 patients. Nineteen age‐matched healthy children, 17 patients with bone marrow failure syndromes, 37 FA heterozygotes, and 37 children with acute leukemia served as negative control groups. The sAFP was measured by particle immunoassay. The level of sAFP was found to be higher than the cut‐off value, 8 IU/mL in 46% and was within normal limits in 54% of the FA patients. The AFP values were within normal limits in all of the subjects belonging to the control groups. This method provided 46% sensitivity and 100% specificity in the diagnosis of FA. The sAFP values were high in 4 of 17 (24%) FA patients who did not receive any androgen therapy, while the sAFP level was high in 7 of 9 (78%) patients who received such a therapy. The statistical analysis of incidence of a high sAFP level between these two groups indicated a significant difference (P = 0.014), suggesting that androgen therapy might be a contributing factor for elevation of sAFP. The comparison of several clinical and laboratory parameters between FA patients with high and normal levels of AFP revealed no statistically significant differences. The level of sAFP was elevated in only 5 of the 11 patients with complementation group A; in addition, variable levels of sAFP were noted among the affected members in 4 families, indicating that complementation groups, type of mutation, or familial factors were not responsible for elevation of sAFP. Am. J. Hematol. 71:275–278, 2002. © 2002 Wiley‐Liss, Inc.
Aplastic anemia in Brazil: Incidence and risk factors
Authors: Maluf Eliane M.C.P. , Pasquini Ricardo , Eluf Jose N. , Kelly Judith , Kaufman David W. 
Published: November 2002

AbstractThis study is the first large‐scale epidemiological investigation of acquired aplastic anemia (AAA) in South America. The objective was to estimate the incidence and to identify risk factors for AAA in Brazil. A national case–control study was conducted to investigate the risk factors for the disease. One hundred twenty‐five cases and 129 controls were included. Multiple logistic regression was used in the estimation of odds ratios (OR) to control confounding. The size of Brazil made it unfeasible to estimate the incidence of AAA in the whole country, and we limited the calculation to the state of Parana. The annual incidence of AAA in Parana was 2.4 cases/106 inhabitants. There was no positive association between chloramphenicol use and AAA (OR 0.4; 95% CI: 0.1–2.9). The OR of AAA associated with household pesticides that include organophosphates in their composition was 2.7 (1.0–8.4). The OR for the usage of unspecified thinner and/or acetone for at least 7 days was 3.0 (1.2–7.3). Cases of AAA in Brazil seem to be associated with some factors traditionally related to this disease, such as certain solvents and the incidence is similar to what has been reported from Europe. Am. J. Hematol. 71:268–274, 2002. © 2002 Wiley‐Liss, Inc.
Urokinase could increase the yield of progenitor cells during red cell depletion in elapsed and anticoagulated cord blood
Authors: Lee Young‐Ho , Han Jin‐Yeong , Seo Su‐Yeong , Kim Kyeong‐Hee , Lee Young‐Ah , Lee Hyung‐Sik , Hur Woo‐Joo , Han Hun , Kwon Hyuk‐Chan , Kim Jae‐Seok , Kim Hyo‐Jin 
Published: November 2002

AbstractWe assessed whether the urokinase could increase the yield of progenitor cells during processing in elapsed, anticoagulated cord blood (CB) after collection, and we also determined the optimal dose of urokinase. The total nucleated cell (TNC) counts after red cell depletion in 48‐hr‐elapsed CB were significantly higher in samples treated with 10,000 and 50,000 IU of urokinase/mL than in untreated samples or treated with 5,000 IU of urokinase/mL. The CD34+ cell counts were significantly higher in samples treated with 10,000 IU of urokinase/mL than in untreated samples and in samples treated with 5,000 or 50,000 IU of urokinase/mL. In 6‐, 12‐, and 24‐hr‐elapsed CB, however, there were no significant differences of TNC, CD34+ cells, or CFU‐GM counts between untreated samples and samples treated with 10,000 IU of urokinase/mL. These findings suggest that the addition of 10,000 IU of urokinase/mL before red cell depletion in 48‐hr‐elapsed, anticoagulated CB could increase the yield of progenitor cells. However, there are no advantages in using urokinase for processing CB prior to 24 hr after collection. Am. J. Hematol. 71:336–339, 2002. © 2002 Wiley‐Liss, Inc.
Randomized trial of fluconazole versus low‐dose amphotericin B in prophylaxis against fungal infections in patients undergoing hematopoietic stem cell transplantation
Authors: Koh L.P. , Kurup A. , Goh Y.T. , Fook‐Chong S.M.C. , Tan P.H.C. 
Published: November 2002

AbstractOver the past decade, invasive fungal infections have become an increasingly important problem in patients undergoing hematopoietic stem cell transplantation (HSCT). The optimal approach for prophylactic antifungal therapy has yet to be determined. To resolve this issue, we performed a prospective randomized study to compare the efficacy of fluconazole (FL) versus low‐dose amphotericin B (AmB) in preventing fungal infections during the first 100 days after HSCT. Patients undergoing allogenic or autologous HSCT were randomized to receive fluconazole 200 mg/day PO or amphotericin B 0.2 mg/kg/day IV beginning 1 day prior to commencement of conditioning regimen and continuing until engraftment, drug‐associated toxicity was suspected, or systemic fungal infection was suspected or proven. High‐dose amphotericin B (0.5–1.0 mg/kg/day) was started for patients with suspected or proven fungal infections. From January 1993 to December 1998, a total of 186 patients were enrolled into the trial, with 100 receiving FL and 86 receiving AmB. Eighty (43%) patients were removed from prophylaxis for persistent fever despite broad‐spectrum antibacterial therapy or suspected fungal infections (FL 46 vs. AmB 34, P > 0.05). The incidence of proven fungal infections (FL 12% vs. AmB 12.8%), suspected fungal infections (FL 4% vs. AmB 2.3%), superficial fungal infections (FL 1% vs. AmB 4.6%) did not show any significant difference. The survival at 100 days post transplant was similar between the 2 groups (FL 78% vs. AmB 70%, P = 0.254). Death attributable to fungal infections was similar in both groups (6% vs. 7%, P > 0.05). We conclude that fluconazole is as effective as low‐dose amphotericin B in prophylaxis against fungal infections in patients undergoing hematopoietic cell transplantation. Am. J. Hematol. 71:260–267, 2002. © 2002 Wiley‐Liss, Inc.
Conservative treatment for patients over 80 years with acute myelogenous leukemia
Authors: Latagliata Roberto , Alimena Giuliana , Carmosino Ida , Breccia Massimo , Borza Paola Anticoli , Bongarzoni Velia , Copia Carolina , Spadea Antonio , Pinazzi Beatrice , Frattarelli Natalia , Ferrara Felicetto , Petti Maria Concetta , Mandelli Franco 
Published: November 2002

AbstractIn order to evaluate the best treatment of very elderly patients with AML, we have retrospectively analyzed 60 cases of patients aged more than 80 years, with a diagnosis of AML and observed from January 1988 to December 1998. Six of these patients were subsequently referred to other centers; of the remaining 54 patients, 20 (37%) received only supportive care, whereas 34 (63%) required palliative chemotherapy to control leukocytosis, after a median time from diagnosis of 9 days (range 0–253). Median overall survival was 13 weeks (range 1–105): 21 (39%) and 6 (11%) patients survived more than 6 and 12 months, respectively. Twenty‐eight patients (51.8%) died from progressive disease, 19 (35.1%) died from AML‐related or unrelated causes in the phase of stable disease, while in 7 patients the cause of death was unknown. In univariate analysis, PS > 2 and WBC > 50 × 109/L had an adverse prognostic significance on survival. Our results, as compared with those reported in the literature for patients over 80 years treated with intensive chemotherapy, support the idea that intensive chemotherapy is usually not indicated in very elderly patients with AML, and that conservative treatment and the primary strategy of “watch‐and‐wait” presently seems to be the best choice. Am. J. Hematol. 71:256–259, 2002. © 2002 Wiley‐Liss, Inc.
Cefepime or carbapenem treatment for febrile neutropenia as a single agent is as effective as a combination of 4th‐generation cephalosporin + aminoglycosides: Comparative study
Authors: Tamura K. , Matsuoka H. , Tsukada J. , Masuda M. , Ikeda S. , Matsuishi E. , Kawano F. , Izumi Y. , Uike N. , Utsunomiya A. , Saburi Y. , Shibuya T. , Imamura Y. , Hanada S. , Okamura S. , Gondoh H. 
Published: November 2002

Abstract1998, a consensus meeting was held in Miyazaki, Japan, to develop an approach to management of febrile neutropenia (FN). The K‐HOT study group decided to examine whether this proposal was applicable to clinical practice in a multicenter study. Patients who developed fever with neutrophil counts <1,000/μL were randomized to receive either a single antibiotic, cefepime or one of the carbapenems, or a combination of cefepime and an aminoglycoside. Patients who became afebrile within the first 3 days were continued on the same treatment. Patients who remained febrile were switched to a combination regimen if they were randomized to receive a single agent, and patients on combination medication were changed from cefepime to another cephalosporin. A total of 165 patients were entered into the trial. One hundred fifty‐three patients were evaluable for response. The average age was 52 years, and 70% of the patients had acute leukemia. Severe neutropenia, defined as <100/μL at the time of FN, was seen in 62% of the patients on entry and during the course of treatment 71% of patients experienced neutrophil counts of <100/μL. Microbiologically documented infection was seen in 6.5% for monotherapy, and 10.5% for a combination treatment, and fever of unknown origin occurred in 75.3% and 59.2% of the patients in each regimen, respectively. Excellent to good response was seen in two‐thirds of the patients in all treatment groups. Adverse events were minimal, and three early deaths were observed at days 9, 16, and 16 among patients treated with a single antibiotic and three in the combination regimen group at days 14, 15, and 20. These results indicate that cefepime or a carbapenem alone is as effective as a combination of cefepime and an aminoglycoside for treating FN. Am. J. Hematol. 71:248–255, 2002. © 2002 Wiley‐Liss, Inc.
Lymphocyte‐specific murine deubiquitinating enzymes induced by cytokines
Authors: Baek Kwang‐Hyun 
Published: November 2002

AbstractIt is becoming clear that a number of proteins regulating cellular mechanisms for homeostasis in all eukaryotes are controlled not only by phosphorylation and dephosphorylation but also by ubiquitination and deubiquitination. This includes most of oncoproteins and signaling components involved in receptor tyrosine kinase (RTK)‐mediated signal transduction pathways. Like protein phosphorylation and dephosphorylation regulated by kinases and phosphatases, respectively, protein ubiquitination and deubiquitination are very dynamic and are regulated by ubiquitin conjugating enzymes and deubiquitinating (DUB) enzymes. A number of deubiquitinating enzymes have been isolated even though little is known about their biological functions. This review concentrates on recent findings and new insights into DUB enzyme subfamily members in lymphocytes. Am. J. Hematol. 71:340–345, 2002. © 2002 Wiley‐Liss, Inc.