bluuebunny/crossref_metadata_2025_split huggingface dataset:
A Deletion 7q in a M0 Adult Acute Myeloid Leukemia (AML) with a Complete Cytogenetic Response to Idarubicin.
Authors: Domke Lewis , Anderson Norwood R. 
Published: October 2019

Abstract
The alteration of chromosome 7 is associated with a reported unfavorable prognosis in acute myeloid leukemia. The deletion of the 7q is seen alone in approximately 1% of AML cases. This finding is associated with an AML M0, M1, and M2 FAB classification. Treatment with idarubicin is thought to be equivalent to other anthracyclines in the induction of a complete remission in acute myeloid leukemia. In previous studies younger patients have responded with improved complete remission rates, when idarubicin is used. A unique case with a 7q single aberration and an AML M0 FAB classification is reported with a complete and rapid response of less than 32 days after one cycle of idarubicin at a dose of 12 mg /m2/day for three days accompanied by a cytosine arabinoside intravenous infusion at a dose of 100 mg/m2/day for seven days. A complete cytologic and genetic remission was obtained after one cycle of induction. The patient received high dose cytosine arabinoside consolidation and will be considered for autologous stem cell transplant. The patient remains in a complete genetic and cytologic remission. In this 25 year old african american female a complete cytologic and genetic remission by FISH is obtained in a rare acute myeloid leukemia genotype with a reportedly unfavorable prognosis using idarubicin as the anthracycline in the 3+7 combination.
Conclusion: Further studies are required to better define the appropriate anthracycline in acute myeloid leukemia by using age and cytogenetic markers. In this rare case presentation, idarubicin is shown to be effective in inducing a complete and rapid cytogenetic remission in a young adult patient with a chromosome 7 cytogenetic aberration and an unfavorable prognosis.
Detection of Aspergillus Species in Blood of Malignant Hemotopoietic Tumor Patients by Two-Step PCR.
Authors: Qian Wenbin , Liu Junqing , Jin Jie 
Published: October 2019

Abstract
Objective To investigate the sensitivity and specificity of two-step PCR in detection of aspergillus species in blood from malignant hemotopoietic tumor patients. Methods Forty-one blood samples from patients at high-risk for invasive fungal disease were detected by two-step PCR. The clinic applicability of two-step PCR for systemic aspergillosis diagnosis was assessed by analyzing computed tomography, aspergillus culture, neutropenia and outcome after anti-fungal therapy. Results Specific band of PCR was obtained from aspergillus strains and the products of PCR had a high homogeneous in sequence with aspergillus species. Sixteen of 41 patients were PCR positive, aspergillosis was proven in 4 of these patients by the results of culture. The controls (12 healthy) were all PCR negative. The eight patients (of the 16 PCR positive patients) had characteristic high-resolution computed tomography findings that suggested invasive aspergillosis. The minimum value of white blood cell (WBC) was 0.3×109/L±0.14×109/L in PCR positive patients, whereas, was 0.5×109/L ±0.26×109/L in PCR negative patients (p=0.0326). The duration with WBC count less than 1.0×109/L was 19±8.313 days and 12.69±6.945 days respectively between two groups (p=0.0472). After antifungal therapy, one of the patients with PCR positive died of chemotherapy-related early death. Conclusions The two-step PCR assays allows for the highly sensitive and specific detection of aspergillus pathogens in vitro and in vivo. A specific two-step PCR assay for the rapid detection of the aspergillus species in blood samples improves early diagnosis for systemic aspergillosis and helps us to make decision for antifungal therapy.
Essential Role of Jun Family Transcription Factors in PU.1-Induced Leukemic Stem Cell Transformation.
Authors: Steidl Ulrich , Rosenbauer Frank , Verhaak Roel G.W , Gu Xuesong , Otu Hasan H. , Kolia Shakir , Owens Bronwyn M. , Klippel Steffen , Wagner Katharina , Aivado Manuel , Passegue Emmanuelle , Libermann Towia A. , Delwel Ruud , Tenen Daniel G. 
Published: October 2019

Abstract
Knockdown of the expression of the myeloid master regulator PU.1 leads to the development of an immature acute myeloid leukemia (AML) in mice. Recent reports suggest that functional inactivation of PU.1 might also play a role in human AML. However, the molecular mechanisms underlying PU.1-mediated malignant transformation are unknown. We examined leukemic PU.1 knockdown mice and found a 3-fold expansion of lin-, c-kit+, Sca1+ (KLS) hematopoietic stem cells (HSC) as compared to wildtype controls, which was not observed during the preleukemic phase. When we transplanted double-sorted leukemic KLS-HSC into NOD-SCID mice the recipients developed AML after 9–12 weeks indicating that the leukemic stem cells derive from the HSC compartment. This finding prompted us to examine the transcriptome of PU.1 knockdown preleukemic HSC to identify early transcriptional changes underlying their malignant transformation. After lineage-depletion and FACS sorting of preleukemic KLS-HSC we performed linear amplification of RNA by 2 cycles of RT-IVT and hybridized the cRNA with Affymetrix Mouse Genome 430 2.0 arrays. Principal component analysis as well as hierarchical cluster analysis clearly distinguished PU.1 knockdown and wildtype HSC. Several in-vitro targets of PU.1 such as c-Fes, BTK, TFEC, CSF2R, and Ebi3 were downregulated demonstrating that those are also affected in HSC in vivo. Differential expression of 16 genes was corroborated by qRT-PCR. Strikingly, several Jun family transcription factors including c-Jun and JunB were downregulated. Retroviral restoration of c-Jun expression in bone marrow cells of preleukemic mice rescued the PU.1-initiated myelomonocytic differentiation block in this early phase. To target cells in the leukemic stage we applied lentiviral vectors expressing c-Jun or JunB. While c-Jun did not affect leukemic proliferation, lentiviral restoration of JunB led to an 80% reduction of clonogenic growth and a loss of leukemic self-renewal capacity in serial replating assays. Expression analysis of 285 patients with AML confirmed the correlation between PU.1 and JunB downregulation and suggests its relevance in human disease. These results delineate a transcriptional pattern that precedes leukemic transformation in PU.1 knockdown HSC and demonstrate that downregulation of c-Jun and JunB contribute to the development of PU.1-induced AML by blocking differentiation (c-Jun) and increasing self-renewal (JunB). Therefore, examination of disturbed gene expression in preleukemic HSC can identify genes whose dysregulation is essential for leukemic stem cell function and are potential targets for therapeutic interventions.
Common Variable Immunodeficiency Presenting with Hodgkin’s Disease.
Authors: Ellwood Amanda , Hamm Caroline 
Published: October 2019

Abstract
We report a case of a 32 year old man with classic Common Variable Immunodeficiency Disease (CVID) and Hodgkin’s disease. Repeated immunoglobulin studies as a child showed hypogammaglobulinemia and decreased mitogen responses, with the suggestion of CVID. He presented to the oncology clinic with nearly three decades of recurrent reactive lymphadenopathy and multiple lymph node biopsies throughout his lifetime, recurrent pulmonary infections, and multiple autoimmune manifestations including diabetes mellitus onset at age 4, autoimmune hemolytic anemia and autoimmune thrombocytopenia. He developed Hodgkin’s disease at the age of 30, which has not previously been described in CVID. We also report two other cases of CVID with the diagnosis of non-Hodgkin’s lymphoma in the same oncology practice. CVID is a relatively common and yet rarely identified syndrome. Specific treatment is recommended. We suggest that oncologists should be aware of this unusual and yet relatively common disorder that carries up to 400 times the risk for the development of lymphoma (Cunningham-Rundles, C, et al. J Clin Immunol1987; 7:294–299).
Tumor Burden Related Immunodeficiency in Lymphoma Patients and Clinical Course.
Authors: Jentsch-Ullrich Kathleen , Gerber Annegret , Reppin Regina , Koenigsmann Michael , Mohren Martin , Franke Astrid 
Published: October 2019

Abstract
The association of lymphoma with a plethora of secondary immune defects is well established. However, most investigations were performed many years ago and little is known about disease specific alterations of the immune system. From January 2003 to August 2004 50 patients with newly diagnosed malignant lymphoma were analyzed and compared with 50 healthy volunteers Corresponding in age and gender. Parameters of cellular and humoral immunity, including CBC, FACS analysis of lymphocyte subpopulations, cytokines (sIL2-R, IL-6, IL-10, TNF-a), serum complement components C3c, C4, CH50 and total concentrations of serum immunoglobulin IgG, IgM, IgA were measured. The median number of CD3+ and CD4+ lymphocyte subsets (p≤0.01) were significantly lower in lymphoma patients compared with matched healthy volunteers. The number of CD8+ T-cells was decreased in patients with bulky disease (p≤0.01). High-intermediate/high risk lymphoma (IPI 3-4 or aaIPI 2-3) had significantly higher CH50 (p=0,02), C3c (p≤0,01), sIL2-R (p≤0,01) and TNF-a levels (p=0,05) than patients with low/low-intermediate risk lymphoma. Infectious complications after chemotherapy (n=14) occurred frequently in patients with high initial LDH (p=0,04), low number of CD4+ (p=0,02) or CD8+ T-cells (p=0,04) and high levels of sIL2-R (p=0,02) or TNF-a (p=0,03). Relapse (n=6) was also observed in patients with decreased CD8+ T-cells (p=0,04) and in patients with high C4 levels (p=0,02). These results indicate that tumor burden in lymphoma patients significantly influenced the number of lymphocyte subsets, serum cytokine levels (sIL2R, TNF- a) and complement components (CH50, C3c). IPI-score and clinical course (infections and relapse) were related to the immunodeficiency. We conclude that measurement of peripheral blood lymphocyte subsets, cytokines and complement factors can be expected to improve existing methods of risk assignment in lymphoma.
Trends in AIDS-Associated Cancers among Patients Attending a Palliative Care Service in Uganda in the ’Pre-HAART’ Era, 1994–2002.
Authors: Ddungu Henry , Crowther Mark A. 
Published: October 2019

Abstract
Objectives: To describe the pattern of AIDS-associated cancers among patients referred to a palliative care service in the absence of HAART therapy.
Design: A systematic review of patient records as part of an ongoing quality assurance project.
Setting: A primary palliative care service centre for cancer and/or AIDS patients in Kampala district, Uganda.
Participants: All patient charts from 1994 to 2002 that met inclusion criteria (positive HIV test, or an AIDS diagnosing condition and AIDS-associated cancer) were selected.
Results – 657 out of 3,706 (17.7%) patients met inclusion criteria. Fifty-two percent were female and the median age was 33 years. Thirty-nine percent were referred for pain and symptom control, 51.9% for pain control only, 7.0% for symptom control only; and 2.3% were referred for other reasons. Ninety-six percent were in pain and 95% of these had moderate-to-severe pain. Kaposi’s sarcoma was the most common AIDS associated malignancy, accounting for 73.2% of cases followed by invasive cancer of the cervix (15.1%), non-Hodgkin’s lymphoma (6.4%) and squamous cell carcinoma of the conjunctiva (1.8%). 83.4% had their pain well controlled at the time of death.
Conclusions: The number and proportion of patients with Kaposi’s sarcoma declined over the study period. Pain control was achievable in most patients despite restrained resources.
Expression of X-Linked Inhibitor of Apoptosis Protein in Reactive Lymphoid Tissues, Non-Hodgkin’s Lymphomas, and Hodgkin’s Disease.
Authors: Strauchen James A. , Burstein David 
Published: October 2019

Abstract
X-linked inhibitor of apoptosis protein (XIAP) is an important regulator of apoptosis which binds to and inhibits caspases-3, -7 and -9, blocking the caspase 9-mediated apoptosis pathway. This pathway is activated by p53 and DNA damage and may be an important determinant of responsiveness to chemotherapy. Apoptosis also plays a major role in the regulation of follicle center B-cell proliferation and BCL2-mediated inhibition of apoptosis is a key factor in B-cell lymphomagenesis. In this study we examined the expression of XIAP in 65 reactive and neoplastic lymphoid proliferations utilizing a monoclonal antibody to XIAP (#610763 BD Biosciences, San Jose, CA) and immunohistochemistry with avidin-biotin-complex immunoperoxidase technique on formalin-fixed, paraffin-embedded sections. In reactive lymph nodes and tonsils, expression of XIAP was limited to large noncleaved cells in follicle centers (5 of 6 cases). XIAP was absent in plasmacytoma (3 cases) and small lymphocytic lymphoma/chronic lymphocytic leukemia (1 case). XIAP was expressed in follicular lymphoma, predominantly in large noncleaved cells (6 of 9 cases) and in diffuse large B cell lymphoma (11 of 16 cases), including cases of T-cell/histiocyte-rich diffuse large B cell lymphoma (2 cases), primary mediastinal large B cell lymphoma (1 case), and posttransplantation diffuse large B cell lymphoma (1 case). XIAP was consistently expressed in Burkitt and Burkitt-like lymphoma (3 of 3 cases) and anaplastic large cell lymphoma (3 of 3 cases) and in one case of adult T cell leukemia/lymphoma. XIAP was variably expressed in marginal-zone B cell lymphoma, predominantly in large blasts (2 of 4 cases) and in mantle cell lymphoma (2 of 3 cases). XIAP was not detected in peripheral T cell lymphoma, unspecified (1 case), extranodal NK/T cell lymphoma, nasal type (1 case), precursor B cell lymphoblastic leukemia (1 case), or granulocytic sarcoma (1 case). XIAP was consistently expressed in the Reed-Sternberg and mononuclear Reed-Sternberg-variant cells of classical Hodgkin disease (9 of 9 cases) and the L+H Reed-Sternberg-variant cells of nodular lymphocyte predominance Hodgkin disease (3 of 3 cases). XIAP is expressed across a broad range of lymphoproliferative disorders, including classical and nodular lymphocyte predominance Hodgkin disease, diffuse large B cell lymphoma, follicular lymphoma, Burkitt lymphoma, marginal-zone and mantle cell lymphoma, and anaplastic large cell lymphoma. XIAP appears to be selectively expressed in the proliferating elements of these lymphomas. The possible prognostic and therapeutic significance of XIAP expression needs to be determined.
Characterization of New Aberrations in 25 Splenic Marginal Zone B-Cell Lymphomas (SMZBCL) Using Spectral Karyotyping Technique (SKY).
Authors: Baro C. , Astier L. , Salido M. , Espinet B. , Domingo A. , Granada I. , Costa D. , Hernandez J.M. , Florensa L. , Salar A. , Serrano S. , Sole F. 
Published: October 2019

Abstract
BACKGROUND/INTRODUCTION
Splenic Marginal Zone B-Cell Lymphoma (SMZBCL) is a specific low grade small B-cell lymphoma described by the World Health Organization (WHO). SMZBCL has distinct features from other B-cell malignancies, but few studies include a large series of patients. Previous reports suggest that SMZBCL constitute a genetically heterogeneous disease being 7q22-q32 deletions and gains of 3q the most frequent cytogenetic aberrations. Karyotypes are often complex and difficult to interpret. Other recurrent chromosomes involved are 1 and 8. Nevertheless, at this cytogenetic level few studies have been reported and the possible chromosome markers are not still well defined.
OBJECTIVES
The aim of this study is to define karyotypes from patients with SMZBCL by SKY technique.
PATIENTS AND METHODS
Among a series of 138 patients we have selected 25 with undefined karyotype. The diagnosis of SMZBCL was confirmed by morphology, histology, phenotype and clinical data. All samples were obtained from peripheral blood, bone marrow or spleen. The current SKY protocol has been used with minor modifications to improve the spectral image. We analyzed a minimum of ten metaphases per karyotype and they were described according to the International System for Human Cytogenetic Nomenclature (ISCN).
RESULTS
All the studied karyotypes were complex with chromosome aberrations which, in most cases, were not fully characterized by G-banding. SKY has allowed to refine chromosomal aberrations and revealed new translocations which were not defined by conventional cytogenetics. In nine cases, the karyotype remained unchanged after SKY analysis and in the remaining cases, new aberrations were described.
We observed that the most common involved chromosomes in translocations were 14, 6, 13 and 17.
CONCLUSIONS
SKY provides a useful complementary technique to routine conventional cytogenetics in the analysis of lymphomas with complex karyotypes. SKY will permit to reveal new translocations associated with SMZBCL. The following new translocations involving 14q32 were found: t(1;14)(p22;q32), t(1;14)(q21;q32), t(9;14)(?;q32), t(9;14)(9pter-9p21::14q32-14q11::9p24-9qter), t(9;14)(p21;q32), t(10;14)(p12;q32), t(14;19)(q32;q13).
High Expression of Human Leukocyte Formin Protein in T Non-Hodgkin’s Lymphomas and in CD19− Cell Population of Normal Tonsils.
Authors: Favaro Patricia M.B. , Traina Fabiola , Crespo Marta , Bosch Francesc , Brousset Pierre , Delsol George , Costa Fernando F. , Saad Sara T.O. 
Published: October 2019

Abstract
We recently described a new gene, denominated human leukocyte formin (hlf), which was overexpressed in lymphoid malignancies and cancer cell lines. In contrast, a low expression of the hlf protein was observed in lymph node and peripheral blood leukocytes in normal tissues. Interestingly, the hlf protein associates with Akt, a protein kinase of an important pathway for cell survival. In order to better characterize the expression of the hlf protein we performed Western blotting in the lymphocytes isolated from 4 tonsils from adult patients obtained during routine tonsillectomy, at the Department of Hematology, Clinic Hospital, Barcelona. Results demonstrated that the CD19− cell population of the tonsil displayed a higher expression of this protein when compared with CD19+ cells. In addition, CD19+ cells were separated into two subpopulations: CD27+ (memory cells) and CD27− (naïve cells), and the CD19+/CD27+ cell presented a higher expression when compared with naïve B cells. Furthermore, we performed Western blotting analysis in frozen biopsies of non-Hodgkin’s lymphoma (NHL) patients obtained from the Department of Pathology, Purpan Hospital (Toulouse, France). The lymph node biopsies were performed at the time of clinical diagnosis and the initial diagnosis was confirmed by immunohistochemical analysis and classified according to REAL classification. Fifty-four patients were studied with ages ranging between 28 and 93 years (median 57 years). The histologic types were: 22 follicular NHL, 15 diffuse large B-cell NHL, 17 T cell NHL (non-otherwise specified). Five reactive lymph nodes were also studied. The expression of the hlf protein was detected in all lymphoma samples studied and also in the 5 reactive lymph nodes. The hlf expression, however, was higher in T cell NHL when compared with the others NHL and reactive lymph nodes (T cell NHL vs reactive lymph node, p=0.002; T cell NHL vs follicular NHL, p=0.0001; T cell NHL vs diffuse large B-cell, p=0.012; Mann Whitney test). The hlf protein may be involved in the anti-apoptosis mechanisms, as it is expressed in all types of lymphoproliferative samples and it is associated with Akt, a pathway that is constitutively activated in some hematologic malignancies. Indeed, the ortholog protein described in mice, presents a role in the protection of the cells from apoptosis, but the pathway is unknown. This report provides a more detailed description of the expression of hlf protein in normal lymphocytes and supports the hypothesis that the hlf protein has a role in the cancer molecular pathology of hematologic malignancies.
Bcl2 Expression Predicts Better Overall Survival in 90 Patients with Mantle Cell Lymphoma.
Authors: Schrader Carsten , Klapper Wolfram , Janssen Dirk , Riis Paul , Meusers Peter , Brittinger Guenter , Siebmann Jens U. , Kneba Michael , Parwaresch Reza 
Published: October 2019

Abstract
The expression of bcl2 was typically observed in follicular lymphoma with a t(14;18) but also in CLL and mantle cell lymphoma (MCL). Up to now no study investigated the expression of bcl2 in correlation with the clinical outcome in a large collective of patients with MCL. We investigated immunohistochemically the expression of the apoptotic marker bcl2 in relation to the clinical course. 90 biopsy specimens were investigated immunohistochemically with antibodies against CD20, CD5, CD3, CD23, cyclin D1 and bcl2. The expression was analyzed in three groups: negative, 5–50% positive cells (weak positive) and more than 50% positive cells (strong positive). The expression profils were compared with the overall survival data analysed according to Kaplan and Meier. In none of thae cases a negative staining was found. 16 cases (18%) had a weak and 74 cases (82%) a strong bcl2 expression. Patients with mantle cell lymphoma that had a weak positive expression (n=16) had a median overall survival time of 21 months compared to 32 months for patients with a strong expression (n=74) (p=0.0402). In contrast to DLBL high levels of bcl2 expression had a better prognosis than pathients with low levels.