X-linked lymphoproliferative disease is a rare T and NK cell immune deficiency which most frequently presents as fulminant infectious mononucleosis following infection with the Epstein-Barr virus (EBV). We report the case of a 4-year-old boy from a Spanish family presenting with severe infectious mononucleosis. In the course of the disease he developed hepatic failure, pancytopenia and neurologic impairment, leading to death after less than 2 months.
View Article and Find Full Text PDFMinimal residual disease (MRD) levels were determined by multi-parameter flow cytometry in 45 younger adult patients ( pound60 years old) with acute myeloid leukemia (AML) in complete remission. Data were collected after induction (MRD1; n=43) and/or at the end of post-remission chemotherapy or before stem cell transplantation (SCT)(MRD2; n=31). Patients with detectable MRD2 who underwent allogeneic or autologous SCT had significantly better 5-year relapse-free survival than patients not transplanted (80%, 53% and 0%, respectively p=0.
View Article and Find Full Text PDFThe present study evaluated the combination of antithymocyte globulin (ATG) and cyclosporine A (CsA) in patients with low-risk myelodysplastic syndromes. Twenty patients (17 with refractory anemia and 3 with refractory anemia with excess blasts) received treatment with rabbit-ATG plus CsA. The overall response rate was 30% (6/20); three of the six responders had a complete response.
View Article and Find Full Text PDFBackground: Some autoimmune and chronic inflammatory disorders are associated with increased risks of non-Hodgkin lymphoma (NHL). Because different NHL subtypes develop at different stages of lymphocyte differentiation, associations of autoimmune and inflammatory disorders with specific NHL subtypes could lead to a better understanding of lymphomagenic mechanisms.
Methods: In a population-based case-control study in Denmark and Sweden, 3055 NHL patients and 3187 matched control subjects were asked about their history of autoimmune and chronic inflammatory disorders, markers of severity, and treatment.
We have analyzed mantle cell lymphomas (MCLs), using high-density DNA microarrays, and confirmed the expression of differentially regulated antigens, using flow cytometry and immunohistochemistry. The results show that MCLs acquire expression of molecules that normally are involved in interaction with other immune cells and, thus, might affect the ability of the tumor to survive. The MCL signature is represented by the overexpression of the chemokine CCL4 (MIP-1beta), implicated in the recruitment of regulatory T cells, as well as CCL5 and 4-1BB-L.
View Article and Find Full Text PDFBackground: A family history of hematopoietic malignancy is associated with an increased risk of non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL), although the magnitude of the relative risk is unclear. We estimated the association between familial hematopoietic cancer and risk of lymphoma using validated, registry-based family data, and we also investigated whether associations between some environmental exposures and risk of lymphoma vary between individuals with and without such a family history.
Methods: In a population-based case-control study of malignant lymphoma, 1506 case patients and 1229 control subjects were linked to the Swedish Multi-Generation Register and then to the Swedish Cancer Register to ascertain history of cancer in first-degree relatives of patients with malignant lymphoma.
Conflicting results from previous epidemiologic studies shed little light on whether medication use is associated with risk of non-Hodgkin's lymphoma (NHL). To investigate this question, the authors conducted a population-based case-control study in Denmark and Sweden from 1999 to 2002, including 3,055 incident NHL cases and 3,187 controls. Participants reported their past use of medications and history of particular medical conditions.
View Article and Find Full Text PDFBackground: Epidemiologic evidence of an association between tobacco smoking and non-Hodgkin's lymphoma has been conflicting. This may reflect that non-Hodgkin's lymphoma comprises several distinct disease entities with different etiologies, as some studies have indicated an association between smoking and follicular lymphoma.
Objective: To investigate the association between cigarette smoking and non-Hodgkin's lymphoma risk, overall and by subtype.
The role of transcript variants of cyclin D1 in cancer biology is unclear. Most tumors with high levels of cyclin D1 express 2 transcripts due to alternative splicing: one full-length transcript of 4.4 kb and one short transcript of approximately 1.
View Article and Find Full Text PDFWe report long-term results of treatment of myelodysplastic syndrome (MDS) with erythropoietin and granulocyte colony-stimulating factor (G-CSF). A total of 129 patients were followed up 45 months after last inclusion in the Nordic MDS Group studies. Erythroid response rate was 39% and median response duration 23 months (range, 3-116 months or more).
View Article and Find Full Text PDFBackground: The incidence of malignant lymphomas has been increasing rapidly, but the causes of these malignancies remain poorly understood. One hypothesis holds that exposure to ultraviolet (UV) radiation increases lymphoma risk. We tested this hypothesis in a population-based case-control study in Denmark and Sweden.
View Article and Find Full Text PDFBackground: Fine-needle aspiration (FNA) with immunophenotyping by immunocytochemistry (IC) on cytospins has recently received increased consideration in the diagnosis of lymphoma. The aim of our study was to establish the diagnostic value of a four-color flow cytometric (FCM) panel, including cytoplasmic Bcl-2, in cytologic diagnosis of malignant non-Hodgkin's lymphoma (NHL) and reactive lymphoid hyperplasia (RH).
Methods: We investigated 424 FNAs from 396 patients.
Substantial research has been dedicated to the study of the relationship between genetic mechanisms regulating cell functions in tumors and how those tumors respond to various treatment regimens. Because these mechanisms are still not well understood, we have chosen to study the genetic makeup of 57 tumor samples from patients with follicular lymphoma (FL). Our goal was to develop a prognostic tool, which can be used as an aid in determining FL patients with tumors genetically predisposed to a successful treatment with the CHOP (cyclophosphamide, vincristine, doxorubicin, prednisone) regimen.
View Article and Find Full Text PDFBackground And Objectives: Mantle cell lymphoma (MCL) is an aggressive disease. Patients with this malignancy have a median survival of 3 years. To better understand disease progression, which is characterized by increased proliferation, we analyzed the gene expression of MCL with different proliferative indices, as determined by immunohistochemical staining for Ki-67.
View Article and Find Full Text PDFBackground And Objectives: Classical Hodgkin's lymphoma (HL) is a malignant disorder characterized by a small number of tumor cells and inflammatory cells. Both the tumor cells and the inflammatory cells produce cytokines which are thought to contribute to the clinical parameters of HL. Quantification of these cytokines at a protein level is still somewhat imprecise.
View Article and Find Full Text PDFIntroduction: Surgical biopsy examination is the gold standard for lymphoma diagnostics. However, fine-needle aspiration cytology (FNAC) offers several advantages in that it is quick, inexpensive, and the aspiration procedure has very few complications. This prospective study compares the diagnostic outcome between FNAC and surgical biopsy.
View Article and Find Full Text PDFThe purpose of this study was to determine the facility and reliability of the World Health Organization (WHO) classification of myelodysplastic syndromes (MDSs) with several observers reviewing the same diagnostic specimens. We also wanted to determine if the WHO classification provided additional information about predictability of clinical response outcome. To accomplish these goals we reviewed 103 previously diagnosed cases of low-risk MDS.
View Article and Find Full Text PDFLimited therapeutic options remain for patients with relapsing lymphoma following chemotherapy and autologous stem cell transplantation (ASCT), hence motivating investigations of complementary treatments. The aim of the present study was to evaluate feasibility and immunological effects of an immunotherapy schedule administered during chemotherapy-induced remission of aggressive non-Hodgkińs lymphoma (NHL). Repeated cycles of rIL-2, rIFN-alpha and histamine were administered to a patient with a grade III follicle center cell lymphoma, following relapse and high-dose chemotherapy with stem cell support.
View Article and Find Full Text PDFA 69-year-old woman was admitted to hospital due to abdominal pain. An enlarged spleen was detected and extirpated. Histological examination and FACS analysis could not clearly differentiate between a splenic marginal zone lymphoma and a mantle cell lymphoma.
View Article and Find Full Text PDFBackground And Objectives: In general, elderly patients with Hodgkin's lymphoma (HL) have a less favorable prognosis than do younger ones. Inadequate therapy, often due to decreased tolerance to treatment, contributes to the poor outcome. This study was undertaken to evaluate potential clinical factors of importance for prognosis with special reference to relative dose intensity (RDI) of chemotherapy (CT).
View Article and Find Full Text PDFMantle cell lymphoma (MCL) is believed to originate from a naive B cell. However, we recently demonstrated that a subset of MCL displayed mutated V(H) genes. We also reported restricted use of certain V(H) genes.
View Article and Find Full Text PDFThe objective of this study was to determine the role of transport proteins in daunorubicin (Dnr) accumulation and efflux in leukemia cells from 36 patients with acute myeloid leukemia (AML). Mononuclear cells were isolated and incubated with 1 microM Dnr with/without addition of 3 microM cyclosporin A (CyA) or metabolic inhibitors (MI). Cellular Dnr concentration in leukemia blast cells was measured with flow cytometry.
View Article and Find Full Text PDFBackground: Morphologically, T-cell-rich B-cell lymphoma (TCRB-NHL) may be indistinguishable from Hodgkin's disease (HD). Immunophenotyping may be helpful in the separation of these entities. TCRB-NHL is occasionally misdiagnosed and treated as HD.
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