National Cancer Institute®
Last Modified: June 1, 2002
UI - 11990706
AU - Kutluk T; Emir S; Karnak I; Gaglar M; Buyukpamukcu M
TI - Mesenteric inflammatory pseudotumor: unusual presentation with leukemoid reaction and massive calcified mass.
SO - J Pediatr Hematol Oncol 2002 Feb;24(2):158-9
AD - Department of Pediatric Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
The authors describe a child with an unusual presentation of mesenteric inflammatory pseudotumor in association with leukemoid reaction. An 11-year-old-boy admitted with short stature was found to have an abdominal mass localized in the right lower quadrant. The leukocyte count was 92,000/mm3 with neutrophilic leukemoid reaction. Abdominal ultrasonography and computed tomography revealed a massive calcified mass in the pelvis. Total resection of the mass was performed and the pathologic diagnosis of inflammatory pseudotumor of the mesentery was made. Leukemoid reaction dramatically resolved within a few days after surgical resection. Physicians should be aware of the association of inflammatory pseudotumor, leukemoid reaction, and massive calcification.
UI - 11923136
AU - Ikeda M; Furukawa H; Imamura H; Shimizu J; Ishida H; Masutani S; Tatsuta
TI - M; Satomi T Poor prognosis associated with thrombocytosis in patients with gastric cancer.
SO - Ann Surg Oncol 2002 Apr;9(3):287-91
AD - Department of Surgery, Sakai Municipal Hospital, Sakai, Japan. email@example.com
BACKGROUND: Thrombocytosis is commonly associated with malignant disease and has recently been suggested to be a poor prognostic indicator in patients with lung cancer and gynecological cancers. The prevalence of thrombocytosis in patients with gastric cancer was reviewed, and its association with poor prognosis was investigated. METHODS: Platelet count (PLT) and hemoglobin concentrations (Hb) were reviewed in 369 consecutive patients with histologically verified gastric cancer from 1994 to 2000. Differences between categories were analyzed with analysis of variance, and survival was compared by using the log-rank test on the Kaplan-Meier life table. Multivariate Cox regression analysis was used to evaluate whether thrombocytosis is an independent prognostic marker. RESULTS: Thrombocytosis was found in 42 patients, and anemia was found in 200 patients. PLT was negatively correlated with Hb. Mean PLT was significantly increased in patients with noncurative operations. There was a positive correlation between the depth of tumor invasion and PLT. One- and 3-year survival expectancies in patients with or without thrombocytosis were 52.4% and 23.4% and 85.7% and 72.9%, respectively. PLT was identified as an independent prognostic factor after lymph node metastasis and depth of tumor invasion. CONCLUSIONS: Thrombocytosis is an independent prognostic indicator of survival in patients with gastric cancer.
UI - 11904740
AU - Pelz AF; Kroning H; Franke A; Wieacker P; Stumm M
TI - High reliability and sensitivity of the BCR/ABL1 D-FISH test for the detection of BCR/ABL rearrangements.
SO - Ann Hematol 2002 Mar;81(3):147-53
AD - Institut fur Humangenetik, Universitatsklinikum Magdeburg, Leipziger Strasse 44, H. 26, 39120 Magdeburg, Germany. Antje-Friederike.Pelz@Medizin.Uni-Magdeburg.de
The BCR/ABL1 fusion gene is mainly caused by the t(9; 22)(q34; q11.2) translocation, which results in the Philadelphia (Ph) chromosome. The Ph chromosome is the typical hallmark in chronic myeloid leukemia (CML), but can also be present in acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). The BCR/ABL1 rearrangement is an important tumor classification marker and a useful prognostic factor allowing an adequate therapy management. Ph chromosome detection by conventional cytogenetics (CC) can be hampered by low quantity and quality of metaphases from tumor cells. Furthermore, BCR/ABL1 rearrangements may be hidden due to cryptic rearrangements or complex aberrations. Therefore, molecular cytogenetic methods turned out to be useful tools for the detection of BCR/ABL1 rearrangements. We performed fluorescent in situ hybridization (FISH) with the recently developed BCR/ABL1 D-FISH probe (QBIOgene, Illkirch, F) on cultured bone marrow and peripheral blood cells of 71 patients with CML, ALL, AML, and myeloproliferative disorder (MPD). FISH results and the results of banding methods were directly compared. Based on the analyses of >200 nuclei per patient, D-FISH correlated closely with CC and allowed an accurate quantification of BCR/ABL1 rearrangements even in a low percentage of aberrant cells. No false-positive or false-negative results were obtained. Furthermore, the D-FISH probe detected three cryptic and one complex BCR/ABL1 rearrangement, which were not visible by CC. We conclude that D-FISH reliably detects standard Ph chromosomes as well as its variant translocations and accurately quantifies BCR/ABL1 rearrangements prior and during cancer treatment as well as in the phase of remission, in daily routine tumor cytogenetic diagnostics.
UI - 11978937
AU - Vantroyen B; Vanstraelen D
TI - Management of essential thrombocythemia during pregnancy with aspirin, interferon alpha-2a and no treatment. A comparative analysis of the literature.
SO - Acta Haematol 2002;107(3):158-69
AD - Department of Internal Medicine, UZ Leuven, Leuven, Belgium.
It is advisable to treat essential thrombocythemia (ET) during pregnancy, because elevated platelet counts can lead to maternal and fetal complications. In order to establish which therapy is more favorable, we undertook a review of the literature. In addition to our own case, we found 27 reports which described 75 cases with 143 pregnancies. We discussed the complications of ET during pregnancy and postpartum, fetal outcome and the therapeutic strategies. Considering the clear risk of complications during pregnancy -- especially the occurrence of spontaneous abortion in the first trimester -- and the risk of intrauterine fetal death, we believe all patients should at least be treated with aspirin unless there is a contraindication. Platelet reduction with interferon-alpha (IFN-alpha) might be able to further reduce the complications of ET during pregnancy and to improve fetal outcome (data from 14 patients). After treatment with IFN-alpha, sufficient numbers of umbilical cord blood stem cells can be collected. Copyright 2002 S. Karger AG, Basel
UI - 11978939
AU - Tzankov A; Krugmann J; Steurer M; Dirnhofer S
TI - Idiopathic myelofibrosis with nodal, serosal and parenchymatous infiltration. Case report and review of the literature.
SO - Acta Haematol 2002;107(3):173-6
AD - Institute of Pathology, University of Innsbruck, Austria. firstname.lastname@example.org
Idiopathic myelofibrosis (IMF) is a breakpoint cluster region rearrangement-negative chronic myeloproliferative disease with progressive bone marrow fibrosis. We report a female patient (65 years old) who was admitted to our hospital in 1996. Trephine bone marrow biopsy revealed diffuse fibrosis with atypical multilobulated megakaryocytes. A cellular phase of IMF was diagnosed. Three years later, despite being at intermediate risk, the patient developed generalized lymphadenopathy and multiple sclerosing tumors throughout the peritoneum and retroperitoneum. Biopsy specimens from these tumors revealed sclerosing hematopoietic infiltrates. The present case demonstrates that IMF not only can progress to acute leukemia but can also spread uncontrollably, or 'metastasize' with extensive sclerosing hematopoietic tumors throughout the body. Copyright 2002 S. Karger AG, Basel
UI - 11999361
AU - Hirayama Y; Koyama R; Nagai T; Matsunaga T; Kogawa K; Sakamaki S; Kokai
TI - Y; Niitsu Y A pleural effusion caused by fibrous hematopoietic tumor successfully treated with prednisolone in a patient with agnogenic myeloid metaplasia with myelofibrosis.
SO - Int J Hematol 2002 Apr;75(3):305-8
AD - Department of Internal Medicine, Higashi Sapporo Hospital, Japan. email@example.com
A 46-year-old woman suffering from agnogenic myeloid metaplasia (AMM) exhibited respiratory failure caused by massive pulmonary effusion, which was speculated to have been produced by chest tumors. A biopsy specimen revealed findings compatible with fibrous hematopoietic tumor (FHT): prominent fibroblasts and fibrosis with scantv megakaryocytes. Serum concentrations of transforming growth factor (TGF)-beta and platelet-derived growth factor (PDGF) were significantly higher than those of other cases of AMM without FHT. The effusion did not respond to administrations of various chemotherapeutic agents, but after prednisolone administration, the effusion disappeared and the tumors also diminished. TGF-beta and PDGF were the possible causes of FHT formation, and for such fibrotic extramedullary regions, the administration of prednisolone should be considered.
UI - 11919391
AU - Macdonald D; Reiter A; Cross NC
TI - The 8p11 myeloproliferative syndrome: a distinct clinical entity caused by constitutive activation of FGFR1.
SO - Acta Haematol 2002;107(2):101-7
AD - Department of Haematology, Faculty of Medicine, Imperial College of Science, Technology and Medicine, Charing Cross Hospital, London, UK.
Several recurrent translocations that involve chromosome band 8p11 have been described in myeloid malignancies. These translocations target two distinct genes: (1) FGFR1, a receptor tyrosine kinase for fibroblast growth factors, and (2) MOZ, a putative histone acetyltransferase whose precise function remains to be defined. Disruption of FGFR1 is associated with a disease entity known as the 8p11 myeloproliferative syndrome (EMS)/stem cell leukemia-lymphoma syndrome, a chronic myeloproliferative disorder that frequently presents with eosinophilia and associated T-cell lymphoblastic lymphoma. The disease is aggressive and rapidly transforms to acute leukaemia, usually of myeloid phenotype. Currently, only allogeneic stem cell transplantation appears to be effective in eradicating or suppressing the malignant clone. To date, four gene fusions associated with distinct translocations have been described in EMS: the t(8;13)(p11;q12), t(8;9)(p11;q33), t(6;8)(q27;p11) and t(8;22)(p11q22) fuse ZNF198, CEP110, FOP and BCR, respectively, to FGFR1. The resulting fusion proteins have constitutive tyrosine kinase activity and activate multiple signal transduction pathways. These pathways and the fusion proteins are attractive targets for targeted signal transduction therapy. Copyright 2002 S. Karger AG, Basel
UI - 11919393
AU - Steer EJ; Cross NC
TI - Myeloproliferative disorders with translocations of chromosome 5q31-35: role of the platelet-derived growth factor receptor Beta.
SO - Acta Haematol 2002;107(2):113-22
AD - Wessex Regional Genetics Laboratory, Salisbury District Hospital, UK.
Acquired reciprocal chromosomal translocations that involve chromosome bands 5q31-33 are associated with a significant minority of patients with BCR-ABL-negative chronic myeloid leukemias. The most common abnormality is the t(5;12)(q33;p13), which fuses the ETV6/TEL gene to the platelet-derived growth factor receptor-beta (PDGFRB), a receptor tyrosine kinase that maps to 5q33. PDGFRB is disrupted by other translocations and to date four additional partner genes (H4, HIP1, CEV14 and Rab5) have been reported. Clinically, most patients present with a myeloproliferative disorder (MPD) with eosinophilia, eosinophilic leukemia or chronic myelomonocytic leukemia and thus fall into the broader category of myeloproliferative disorders/myelodysplastic syndromes (MPD/MDS). With the advent of targeted signal transduction therapy, patients with rearrangement of PDGFRB might be better classified as a distinct subgroup of MPD/MDS. Copyright 2002 S. Karger AG, Basel
UI - 11919389
AU - Roy S; Szer J; Campbell LJ; Juneja S
TI - Sequential transformation of t(8;13)-related disease: a case report.
SO - Acta Haematol 2002;107(2):95-7
AD - Royal Melbourne Hospital, Melbourne, Australia.
We report a case of an atypical myeloproliferative disorder with t(8;13) that presented as B-lineage acute lymphoblastic leukaemia (B-ALL). Following induction chemotherapy, the disease manifested as chronic myeloproliferative state, which responded to hydroxyurea. Terminally, the disease transformed into acute myeloid leukaemia (AML) with additional chromosomal abnormalities including monosomy 7. To our knowledge, this is the first case of this rare atypical myeloproliferative disorder with t(8;13) that presented as B-ALL and terminally transformed to AML with additional chromosomal abnormalities. Copyright 2002 S. Karger AG, Basel
UI - 11919390
AU - JabbarAl-Obaidi M; Rymes N; White P; Pomfret M; Smith H; Starczynski J;
TI - Johnson R A fourth case of 8p11 myeloproliferative disorder transforming to B-lineage acute lymphoblastic leukaemia. A case report.
SO - Acta Haematol 2002;107(2):98-100
AD - Department of Haematology, Birmingham Heartlands Hospital, Birmingham, UK.
A 56-year-old male presented with inguinal lymphadenopathy and leucocytosis (WBC 98 x 10(9)/l). Bone marrow morphology showed myeloid hyperplasia, with eosinophilia. Cytogenetic analysis showed no evidence of the Philadelphia chromosome, and fluorescence in situ hybridisation studies for the BCR/ABL fusion were negative. All cells examined showed the t(8;13)(p11;q12) translocation. Six weeks after presentation, the disease progressed to an acute lymphoblastic leukaemia (ALL). The lymphoblasts were CD19/CD10 dual positive. Cytogenetic analysis again showed the t(8;13) translocation, with no additional abnormalities. There have been at least 14 reported cases of the t(8;13) myeloproliferative disorder to date, of which only 3 transformed to B-lineage ALL: our case is the 4th. Copyright 2002 S. Karger AG, Basel
UI - 10609780
AU - Matsumoto K; Anasetti C
TI - The role of T cell costimulation by CD80 in the initiation and maintenance of the immune response to human leukemia.
SO - Leuk Lymphoma 1999 Nov;35(5-6):427-35
AD - Clinical Research Division, Fred Hutchinson Cancer Research Center, Seattle, WA 98109, USA.
Most human myeloid leukemias express both class I and class II HLA and it has been postulated that leukemia-associated peptides are presented by those molecules. It is possible, however, that leukemia cells escape the immune surveillance by lacking expression of "costimulatory" molecules required for activating the immune response. Human erythroleukemia line (HEL) has been the subject of previous detailed studies demonstrating surface expression of bona fide HLA molecules but inability to stimulate allogeneic response of proliferative or cytolytic T cells. We found that an HLA-DR+ subclone (HEL-DR+) expresses LFA-1, LFA-3, ICAM-1, ICAM-3, but neither CD80 nor CD86 on the surface. Transfection of CD80 cDNA into HEL-DR+ cells induced the allogeneic response of purified T cells from both cord blood and peripheral blood of adult donors, demonstrating that CD80 expression could lead to accessory cell-independent activation of naive T cells. Priming allogeneic peripheral blood T cells by HEL-DR+/CD80+ also lead to generation of cytotoxic T lymphocytes that lysed both HEL-DR+/CD80+ and wild type HEL-DR+ equally well, confirming CD80 expression is required only in the CTL induction phase but not in the CTL effector phase. We established and maintained alloproliferative T cell clones from adult blood by stimulation with the HEL-DR+/CD80+ line. The clones could respond not only to HEL-DR+/CD80+ line but also to the HEL-DR+ line; however, the proliferative response to HEL-DR+/CD80+ was amplified and sustained compared to the short-lived response to wild type HEL-DR+ cells. Therefore, expression of CD80 by HEL-DR+ cells was determinant both to initiate and sustain the T cell response. These experiments support the hypothesis that lack of expression of "costimulatory" molecules for T cells contributes to leukemia escape from immune surveillance, and provide preliminary data for the use of CD80 transfection in the immunotherapy of human leukemia.
UI - 10848954
AU - Pahl HL
TI - Towards a molecular understanding of polycythemia rubra vera.
SO - Eur J Biochem 2000 Jun;267(12):3395-401
AD - Division of Experimental Anaesthesiology, University Hospital Freiburg, Center for Tumor Biology, Germany. firstname.lastname@example.org
Polycythemia rubra vera (PV) is one of four diseases collectively called the myeloproliferative disorders (MPDs). Each disorder leads to an increased production of one or several hematopoietic cell lineages. MPDs arise from acquired mutations in a pluripotent hematopoietic stem cell. However, the molecular mechanisms leading to the development of these diseases are poorly understood. This review will summarize and evaluate recent advances in our understanding of one particular MPD, PV.
UI - 2836003
AU - Avalos BR; Kaufman SE; Tomonaga M; Williams RE; Golde DW; Gasson JC
TI - K562 cells produce and respond to human erythroid-potentiating activity.
SO - Blood 1988 Jun;71(6):1720-5
AD - Department of Medicine, UCLA School of Medicine.
Human erythroid-potentiating activity (EPA) is a 28,000 mol wt glycoprotein that stimulates the growth of erythroid progenitors in vitro and enhances colony formation by the K562 human erythroleukemia cell line. EPA has potent protease inhibitory activity, and is also referred to as tissue inhibitor of metalloproteinases (TIMP). We observed that colony formation by K562 cells in semi-solid medium containing reduced fetal calf serum (FCS) is not directly proportional to the number of cells plated, suggesting production of autostimulatory factors by K562 cells. Using radioimmunoprecipitation and a bioassay for EPA, medium conditioned by K562 cells was found to contain high levels of biologically active EPA; Northern hybridization analysis confirmed the expression of EPA mRNA. Radiolabeled EPA was used to identify cell surface receptors on K562 cells. Together, these results suggest that EPA may act as an autocrine growth factor for K562 cells.
UI - 12047134
AU - Douglas VK; Tallman MS; Cripe LD; Peterson LC
TI - Thrombopoietin administered during induction chemotherapy to patients with acute myeloid leukemia induces transient morphologic changes that may resemble chronic myeloproliferative disorders.
SO - Am J Clin Pathol 2002 Jun;117(6):844-50
AD - Department of Pathology, Immunology, and Laboratory Medicine, University of Florida College of Medicine, Gainesville 32610-0275, USA.
Thrombopoietin (TPO), a potent stimulator of megakaryocyte and platelet production, has been used in clinical trials to reduce thrombocytopenia after chemotherapy in patients with acute myeloid leukemia (AML). We report that TPO therapy is associated with peripheral blood and bone marrow findings that can mimic myeloproliferative disorders. Peripheral blood and bone marrow samples of 13 patients with AML who received TPO were examined. A subset of bone marrow samples exhibited hypercellularity, megakaryocytic hyperplasia, and reticulin fibrosis after TPO administration. Cases demonstrated as many as 58.4 megakaryocytes per high-powerfield (MHPF) compared with 3.7 MHPF in the control group. Megakaryocytic atypia, increased mitoses, emperipolesis, intrasinusoidal megakaryocytes, and thickened trabeculae also were seen. Peripheral blood findings included leukoerythroblastosis, leukocytosis, thrombocytosis, and circulating megakaryocyte nuclei. Changes resolved within 3 months after discontinuation of TPO. This rapid resolution of the morphologic abnormalities induced by TPO distinguishes these findings from those seen in true chronic myeloproliferative disorders.
UI - 12035366
AU - Mohanty R; Dash B; Chawla SC; Paul HS
TI - Acute erythroleukemia--M6B.
SO - Indian J Pathol Microbiol 2001 Oct;44(4):461-2
AD - Department of Pathology, Tata Main Hospital, Jamshedpur.
Acute Erythroleukemia is a rare disorder of hematopoietic system, accounts for 1-3% of all acute leukemia and 15% of myeloid leukemia. Recently, the World Health Organisation & Society of Haematopathology proposed a change in the categorization, with M6a and M6b subgroups of the original FAB classification. Hereby we report a case of acute erythroleukemia--M6b subtype, presented with pain abdomen and vomiting. The patient died within two days. The case is being reported for its rarity and uncommon presentation.
UI - 12068799
AU - Elliot MA; Yoon SY; Kao P; Li CY; Tefferi A
TI - Simultaneous measurement of serum thrombopoietin and expression of megakaryocyte c-Mpl with clinical and laboratory correlates for myelofibrosis with myeloid metaplasia.
SO - Eur J Haematol 2002 Mar;68(3):175-9
AD - Division of Hematology and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
OBJECTIVES: We sought to investigate potential mechanisms of increased serum thrombopoietin (TPO) concentrations in myelofibrosis with myeloid metaplasia (MMM) by simultaneously measuring serum TPO, platelet and megakaryocyte (MK) numbers, and MK c-Mpl expression. METHODS: We studied 17 consecutive patients who had MMM and were not receiving therapy at the time of evaluation. Serum TPO was measured by a two-site immunochemiluminometric assay. Immunohistochemical staining of c-Mpl was accomplished with an immunoperoxidase method on simultaneously obtained bone marrow specimens. RESULTS: Our findings confirmed the presence of inappropriately increased serum TPO despite mostly normal or increased peripheral platelet counts and markedly increased bone marrow MK numbers. In addition, we found an inverse correlation between platelet count and serum TPO (P<0.03) and splenic size (P<0.04). However, serum TPO did not correlate with either bone marrow MK number or c-Mpl expression. The lack of correlation of serum TPO and bone marrow megakaryocyte number may be accounted for by the unavoidable inaccuracies in quantifying megakaryocytopoiesis in a disorder of known altered hematopoietic progenitor cell distribution, both intramedullary and extramedullary. The significant inverse correlation between serum TPO and spleen size suggests that this site of extramedullary megakaryocytopoiesis may assume a role in the dysfunctional TPO regulatory axis. CONCLUSIONS: These observations suggest some preservation of the negative feedback regulation that appears to be dysfunctional at the MK c-Mpl level. Consistent with previous observations in animal models, our observations suggest the possibility that altered TPO regulation resulting in sustained ligand excess may have pathogenetic relevance in MMM.
UI - 12051745
AU - Umenishi F; Schrier RW
TI - Induction of human aquaporin-1 gene by retinoic acid in human erythroleukemia HEL cells.
SO - Biochem Biophys Res Commun 2002 May 10;293(3):913-7
AD - Division of Renal Diseases and Hypertension, Department of Medicine, University of Colorado Health Sciences Center, Denver, CO 80262, USA. email@example.com
Retinoids have been implicated in the control of cell proliferation, differentiation, and developmental processes. We report here that aquaporin-1 (AQP1) is specifically induced by retinoic acid (RA) in human erythroleukemia HEL cells. Both all-trans-RA (ATRA) and 9-cis-RA (9CRA) strongly induced the AQP1 mRNA and protein in a dose-dependent manner. AQP1 protein was mainly expressed in plasma membrane in cells induced by RAs. To identify the RA response element (RARE) in the human AQP1 promoter, the 5(')-flanking region of AQP1 promoter was isolated and transient transfection experiment in HEL cells was performed. Deletion analysis of the AQP1 promoter revealed that one putative DR5-like RARE with five spaces was located in the region from -2218 to -2202; AGGGCAgggacAGGTGA. Electrophoretic mobility shift assay (EMSA) experiment demonstrated that two slowly migrated complexes (C1 and C2) capable of binding the RARE sequence were present in nuclear extracts prepared from cells and the complex C1 was strongly increased in nuclear extracts by RA stimulation. The complexes C1 and C2 were significantly abolished by an excess unlabeled probe. These results indicate that RAs strongly stimulate the human AQP1 gene expression through the RARE and define a novel role in the regulation of erythropoiesis. (c) 2002 Elsevier Science (USA).
UI - 11920280
AU - Dobo I; Mossuz P; Campos L; Girodon F; Allegraud A; Latger-Cannard V;
TI - Boiret N; Pineau D; Wunder E; Zandecki M; Praloran V; Hermouet S Comparison of four serum-free, cytokine-free media for analysis of endogenous erythroid colony growth in polycythemia vera and essential thrombocythemia.
SO - Hematol J 2001;2(6):396-403
AD - Laboratoire d'Hematologie, Centre Hospitalier Universitaire d'Angers, Angers, France.
INTRODUCTION: The assay of endogenous erythroid colony formation (EEC), a characteristic of polycythemia vera and essential thrombocythemia, is not standardized. In this multicentric study, we tested four semisolid, serum-free, cytokine-free media based on either methylcellulose (M1, M2) or collagen (C1, C2) commercialized for the EEC assay. MATERIALS AND METHODS: Bone marrow mononuclear cells (BMMC) from 73 individuals (62 patients with either polycythemia vera (26), essential thrombocythemia (19), secondary polyglobuly (17) or chronic myeloid leukemia (2) and 11 healthy donors) were grown in parallel in the four media without, or with 0.01 U/ml erythropoietin (EPo). RESULTS: In all four media EEC formation was specific, as it was not observed in cultures of patients with secondary polyglobuly or chronic myeloid leukemia, nor of healthy donors. Analysis of fresh or MGG-stained collagen gel cultures allowed detection of EEC formation significantly more frequently than methylcellulose-based media; addition of 0.01 U/ml of EPo had little or no effect on EEC formation. Collagen-based medium C1 gave better results than the other media tested: the 'C1' EEC assay was positive for 68.2% of polycythemia vera cultures with significantly higher median EEC numbers (6.5/10(5) BMMC for patients with one major criteria of polycythemia vera and 19 and 21/10(5) BMMC for patients with two or three major criteria, respectively). Medium C1 was also better for essential thrombocythemia cultures with 47.4% of positive results but with a low median EEC number (6.7/10(5) BMMC). When associated with the ELISA dosage of serum EPo, the 'C1' EEC assay allowed confirmation or elimination of the diagnosis of polycythemia vera for 91% (20/22) of polyglobulic patients. CONCLUSION: We propose that serum-free collagen-based culture systems be considered to standardize the EEC assay, now part of the new criteria of polycythemia vera.
UI - 12061320
AU - Hirose Y; Masaki Y; Sugai S
TI - Leukemic transformation with trisomy 8 in essential thrombocythemia: a report of four cases.
SO - Eur J Haematol 2002 Feb;68(2):112-6
AD - Division of Hematology and Immunology, Department of Internal Medicine, Kanazawa Medical University, Ishikawa, Japan.
Karyotype analysis of bone marrow samples was performed in 20 cases of essential thrombocythemia (ET) at the time of diagnosis. Three patients had karyotype abnormalities at the time of diagnosis; trisomy 8, deletion of Y, and del(9)(q?) in each. The patient who had trisomy 8 at the time of diagnosis underwent myeloid blastic transformation in 35 months. Three patients whose karyotypes were normal at the time of diagnosis developed a chromosome abnormality with trisomy 8 when they developed myeloid blastic transformation 38, 79 and 86 months after initial diagnosis of ET. Two patients with blastic transformation had been treated with busulfan, one with hydroxyurea and one with methyl-6-[3-(2-chloroethyl)-3-nitorosoureidol]-6-deoxy-alpha-D-glucopyra noside (MCNU). It is suggested that progression of the disease may have increased the incidence of trisomy 8 and the development of leukemic transformation.
The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.