National Cancer Institute®
Last Modified: November 21, 2001
UI - 20389780
AU - Symbas NP; Townsend MF; El-Galley R; Keane TE; Graham SD; Petros JA
TI - Poor prognosis associated with thrombocytosis in patients with renal cell carcinoma.
SO - BJU Int 2000 Aug;86(3):203-7
AD - Department of Urology, Emory University School of Medicine, Atlanta, Georgia, and The Virginia Medical Center, USA.
OBJECTIVES: To better define the relationship between platelet count and survival using a retrospective analysis in patients with thrombocytosis and metastatic renal cell carcinoma (RCC), some of whom had a shorter life expectancy than those with a normal platelet count. PATIENTS AND METHODS: The records were reviewed of patients with stage IV RCC who had undergone a variety of adjuvant therapies after nephrectomy between 1972 and 1992. Entry criteria included a tissue diagnosis of RCC, at least one platelet count and a complete follow-up until the time of death. Of 350 patients available for review, 259 met the entry criteria. Patients were divided into two groups: group 1 included 112 patients whose platelet counts remained at < 4 x 105/microL between the time of nephrectomy and the time of death; group 2 included 147 patients with at least one platelet count of > 4 x 105/microL (mean age in each group 57 years). RESULTS: The mean (SD) survival for group 1 was 151 (34) months, compared with 92 (18) months for those in group 2. Using the log-rank chi-square test the difference in survival between the groups was significant (P = 0.005). Controlling for established prognostic indicators of pathological stage, nuclear grade and cell type, using Cox's regression technique, the difference in survival between the groups remained significant (P = 0.015). CONCLUSIONS: These results suggest that patients with metastatic RCC who receive adjuvant therapy and have a persistently normal platelet count have a 64% longer life expectancy than those with thrombocytosis. The difference is highly statistically significant when controlled for nuclear grade, cell type and pathological stage.
UI - 21240459
AU - Tiribelli M; Michelutti A; Damante G; Pellizzari L; Martinelli G;
TI - Amabile M; Russo D Screening of Bcr-Abl transcripts in Philadelphia negative essential thrombocythemia.
SO - Leuk Lymphoma 2000 Oct;39(3-4):339-41
AD - Chair and Division of Hematology, Department of Medical and Morphological Research, University of Udine, Italy.
Essential thrombocythemia (ET) is a chronic myeloproliferative disorder characterised by the absence of the Philadelphia (Ph+) chromosome. Recent studies have reported controversial results relating to BCR-ABL rearrangements in ET patients. We studied 44 Ph-negative ET patients with the RT-PCR technique at diagnosis or during the follow-up. None of them showed any of the BCR-ABL transcript actually described by others in ET; neither the "classical" P210 nor the P190 or P230 variants. Our results confirm the absence of BCR-ABL abnormalities in Ph-negative ET patients.
UI - 21240470
AU - Kasahara S; Tsurumi H; Hara T; Goto H; Moriwaki H
TI - Idiopathic myelofibrosis developing isolated granulocytic sarcoma with der (1;7)(q10; p10) after splenectomy and finally transforming to acute myelogenous leukemia.
SO - Leuk Lymphoma 2000 Oct;39(3-4):427-33
AD - First Department of Internal Medicine, Gifu University School of Medicine, 40 Tsukasa-machi, Gifu 500-8705, Japan.
A 47-year-old female with idiopathic myelofibrosis developed isolated granulocytic sarcoma with der (1; 7)(q10; p10) after splenectomy, followed by acute myelogenous leukemia. The patient had myelofibrosis since 22 years of age, received splenectomy at 47 years, and developed isolated submandibular granulocytic sarcoma, 8 months later. Although the initial tumor disappeared after irradiation, recurrent tumors selectively appeared in the areas of operative scars. She subsequently developed blastic transformation with der (1; 7)(q10; p10), and the blasts were refractory to different chemotherapy. This case is very rare in the following aspects: the onset of myelofibrosis occurred at a relatively young age; isolated granulocytic sarcoma after splenectomy preceded the transformation to acute leukemia; and the subcutaneous tumors developed in areas of operative scars.
UI - 21240497
AU - Kiss E; Gal I; Simkovics E; Kiss A; Banyai A; Szakall S; Szegedi G
TI - Myelofibrosis in systemic lupus erythematosus.
SO - Leuk Lymphoma 2000 Nov;39(5-6):661-5
AD - 3rd Department of Internal Medicine, National Institute of Haematology and Blood Transfusion, Budapest, Hungary. email@example.com
In this study we present a case of coexisting systemic lupus erythematosus (SLE) and myelofibrosis. Literature review supports the fact that the two diseases rarely occur together in the same patient. The young female patient studied was admitted with pancytopenia and a clinical picture which met the criteria of SLE. Histological examination of the bone marrow biopsy revealed severe myelofibrosis with hypocellularity of the myeloid cell lines. Treatment with immunosuppressive and colony stimulating factor led to slow but complete regeneration of the bone marrow and subsequently to an improved haematological status, and the patient was spared bone marrow transplantation.
UI - 21282490
AU - Yonezumi M; Miyagishima T; Kudo M; Choi GH; Kishimoto A; Miura Y;
TI - Nakagawa M; Okabe M A case of de novo early erythroblastic leukemia supporting a proposal of AML M6 'variant'.
SO - Leuk Lymphoma 2000 Nov;39(5-6):667-8
UI - 20346934
AU - Tefferi A; Yoon SY; Li CY
TI - Immunohistochemical staining for megakaryocyte c-mpl may complement morphologic distinction between polycythemia vera and secondary erythrocytosis.
SO - Blood 2000 Jul 15;96(2):771-2
AD - Division of Hematology and Internal Medicine, Mayo Clinic and Mayo Foundation, Rochester, MN 55095, USA.
Recent studies have shown decreased megakaryocyte expression of the thrombopoietin receptor (c-mpl) in patients with polycythemia vera (PV) but not in those with reactive erythrocytosis. We examined the diagnostic utility of this observation in 22 patients with PV, 7 patients with secondary erythrocytosis (SE), and 10 normal controls. Commercial antibodies against c-mpl were used with standard immunoperoxidase methods. Megakaryocyte c-mpl staining intensity was uniformly moderate-to-strong in the healthy controls and in all the patients with SE. In contrast, staining intensity in 9 patients with PV (41%) was uniformly weak. Furthermore, in 12 of the remaining 13 patients with PV, the c-mpl staining pattern in each case was heterogeneous and was associated with weak staining intensity in more than 20% of the megakaryocyte population. These preliminary data suggest that c-mpl immunostains may complement bone marrow histopathology in distinguishing PV from nonclonal causes of erythrocytosis. (Blood. 2000;96:771-772)
UI - 21435099
AU - Frankova H; Gaja A; Hejlova N
TI - Pulmonary sarcoidosis in a patient with essential thrombocythemia treated with interferon alpha: a short case report.
SO - Med Sci Monit 2000 Mar-Apr;6(2):380-2
AD - Masaryk Memorial Cancer Institute, Zluty kopec 7, 656 53 Bmo, Czech Republic.
A patient with essential thrombocythemia was diagnosed with pulmonary sarcoidosis after interferon alpha therapy. Following interferon treatment the miliary pulmonary dissemination has appeared and after disruption of this therapy it resolved during two months. Few cases of sarcoidosis associated with interferon alpha treatment have been reported. These patients were treated for chronic myelogenous leukemia, chronic hepatitis C, and renal cell carcinoma. We report the first case of interferon-related sarcoidosis in an essential thrombocythemia patient.
UI - 21241654
AU - Meletis J; Terpos E; Samarkos M; Meletis C; Konstantopoulos K; Komninaka
TI - V; Apostolidou E; Benopoulou O; Korovesis K; Mavrogianni D; Variami E; Yataganas X; Loukopoulos D Detection of CD55 and/or CD59 deficient red cell populations in patients with aplastic anaemia, myelodysplastic syndromes and myeloproliferative disorders.
SO - Haematologia (Budap) 2001;31(1):7-16
AD - First Department of Internal Medicine, University of Athens School of Medicine, Laiko General Hospital, Greece. firstname.lastname@example.org
Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal stem cell disorder characterized by intravascular haemolysis, venous thrombosis, marrow hypoplasia, frequent episodes of infection, and rarely leukaemic conversion. At the cellular level, PNH is characterized by the decrease or absence of glycosylphosphatidylinositol (GPI)-anchored molecules, such as CD55 and CD59, from the cell surface. PNH-like clones have been described in various haematological disorders. The link between PNH and aplastic anaemia (AA) has been established but the relationship of PNH with myelodysplastic syndromes (MDS) or myeloproliferative disorders (MPD) remains unclear. In this study, the presence of CD55 and/or CD59 defective (PNH-like) red cell populations was evaluated in 21 patients with AA, 133 with MDS, 197 with MPD, 7 with PNH and in 121 healthy blood donors using the Sephacryl Gel Test microtyping system. Red cell populations deficient in both molecules CD55 and CD59 were detected in 33.3% of AA patients, in 16.5% of MDS patients (50% with hypoplastic bone marrow), in 14.2% of MPD patients (more often in essential thrombocythemia, 21.2%) and in all PNH patients. CD55 deficient red cell populations were found in 14.2% of patients with AA, 12.7% of patients with MDS and 21.3% of patients with MPD. CD59 deficient populations were found in 9.5% of AA patients, 2.2% of MDS patients and 2% of MPD patients. These results indicate an association between PNH, AA and MDS or even between PNH and MPD. Further investigation is necessary to work out the mechanisms of this association, and to define classification criteria for borderline cases, where diagnosis is difficult.
UI - 21449247
AU - Heller P; Kornblihtt LI; Cuello MT; Larripa I; Najfeld V; Molinas FC
TI - BCR-ABL transcripts may be detected in essential thrombocythemia but lack clinical significance.
SO - Blood 2001 Sep 15;98(6):1990
UI - 21437187
AU - Virchis A; Massey E; Butler T; Devaraj P; Wright F; Secker-Walker L;
TI - Prentice HG; Mehta A Acute myeloblastic leukaemias of FAB types M6 and M4, with cryptic PML/RARalpha fusion gene formation, relapsing as acute promyelocytic leukaemia M3.
SO - Br J Haematol 2001 Sep;114(3):551-6
AD - Department of Haematology, The Royal Free and University College School of Medicine, Royal Free Campus, University College London, UK. email@example.com
Demonstration of either the translocation t(15;17)(q22;q21) or the fusion of PML and RARalpha genes is regarded as diagnostic for acute myeloid leukaemia (AML) of FAB type M3, but has occasionally been seen in other FAB types. We present two such cases. Case 1 presented with FAB type M6 and a complex karyotype involving chromosomes 1, 2, 11 and 17. Bone marrow relapse of FAB type M3 followed autologous bone marrow transplantation. Subsequent marrow dysplasia and an M6 relapse were accompanied by a new cytogenetic clone involving chromosomes X, 2, 4, 6, 7 and 16. Fluorescence in situ hybridization (FISH) of metaphase chromosomes at diagnosis showed insertion of material from chromosome 17 into a 'normal' 15 with juxtaposition of PML and RARalpha. Case 2 presented as AML M4 and relapsed as M3. Cytogenetic analysis at diagnosis and in relapse showed 46,XY,t(15;17)(q22;q11),del(16)(q22). FISH analysis showed this to be a three-way translocation involving chromosomes 15, 16 and 17 again with juxtaposition of PML and RARalpha. Reverse transcription-polymerase chain reaction (RT-PCR) revealed PML/RARalpha fusion at diagnosis, in remission and in first relapse. These examples strengthen the case for RT-PCR screening of all AML patients for these fusion genes.
UI - 21437199
AU - Camba L; Aldrighetti L; Ciceri F; Bernardi M; Marktel S; Angeli E;
TI - Giacomelli M Locoregional intrasplenic chemotherapy for hypersplenism in myelofibrosis.
SO - Br J Haematol 2001 Sep;114(3):638-40
AD - Department of Haematology, Ospedale S. Raffaele, Milan, Italy. firstname.lastname@example.org
A 79-year-old patient with post-polycythaemic myelofibrosis presented with severe hypersplenism. After splenic artery catheterization, starting at 20 mg/m2/d and tapering by 5 mg/m2 every 2 weeks to a final daily dose of 5 mg/m2/d. The drug was then stopped. The spleen had decreased to one third of the initial volume. Clinical conditions and haematological indices improved substantially. Intrasplenic therapy could be a new therapeutic tool for hypersplenism in chronic idiopathic and post-myeloproliferative myelofibrosis.
UI - 21424591
AU - Steensma DP; Hanson CA; Letendre L; Tefferi A
TI - Myelodysplasia with fibrosis: a distinct entity?
SO - Leuk Res 2001 Oct;25(10):829-38
AD - Department of Internal Medicine, Division of Hematology, West 10, Mayo Clinic Rochester, 200 First Street SW, Rochester, MN 55905, USA. email@example.com
UI - 21424597
AU - Kabutomori O; Kanakura Y; Iwatani Y
TI - Increase in platelet-large cell ratio in chronic myeloid leukemia.
SO - Leuk Res 2001 Oct;25(10):873
AD - Central Laboratory for Clinical Investigation, Osaka University Hospital, 2-15 Yamada-oka, Suita, 565-0871, Osaka, Japan.
UI - 97465524
AU - Blickstein D; Aviram A; Luboshitz J; Prokocimer M; Stark P; Bairey O;
TI - Sulkes J; Shaklai M BCR-ABL transcripts in bone marrow aspirates of Philadelphia-negative essential thrombocytopenia patients: clinical presentation.
SO - Blood 1997 Oct 1;90(7):2768-71
AD - Division of Hematology, Rabin Medical Center, Beilinson Campus, Petah-Tikva, Israel.
One of the diagnostic criteria of essential thrombocythemia (ET) is the absence of the Philadelphia chromosome (Ph-neg). On the molecular level, Ph-neg ET patients may carry BCR-ABL transcript. The natural history of BCR-ABL positive Ph-neg ET patients is undetermined. We examined the BCR-ABL status by reverse transcriptase two-step nested polymerase chain reaction in bone marrow aspirates of 25 Ph-neg ET patients. We found 12 BCR-ABL positive and 13 BCR-ABL negative patients in the study group. The comparison showed that the two groups had similar clinical and laboratory characteristics, except for a significant increased patients' age and decreased polymorphonuclear cell count in the BCR-ABL positive group. During a median follow-up of 20 and 22.5 months for the BCR-ABL negative and positive groups, respectively, there was neither blastic transformation nor unrelated death in both groups. We conclude that it is important to look for BCR-ABL transcript in Ph-neg ET patients and to follow them closely to investigate the nature of this translocation in this group of patients.
UI - 21180601
AU - Emilia E; Marasca R; Zucchini P; Temperani P; Luppi M; Torelli G; Lanza
TI - F; De Angelis C; Gandini D; Castoldi GL; Vallisa D; Cavanna L; del Senno L BCR-ABL rearrangement is not detectable in essential thrombocythemia.
SO - Blood 2001 Apr 1;97(7):2187-9
UI - 21291607
AU - Colita A; Belhabri A; Chelghoum Y; Charrin C; Fiere D; Thomas X
TI - Prognostic factors and treatment effects on survival in acute myeloid leukemia of M6 subtype: a retrospective study of 54 cases.
SO - Ann Oncol 2001 Apr;12(4):451-5
AD - Service d'Hematologie, Hjpital Edouard Herriot, Lyon, France.
classification system of acute myeloid leukemia (AML) which designates it as M6 AML. This report describes the data of 54 patients with newly Median age was 59 years. Pancytopenia was the most common feature at diagnosis. Twenty-six percent of cases presented with secondary AML. Karyotype was successfully performed in 35 cases. Eleven patients presented with normal karyotype, nine with simple karyotypic abnormalities, and fifteen with major karyotypic abnormalities. Fifty of the fifty-four patients received one or two courses of induction chemotherapy combining anthracyclines with cytarabine according to different successive protocols. One elderly patient only received low-dose cytarabine, and three patients died before any chemotherapy could be given. RESULTS: Complete remission (CR) was achieved in 29 cases (54%, 95% confidence interval (CI): 40%-67%). As post-remission therapy, four patients could be allografted, and two underwent autologous transplantation. All other treated patients received continuation chemotherapy. Twenty-one patients have relapsed (72%). Median time to relapse was six months. Among those patients, only eight achieved a second CR (38%). The median disease-free survival (DFS) was eight months (95% CI: 4-10 months) with a five-year survival rate of 17%. Median overall survival (OS) was nine months (95% CI: 5-12 months) with a five-year survival rate of 13%. In univariate analysis, poor prognostic factors for DFS were secondary AML (P = 0.05) and initial platelet count <50 x 109/l (P = 0.02). Poor prognostic factors for OS were age > or = 60 years (P = 0.005), secondary AML (P = 0.05), initial 'blastic' fever (P = 0.0004), and initial haemoglobin level < 90 g/l (P = 0.03). All factors, but haemoglobin level, remained significant in the multivariate analysis. Although it was not statistically significant, there was a trent for a better prognosis of M6 patients presenting with normal karyotype as compared to those displaying chromosomal abnormality. CONCLUSIONS: This retrospective analysis points to a somewhat heterogenous group of AML in terms of clinical and biological features, and outcome. Distinctive subgroups can be identified according to prognostic factors related to survival. A larger multicenter study with well-defined diagnostic criteria is warranted to further clarify treatment effects.
UI - 21439904
AU - Wall DB; Kachman MT; Gong SS; Parus SJ; Long MW; Lubman DM
TI - Isoelectric focusing nonporous silica reversed-phase high-performance liquid chromatography/electrospray ionization time-of-flight mass spectrometry: a three-dimensional liquid-phase protein separation method as applied to the human erythroleukemia cell-line.
SO - Rapid Commun Mass Spectrom 2001;15(18):1649-61
AD - Department of Chemistry, The University of Michigan, Ann Arbor, MI 48109-1055, USA.
A liquid-phase three-dimensional protein separation method has been developed that is used to separate the cytosolic fraction of a HEL cell lysate via isoelectric focusing (IEF), nonporous silica (NPS) reversed-phase high-performance liquid chromatography (RP-HPLC) and electrospray ionization time-of-flight mass spectrometry (ESI-TOFMS), respectively. Several hundred unique protein molecular weights were observed in a pI range from 4.8 to 8.5 and a mass range from 5 to 85 kDa. Proteins were positively identified by analysis of the pI (+/-0.5 pI units), an intact protein molecular weight (+/-150 ppm), and peptide mass mapping results. Using the molecular weight (MW) and peptide mapping results of identified proteins it was possible to characterize their posttranslational (PTMs) and/or sequence modifications. PTMs were detected on both forms of cytosolic actin, heat shock 90 beta, HINT and alpha-enolase. Sequence modifications or conflicts were observed for beta-and gamma-actin, ATP beta-synthase and heat shock 90 beta. IEF-NPS-RP-HPLC/ESI-TOFMS was used to determine experimental pI, MW and relative hydrophobicity values for each protein detected. This data was used to generate a 2-D pI-MS protein map, where proteins are displayed according to their pI and molecular weight. Protein molecular weight peaks are represented as bands in the 2-D pI-MS image where the gray scale of each band is proportional to the intensity of the protein molecular weight peak. In addition, a third hydrophobicity dimension (%B) was added as the % acetonitrile elution to generate a 3-D pI-MS-%B plot where each protein can be tagged according to three parameters. Copyright 2001 John Wiley & Sons, Ltd.
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.