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NCI CANCERLIT® Search: Myeloproliferative Disorders - October 2001
National Cancer Institute®
Last Modified: November 21, 2001
Table of Contents
1
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.
2
UI - 21248363
AU - Jefferson K; Persad R
TI -
Poor prognosis associated with thrombocytosis in patients with renal
cell carcinoma.
SO - BJU Int 2001 May;87(7):715-6
3
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.
4
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.
5
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. kiss@iiibel.dote.hu
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.
6
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
7
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)
8
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.
9
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. imeletis@cc.uoa.gr
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.
10
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
11
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.
andres@talk21.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.
12
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.
l.camba@hsr.it
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.
13
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.
steensma.david@mayo.edu
14
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.
15
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.
16
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
17
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.
18
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.
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