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Tipos de Cancer / Leucemia / Desórdenes Mieloproliferativos / Recursos de NCI

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NCI CANCERLIT^® Search: Myeloproliferative Disorders - March 2002

Imprima English

National Cancer Institute^®
Ultima Vez Modificado: 1 de marzo del 2002

Early elevation of serum thrombopoietin levels and subsequent thrombocytosis in healthy preterm infants.

[Polycythemia vera]

Skin lesion caused by hydroxyurea.

[Association between hepatocellular carcinoma and polycythemia vera]

Transforming growth factor-beta1 transcriptionally activates CD34 and prevents induced differentiation of TF-1 cells in the absence of any

Characterization of functional VIP/PACAP receptors in the human erythroleukemic HEL cell line.

Pseudohyperkalemia due to reactive thrombocytosis in an infant with yolk sac tumor.

Lack of pathogenic mutations in the 5'-untranslated region of the thrombopoietin gene in patients with non-familial essential

The optimal management of polycythaemia vera.

Table of Contents

1
UI - 11843834
AU - Matsubara K; Baba K; Nigami H; Harigaya H; Ishiguro A; Kato T; Miyazaki
TI - H Early elevation of serum thrombopoietin levels and subsequent thrombocytosis in healthy preterm infants.
SO - Br J Haematol 2001 Dec;115(4):963-8
AD - Department of Paediatrics, Nishi-Kobe Medical Centre, 5-7-1 Kojidai, Nishi-ku, Kobe 651-2273, Japan. kskmatsu@portnet.ne.jp
To verify pathophysiological mechanisms underlying thrombocytosis in low-birth-weight (LBW) preterm babies, we evaluated kinetic changes in platelet counts and thrombopoietic cytokines including thrombopoietin (TPO), interleukin 6 (IL-6) and IL-11 in 24 uncomplicated preterm infants. Platelet counts in cord blood (CB) (265 +/- 64 x 10(9)/l) were similar to adult levels, increased by d 14 (473 +/- 140 x 10(9)/l), and then remained fairly constant. Thrombocytosis (> 500 x 10(9)/l) was observed in 9/24 (38%) subjects. Mean TPO level in CB was 5.11 +/- 1.51 fmol/ml, peaked at d 2 (7.64 +/- 3.28 fmol/ml), decreased at d 5 (3.93 +/- 1.67 fmol/ml), and thereafter kept fairly constant during the remaining neonatal period. Compared with term infants, mean TPO levels of preterm infants in CB and at d 2 were significantly higher (P < 0.01). There was an inverse correlation between platelet counts and TPO levels (r = 0.45, P < 0.001, n = 88). Preterm neonates with thrombocytosis had significantly higher TPO values in CB than those without thrombocytosis (P < 0.05). There was no significant relationship between platelet counts and IL-6. IL-11 was not detectable. These results suggest that an early elevation of serum TPO levels is related to the subsequent thrombocytosis in LBW preterm infants.

2
UI - 11808159
AU - Urabe A
TI - [Polycythemia vera]
SO - Nippon Rinsho 2001 Nov;59 Suppl 7():498-503
AD - Division of Hematology, NTT Kanto Medical Center.

3
UI - 11872082
AU - Hirri HM; Green PJ
TI - Skin lesion caused by hydroxyurea.
SO - Eur J Haematol 2001 Nov-Dec;67(5-6):328-9

4
UI - 11850996
AU - Gensini GF; Conti AA
TI - [Association between hepatocellular carcinoma and polycythemia vera]
SO - Recenti Prog Med 2002 Jan;93(1):25-7
AD - Clinica Medica Generale e Cardiologia, Universita, Firenze. g.gensini@dfc.unifi.it
The association of hepatocellular carcinoma (HCC) and polycythemia vera in a middle-aged caucasian man is described for the first time in this case report. The report underlines the necessity and the importance of a full, evidence-based investigation of the nature of polyglobuly in the course of HCC, a condition known to be associated to erythrocytosis, so far usually considered secondary to HCC, and not primary.

5
UI - 11840268
AU - Marone M; Scambia G; Bonanno G; Rutella S; de Ritis D; Guidi F; Leone G;
TI - Pierelli L Transforming growth factor-beta1 transcriptionally activates CD34 and prevents induced differentiation of TF-1 cells in the absence of any cell-cycle effects.
SO - Leukemia 2002 Jan;16(1):94-105
AD - Dept of Gynecology, Catholic University, Rome Italy.
A number of cytokines modulate self-renewal and differentiation of hematopoietic elements. Among these is transforming growth factor beta1 (TGF-beta1), which regulates cell cycle and differentiation of hematopoietic cells, but has pleiotropic activities depending on the state of responsiveness of the target cells. It has been previously shown by us and other authors that TGF-beta1 maintains human CD34(+) hematopoietic progenitors in an undifferentiated state, independently of any cell cycle effects, and that depletion of TGF-beta1 triggers differentiation accompanied by a decrease in CD34 antigen expression. In the present work, we show that exogenous TGF-beta1 upregulates the human CD34 antigen in the CD34(+) cell lines TF-1 and KG-1a, but not in the more differentiated CD34(-) cell lines HL-60 and K-562. We further studied this effect in the pluripotent erythroleukemia cell line TF-1. Here, TGF-beta1 did not effect cell growth, but induced transcriptional activation of full-length CD34 and prevented differentiation induced by differentiating agents. This effect was associated with nuclear translocation of Smad-2, activation of TAK-1, and with a dramatic decrease in p38 phosphorylation. In other systems TGF-beta1 has been shown to activate a TGF-beta-activated kinase 1 (TAK1), which in turn, activates p38. The specific inhibitor of p38 phosphorylation, SB202190, also increased CD34 RNA expression, indicating the existence of a link between p-38 inhibition by TGF-beta1 and CD34 overexpression. Our data demonstrate that TGF-beta1 transcriptionally activates CD34 and prevents differentiation of TF-1 cells by acting independently through the Smad, TAK1 and p38 pathways, and thus provide important clues for the understanding of hematopoietic development and a potential tool to modify response of hematopoietic cells to mitogens or differentiating agents.

6
UI - 11786204
AU - Lema-Kisoka R; Hayez N; Langer I; Robberecht P; Sariban E; Delporte C
TI - Characterization of functional VIP/PACAP receptors in the human erythroleukemic HEL cell line.
SO - Peptides 2001 Dec;22(12):2155-62
AD - Department of Biochemistry and Nutrition, Faculty of Medicine, Universite Libre de Bruxelles, Brussels, Belgium.
The presence of VIP/PACAP receptors was investigated on the human erythroleukemic cell line HEL. Specific binding of [125I]-PACAP or [125I]-VIP on HEL cells or membranes was very low and did not allow to perform competition curves. At 37 degrees C PACAP transiently increased cAMP levels in the presence of the non-specific phosphodiesterase inhibitor IBMX, suggesting rapid desensitization. Kinetic studies revealed that optimal conditions to measure the EC(50) of PACAP(1-27) were 10 min at 20 degrees C. Under those conditions, PACAP-related peptides increased cAMP levels with EC(50) in agreement with the pharmacological profile of the VPAC(1) receptor subtype: PACAP = VIP > [K(15), R(16,) L(27)]VIP(1-7)/GRF(8-27) = [R(16)]ChSn (two VPAC(1) agonists) >> helodermin = secretin. RO 25-1553, a selective activator of VPAC(2) receptor was inactive at 1 microM. Dose-response curves of VPAC(1) agonist molecules (PACAP, VIP, [K(15), R(16), L(27)]VIP(1-7)/GRF(8-27), [R(16)]ChSn) were shifted to the right by the VPAC(1) receptor antagonist [AcHis(1), D-Phe(2), Lys(15), Leu(17)]VIP(3-7)/GRF(8-27), with a K(i) of 3 +/- 1 nM (n = 3). The presence of VPAC(1) receptor mRNA was confirmed by RT-PCR. Preincubation with PACAP or PMA showed that VPAC(1) receptors underwent homologous and heterologous desensitization.This study provides the first evidence for the expression of functional VPAC(1) receptors undergoing rapid desensitization in HEL cells.

7
UI - 11400658
AU - Bakkaloglu SA; Soylemezoglu O; Karadeniz C; Oguz A; Buyan N; Hasanoglu E
TI - Pseudohyperkalemia due to reactive thrombocytosis in an infant with yolk sac tumor.
SO - Pediatr Hematol Oncol 2001 Jun;18(4):303-5
AD - Department of Pediatric Nephrology, Gazi University Faculty of Medicine, Ankara, Turkey. sevcan@med.gazi.edu.tr

8
UI - 11860444
AU - Allen AJ; Gale RE; Harrison CN; Machin SJ; Linch DC
TI - Lack of pathogenic mutations in the 5'-untranslated region of the thrombopoietin gene in patients with non-familial essential thrombocythaemia.
SO - Eur J Haematol 2001 Oct;67(4):232-7
AD - Department of Haematology, University College London, London, UK.
Thrombopoietin (TPO) is thought to be the major physiological regulator of thrombopoiesis, and, in general, circulating levels are inversely proportional to megakaryocyte and platelet mass. However, normal or elevated TPO levels are found in patients with essential thrombocythaemia (ET) and the reason for this is not fully understood. Recent studies have shown that four kindreds with hereditary thrombocythaemia (HT) have point mutations in the 5'-untranslated region (UTR) of the TPO gene which lead to increased TPO translation. In order to determine whether similar mutations are present in apparently acquired ET, in particular in those patients with polyclonal myelopoiesis, we have studied this region in 50 ET patients using neutrophil DNA. The known HT mutations were investigated using polymerase chain reaction with mismatch primers and restriction enzyme digestion; only wild-type alleles were detected. Single-stranded conformation polymorphism (SSCP) analysis of exons 1-4 identified a C-->T substitution at nucleotide 3767. However, this appears to be a common polymorphism, as it was present at the same frequency in haematologically normal controls and is unlikely to be of pathological significance. These results demonstrate that mutations in the 5' UTR of the TPO gene are not the cause of the normal or elevated TPO levels in acquired ET.

9
UI - 11841424
AU - Spivak JL
TI - The optimal management of polycythaemia vera.
SO - Br J Haematol 2002 Feb;116(2):243-54
AD - Division of Hematology, Johns Hopkins University School of Medicine, Baltimore, MD, USA. jlspivak@mail.jhmi.edu

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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