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NCI CANCERLIT^® Search: Myeloproliferative Disorders - May 2002

Imprima English

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

Erythroleukemia: a comparison between the previous FAB approach and the WHO classification.

On the diagnosis of polycythaemia vera as assessed in the health and medical care in the Vastra Gotaland region, Sweden.

Allogeneic blood cell transplantation following reduced-intensity conditioning is effective therapy for older patients with myelofibrosis

Symptoms, symptom distress and health-related quality of life in patients with polycythaemia vera or essential thrombocythaemia during

Serum erythropoietin values in erythrocytoses and in primary thrombocythaemia.

Table of Contents

1
UI - 11916513
AU - Park S; Picard F; Azgui Z; Viguie F; Merlat A; Guesnu M; Leblond V;
TI - Dreyfus F Erythroleukemia: a comparison between the previous FAB approach and the WHO classification.
SO - Leuk Res 2002 May;26(5):423-9
AD - Service d'hematologie, Hopital Cochin, 27 rue du Faubourg St-Jacques, 75014 Paris, France. parksophie@yahoo.com
Erythroleukemia is, within FAB classification, a proliferation of erythroblasts superior to 50% and of myeloblasts superior to 30%. The new WHO classification abolishes the frontier between RAEB-t with 20% and leukemia with 30% of blasts. AML6 variant is a new entity characterized by the proliferation of immature erythroblasts and the absence of non-erythroid blast cells. We analyzed 16 erythroleukemia, 5 RAEB-t and 2 AML6 variants to clarify their relationship.We suggest on survival, karyotype and cytologic characteristics that secondary erythroleukemia are the same entity as RAEB-t, confirming the WHO classification and that amongst de novo erythroleukemia, there is 'AML6 variant' with pure erythroid lineage proliferation.

2
UI - 11952886
AU - Johansson P; Andreasson B; Safai-Kutti S; Rhedin C; Vilen L; Vaart J;
TI - Kutti J On the diagnosis of polycythaemia vera as assessed in the health and medical care in the Vastra Gotaland region, Sweden.
SO - J Intern Med 2002 Apr;251(4):348-54
AD - Haematology Section, Department of medicine, Sahlgrenska University hospital, Goteborg, Sweden. peter.l.johansson@vgregion.se
OBJECTIVES: The aim was to assess how the diagnosis of polycythaemia vera (PV) was established and to study to which extent the classic Polycythemia Vera Study Group (PVSG) criteria and the revised criteria for the diagnosis of PV as proposed by Pearson and Messinezy in 1996 (PM criteria) were fulfilled when the diagnosis of PV was made. DESIGN AND SETTING: A questionnaire was sent to physicians in charge of haematological patients at the departments of medicine in 12 hospitals in the Health and Medical Care in the Vastra Gotaland Region (VGR), Sweden, with a population of 1.5 million inhabitants; they were asked to provide reports as regards all patients with an unequivocal diagnosis of Full reports were obtained from six hospitals, which serve about 1.1 million inhabitants. The results from a total of 129 PV patients, 62 from two university hospitals (UHs) and 67 from four county hospitals (CHs), were the subject of the present analysis. Results. It was shown that measurement of the red cell mass (RCM) had been carried out in 61 of 62 (98%) patients in UHs compared with 24 of 67 (36%) patients at CHs (P < 0.01). By using ultrasound imaging and/or scintigraphy the spleen size had been determined in 55 of 62 (89%) patients at UHs and in 24 of 67 (36%) patients at CHs (P < 0.01). At the UHs, arterial oxygen saturation had been measured in 32 of 62 (52%) patients; the corresponding figure for the CH patients was 22 of 67 (33%). Plasma or serum erythropoietin (EPO) concentrations had been measured in 44 of 62 (71%) of PV patients at UHs and in 31 of 67 (46%) of patients at CHs; in all these cases the EPO concentrations were subnormal or not measurable. CONCLUSIONS: At the UHs only 37% of the PV patients fulfilled the PVSG criteria for the diagnosis of PV whereas the corresponding figure for CHs was 4% (P < 0.01). The adherence to PM criteria was, however, 71% at UHs compared with 16% at CHs (P < 0.01).

3
UI - 11877308
AU - Devine SM; Hoffman R; Verma A; Shah R; Bradlow BA; Stock W; Maynard V;
TI - Jessop E; Peace D; Huml M; Thomason D; Chen YH; van Besien K Allogeneic blood cell transplantation following reduced-intensity conditioning is effective therapy for older patients with myelofibrosis with myeloid metaplasia.
SO - Blood 2002 Mar 15;99(6):2255-8
AD - Blood and Marrow Transplantation Program, Section of Hematology/Oncology, University of Illinois College of Medicine, 840 South Wood Street, M/C 787, Chicago, IL 60612, USA. sdevine@uic.edu
Standard myeloablative conditioning prior to allogeneic hematopoietic stem cell (HSC) transplantation has been associated with significant toxicity in patients older than 45 years of age with myelofibrosis with myeloid metaplasia (MMM). We sought to evaluate the efficacy of a reduced-intensity conditioning regimen for allogeneic HSC transplantation in this setting. A regimen consisting of fludarabine (30 mg/m(2) intravenously daily for 5 days) and melphalan (70 mg/m(2) intravenously daily for 2 days) followed by transplantation of filgrastim-mobilized peripheral blood cells from HLA-identical siblings was administered to 4 older patients (median age, 56 years; range, 48-58 years) with advanced MMM. All patients achieved prompt neutrophil and platelet engraftment and have experienced a significant regression of splenomegaly and bone marrow fibrosis. All now have normal bone marrow cellularity. With a median follow-up of 13 months (range, 11-19 months), all 4 patients are alive with stable full-donor hematopoietic chimerism. These results support the feasibility and effectiveness of reduced-intensity conditioning prior to allogeneic HSC transplantation for older patients with advanced MMM.

4
UI - 11990518
AU - Merup M; Aberg W; Lofvenberg E; Svensson E; Engman K; Paul C; Gardulf A
TI - Symptoms, symptom distress and health-related quality of life in patients with polycythaemia vera or essential thrombocythaemia during treatment with interferon-alpha.
SO - Acta Oncol 2002;41(1):50-5
AD - Department of Medicine, Karolinska Institutet at Huddinge University Hospital, Sweden. mats.merup@medhs.ki.se
Sixteen patients with polycythaemia vera or essential thrombocythaemia were treated with interferon-alpha in order to normalize elevated platelets. Patients were followed for 6 months and the frequency and intensity of symptoms and side effects were recorded before and during the study period by the patients and by the doctor. Health-related quality of life was also assessed. The most frequently reported pretreatment symptoms were fatigue, headache and muscle pain. The intensity of fatigue initially increased during treatment and there was no relief of any of the three most frequent symptoms during the treatment period. Common interferon-related symptoms such as fever and chills were most frequently reported after one week. After one month of treatment, symptoms related to the gastrointestinal tract reached a peak. Two patients discontinued treatment during the study period. Another patient suffered severe depression after the study period when still on interferon. There was no difference between the frequency of symptoms recorded by the doctor and that reported by the patients.

5
UI - 11918532
AU - Messinezy M; Westwood NB; El-Hemaidi I; Marsden JT; Sherwood RS; Pearson
TI - TC Serum erythropoietin values in erythrocytoses and in primary thrombocythaemia.
SO - Br J Haematol 2002 Apr;117(1):47-53
AD - Department of Haematological Medicine and Department of Clinical Pathology, Guy's, King's and St Thomas' School of Medicine, London, UK.
Serum erythropoietin (Epo) values were estimated in samples from 125 patients with erythrocytosis to examine the specificity and sensitivity of reduced and raised values in the diagnosis of polycythaemia vera (PV) and secondary erythrocytosis (SE) respectively. Additionally, Epo values were estimated in samples from 49 patients with primary thrombocythaemia (PT) to determine whether Epo values were altered. We found high specificity (92%) and moderate sensitivity (64%) of low serum Epo values (below the reference range) in the diagnosis of PV, and also poor sensitivity (47%) of raised Epo values in the diagnosis of SE. Raised Epo values were not observed in PV patients with Hb > 14.0 g/dl and were only observed in one PV patient with a relatively low Hb recovering from a gastro-intestinal haemorrhage. Raised Epo values occurred in some patients with apparent erythrocytosis (AE) and idiopathic erythrocytosis (IE), mainly at normal (rather than raised) Hb values (< 16 g/dl). Low Epo values occurred in a few AE, IE and SE patients at higher Hb values (> 16 g/dl). Low Epo values were less specific for PV when the Hb was raised, while raised Epo values were less specific for SE when the Hb was not raised. Approximately one third of patients with PT had a low (below the reference range) Epo value, this being associated with a high normal Hb (> 14 g/dl, P < 0.001) and showing a trend towards association with absence of treatment. The high normal Hb values were in turn associated with an increased incidence of thrombotic events (P < 0.05). These findings could influence the future investigation and management of PT patients.

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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