Hemorrhagic Thrombocythemia: A Critical Review

Gunz, F. W.

doi:10.1182/blood.v15.5.706.706

Cited by 175 publications

(21 citation statements)

References 16 publications

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“…Our findings of incidences around 50% represent almost a median of previous reported results. The IT group in the present study, showing increased NAP scores in 25% of the cases, is very small and no other reports, except 2 cases with very high NAP scores reported by Gum (17), are available for comparison. We have been able to show a statistically significant difference in the incidences of decreased and increased NAP scores between CML and the other representatives of the myeloproliferative syndrome (p(O.001).…”

Section: Single Cell N a P Activity;contrasting

confidence: 61%

Evaluation of neutrophil alkaline phosphatase (NAP) activity in untreated myeloproliferative syndromes and in leukaemoid reactions

Bendix-Hansen

Bergmann²

1985

Scandinavian Journal of Haematology

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Neutrophil alkaline phosphatase activity was estimated in 194 patients; 59 cases of chronic myeloid leukaemia (CML), 42 cases of polycythaemia vera (PV), 24 cases of primary myelofibrosis, 7 cases of idiopathic thrombocythaemia, 6 cases of leukaemoid reaction, 19 cases of secondary polycythaemia (PS) and 37 cases of the primary myelodysplastic syndrome (MDS). According to NAP activities the groups proved to be separate entities (p < 0.00025). The incidence of decreased NAP score in the CML group was 85% and differed significantly from the other groups as a whole, as well as separately (p< 0.001). The MDS group, the only group besides CML that showed decreased scores, also differed significantly from the others (p < 0.001). The PS group, nearly always showing normal scores, differed significantly from the PV group (p<0.0052). A method evaluating single cell NAP activity proved superior to the score method in discriminating between the different groups. Thus, the incidence of decreased activity in the CML group was 93 % compared with 85% by the score method and the incidences of increased activity in the PV, MP, IT, and LR groups were 79% to 100% compared with 25% to 67% by the score method. The latter difference was statistically significant (p=0.029).

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Section: Single Cell N a P Activity;contrasting

confidence: 61%

Evaluation of neutrophil alkaline phosphatase (NAP) activity in untreated myeloproliferative syndromes and in leukaemoid reactions

Bendix-Hansen

Bergmann²

1985

Scandinavian Journal of Haematology

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“…Note clusters of megakaryocytes (arrows) and atypical forms. Hematoxylin and eosin stain; original magnification x 100; reduced by 25 percent.karyocytic preponderance and increased reticulin deposition (FIGS 3. and 4).A bone marrow aspirate and biopsy from the iliac crest confirmed the initial impression of a myeloproliferative disease.…”

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confidence: 88%

Essential Thrombocythemia With Transition Into Acute Leukemia

Raman

Mahmood

Slyck

et al. 1981

Annals of the New York Academy of Sciences

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A 57-year-old black man with sustained platelet count of 2 million/mm3 and evidence of intermittent gastrointestinal bleeding was diagnosed as having essential thrombocythemia. Studies of bone marrow morphology, platelet aggregation, and other variables were confirmatory of the disease. The patient was treated briefly with low doses of Myleran for less than three weeks. He was then lost to follow-up study. Approximately 16 months later he reappeared complaining of recurrent nose bleeds. He was found to be pancytopenic and diagnosis of acute leukemia was made on the basis of bone marrow aspiration.

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“…With busulfan, most patients also reached CR, and remained in CR for a prolonged period after the drug was discontinued. The efficacy of busulfan3 or other alkylating drugs, such as No. 3 ESSENTIAL THROMBOCYTHEMIA -Fenazu et al 555 melphalan, chlorambucil, or lomustine (CCNU),I5 has also been shown in ET.…”

Section: Vol 66mentioning

confidence: 99%

Clinical course of essential thrombocythemia in 147 cases

Fenaux

Simon

Caulier

et al. 1990

Cancer

284

191

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The authors retrospectively analyzed the initial characteristics, treatment, and clinical course in 147 patients with essential thrombocythemia (ET). Median age was 60 years and the M:F ratio was 0.69. At diagnosis, 53 patients were asymptomatic; 50 patients had functional symptoms (mainly vasomotor disturbances); 27 patients had large vessel thrombosis; 27 patients had a bleeding diathesis; and seven patients had both bleeding and thrombosis. The platelet count ranged from 0.7 to 2.92 X 10(12)/l. Forty-five of the 61 tested patients (61%) had prolonged bleeding time and/or platelet hypoaggregation. Three patients had in vitro spontaneous aggregation. No significant correlations were found between hemostatic findings and in vivo bleeding or thrombosis. The incidence of bleeding, however, was higher in patients with more than 2 x 10(12)/l platelets. Of 87 karyotypes performed with banding techniques, only four were abnormal. One hundred twenty-nine patients received one or more cytoreductive agents at diagnosis or during follow-up. Sixty patients received an antiaggregating agent. First-line therapy was radiophosphorus (32P) in 22 patients; busulfan in 35 patients; and hydroxyurea in 72 patients. Hydroxyurea required continuous maintenance therapy and had to be changed to another treatment in 12 of the initial responders because of inadequate control of thrombocythemia. During follow-up, 14 treated patients experienced one or several major thrombotic events. Two untreated patients also had major thrombosis. Only one major bleeding event was seen during follow-up. Median actuarial survival was 73.5% at 7 years and only one patient progressed to acute non-lymphocytic leukemia (ANLL). These results suggest that large vessel thrombosis is the main complication of ET. It appears largely unpredictable in a given patient at diagnosis but can be largely prevented by the control of thrombocythemia. Because of the low incidence of side effects of treatment in this experience, the authors believe that cytoreductive therapy is indicated in most patients with ET, as long as a group of patients with very low risk of thrombosis is not defined in prospective studies.

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Hemorrhagic Thrombocythemia: A Critical Review

Cited by 175 publications

References 16 publications

Evaluation of neutrophil alkaline phosphatase (NAP) activity in untreated myeloproliferative syndromes and in leukaemoid reactions

Evaluation of neutrophil alkaline phosphatase (NAP) activity in untreated myeloproliferative syndromes and in leukaemoid reactions

Essential Thrombocythemia With Transition Into Acute Leukemia

Clinical course of essential thrombocythemia in 147 cases

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