Acquired Pyruvate Kinase Deficiency with Hemolysis in Preleukemia

Helmstädter, V.; Arnold, H.; Blume, K. G.; N, Uhl; Hünstein, W.

doi:10.1159/000207900

Cited by 8 publications

(8 citation statements)

References 6 publications

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“…It is not the only red cell protein anomaly observed in patients with myelodysplastic syndrome. There are also reports on imbalances of a-and b-globin chains [16,22], abnormal membrane protein composition, and abnormally reduced erythrocyte enzyme activities [9,18]. It is not known whether hemoglobin F is synthesized by the abnormal clonogenic cells of the myelodysplastic syndrome or whether it represents the ability of the bone marrow to reactivate "fetal erythrocyte progenitors" [24].…”

Section: Discussionmentioning

confidence: 99%

Hemoglobin F in myelodysplastic syndrome

Reinhardt

Haase

Schoch

et al. 1998

Annals of Hematology

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Reactivation of fetal hemoglobin synthesis in adulthood can be seen in hematological disorders affecting the erythropoietic system. The objective of the present study was to evaluate the incidence and prognostic significance of increased hemoglobin F in patients with myelodysplastic syndrome. Hemoglobin F concentrations and Ggamma/Ggamma + A gamma-globin chain ratios were determined in 26 patients with primary myelodysplastic syndrome. Median age of the patients was 65 years; all FAB subtypes were included. Increased hemoglobin F concentration of up to 20% of total hemoglobin (normal: below 2%) was seen in 16 patients; ten patients had normal values. There was a significant relation between hemoglobin F concentration and the course of disease, e.g., 12 of the 16 patients with elevated hemoglobin F survived at least 1 year after the examination, in contrast to only three of the ten patients with normal hemoglobin F (p < 0.025). All of six patients with hemoglobin F above 5% survived at least 1 year. There was no significant difference in the hemoglobin F concentration between patients with and without cytogenetic anomalies. The Ggamma/Ggamma + A gamma-globin chain ratio was slightly elevated in all patients, with a weak correlation to the degree of hemoglobin F elevation. The values were not of additional prognostic significance. The data of the present study suggest that the hemoglobin F concentration may be a prognostic parameter in myelodysplastic syndrome; increased hemoglobin F concentration may indicate a better prognosis.

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Section: Discussionmentioning

confidence: 99%

Hemoglobin F in myelodysplastic syndrome

Reinhardt

Haase

Schoch

et al. 1998

Annals of Hematology

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“…A decreased PK activity has been described in patients with preleukaemia and acute leukaemia (25·0% in 140 cases) or bone marrow failure (50·3% in 201 cases), but less frequently in myeloproliferative neoplasms (3·4% in 59 cases) (Table S2). 5–10 However a decreased G6PD activity has only been reported in rare patients with leukaemia (2·8% in 72 cases) 8 …”

Section: Figurementioning

confidence: 99%

“…A decreased PK activity has been described in patients with preleukaemia and acute leukaemia (25•0% in 140 cases) or bone marrow failure (50•3% in 201 F I G U R E 1 Main biologic features during follow-up 1A: G6PD gene analysis; 1B: Evolution of haemoglobin and reticulocytes count cases), but less frequently in myeloproliferative neoplasms (3•4% in 59 cases) (Table S2). [5][6][7][8][9][10] However a decreased G6PD activity has only been reported in rare patients with leukaemia (2•8% in 72 cases). 8 Residual activities vary in these studies, from 20% to 80%, higher than 50% in most cases, and without clinical and biological impact.…”

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Acquired glucose 6‐phosphate dehydrogenase (G6PD) deficiency in a patient with Chronic Myelomonocytic Leukemia

et al. 2022

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“…PK enzymopathy is mainly found in sideroblas tic anemia, acute leukemias, and the socalled preleukemic states [4][5][6].…”

Section: Introductionmentioning

confidence: 99%

Red Cell Pyruvate Kinase in Acute Leukemia

Labar¹,

Stavljenić²,

Jusufhodzić³

1984

Enzyme

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The red cell pyruvate kinase (PK) activity, K(M) for phosphoenolpyruvate (PEP) and adenosine diphosphate (ADP), and thermostability properties were studied in patients with acute leukemias. The acquired PK defect was found in patients with acute myeloid leukemia, while it was normal in acute non-myeloid leukemia enzyme kinetic tests. PK abnormality was expressed in 17 patients as deficient PK activity, in 15 patients as decreased enzyme thermostability, and in 11 patients as altered PK affinity for ADP.

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Acquired Pyruvate Kinase Deficiency with Hemolysis in Preleukemia

Cited by 8 publications

References 6 publications

Hemoglobin F in myelodysplastic syndrome

Hemoglobin F in myelodysplastic syndrome

Acquired glucose 6‐phosphate dehydrogenase (G6PD) deficiency in a patient with Chronic Myelomonocytic Leukemia

Red Cell Pyruvate Kinase in Acute Leukemia

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