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Paroxysmal nocturnal hemoglobinuria: a single Spanish center's experience over the last 40 yr.

Muñoz-Linares C, Ojeda E, Forés R, Pastrana M, Cabero M, Morillo D, Bautista G, Baños I, Monteserín C, Bravo P, Jaro E, Cedena T, Steegmann JL, Villegas A, Cabrera JR - Eur. J. Haematol. (2014)

Bottom Line: The average clonal size upon diagnosis was 48%, presenting a variable evolution.Thrombotic episodes and cancer were five each, and the main causes of death among our patients were equal at 8.9%.Sixteen patients have been treated with eculizumab so far in our series, and being a safe drug, it provides improvement in the patients' quality of life, and the disappearance of clinical symptoms, and avoids the emergence of new thrombosis.

View Article: PubMed Central - PubMed

Affiliation: Hematology Service, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.

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Related in: MedlinePlus

Lineal relationship between paroxysmal nocturnal hemoglobinuria (PNH) clone and lactate dehydrogenase (LDH) levels. LDH levels were correlated with the PNH clone in granulocytes in the same day in 199 samples of patients with PNH. Patients on eculizumab were excluded. LDH levels over normal could be calculated in our series in base to PNH clone in granulocytes with a mathematic equation as follows: LDH = 0.485 + 0.067 × PNH clone. r = 0.651 P < 0.001.
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fig01: Lineal relationship between paroxysmal nocturnal hemoglobinuria (PNH) clone and lactate dehydrogenase (LDH) levels. LDH levels were correlated with the PNH clone in granulocytes in the same day in 199 samples of patients with PNH. Patients on eculizumab were excluded. LDH levels over normal could be calculated in our series in base to PNH clone in granulocytes with a mathematic equation as follows: LDH = 0.485 + 0.067 × PNH clone. r = 0.651 P < 0.001.

Mentions: The most severe clinical expressivity attributable to hemolysis, including thrombotic events, is represented in the group of patients with classic disease forms in which lactate dehydrogenase (LDH) levels are significantly higher. A linear relationship is established between levels of LDH and size of clone PNH, represented in Fig.1.


Paroxysmal nocturnal hemoglobinuria: a single Spanish center's experience over the last 40 yr.

Muñoz-Linares C, Ojeda E, Forés R, Pastrana M, Cabero M, Morillo D, Bautista G, Baños I, Monteserín C, Bravo P, Jaro E, Cedena T, Steegmann JL, Villegas A, Cabrera JR - Eur. J. Haematol. (2014)

Lineal relationship between paroxysmal nocturnal hemoglobinuria (PNH) clone and lactate dehydrogenase (LDH) levels. LDH levels were correlated with the PNH clone in granulocytes in the same day in 199 samples of patients with PNH. Patients on eculizumab were excluded. LDH levels over normal could be calculated in our series in base to PNH clone in granulocytes with a mathematic equation as follows: LDH = 0.485 + 0.067 × PNH clone. r = 0.651 P < 0.001.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4232878&req=5

fig01: Lineal relationship between paroxysmal nocturnal hemoglobinuria (PNH) clone and lactate dehydrogenase (LDH) levels. LDH levels were correlated with the PNH clone in granulocytes in the same day in 199 samples of patients with PNH. Patients on eculizumab were excluded. LDH levels over normal could be calculated in our series in base to PNH clone in granulocytes with a mathematic equation as follows: LDH = 0.485 + 0.067 × PNH clone. r = 0.651 P < 0.001.
Mentions: The most severe clinical expressivity attributable to hemolysis, including thrombotic events, is represented in the group of patients with classic disease forms in which lactate dehydrogenase (LDH) levels are significantly higher. A linear relationship is established between levels of LDH and size of clone PNH, represented in Fig.1.

Bottom Line: The average clonal size upon diagnosis was 48%, presenting a variable evolution.Thrombotic episodes and cancer were five each, and the main causes of death among our patients were equal at 8.9%.Sixteen patients have been treated with eculizumab so far in our series, and being a safe drug, it provides improvement in the patients' quality of life, and the disappearance of clinical symptoms, and avoids the emergence of new thrombosis.

View Article: PubMed Central - PubMed

Affiliation: Hematology Service, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain.

Show MeSH
Related in: MedlinePlus