You don't have or don't remember the password! Click Here
Editor-in-chief
Maria Stella Graziani
Deputy Director
Martina Zaninotto
Associate Editors
Ferruccio Ceriotti
Davide Giavarina
Bruna Lo Sasso
Giampaolo Merlini
Martina Montagnana
Andrea Mosca
Paola Pezzati
Rossella Tomaiuolo
Matteo Vidali
EIC Assistant
Francesco Busardò
International Advisory Board
Khosrow Adeli Canada
Sergio Bernardini Italy
Marcello Ciaccio Italy
Eleftherios Diamandis Canada
Philippe Gillery France
Kjell Grankvist Sweden
Hans Jacobs The Netherlands
Eric Kilpatrick UK
Magdalena Krintus Poland
Giuseppe Lippi Italy
Mario Plebani Italy
Sverre Sandberg Norway
Ana-Maria Simundic Croatia
Tommaso Trenti Italy
Cas Weykamp The Netherlands
Maria Willrich USA
Paul Yip Canada
Publisher
Biomedia srl
Via L. Temolo 4, 20126 Milano
Responsible Editor
Giuseppe Agosta
Editorial Secretary
Chiara Riva
Biomedia srl
Via L. Temolo 4, 20126 Milano
Tel. 0245498282
email: biochimica.clinica@sibioc.it
<p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematological disease of the hematopoietic stem cell. PNH arises as a consequence of non-malignant clonal expansion of hematopoietic stem cells and progeny mature blood cells of both myeloid and monocyte lineage, which are deficient in some surface proteins, including the two complement regulators CD55 and CD59. As a result, PNH erythrocytes are incapable to modulate on their surface physiologic complement activation, leading to complement-mediated intravascular hemolysis, which is the central clinical feature of PNH. Diagnosis and monitoring of PNH clones currently rely on the analysis of CD59 expression on red blood cells and FLAER (fluorescent aerolysin) and some glycophosphatidylinositol-anchored proteins on granulocyte and monocyte lineages by flow cytometry. Despite the availability of safe and effective targeted therapy that controls intravascular hemolysis, the management of PNH remains difficult because of disease heterogeneity and close association with bone marrow failure syndromes.</p>
<p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired hematological disease of the hematopoietic stem cell. PNH arises as a consequence of non-malignant clonal expansion of hematopoietic stem cells and progeny mature blood cells of both myeloid and monocyte lineage, which are deficient in some surface proteins, including the two complement regulators CD55 and CD59. As a result, PNH erythrocytes are incapable to modulate on their surface physiologic complement activation, leading to complement-mediated intravascular hemolysis, which is the central clinical feature of PNH. We present a case of a 16 years old Ukrainian boy who presented with a diagnosis of myelodysplastic syndrome and who was found to be affected by PNH.</p>
Biochimica Clinica ; 37(4) 326-328 Casi clinici - Case Report
Gentile utente, le ricordiamo che l'accesso alla rivista è riservato ai soci SIBioC in regola con la quota associativa.
Per accedere alla pagina richiesta è necessario inserire il suo nome utente e la password nella sezione "AREA SOCI".
A disposizione per ulteriori informazioni.
La Segreteria SIBioC
Dear User, please note that access to the Journal is for SIBioC members who have renewed their membership fee.
To access the requested page you need to enter username and password in "Area Soci".
At your disposal for further information.
SIBioC Secretariat