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

International Advisory Board Khosrow Adeli Canada
Sergio Bernardini Italy
Marcello Ciaccio Italy
Eleftherios Diamandis Canada
Kjell Grankvist Sweden
Hans Jacobs The Netherlands
Eric Kilpatrick UK
Magdalena Krintus Poland
Giuseppe Lippi Italy
☩Howard Morris Australia
Mario Plebani Italy
Sverre Sandberg Norway
Ana-Maria Simundic Croatia
☩Jill Tate Australia
Tommaso Trenti Italy
Cas Weykamp The Netherlands
Maria Willrich USA
Paul Yip Canada


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Biomedia srl
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Responsible Editor
Giuseppe Agosta

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Arianna Lucini Paioni
Biomedia srl
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email: biochimica.clinica@sibioc.it



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BC: Articoli scritti da M.C. Sanzari

Emoglobinuria parossistica notturna
Paroxysmal nocturnal hemoglobinuria
<p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired&nbsp;hematological disease of the hematopoietic stem cell. PNH arises as a consequence of non-malignant clonal&nbsp;expansion of hematopoietic stem cells and progeny mature blood cells of both myeloid and monocyte lineage, which&nbsp;are deficient in some surface proteins, including the two complement regulators CD55 and CD59. As a result, PNH&nbsp;erythrocytes are incapable to modulate on their surface physiologic complement activation, leading to complement-mediated&nbsp;intravascular hemolysis, which is the central clinical feature of PNH. Diagnosis and monitoring of PNH&nbsp;clones currently rely on the analysis of CD59 expression on red blood cells and FLAER (fluorescent aerolysin) and&nbsp;some glycophosphatidylinositol-anchored proteins on granulocyte and monocyte lineages by flow cytometry. Despite&nbsp;the availability of safe and effective targeted therapy that controls intravascular hemolysis, the management of PNH&nbsp;remains difficult because of disease heterogeneity and close association with bone marrow failure syndromes.</p>
Biochimica Clinica ; 37(4) 268-274
Rassegne - Reviews
 
Un caso di emoglobinuria parossistica notturna associata a mielodisplasia
A case of paroxysmal nocturnal hemoglobinuria in a patient with myelodysplasia
<p>Paroxysmal nocturnal&nbsp;hemoglobinuria (PNH) is a rare acquired hematological disease of the hematopoietic stem cell. PNH arises as a&nbsp;consequence of non-malignant clonal expansion of hematopoietic stem cells and progeny mature blood cells of both&nbsp;myeloid and monocyte lineage, which are deficient in some surface proteins, including the two complement regulators&nbsp;CD55 and CD59. As a result, PNH erythrocytes are incapable to modulate on their surface physiologic complement&nbsp;activation, leading to complement-mediated intravascular hemolysis, which is the central clinical feature of PNH. We&nbsp;present a case of a 16 years old Ukrainian boy who presented with a diagnosis of myelodysplastic syndrome and&nbsp;who was found to be affected by PNH.</p>
Biochimica Clinica ; 37(4) 326-328
Casi clinici - Case Report