Diagnostic screening of paroxysmal nocturnal hemoglobinuria: Prospective multicentric evaluation of the current medical indications

Abstract

In summary, our results demonstrate that the current medical indications for PNH screening by FCM are highly efficient, although improved screening algorithms are needed for patients presenting with thrombosis and normal blood cell counts. © 2016 International Clinical Cytometry Society.

The Monoclonal Anti‐CD157 Antibody Clone SY11B5, Used for High Sensitivity Detection of PNH Clones on WBCs, Fails to Detect a Common Polymorphic Variant Encoded byBST‐1

“…The other main symptoms belonging to the classical clinical triad of PNH are bone marrow failure and thrombosis especially at unusual sites which is the single most frequent cause of death in PNH patients (3)(4)(5). Furthermore, flow cytometry is preferentially used for follow-up of PNH patients or patients that were suffering from other bone marrow failures like aplastic anemia or refractory cytopenia with unilineage dysplasia (RCUD) (3,8) as there is a well-known association between PNH and these other bone marrow diseases (10)(11)(12)(13)(14). Flow cytometry is the gold standard for identification and quantification of PNH phenotypes (7)(8)(9).…”

“…Using this method even small populations of GPI-deficient cells can be detected, which allows a quantitative assessment of the PNH population. Furthermore, flow cytometry is preferentially used for follow-up of PNH patients or patients that were suffering from other bone marrow failures like aplastic anemia or refractory cytopenia with unilineage dysplasia (RCUD) (3,8) as there is a well-known association between PNH and these other bone marrow diseases (10)(11)(12)(13)(14). Diverse markers and staining strategies for PNH testing are discussed in different manuscripts (7,9,11,(15)(16)(17)(18)(19).…”

Myeloproliferative neoplasms, thrombosis and paroxysmal nocturnal hemoglobinuria: Is this triad more frequent than we thought?

“…Consensus Recommendations [6,7] indicate that patients requiring a PNH screening include those presenting with thrombosis associated with signs of hemolytic anemia or with unexplained cytopenia, or thrombosis at unusual sites (such as splanchnic veins or cerebral sinuses); the latter recommendation has been summarized under the term 'unexplained thrombosis' in the Consensus Recommendations text itself [6], and in the subsequent scientific papers or specialized educational material [8][9][10].…”

Flow cytometry—Recognizing unusual populations in leukemia and lymphoma diagnosis

“…The monocyte population was confirmed with a CD14/FLAER‐negative population gated on CD64‐positive cells (data not shown). In a recent study from Spain and Brazil which examined PNH clones in almost 4000 patients, 45% of 541 patients with aplastic anemia had a PNH clone present, indicating the utility of screening for PNH in this patient group.…”