scholarly journals Impact of Whole Blood Storage Conditions in Flow Cytometric Analysis for Paroxysmal Nocturnal Hemoglobinuria

2021 ◽  
Vol 43 (3) ◽  
pp. 143-151
Author(s):  
Sewon Yoon ◽  
Jimyung Kim ◽  
Seon Young Kim ◽  
Jinsook Lim ◽  
Gye Cheol Kwon ◽  
...  
PLoS ONE ◽  
2011 ◽  
Vol 6 (3) ◽  
pp. e17813 ◽  
Author(s):  
Urban Sester ◽  
Mathias Fousse ◽  
Jan Dirks ◽  
Ulrich Mack ◽  
Antje Prasse ◽  
...  

Medicine ◽  
2004 ◽  
Vol 83 (3) ◽  
pp. 193-207 ◽  
Author(s):  
Jun-Ichi Nishimura ◽  
Yuzuru Kanakura ◽  
Russell E. Ware ◽  
Tsutomu Shichishima ◽  
Hideki Nakakuma ◽  
...  

2017 ◽  
Vol 37 (4) ◽  
pp. 855-867 ◽  
Author(s):  
Mike Keeney ◽  
Andrea Illingworth ◽  
D. Robert Sutherland

Blood ◽  
1993 ◽  
Vol 81 (7) ◽  
pp. 1855-1862 ◽  
Author(s):  
T Shichishima ◽  
T Terasawa ◽  
C Hashimoto ◽  
H Ohto ◽  
M Takahashi ◽  
...  

We performed a flow cytometric analysis using monoclonal antibodies to decay accelerating factor (DAF) and CD59/membrane attack complex inhibitory factor (CD59/MACIF) in order to investigate the leukemic cells and erythrocytes from a patient with paroxysmal nocturnal hemoglobinuria (PNH) who developed acute myelocytic leukemia. In May 1990, the leukemic cells comprised 70% of the mononuclear cells in the bone marrow and 76% of those in the peripheral blood. They consisted of a mixture of positive and negative populations, including single DAF- positive cells. In August 1990, almost 100% of the peripheral mononuclear cells were leukemic blasts, and these consisted of a single population with reduced DAF expression. Single-color flow cytometric analysis showed that the leukemic cells lacked CD59/MACIF, while control leukemic cells (n = 3) expressed both DAF and CD59/MACIF. Leukemic blasts from this patient and six control patients expressed lymphocyte function-associated antigen 3 and FcIII receptors (CD 16) both before and after treatment with phosphatidylinositol-specific phospholipase C. The patient's erythrocytes lacking DAF and CD59/MACIF expression corresponded to the proportion of complement-sensitive cells at the onset of acute leukemia. These DAF- and CD59/MACIF-deficient erythrocytes disappeared almost completely with progression of the leukemia. In conclusion, it appears that the expression of glycosylphosphatidylinositol-linked membrane proteins by leukemic cells was heterogeneous and discordant in our patient, and that the leukemic cells were derived from the PNH clone because of their deficiency of CD59/MACIF. It is also suggested that DAF could compete more effectively than CD59/MACIF for a limited number of anchor molecules available on the proliferating leukemic cells.


TH Open ◽  
2020 ◽  
Vol 04 (01) ◽  
pp. e36-e39
Author(s):  
Christina Griesser ◽  
Michael Myskiw ◽  
Werner Streif

AbstractParoxysmal nocturnal hemoglobinuria (PNH) is a chronic disease caused by complement-mediated hemolysis. Clinical symptoms include intravascular hemolysis, nocturnal hemoglobinuria, thromboses, cytopenia, fatigue, abdominal pain, and a strong tendency toward bone marrow failure. It is a rare disease, especially in children, with high mortality rates without appropriate treatment.We here present the case of a 17-year-old girl with unprovoked muscle vein thrombosis. Flow cytometric analysis showed deficiency of glycosyl-phosphatidylinositol-anchored membrane proteins on all three hematopoietic cell lines and confirmed the diagnosis of PNH. Treatment with the monoclonal antibody eculizumab achieved long-term remission.As flow cytometry is normally not part of the routine diagnostics for pediatric thrombosis, awareness is crucial and PNH is important to consider in all children with thrombosis at atypical sites and abnormalities in blood counts with regard to hemolysis and cytopenia.


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