Detailed immunophenotypic characterization of different major and minor subsets of peripheral blood cells in patients with paroxysmal nocturnal hemoglobinuria

Hernández-Campo, Pilar; Almeida, Júlia; Acevedo, María José; Sánchez, María Luz; Alberca, Ignacio; Vidriales, María‐Belén; Martínez, Elvira Aguilar; Romero, Juan R; Órfão, Alberto

doi:10.1111/j.1537-2995.2008.01686.x

Cited by 26 publications

(29 citation statements)

References 33 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…But studies of recent years have shown that CD59 ? antigen is stable on cell surface in ex vivo experiments [12,35]. Our study also showed that although the percentage of CD34 ?…”

Section: Discussionsupporting

confidence: 57%

See 1 more Smart Citation

Ex vivo expansion and long-term hematopoietic reconstitution ability of sorted CD34+CD59+ cells from patients with paroxysmal nocturnal hemoglobinuria

Han

Zhong

et al. 2010

Int J Hematol

View full text Add to dashboard Cite

Autologous bone marrow transplantation (ABMT) for paroxysmal nocturnal hemoglobinuria (PNH) remains difficult so far. To expand residual normal CD34(+)CD59(+) cells isolated from patients with PNH and observe the long-term hematopoietic reconstruction ability of the expanded cells both ex vivo and in vivo, CD34(+)CD59(+) cells from 13 PNH patients and CD34(+) cells from 11 normal controls were separated from bone marrow mononuclear cells first by immunomagnetic microbeads and then by flow cytometry autoclone sorting. The cells were then cultivated under different conditions. The long-term hematopoietic supporting ability of expanded CD34(+)CD59(+) cells was evaluated by long-term culture in semi-solid medium in vitro and long-term engraftment in irradiated severe combined immunodeficiency (SCID) mice in vivo. The best combination of hematopoietic growth factors for ex vivo expansion was SCF + IL-3 + IL-6 + FL + Tpo + Epo. The most suitable time for harvest was on day 7. CD34(+)CD59(+) PNH cells retained strong colony-forming capacity even after expansion. The survival rate, complete blood cell count recovery on day 90, and human CD45 expression in different organs were similar between the irradiated SCID mice transplanted with expanded CD34(+)CD59(+) PNH cells and those with normal CD34(+) cells (P > 0.05) both in primary and secondary transplantation. These data provided a new potential way of managing PNH with ABMT.

show abstract

“…But studies of recent years have shown that CD59 ? antigen is stable on cell surface in ex vivo experiments [12,35]. Our study also showed that although the percentage of CD34 ?…”

Section: Discussionsupporting

confidence: 57%

“…Monoclonal antibodies to GPIanchored proteins (e.g. anti-CD59 and anti-CD55) began to replace the sucrose hemolysis and Ham test in the diagnosis of PNH in the early 1990s [10][11][12]. Blood cells with PNH phenotype (GPI-AP-) and those with normal phenotype (GPI-AP?)…”

Section: Introductionmentioning

confidence: 99%

Ex vivo expansion and long-term hematopoietic reconstitution ability of sorted CD34+CD59+ cells from patients with paroxysmal nocturnal hemoglobinuria

Han

Zhong

et al. 2010

Int J Hematol

View full text Add to dashboard Cite

show abstract

“…Other researchers have excluded this possibility, since different patterns of diminished expression of Cregs were observed on each cell type, strongly suggesting the participation of different lineage-specific physiopathology processes [65]. Nevertheless, similar variability has been described in the pattern of expression of GPI-anchored proteins (GPI-APs) explored among different hematopoietic cell-lineage subpopulations in patients with PNH and normal individuals [80,81]. Moreover, Hernandez-Campo et al reported that DAF and MIRL expression is highly variable among different cell-lineage populations, while the latter shows higher amounts on RBCs.…”

Section: Discussionmentioning

confidence: 99%

The presence of CD55- and/or CD59-deficient erythrocytic populations in patients with rheumatic diseases reflects an immune-mediated bone-marrow derived phenomenon

et al. 2014

View full text Add to dashboard Cite

BackgroundComplement has the potential to provoke severe impairment to host tissues, as shown in autoimmune diseases where complement activation has been associated with diminished CD55 and/or CD59 expression on peripheral blood cell membranes. The aim of this study was to evaluate the presence of CD55- and/or CD59-deficient erythrocytic populations in patients with different rheumatic diseases and to investigate possible correlations with clinical or laboratory parameters.Material/MethodsCD55 and CD59 expression was evaluated in erythrocytes of 113 patients with rheumatic diseases, 121 normal individuals, and 10 patients with paroxysmal nocturnal hemoglobinuria (PNH) using the Sephacryl gel microtyping system. Ham and sucrose tests were also performed.ResultsInterestingly, the majority of patients (104/113, 92%) demonstrated CD55- and/or CD59-deficient erythrocytes: 47 (41.6%) with concomitant deficiency of CD55 and CD59, 50 (44.2%) with isolated deficiency of CD55, and 6 (6.2%) with isolated deficiency of CD59. In normal individuals, only 2 (1%) had concomitant CD55/CD59 negativity and 3 (2%) had isolated CD55 or CD59 deficiency. All PNH patients exhibited simultaneous CD55/CD59 deficiency. Positive Ham and sucrose tests were found only in PNH patients. There was no association between the CD55- and/or CD59-deficient erythrocytes and hemocytopenias or undergoing treatment. However, CD55 expression significantly influenced hemoglobin values (F=6.092, p=0.015).ConclusionsThis study provides evidence supporting the presence of erythrocytes with CD55 and/or CD59 deficiency in patients with rheumatic diseases. Moreover, CD55 deficiency on red cells influences hemoglobin concentration. Further studies using molecular techniques will clarify the exact pathophysiological mechanisms of this deficiency.

show abstract

“…Its prevalence is about 5-15 per million adults (5,6). PNH is a rare, acquired clonal hematopoietic stem cell disorder characterized by chronic intravascular hemolysis findings, BM failure and thrombosis, and the prevalence of the disease was considered to be 1-10 per million (7,8). pHT is also very rare in patients with PNH, yet there are no reports of prevalence.…”

Section: Discussionmentioning

confidence: 99%

The Prevalence of Paroxysmal Nocturnal Hemoglobinuria Clone in Adult Patients with Idiopathic Pulmonary Hypertension

Ayer¹,

Elibol²,

Sinan³

et al. 2018

Haseki

View full text Add to dashboard Cite

Aim:Paroxysmal nocturnal hemoglobinuria (PNH) a is a clonal disorder that may lead to several conditions such as thromboses, Budd-Chiari syndrome, renal failure, impotence, and pulmonary hypertension (pHT). Since the presentation of PNH may be occult, monitoring for clonal evolution is recommended in certain situations including aplastic anemia, Myelodysplastic syndrome, and unexplained cytopenia, and thrombosis. The prevalence of PNH clone in patients with idiopathic pHT is unknown. We designed a study to determine the prevalence of PNH clone in patients with idiopathic pHT, since it may be the first isolated presentation of the disease. Methods:A total of 45 patients with pHT were screened for PNH clone by proaerolysin conjugated with fluorescein.Results: Only two out of 45 patients had elevated lactate dehydrogenase (LDH) levels at presentation. PNH clone was detected in none of the patients. Conclusion:Screening for PNH clone in patients with pHT, who have normal LDH levels is unnecessary. Keywords: Paroxysmal nocturnal hemoglobinuria, pulmonary hypertension, cloneAmaç: Paroksismal noktürnal hemoglobinüri (PNH) tromboz, Budd-Chiari sendromu, böbrek yetmezliği, impotans, pulmoner hipertansiyon (pHT) gibi çeşitli hastalıklara sebebiyet verebilen klonal bir hastalıktır. PNH'nin prezentasyonu sessiz olabileceğinden, aplastik anemi, Miyelodisplastik sendrom, açıklanamaya sitopeniler ve tromboz gibi çeşitli durumlarda PNH klonu taranması önerilmektedir. İdiyopatik pHT'si olan hastalarda PNH klonu prevalansı ise bilinmemektedir. PNH'li hastalarda pHT hastalığın ilk bulgusu olabileceğinden, pulmoner hipertansiyonu olan hastalarda PNH klonu prevelansını belirlemek amacıyla bir çalışma tasarladık.Yöntemler: pHT'si olan toplam 45 hastada florasanla konjuge proaerolizin yöntemiyle PNH klonu tarandı. Bulgular: Kırk beş hastanın yalnızca ikisinde başlangıç laktat dehidrogenaz (LDH) düzeyi normalin üzerindeydi. PNH klonu hiçbir hastada saptanmadı.Sonuç:LDH düzeyi normal olan pHT hastalarında PNH klonu taranması gerekli görülmemiştir.AnahtarSözcükler: Paroksismal noktürnal hemoglobinüri, pulmoner hipertansiyon, klon

show abstract

Detailed immunophenotypic characterization of different major and minor subsets of peripheral blood cells in patients with paroxysmal nocturnal hemoglobinuria

Cited by 26 publications

References 33 publications

Ex vivo expansion and long-term hematopoietic reconstitution ability of sorted CD34+CD59+ cells from patients with paroxysmal nocturnal hemoglobinuria

Ex vivo expansion and long-term hematopoietic reconstitution ability of sorted CD34+CD59+ cells from patients with paroxysmal nocturnal hemoglobinuria

The presence of CD55- and/or CD59-deficient erythrocytic populations in patients with rheumatic diseases reflects an immune-mediated bone-marrow derived phenomenon

The Prevalence of Paroxysmal Nocturnal Hemoglobinuria Clone in Adult Patients with Idiopathic Pulmonary Hypertension

Contact Info

Product

Resources

About