The role of paroxysmal nocturnal hemoglobinuria clones in response to immunosuppressive therapy of patients with severe aplastic anemia

Annals of Hematology
Xin ZhaoFeng-kui Zhang

Abstract

Whether paroxysmal nocturnal hemoglobinuria (PNH) clone in aplastic anemia (AA) is a prognostic factor to immunosuppressive therapy is a subject of debate. We evaluated hematological responses to immunosuppressive therapy (IST) in severe AA (SAA) patients with or without the presence of a PNH clone. In 97 SAA patients who received first-line IST between January and December 2011, 24 (24.7 %) had a PNH clone prior to treatment, with a median clone size of 7.82 % (range 1.19-45.46 %). The response rates to IST for patients with or without a PNH clone were 66.7 and 50.7 % (P < 0.172), 79.2 and 57.5 % (P < 0.057), and 79.2 and 67.1 % (P < 0.264) at 3, 6, and 12 months, respectively. Combined rate of complete and good partial responses differed between patients with or without a PNH clone: insignificantly at 3 months (41.7 vs. 21.9 %, P < 0.058), but significantly at 6 (66.7 vs. 31.5 %, P < 0.002) and 12 (75.0 vs. 46.6 %, P < 0.015) months. Multivariate analysis revealed that a pretreatment neutrophil count of >0.2 × 10(9)/L is indicative of a better response, while the presence of a PNH clone is predictive to a higher combined rate of complete and good partial responses. This study demonstrated that the presence of a PNH clone coul...Continue Reading

References

Sep 25, 1999·Annals of Internal Medicine·D E DunnN S Young
Feb 17, 2000·Seminars in Hematology·G SociéA Tichelli
Mar 8, 2000·Acta Haematologica·B M Camitta
Nov 28, 2008·British Journal of Haematology·Phillip ScheinbergNeal S Young
Aug 14, 2009·British Journal of Haematology·Judith C W MarshUNKNOWN British Committee for Standards in Haematology
May 25, 2011·Haematologica·Manuel G AfableJaroslaw P Maciejewski

❮ Previous
Next ❯

Citations

Apr 20, 2016·International Journal of Hematology·Atsushi Narita, Seiji Kojima
Sep 7, 2016·Cytometry. Part B, Clinical Cytometry·Marta MoradoUNKNOWN PNH working group of the Iberian Society of Cytometry (SIC)
Jan 21, 2017·British Journal of Haematology·Natasha StanleyDaria V Babushok
Oct 27, 2017·Journal of Pediatric Hematology/oncology·Shuanglong LuXiaotian Xie
Nov 17, 2017·Current Treatment Options in Oncology·Scott A PeslakDaria V Babushok

❮ Previous
Next ❯

Related Concepts

Related Feeds

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.