Kidney Week

Abstract: SA-PO424

Natural Killer Subsets in Patients with Fabry Disease

Session Information

• Genetic Diseases of the Kidney - III
  November 09, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Genetic Diseases of the Kidneys

• 1002 Genetic Diseases of the Kidneys: Non-Cystic

Authors

• Turkmen, Kultigin, Necmettin Erbakan Universtity Meram School of Medicine, Konya, Turkey

Kultigin Turkmen,

• Karaselek, Mehmet Ali, Necmettin Erbakan University, Konya, Turkey

Mehmet Ali Karaselek,

• Baloglu, Ismail, Necmettin Erbakan Universtity Meram School of Medicine, Konya, Turkey

Ismail Baloglu,

• Celik, Seyma, Necmettin Erbakan University, Konya, Turkey

Seyma Celik,

• Reisli, Ismail, Necmettin Erbakan University, Konya, Turkey

Ismail Reisli,

• Guner, Sukru, Necmettin Erbakan University, Konya, Turkey

Sukru Guner,

• Keles, Sevgi, Necmettin Erbakan University, Konya, Turkey

Sevgi Keles,

Background

Fabry Disease (FD) is a storage disorder which affects mostly kidney, kardio and serebrovascular systems. The lysosome, whose function is impaired in FD and also is an important compartment for the innate immune responses. To date, invariant natural killer (NK) T cell functions was found be impaired in FD. Hovewer, there is no data regarding NK cell subsets in patients with FD. We aimed to analyze subtypes of NK cell in patients with FD and compared these results with healthy subjects.

Methods

15 patients with FD and 10 healthy subjects were included in the study. Of these 15 patients 8 patients were receiving agalsidase alfa or beta. Blood samples obtained from the patients with FD and healthy subjects were taken into 2 ml EDTA tube to evaluate peripheral NK cell subgroups according to CD56 and CD16. These cells were evaluated by flow cytometry technique.

Results

The characteristic and demographic features of patients with FD and controls are depicted in Table 1. According to flow-cytometric analysis, total percentage of NK cells of FD patients are similar to healthy controls (11.6% vs 10.7%, p: >0.05, respectively). When we analyzed subgroups of NK cells, we determined that CD56^dimCD16^dim and CD56^brightCD16^dim NK cells were increased, however, CD56^dimCD16^bright NK cells were decreased when compared with healthy controls (59% vs 38%, p:, 13.4% vs 7.3%, p:, 18.9% vs 49.2% p:0.025, respectively).

Conclusion

CD56^brightCD16^dim subtype of NK cells which can secrete cytokines in normal population are increased in patients with FD. Additionaly CD56^dimCD16^dim subtype of NK cells are also decreased in these patients which is closely associated with cellular cytotoxity in normal population. Further studies are needed to clarify the roles of NK cells in patients with FD.

Demographic and Laboratory Features of Patients with Fabry Disease and Healthy Controls

Table 1

Funding

• Government Support - Non-U.S.