Contributions
Abstract: EP1218
Type: E-Poster Presentation
Session title: Sickle cell disease
Background
With the advances in sickle cell disease (SCD), the patients' life expectancy has increased, and chronic disease complications have become more prominent.
Aims
The National Hemoglobinopathy Register of the Turkish Society of Hematology (TSH) aims to evaulate the frequency of chronic complications and long term outcomes of the patients with SCD in Turkey.
Methods
The physicians from 35 hemoglobinopathy centers registered 480 SCD (75%) and 160 (25%) HbSB patients' hospital records into the web-based system.
Results
The patients (305 males, 335 females) were 32 ± 15 years-old. Musculoskeletal disorders (44%), osteopenia (42%), and avascular necrosis (34%) were the most common complications. Musculoskeletal complications were significantly higher in patients with HbSS (48.5%) than the patients with HBSB (34.8%) (p = 0.006). Infectious complications affected 26.5% of patients, and 3.4% of patients suffered from sepsis. Overall, 3.8% of the patients had transfusion-transmitted infections, and the majority (90%) was Hepatitis-C virus infection. 17% of patients with SCD had liver and gall bladder complications. The biopsy-proven cirrhosis was 1.6%. The cardiovascular complications, including heart failure, thromboembolism, arrhythmia, and pulmonary hypertension, affected 4.9%, 4.4%, 3.2%, 1.5% of the patients, respectively. Each of the urogenital and cerebrovascular complications affected 9.5% of the patients. 2.3% of the patients showed chronic renal failure. Endocrine complications were more prominent in patients with HbSB than SS (14.7% versus 6.5%; p = 0.006). 38% of patients aged >18 years got married at the mean age of 26.7 ± 5.2 years (range 18-48). In 11.3% of these marriages, the couples were the patients with hemoglobinopathy. 85.7% of patients with a healthy partner had at least one child indicating patients' reproductive potential. Three patients had breast cancer, non-Hodgkin lymphoma, and neurofibromatosis type 1, one in each. Twenty-seven patients (4.2%) died of SCD-related complications at the mean age of 38.9± 15.4 years (range 10-67). Among the causes of death, cardiovascular complications were the most common (22%), followed by infections (18.5%) and thromboembolic events (11%). Nine patients aged between 4 and 32 years of age had bone marrow transplantation (BMT) from a matched sibling (n=7) and unrelated (n=2) donors. All transplanted patients alive, and all but one had BMT from an unrelated donor were disease-free.
Conclusion
As the data showed, adolescents and young adults are composed of most SCD population in Turkey. It indicates the need for consideration of an effective transitioning program from pediatric to adult care. The organization of specialized centers for patients with SCD and improving access to these centers for regular and comprehensive follow-up should positively impact the patients' survival by controlling the disease's life-threatening complications.
Keyword(s): Complications, Sickle cell disease
Abstract: EP1218
Type: E-Poster Presentation
Session title: Sickle cell disease
Background
With the advances in sickle cell disease (SCD), the patients' life expectancy has increased, and chronic disease complications have become more prominent.
Aims
The National Hemoglobinopathy Register of the Turkish Society of Hematology (TSH) aims to evaulate the frequency of chronic complications and long term outcomes of the patients with SCD in Turkey.
Methods
The physicians from 35 hemoglobinopathy centers registered 480 SCD (75%) and 160 (25%) HbSB patients' hospital records into the web-based system.
Results
The patients (305 males, 335 females) were 32 ± 15 years-old. Musculoskeletal disorders (44%), osteopenia (42%), and avascular necrosis (34%) were the most common complications. Musculoskeletal complications were significantly higher in patients with HbSS (48.5%) than the patients with HBSB (34.8%) (p = 0.006). Infectious complications affected 26.5% of patients, and 3.4% of patients suffered from sepsis. Overall, 3.8% of the patients had transfusion-transmitted infections, and the majority (90%) was Hepatitis-C virus infection. 17% of patients with SCD had liver and gall bladder complications. The biopsy-proven cirrhosis was 1.6%. The cardiovascular complications, including heart failure, thromboembolism, arrhythmia, and pulmonary hypertension, affected 4.9%, 4.4%, 3.2%, 1.5% of the patients, respectively. Each of the urogenital and cerebrovascular complications affected 9.5% of the patients. 2.3% of the patients showed chronic renal failure. Endocrine complications were more prominent in patients with HbSB than SS (14.7% versus 6.5%; p = 0.006). 38% of patients aged >18 years got married at the mean age of 26.7 ± 5.2 years (range 18-48). In 11.3% of these marriages, the couples were the patients with hemoglobinopathy. 85.7% of patients with a healthy partner had at least one child indicating patients' reproductive potential. Three patients had breast cancer, non-Hodgkin lymphoma, and neurofibromatosis type 1, one in each. Twenty-seven patients (4.2%) died of SCD-related complications at the mean age of 38.9± 15.4 years (range 10-67). Among the causes of death, cardiovascular complications were the most common (22%), followed by infections (18.5%) and thromboembolic events (11%). Nine patients aged between 4 and 32 years of age had bone marrow transplantation (BMT) from a matched sibling (n=7) and unrelated (n=2) donors. All transplanted patients alive, and all but one had BMT from an unrelated donor were disease-free.
Conclusion
As the data showed, adolescents and young adults are composed of most SCD population in Turkey. It indicates the need for consideration of an effective transitioning program from pediatric to adult care. The organization of specialized centers for patients with SCD and improving access to these centers for regular and comprehensive follow-up should positively impact the patients' survival by controlling the disease's life-threatening complications.
Keyword(s): Complications, Sickle cell disease