TITLE

Double-faced cell-mediated immunity in β-thalassemia major: stimulated phenotype versus suppressed activity

AUTHOR(S)
Gharagozloo, Marjan; Karimi, Mehran; Amirghofran, Zahra
PUB. DATE
January 2009
SOURCE
Annals of Hematology;Jan2009, Vol. 88 Issue 1, p21
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
In this study, the immunologic abnormalities of Iranian β-thalassemia major patients were characterized, considering their clinical parameters including splenectomy status and iron overload. Serum samples and peripheral blood mononuclear cells were collected from 28 patients and 30 age- and sex-matched healthy individuals. Patients with thalassemia showed significantly increased absolute lymphocyte counts compared with the control group. An increased number of activated T cells and higher levels of serum neopterin were also observed in thalassemia patients, which suggest chronic stimulation of immune system. On the contrary, T-cell proliferation and interleukin 2 (IL-2), interferon gamma (IFN-γ), and IL-4 production were suppressed in patients compared to controls. Patients with high serum ferritin levels produced significantly less IFN-γ and IL-2, indicating the immunosuppressive effect of iron overload in β-thalassemia patients. The serum levels of tumor necrosis factor alpha and absolute counts and percentages of B and T cells were higher in splenectomized patients; however, serum levels of neopterin significantly decreased in splenectomized patients compared to the non-splenectomized group. Taken together, T lymphocytes express activated phenotype in polytransfused β-thalassemia major patients, while T cell proliferation and effector function are significantly suppressed. Multiple blood transfusion and continuous immune stimulation could be responsible for making such a double-faced immune response.
ACCESSION #
35387726

 

Related Articles

  • Renal tubular dysfunction in a-thalassemia. Sumboonnanonda, Achra; Malasit, Prida; Tanphaichitr, Voravarn S.; Ong�ajyooth, Sompong; Petrarat, Siripan; Vongjirad, Arun // Pediatric Nephrology;Mar2003, Vol. 18 Issue 3, p257 

    Shortened red cell life span and excess iron cause functional and physiological abnormalities in various organ systems in thalassemia patients. In an earlier study, we showed that �-thalassemia patients have a high prevalence of renal tubular abnormalities. The severity correlated with the...

  • Occurrence of overwhelming gram-negative infections in splenectomised patients with thalassaemia major. Alebouyeh, Mardawig; Moussavi, Farideh // European Journal of Pediatrics;2003, Vol. 162 Issue 9, p637 

    Discusses post-splenectomy gram-negative infections in thalassaemics. Signs and symptoms of thalassaemia major; Intravenous antibiotic treatments; Clinical characteristics of gram-negative infections.

  • Anesthetic management of a patient with β-thalassemia intermedia undergoing splenectomy: a case report. Kitoh, Takeshi; Tanaka, Satoshi; Ono, Koichi; Hasegawa, Joe; Otagiri, Tetsutaro // Journal of Anesthesia;2005, Vol. 19 Issue 3, p252 

    A 37-year-old man with β-thalassemia intermedia (βTI), a rare disease caused by partial or complete deficiency of β-globin chain synthesis, fell into a hemolytic crisis. Severe anemia persisted despite frequent transfusions. Therefore, he was scheduled for splenectomy to alleviate the...

  • Prevention of overwhelming postsplenectomy infection in thalassemia patients by partial rather than total splenectomy. Sheikha, Anwar K.; Salih, Ziyan T.; Kasnazan, Kalandar H.; Khoshnaw, Mohammad K.; Al-Maliki, Talal; Al-Azraqi, Tarek A.; Zafer, Mubarak H. // Canadian Journal of Surgery;Oct2007, Vol. 50 Issue 5, p382 

    Objective: We aimed to evaluate the protective role of partial versus total splenectomy against sepsis in patients with thalassaemia when other preventive measures are not available. Overwhelming postsplenectomy infection is a serious complication of splenectomy in these patients, and most...

  • Impact of Splenectomy and Chelating Agents on Serum Cystatin C Levels in Egyptian Children With Beta-Thalassemia. Ismail, Nagwa Abdallah; Moustafa, Naglaa Omar; Habib, Sonia Adolf; Mohammad, Nagwa Abd El-Ghaffar; El Kafoury, Mona Raafat; Talaat, Ahmed A. // Australian Journal of Basic & Applied Sciences;Feb2012, Vol. 6 Issue 2, p85 

    The outlook for patients with thalassemia has improved in recent decades with the use of modern transfusion practice and iron chelation, but patients continue to be at high risk for iron overload and its toxicities. Cystatin C (CysC) has been suggested as a sensitive marker of glomerular...

  • A comparative study of partial vs total splenectomy in thalassemia major patients. Bahador, Ali; Banani, Seyed Abbass; Foroutan, Hamid Reza; Hosseini, Seyed Mohammad Vahid; Davani, Sam Zeraatian Nejad // Journal of Indian Association of Pediatric Surgeons;Jul-Sep2007, Vol. 12 Issue 3, p133 

    Background: In this study, we show the advantages of partial splenectomy (PS) over total splenectomy (TS) regarding the chances of overwhelming postsplenectomy sepsis (OPSI). Materials and Methods: From February 1991 to December 1999, 143 cases of β-thalassemia underwent PS. 1/3, ¼ of the...

  • Impact of splenectomy on quality of life of children with β-thalassemia. Bhosale, Minakshi; Chandanwale, Ajay; Kinikar, Aarti; Singh, Dasmit; Chaudhary, Rohan // International Journal of Medicine & Public Health;Oct-Dec2015, Vol. 5 Issue 4, p322 

    Context: About 10,000 babies with â-thalassemia major are born every year in India, posing a major healthcare burden on the country. In clinically symptomatic individuals, monthly blood transfusions, chelation therapy and splenectomy are the mainstay of therapy at present. Aims: To...

  • Matriptase is highly upregulated in chronic lymphocytic leukemia and promotes cancer cell invasion. Gao, L; Liu, M; Dong, N; Jiang, Y; Lin, C-Y; Huang, M; Wu, D; Wu, Q // Leukemia (08876924);May2013, Vol. 27 Issue 5, p1191 

    A letter to the editor is presented regarding the role of matriptase in chronic lymphocytic leukemia (CLL) and in promoting cancer cell invasion.

  • Serum Immunoglobulin Levels in Splenectomized and Non- Splenectomized Patients with Major Beta-Thalassemia. Kiani-amin, Mojgan; Daneshi, Mohammadmehdi; Ayazi, Parviz; Mohammadian, Shima; Rezaei, Nima // Iranian Journal of Pediatrics;Mar2011, Vol. 21 Issue 1, p95 

    Objective: Thalassemia is a common disease in many countries, in which several complications such as infections can occur. Although aberration in the function of the immune system could be a reason for such complication, a little is known about the status of humoral immune system in major beta...

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics