Pneumococcal vaccination for splenectomized patients with thalassemia major in Indonesia

Sari, Teny Tjitra; Akib, Arwin Ap; Gatot, Djajadiman; Harahap, Alida Roswita; Bardosono, Saptawati; Hadinegoro, Sri Rezeki

doi:10.1016/j.vaccine.2017.07.011

Cited by 6 publications

(5 citation statements)

References 19 publications

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“…Also, this investigation demonstrated a significant correlation between splenectomy, and a downward trend in IgG, CD16 levels, and neutrophils count, which cause infection in these patients. Sari's examination indicated that neutrophil count was lower in patients with thalassemia major and who had a splenectomy than in patients without splenectomy 29 . Based on previous studies of chemotaxis, phagocytosis of neutrophils and macrophages was impaired in patients with thalassemia major.…”

Section: Discussionmentioning

confidence: 98%

“…Sari's examination indicated that neutrophil count was lower in patients with thalassemia major and who had a splenectomy than in patients without splenectomy. 29 Based on previous studies of chemotaxis, phagocytosis of neutrophils and macrophages was impaired in patients with thalassemia major. Due to inefficient hematopoiesis, the function of the phagocytic system against microorganisms was reduced.…”

Section: Discussionmentioning

confidence: 99%

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Evaluation of immune system in patients with transfusion‐dependent beta‐thalassemia in Rasoul‐e‐Akram Hospital in 2021: A descriptive cross‐sectional study

Ehsanipour

Faranoush

Foroughi‐Gilvaee

et al. 2022

Health Science Reports

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Background and aims Thalassemia syndromes are the most common hemoglobinopathy globally related to blood transfusion and iron overload in the body. Splenectomy, excessive iron overload, and repeated exposure to antigens in blood transfusions can cause severe damage to the patient's immune system making the patient prone to frequent infection. This study evaluates the immune system status and infection rate in beta‐thalassemia major patients receiving iron chelators. Methods This descriptive cross‐sectional study was performed in Rasoul‐e‐Akram Hospital on patients with a beta‐thalassemia major who had iron overload due to frequent blood transfusions. The percentage of lymphocyte markers was determined by flow cytometry. Serum levels of immunoglobin were measured by nephelometric assay. Also, Nitro blue tetrazolium and dihydrorhodamine assays were used to evaluate the phagocytic function. Results Of the 106 patients participating in this study, 59 (55.7%) and 47 (44.3%) are male and female, respectively. The mean age ± SD of participants was 24.7 ± 12.1 years with 4 to 55 years. There was no significant correlation between sex, the C3 and C4 complements, the lymphocyte markers, and the immunoglobulin levels. Furthermore, all of these variables increased significantly over 30 ( p < 0.05). Moreover, there was a strong positive correlation between splenectomy and IgG immunoglobulin ( p < 0.001) and CD16 ( p = 0.005) lymphocyte marker. Conclusion Iron chelator agents effectively improve patients' immune system with thalassemia major. The increase in IgG and IgM immunoglobulins levels is due to frequent blood transfusions, which stimulate the immune system.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Evaluation of immune system in patients with transfusion‐dependent beta‐thalassemia in Rasoul‐e‐Akram Hospital in 2021: A descriptive cross‐sectional study

Ehsanipour

Faranoush

Foroughi‐Gilvaee

et al. 2022

Health Science Reports

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“…Although not all patients respond equally to splenectomy. Moreover, splenectomy might facilitate many unfavorable consequences like postoperative infections and thromboembolic events [57]. In recent years, the use of splenectomy is characteristically reduced due to an adequate amount of blood transfusion.…”

Section: Splenectomymentioning

confidence: 99%

The Key Genetic Determinants Behind the Phenotypic Heterogeneity of HbE/β-thalassemia Patients and the Probable Management Strategy

Panja¹,

Das²,

Dolai³

et al. 2023

Thalassemia Syndromes - New Insights and Transfusion Modalities

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HbE/β-thalassemia is the most common severe form of thalassemia which is very prominent in South East Asian countries. It is responsible for nearly one-half of all the severe types of β-thalassemia all over the world. It is also known to represent a wide range of phenotypic diversity which varies from asymptomatic to transfusion-dependent severe phenotype. The most important predictive factor is mutations within the beta-globin gene (HBB). Apart from the primary genetic modifiers, there are certain other determinants regulating the phenotypic heterogeneity including, co-inheritance of alpha thalassemia mutations and other secondary modifiers including Xmn1 polymorphism, HBS1L-MYB, GATA-1, BCL11A polymorphism, and presence of HPFH mutations. Although the degree of severity is also determined by other tertiary genetic modifiers like increase in serum erythropoietin due to anemia, previous infection with malaria, environmental factors, splenectomy, etc. This review aimed to reveal the potential genetic predictors of HbE/β-thalassemia patients and the probable management strategy. This also enhances the generation of “personalized medicine” for better patient care. The instability of clinical phenotype and remarkable variation indicate careful monitoring of treatment for each patient and the therapeutic approaches should be monitored over time.

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“…Multiple clinical complications including infections and those related to splenomegaly and splenectomy, which occur in addition to iron overload toxicity, are also a major target of therapeutic interventions in TM [29,47,74,80,[150][151][152][153][154][155]. In general, TM patients prior to splenectomy are treated for immunoprophylaxis with vaccination against the pneumococcal, Haemophilus influenza and Neisseria meningitides viruses [156]. Splenectomised patients receive a number of prophylactic and other treatments including penicillin and other antibiotics for infections and sepsis, aspirin for thrombocytosis and anti-coagulant prophylaxis for hypercoagulability and thromboembolic complications [157,158].…”

Section: Pharmacotherapies In Thalassaemia Not Related To the Elimina...mentioning

confidence: 99%

Benefits and Risks in Polypathology and Polypharmacotherapy Challenges in the Era of the Transition of Thalassaemia from a Fatal to a Chronic or Curable Disease

Kolnagou¹,

Kleanthous²,

Kontoghiorghes³

2022

Front. Biosci. (Elite Ed)

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Beta thalassaemia major (TM), a potentially fatal haemoglobinopathy, has transformed from a fatal to a chronic disease in the last 30 years following the introduction of effective, personalised iron chelation protocols, in particular the use of oral deferiprone, which is most effective in the removal of excess iron from the heart. This transition in TM has been achieved by the accessibility to combination therapy with the other chelating drugs deferoxamine and deferasirox but also therapeutic advances in the treatment of related co-morbidities. The transition and design of effective personalised chelation protocols was facilitated by the development of new non-invasive diagnostic techniques for monitoring iron removal such as MRI T2*. Despite this progress, the transition in TM is mainly observed in developed countries, but not globally. Similarly, potential cures of TM with haemopoietic stem cell transplantation and gene therapy are available to selected TM patients but potentially carry high risk of toxicity. A global strategy is required for the transition efforts to become available for all TM patients worldwide. The same strategy could also benefit many other categories of transfusional iron loaded patients including other thalassaemias, sickle cell anaemia, myelodysplasia and leukaemia patients.

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Pneumococcal vaccination for splenectomized patients with thalassemia major in Indonesia

Cited by 6 publications

References 19 publications

Evaluation of immune system in patients with transfusion‐dependent beta‐thalassemia in Rasoul‐e‐Akram Hospital in 2021: A descriptive cross‐sectional study

Evaluation of immune system in patients with transfusion‐dependent beta‐thalassemia in Rasoul‐e‐Akram Hospital in 2021: A descriptive cross‐sectional study

The Key Genetic Determinants Behind the Phenotypic Heterogeneity of HbE/β-thalassemia Patients and the Probable Management Strategy

Benefits and Risks in Polypathology and Polypharmacotherapy Challenges in the Era of the Transition of Thalassaemia from a Fatal to a Chronic or Curable Disease

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