Thalassemia (low hemogloben problem) and Vitamin D

"Thalassemia (thal-uh-SEE-me-uh) is an inherited blood disorder that causes your body to have less hemoglobin than normal. Hemoglobin enables red blood cells to carry oxygen. Thalassemia can cause anemia, leaving you fatigued. If you have mild thalassemia, you might not need treatment" Mayo Clinic

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Thalassemia associated with Low Vitamin D - June 2021

An urgent need for improving thalassemia care due to the wide gap in current real-life practice and clinical practice guidelines
Sci Rep. 2021 Jun 24;11(1):13283. doi: 10.1038/s41598-021-92715-w.

Supachai Ekwattanakit 1, Chattree Hantaweepant 2, Archrob Khuhapinant 2, Noppadol Siritanaratkul 2, Vip Viprakasit 3 4
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Based on Thalassemia International Federation clinical practice guidelines (CPG) for non-transfusion dependent and transfusion dependent thalassemia, several measures should be routinely implemented such as monitoring and surveillance of thalassemia related complications for early detection and proper clinical management. To evaluate the prevalence and the performance of routine surveillance for thalassemia related complications during 2 periods; before and after published CPGs (2012-2014 vs 2015-2017), data from 524 adult thalassemia patients attended at Siriraj hospital were compared among different treating physician groups; thalassemia, private hematology, and internal medicine clinics. Three most common complications were osteopenia/osteoporosis (69.8%), gallstones (67.6%) and abnormal vitamin D level (67.6%). Iron overload has been widely evaluated (93.1%) followed by liver function test (82.3%). However, the rate of evaluation for other complications were significantly reduced and < 25% of patients were evaluated in several complications. Comparing among clinics, the surveillance rate has increased significantly for several endocrine complications only in patients treated at thalassemia clinic but not in others. This study was the first study that evaluated real-world practical management of thalassemia patient in terms of complication surveillance. This different clinical practice has called for an immediate policy change to improve and standardize a care for thalassemia patients in Thailand.

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Thalassemia Major associated with bone problems, need Vitamin D - 2013

Vitamin D Status in Thalassemia Major: an Update
Mediterr J Hematol Infect Dis. 2013; 5(1): e2013057. Sep 2. doi: 10.4084/MJHID.2013.057
Ashraf Soliman,1 Vincenzo De Sanctis,2 and Mohamed Yassin3

The survival of patients with thalassemia major has progressively improved with advances in therapy; however, osteoporosis and cardiac dysfunction remain frequent complications. Adequate circulating levels of vitamin D are essential for optimal skeletal health and reducing fracture risk. Vitamin D deficiency and insufficiency is reported to be high in thalassemic patients in many countries despite the presence of good sunshine and routine prescription of 400–1,000 IU vitamin D per day.
The risk of vitamin D deficiency in thalassemia and its relation to bone disease; including osteoporosis, rickets, scoliosis, spinal deformities and fractures as well as to cardiac dysfunction is discussed in this mini-review. Monitoring and maintaining normal serum level of 25-OH vitamin D through oral intake of vitamin D and early correction of VDD by oral or parental use of vitamin D may significantly improve bone mineral accretion and ameliorate cardiac function.
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Thalassemia Major, Vitamin D and Receptor 2020

Analysis of VDR Gene Polymorphism in Beta Thalassemia Major (Beta Thalassemia Major / Vitamin D / Calcium / 825T/T Vitamin D Receptor Gene)
Source: Systematic Reviews in Pharmacy . 2020, Vol. 11 Issue 4, p268-274
Author(s): Riyanti, Eriska; Achmad, Muhammad Harun; Safari, Mieke Hemiawati; Gartika, Meirina; Ramadhany, Yunita Feby

Vitamin D Receptor (VDR) Gene has an important role in calcium and bone metabolism because is required in mineralization of normal bone, calcium absorption, organizing calcium and phosphate homeostasis, and regulating the secretion of parathyroid hormone. Genetics research found that BsmI polymorphism region in VDR gene relates to osteoporosis, other bone diseases, and cartilage metabolism in particular allele. The aim of this research is to analyze polymorphism of VDR (825G/T) gene in the eighth intron of beta thalassemia major on Sundanese population. In this case-control study, SNP 825G/T of 66 beta thalassemia major patients (case group) and 15 controls (control group) were evaluated using PCR-RFLP with restriction of enzyme BsmI. Vitamin D and calcium level were analyzed using ELISA. Genotype distributions and allele frequencies were analyzed chisquared test.
This study found that the vitamin D level was significantly lower on case group than on control group (vitamin D 56.1 pmol/L 80.30%; p&lt;0.001), and calcium level was significantly higher on case group than on control group (&gt;8.5 mg/dL; p=0.025).
Genotype frequency 825 VDR on case group was compared with control group, and there was no BB genotype in control group. There is only one case on case group (OR, 2.63; 95%CI, 0.73-9.64). B allele is significant in both case and control groups. There was no significant difference between case and control groups (OR, 2, 95%CI, 0.71-5.92). There was a significant difference in level of vitamin D and calcium, and also in allele frequency between on case and control groups.
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thalassemia.com: Nutrition including vitamin D - 2020

Living with Thalassemia - Nutrition and Diet
Nutritional deficiencies are common in thalassemia, due to hemolytic anemia, increased nutritional requirements, and morbidities such as iron overload, diabetes, and chelator use.

Patients should be evaluated annually by a registered dietitian regarding adequate dietary intake of calcium, vitamin D, folate, trace minerals (copper, zinc, and selenium) and antioxidant vitamins (E and C). Annual nutritional laboratory testing should include albumin, 25-hydroxy vitamin D, fasting glucose, fasting plasma zinc, serum copper, ceruloplasmin, serum selenium, alpha and gamma tocopherol, plasma ascorbate, and serum folate. (See nutrition table below.)

Recommendations for dietary supplementation should be made as indicated by nutritional history, complications of the disease, and, in children, growth status. Typically multivitamin supplementation without iron is suggested (e.g., Centrum Silver in tablet or chewable form is now available).

For nontransfused thalassemia patients, folate supplementation (1 mg daily) is recommended, and consuming a moderately low-iron diet is encouraged—that is, avoiding iron-fortified cereals and other products and excessive consumption of red meat. Drinking black tea with meals is recommended to reduce iron absorption from food.

For transfused patients on chelation therapy, a low-iron diet is unnecessary and may decrease the quality of life for some patients. The amount of iron obtained from just one unit of packed red cells (200 mg) far outweighs the amount of iron obtained from a 3-ounce steak (5 mg).

Vitamin D supplementation (50,000 IU once a week until levels normalize) is recommended for patients with a 25-hydroxy vitamin D less than 20 ng/dL. Calcium supplementation should be encouraged if dietary intake is insufficient.

Counseling should be offered for patients with special dietary needs. These include patients with diabetes or lactose intolerance, those who practice vegetarianism, those who are pregnant, or those on oral chelators or bisphosphonate medications.

Alcohol consumption and cigarette smoking are to be discouraged. Alcohol potentiates the oxidative damage of iron and aggravates the effect of hepatitis B and C on liver tissue. Cigarette smoking affects bone remodeling and is associated with osteoporosis.

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Thalessemia Major children have 16 ng of vitamin D - July 2019

Study of Vitamin D Levels in Thalessemia Major Patients in Children
J of Medical Science and clinical Research Vol 07 Issue 07 July 2019 DOI: https://dx.doi.org/10.18535/jmscr/v7i7.49
Shruti Dhale, Subash Valinjkar, Jyothi Janardhanan, Sagar Walhekar

Introduction: Thalassemia is an inherited disorder of hemoglobin synthesis. Regular blood transfusions and chelation therapy have noticeably prolonged survival in thalassemic patients.[1] Despite a significant increase in the lifespan of these patients, they suffer from multiple abnormalities probably due to iron overload, including endocrinal abnormalities such as

• hypogonadism,
• diabetes mellitus,
• hypothyroidism and
• hypoparathyroidism.[2] The pattern of levels of vitamin D levels among the patients with thalassemia major undergoing repeated blood transfusions remain unexplored. Very few studies have been undertaken among Indian population.

Methodology: It is a prospective study and was conducted at St George hospital, Mumbai,on diagnosed patients of thalassemia major admitted to the pediatric wards. Duration of the study was 3 months and 36 patients were included.

Inclusion Criteria
All admitted patients of thalassemia major aged 12 years and below.

Exclusion Criteria:
Chronic hemolyticanemia apart from thalassemia major
Those that was very sick
Those suffering from malnutrition
Those who were on supplementation of calcium, phosphorus and vitamin d .
Patients who were willing to participate and sign the inform consent were enrolled in the study. Serum 25 hydroxyvit D levels were done using CLIA.

Results: The mean age of the studied thalassemia patients was 8.76 ± 3.7 years with no gender preponderance.
Mean (SD) Vitamin D levels was 16.26 ±8.75 and was found to be statistically significant (p<0.001).

Conclusion: It is evident from the present study that the levels of vitamin D are deficient among β-thalassemia major patients on repeated blood transfusion. The deficiencies may be due to iron overload or due to nutritional deficiency. Frequent monitoring and supplementation in deficient states is recommended.
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Vitamin D and bone health status in beta thalassemia patients-systematic review - Jan 2021

Manolopoulos PP 1, Lavranos G 1, Mamais I 2, Angouridis A 1, Giannakou K 2, Johnson EO 1
Osteoporosis International DOI: 10.1007/s00198-021-05821-w PMID: 33423084

Thalassemia is a chronic congenital disease characterized by a combination of endocrine and metabolic disorders. Bone disease is a very common complication related to the poor absorption of calcium, the secondary chronic renal disease with low vitamin D, as well as multiple endocrine risk factors. The aim of this systematic review was to estimate the prevalence of vitamin D deficiency in thalassemia, as well as its association with osteoporosis/low bone mass. A systematic review was carried out using PubMed/Medline, Cochrane, and EBSCO databases. The methodological quality of the included studies was assessed with the validated Newcastle-Ottawa Quality Assessment Scale adapted for cross-sectional studies and cohort studies respectfully and the Cochrane Collaboration for clinical trials. After application of predetermined exclusion criteria compatible with the PICOS process, a total of 12 suitable articles were identified.
The prevalence of vitamin D deficiency varied considerably. Only five of the reviewed studies examined the correlation between vitamin D levels and BMD of which just three showed a statistically significant positive association of mild/moderate grade.
Vitamin D deficiency is a common comorbidity in patients with thalassemia. However, both its prevalence and its severity vary considerably in different populations, and existing evidence is insufficient to conclude whether vitamin D supplementation is also associated with BMD improvement in this special population group.

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Iran COVID-19 Morality rate 16% TM, 6% in general population - Dec 2020

Coronavirus Disease 2019 (COVID-19) Severity in Patients with Thalassemias: A Nationwide Iranian Experience
Mediterranean Journal of Hematology and Infectious Diseases, 31 Dec 2020, 13(1):e2021008 DOI: 10.4084/mjhid.2021.008
Karimi M1, Haghpanah S1, Azarkeivan A2, Matin S3, Safaei A1, De Sanctis V4
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CDC listed Thalassemia as one of the risk-factors for COVID-19 - Dec 2020

26 health factors increase the risk of COVID-19 – all are proxies for low vitamin D

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Thalassemia is related to sickle cell anemia (which is also helped by Vitamin D)

• Overview Sickle Cell and Vitamin D in Vitamin D Life
• About Thalassaemia and Sickle Cell Disease
  • α-thalassaemia is characterised by a decrease or complete absence of expression from one or more of the four α-globin genes and may be brought about by a deletion or a nondeletion mutation in the α-globin genes.
  • β-thalassaemia is characterised by the reduced synthesis (β+) or absence (β0) of the β-globin chains in the Hb molecule, resulting in accumulation of unbound α-globin chains that precipitate in erythroid precursors in the bone marrow and in the mature erythrocytes, leading to ineffective erythropoiesis and peripheral haemolysis.
  • Sickle cell disease is caused by one particular mutation on the HBB gene, producing an abnormal version of β-globin known as haemoglobin S (HbS) which can distort red blood cells into a sickle shape. The sickle-shaped red blood cells die prematurely, which can lead to anemia. Sometimes the inflexible, sickle-shaped cells get stuck in small blood vessels and can cause serious medical complications.

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