Mediterranean Journal of Hematology and Infectious Diseases, 2022
Background: The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavir... more Background: The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to high morbidity and mortality worldwide. Vaccination against SARS-CoV-2 is a leading strategy to change the course of the COVID-19 pandemic. Aims of study: Our aim was to investigate the efficacy and side effects of Sinopharm vaccine in patients with hemoglobinopathies in Iran, and the frequency of breakthrough infection after a full course of vaccination. Methods: A multicenter cross-sectional study of 434 patients with hemoglobinopathies (303 β-thalassemia major, 118 β-thalassemia intermedia, and 13 sickle-thalassemia) were conducted from March to July 2021 in IRAN. All patients have received the first dose of China Sinopharm vaccine and received the second dose of the vaccine 28 days apart. Antibody testing: Detection of immunity after vaccination was evaluated by commercial enzyme- linked immunosorbent assay (Pishtazteb ELISA commercial kit), including ...
We’re all flying blind regarding coronavirus, but it’s fair to think if thalassemic patients are ... more We’re all flying blind regarding coronavirus, but it’s fair to think if thalassemic patients are particularly vulnerable to SARS-COV-2 infection or are at potential higher risk of complications from COVID-19 than normal population, specially when they become older. The frustrating thing is that, right now, this virus is still new. It only came to the attention of the World Health Organization at the end of December. Very few cases in thalassemia have so far been reported; is this due to lack of testing or a true lack of infection/susceptibility? However, we believe that more data should be collected to better characterise the impact of SARS-CoV-2 infection in patients with thalassemias. Therefore, a multicenter registry and the collection of comprehensive data from both positive COVID-19 thalassemia major and non-transfusion dependent thalassemia are necessary to clarify debated issues. In the meantime an early and vigilant monitoring along with high quality supportive care are need...
We present a 34-year-old man with combination of beta thalassemia intermedia and coagulation fact... more We present a 34-year-old man with combination of beta thalassemia intermedia and coagulation factor VII deficiency who was presented with pallor and irregular nose bleeding episodes. On physical examination, he had splenomegaly and yellow sclera. Pallor and splenomegaly could be reminder of thalassemic syndromes or hemoglobinopathies including thalassemia intermedia. Association with unusual bleeding tendency such as prolonged and repeated episodes of epistaxis without any evidence of liver failure in hemoglobinopathies suggest the possibility of coinheritance of a bleeding disorder. As a result, coagulation assay for proper diagnosis, management and prevention of probable life-threatening bleeding episodes is suggested. Iranian Journal of Blood & Cancer
This article reviews the current knowledges of congenital bleeding disorders (CBD) amid the COVID... more This article reviews the current knowledges of congenital bleeding disorders (CBD) amid the COVID-19 pandemic. It appears that CBD is not associated with higher risk of getting COVID-19 and so the prevalence of COVID-19 among them seems not higher compared to the general population. In absence of specific therapeutic recommendations, it is essential to make a correct assessment of the risk of haemorrhage/thrombosis. Based on expert opinion, strategies for outpatient management include adherence to prescribed regimens, telemedicine, and communication about COVID-19 in patients with CBD. More data should be also collected to better characterize the impact of COVID-19 on patients with CBD. The current findings encourage further studies to determine the prevalence of SARS-CoV2 infection in CBD patients to understand more fully the burden of this novel pathogen and to develop adequate preventive measures against this infection. (www.actabiomedica.it)
Although the improvements in the treatment and management of thalassemia patients in new years le... more Although the improvements in the treatment and management of thalassemia patients in new years lead to the improved survival and quality of life (QOL) in this group of patients, QOL is still is an important dimension of care in thalassemic patients. WHO defines QOL as “an individual’s perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards, and concerns”. Thalassemia is a chronic disease needs life-long care with multiple physical, mental and social complications that affect QOL in patients. The most important factors which affect QOL in thalassemia are: effects of the disease on family, skeletal and face changes, frequent blood transfusion and drug infusion, sexual impairment and infertility, heart and liver disease as well as endocrine disorders, anxiety and low life expectancy.
The thalassemias are among the most common genetic disorders worldwide, occurring more frequently... more The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran. A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of ß- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO) dose, mean daily doses of DFO (mg/kg) and audiometric variables was recorded. Out of 308 cases, 283 (96.5%) had normal hearing and 10 (3.5%) sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion. We found the lowest incidence...
Mediterranean journal of hematology and infectious diseases, 2014
IGF-1 deficiency in TM patients in children and adolescents has been attributed to chronic anemia... more IGF-1 deficiency in TM patients in children and adolescents has been attributed to chronic anemia and hypoxia, chronic liver disease, iron overload and other associated endocrinopathies, e.g. growth hormone deficiency (GHD). Few data are available in the literature regarding adult TM patients and growth disorders. The aim of this study was to measure IGF-1 values and other clinical data in a large number of adult patients with TM to evaluate the possible relationships between them. A cohort of 120 adult patients with TM was studied for plasma levels of IGF-1. Plasma total IGF-1 was determined by chemiluminescent immunometric assay (CLIA) method. In eleven patients (3 females) the GH response during glucagon stimulation test (GST) was also evaluated. Fifty percent of patients (33 males and 27 females) had IGF-1 levels <- 2 SDs below normative values for healthy subjects matched for age and sex. In these patients endocrine complications and elevations of aminotransferases (ALT) wer...
Background: Beta-thalassemia major is one of the major health problems in our country. Many studi... more Background: Beta-thalassemia major is one of the major health problems in our country. Many studies have confirmed the fact that, these patients have an increased susceptibility to bacterial infections. Objective: In this study, we have assessed the ...
Extramedullary hematopoiesis (EMH) is a physiological compensatory phenomenon occurring in many h... more Extramedullary hematopoiesis (EMH) is a physiological compensatory phenomenon occurring in many hemolytic anemias including thalassemia. Besides transfusion, radiotherapy, surgery or a combination of these modalities, hydroxyurea (HU) as an optimal treatment has been described occasionally. We described a case of beta-thalassemia major who has been on regular blood transfusion and developed EMH that was treated with HU combined with radiotherapy. EMH should also be considered in thalassemia major patients, although it is more common in non-transfusion dependent thalassemia patients. HU combined with low dose radiotherapy along with regular blood transfusion are suggested as good treatment options for patients with EMH. Iranian Journal of Blood & Cancer
One recurring question is about the size of the clot (>2 cm) in conjunction with several morph... more One recurring question is about the size of the clot (>2 cm) in conjunction with several morphological characteristics and mobility criteria of the clot that would deem the patient with RAT to be considered ‘high-risk’. The upper limit of 2-cm clot size cannot be applied to all children due to varying sizes of the right atrium. The critical clot size of 2 cm is approximately 2 SD below the mean size of the pulmonary valve of an adult (size of 1.6 cm/m) [2]. This is thought to be the critical size of a clot that can completely occlude the pulmonary valve and cause sudden death. Although the size serves as the utmost maximum, as the data were derived from adult studies, our aim is to provide size parameters for younger children and infants. We propose an equivalent critical clot size in children according to their expected pulmonary valve size on the basis of their weight (Table 1) and hope the reader will find it to be a useful reference.
Mediterranean Journal of Hematology and Infectious Diseases, 2022
Background: The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavir... more Background: The ongoing COVID-19 pandemic, caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to high morbidity and mortality worldwide. Vaccination against SARS-CoV-2 is a leading strategy to change the course of the COVID-19 pandemic. Aims of study: Our aim was to investigate the efficacy and side effects of Sinopharm vaccine in patients with hemoglobinopathies in Iran, and the frequency of breakthrough infection after a full course of vaccination. Methods: A multicenter cross-sectional study of 434 patients with hemoglobinopathies (303 β-thalassemia major, 118 β-thalassemia intermedia, and 13 sickle-thalassemia) were conducted from March to July 2021 in IRAN. All patients have received the first dose of China Sinopharm vaccine and received the second dose of the vaccine 28 days apart. Antibody testing: Detection of immunity after vaccination was evaluated by commercial enzyme- linked immunosorbent assay (Pishtazteb ELISA commercial kit), including ...
We’re all flying blind regarding coronavirus, but it’s fair to think if thalassemic patients are ... more We’re all flying blind regarding coronavirus, but it’s fair to think if thalassemic patients are particularly vulnerable to SARS-COV-2 infection or are at potential higher risk of complications from COVID-19 than normal population, specially when they become older. The frustrating thing is that, right now, this virus is still new. It only came to the attention of the World Health Organization at the end of December. Very few cases in thalassemia have so far been reported; is this due to lack of testing or a true lack of infection/susceptibility? However, we believe that more data should be collected to better characterise the impact of SARS-CoV-2 infection in patients with thalassemias. Therefore, a multicenter registry and the collection of comprehensive data from both positive COVID-19 thalassemia major and non-transfusion dependent thalassemia are necessary to clarify debated issues. In the meantime an early and vigilant monitoring along with high quality supportive care are need...
We present a 34-year-old man with combination of beta thalassemia intermedia and coagulation fact... more We present a 34-year-old man with combination of beta thalassemia intermedia and coagulation factor VII deficiency who was presented with pallor and irregular nose bleeding episodes. On physical examination, he had splenomegaly and yellow sclera. Pallor and splenomegaly could be reminder of thalassemic syndromes or hemoglobinopathies including thalassemia intermedia. Association with unusual bleeding tendency such as prolonged and repeated episodes of epistaxis without any evidence of liver failure in hemoglobinopathies suggest the possibility of coinheritance of a bleeding disorder. As a result, coagulation assay for proper diagnosis, management and prevention of probable life-threatening bleeding episodes is suggested. Iranian Journal of Blood & Cancer
This article reviews the current knowledges of congenital bleeding disorders (CBD) amid the COVID... more This article reviews the current knowledges of congenital bleeding disorders (CBD) amid the COVID-19 pandemic. It appears that CBD is not associated with higher risk of getting COVID-19 and so the prevalence of COVID-19 among them seems not higher compared to the general population. In absence of specific therapeutic recommendations, it is essential to make a correct assessment of the risk of haemorrhage/thrombosis. Based on expert opinion, strategies for outpatient management include adherence to prescribed regimens, telemedicine, and communication about COVID-19 in patients with CBD. More data should be also collected to better characterize the impact of COVID-19 on patients with CBD. The current findings encourage further studies to determine the prevalence of SARS-CoV2 infection in CBD patients to understand more fully the burden of this novel pathogen and to develop adequate preventive measures against this infection. (www.actabiomedica.it)
Although the improvements in the treatment and management of thalassemia patients in new years le... more Although the improvements in the treatment and management of thalassemia patients in new years lead to the improved survival and quality of life (QOL) in this group of patients, QOL is still is an important dimension of care in thalassemic patients. WHO defines QOL as “an individual’s perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards, and concerns”. Thalassemia is a chronic disease needs life-long care with multiple physical, mental and social complications that affect QOL in patients. The most important factors which affect QOL in thalassemia are: effects of the disease on family, skeletal and face changes, frequent blood transfusion and drug infusion, sexual impairment and infertility, heart and liver disease as well as endocrine disorders, anxiety and low life expectancy.
The thalassemias are among the most common genetic disorders worldwide, occurring more frequently... more The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major ß- thalassemias transfusion dependent patients in south of Iran. A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of ß- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO) dose, mean daily doses of DFO (mg/kg) and audiometric variables was recorded. Out of 308 cases, 283 (96.5%) had normal hearing and 10 (3.5%) sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion. We found the lowest incidence...
Mediterranean journal of hematology and infectious diseases, 2014
IGF-1 deficiency in TM patients in children and adolescents has been attributed to chronic anemia... more IGF-1 deficiency in TM patients in children and adolescents has been attributed to chronic anemia and hypoxia, chronic liver disease, iron overload and other associated endocrinopathies, e.g. growth hormone deficiency (GHD). Few data are available in the literature regarding adult TM patients and growth disorders. The aim of this study was to measure IGF-1 values and other clinical data in a large number of adult patients with TM to evaluate the possible relationships between them. A cohort of 120 adult patients with TM was studied for plasma levels of IGF-1. Plasma total IGF-1 was determined by chemiluminescent immunometric assay (CLIA) method. In eleven patients (3 females) the GH response during glucagon stimulation test (GST) was also evaluated. Fifty percent of patients (33 males and 27 females) had IGF-1 levels <- 2 SDs below normative values for healthy subjects matched for age and sex. In these patients endocrine complications and elevations of aminotransferases (ALT) wer...
Background: Beta-thalassemia major is one of the major health problems in our country. Many studi... more Background: Beta-thalassemia major is one of the major health problems in our country. Many studies have confirmed the fact that, these patients have an increased susceptibility to bacterial infections. Objective: In this study, we have assessed the ...
Extramedullary hematopoiesis (EMH) is a physiological compensatory phenomenon occurring in many h... more Extramedullary hematopoiesis (EMH) is a physiological compensatory phenomenon occurring in many hemolytic anemias including thalassemia. Besides transfusion, radiotherapy, surgery or a combination of these modalities, hydroxyurea (HU) as an optimal treatment has been described occasionally. We described a case of beta-thalassemia major who has been on regular blood transfusion and developed EMH that was treated with HU combined with radiotherapy. EMH should also be considered in thalassemia major patients, although it is more common in non-transfusion dependent thalassemia patients. HU combined with low dose radiotherapy along with regular blood transfusion are suggested as good treatment options for patients with EMH. Iranian Journal of Blood & Cancer
One recurring question is about the size of the clot (>2 cm) in conjunction with several morph... more One recurring question is about the size of the clot (>2 cm) in conjunction with several morphological characteristics and mobility criteria of the clot that would deem the patient with RAT to be considered ‘high-risk’. The upper limit of 2-cm clot size cannot be applied to all children due to varying sizes of the right atrium. The critical clot size of 2 cm is approximately 2 SD below the mean size of the pulmonary valve of an adult (size of 1.6 cm/m) [2]. This is thought to be the critical size of a clot that can completely occlude the pulmonary valve and cause sudden death. Although the size serves as the utmost maximum, as the data were derived from adult studies, our aim is to provide size parameters for younger children and infants. We propose an equivalent critical clot size in children according to their expected pulmonary valve size on the basis of their weight (Table 1) and hope the reader will find it to be a useful reference.
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