Reduction of the Clinical Severity of Sickle Cell/β-Thalassemia with Hydroxyurea: The Experience of a Single Center in Greece

Loukopoulos, Dimitris; Voskaridou, Ersi; Kalotychou, Vassiliki; Schina, Maria; Loutradi, Aphrodite; Theodoropoulos, Ilias

doi:10.1006/bcmd.2000.0328

Cited by 45 publications

(41 citation statements)

References 19 publications

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“…The assay of sTfR may thus be useful to better individualize the transfusion regimen. The same conclusions were derived in patients with sickle cell anemia [30], although similar myelosuppression with increased fetal hemoglobin can be achieved with hydroxyurea [31].…”

Section: Main Characteristicssupporting

confidence: 64%

Soluble transferrin receptor for the evaluation of erythropoiesis and iron status

Béguin

2003

Clinica Chimica Acta

395

316

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Iron transport in the plasma is carried out by transferrin, which donates iron to cells through its interaction with a specific membrane receptor, the transferrin receptor (TfR). A soluble form of the TfR (sTfR) has been identified in animal and human serum. Soluble TfR is a truncated monomer of tissue receptor, lacking its first 100 amino acids, which circulates in the form of a complex of transferrin and its receptor. The erythroblasts rather than reticulocytes are the main source of serum sTfR. Serum sTfR levels average 5.0 F 1.0 mg/l in normal subjects but the various commercial assays give disparate values because of the lack of an international standard. The most important determinant of sTfR levels appears to be marrow erythropoietic activity which can cause variations up to 8 times below and up to 20 times above average normal values. Soluble TfR levels are decreased in situations characterized by diminished erythropoietic activity, and are increased when erythropoiesis is stimulated by hemolysis or ineffective erythropoiesis. Measurements of sTfR are very helpful to investigate the pathophysiology of anemia, quantitatively evaluating the absolute rate of erythropoiesis and the adequacy of marrow proliferative capacity for any given degree of anemia, and to monitor the erythropoietic response to various forms of therapy, in particular allowing to predict response early when changes in hemoglobin are not yet apparent. Iron status also influences sTfR levels, which are considerably elevated in iron deficiency anemia but remain normal in the anemia of inflammation, and thus may be of considerable help in the differential diagnosis of microcytic anemia. This is particularly useful to identify concomitant iron deficiency in a patient with inflammation because ferritin values are then generally normal. Elevated sTfR levels are also the characteristic feature of functional iron deficiency, a situation defined by tissue iron deficiency despite adequate iron stores. The sTfR/ferritin ratio can thus describe iron availability over a wide range of iron stores. With the exception of chronic lymphocytic leukemia (CLL) and high-grade non-Hodgkin's lymphoma and possibly hepatocellular carcinoma, sTfR levels are not increased in patients with malignancies. We conclude that soluble TfR represents a valuable quantitative assay of marrow erythropoietic activity as well as a marker of tissue iron deficiency. D

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Section: Main Characteristicssupporting

confidence: 64%

Soluble transferrin receptor for the evaluation of erythropoiesis and iron status

Béguin

2003

Clinica Chimica Acta

395

316

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“…Hydroxyurea has been suspected to promote ulceration in patients with SCD [66][67][68] and other hematological disorders [69][70][71]. A recent study of 3,411 patients with Philadelphia chromosome negative myeloproliferative neoplasms treated with hydroxyurea reported 5% incidence of mucocutaneous complications, 80% of which were leg ulcers [72].…”

Section: Systemic and Local Therapiesmentioning

confidence: 99%

Critical Reviews: How we treat sickle cell patients with leg ulcers

Minniti

Kato

2015

American J Hematol

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The past five decades have seen an improvement in the mortality and morbidity of sickle cell disease (SCD) because of prophylaxis against infectious complications, improved and expanded red cell transfusions, implementation of hydroxyurea therapy, and advances in supportive care. Now that the majority of patients in the western hemisphere reaches adulthood, end organ diseases are frequent, which include vasculopathic complications such as chronic leg ulcers. The management of patients with leg ulcers requires the hematologist to lead a team of health care professionals, and investigates the presence of associated, but potentially still occult signs of vasculopathy, such as pulmonary hypertension, renal disease, priapism and retinopathy. These complications may be asynchronous, and long term careful screening is indicated, in order to ensure early diagnosis and intervention. It is crucial to address both the immediate consequences of pain, infection and disability, and long term effects on quality of life, employment and stigma associated with chronic ulceration. Recent insights into their pathophysiology may have practical implications. We propose a holistic approach to the management of patients' physical and emotional problems and mechanisms of ulcers formation and delayed healing. An overview of topical and systemic therapies for chronic ulcers is given, with the understanding that wound care therapy is best left to the wound specialists, medical and surgical, with whom the hematologist must keep an open line of communication. In the absence of evidence-based guidelines, our opinion is based on both a critical review of the literature and our personal clinical and research experience. Am. J. Hematol. 91:22-30, 2016. V C 2015 Wiley Periodicals, Inc. Clinical CasesCase 1. A 49 year old African American woman with sickle cell anemia, bilateral hip replacements for avascular necrosis of the femoral head, pulmonary arterial hypertension documented by pulmonary artery catheterization, and history of laser treatment and vitrectomy for proliferative retinopathy and vitreous hemorrhage, presented with a very painful ulcer at the right malleolus. She has had three previous ulcers, on both legs, the first at age 32 years, attributed to excessive standing at her job, no trauma, which had responded to topical care and healed within few months of presentation. At that time she was not taking hydroxyurea, but has been taking it now for longer than 10 years with good fetal hemoglobin response: 17%. This current ulcer developed while she was experiencing high personal stress but no physical trauma. She rarely experiences vaso-occlusive pain crisis and has very infrequent hospitalizations. She was referred to the wound care service and after 7 months of unfruitful therapies, including surgical debridement, MIST ultrasound therapy, antibiotics, and compressions, she was started on monthly transfusions and her ulcer healed 5 months later. She has had consistently elevated serum lactate dehydrogenase (LDH) (>600 IU/L), serum dire...

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“…[14] It is apparent from (Table No 6) that the majority of the Sickle Cell Disease and sickle cell-β -thalassemia cases showed reduced values of red cell indices like MCV, MCH and MCHC suggestive of hypochromic and microcytic anaemia (15) . Following Hydroxyurea treatment, there are overall increases in HbF in most of the cases with reductions in the frequency of vasoocclusive crisis & avascular necrosis as hydroxyurea increases the red cells containing an increased amount of fetal hemoglobin, which inhibits HbS polymerization, and decrease of leukocytes and platelets, which significantly limits their adherence to the vascular wall (16,17) . (Table7).…”

Section: Discussionmentioning

confidence: 99%

“…(Table7). There are marked reduction in requirement of blood transfusion due to increase in HbF level (16) .…”

Section: Discussionmentioning

confidence: 99%

Clinico- haematological Profile of Sickle Cell Disease and Sickle Cell BetaThalassaemia in the State of Odisha

Mishra¹

2017

jmscr

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Reduction of the Clinical Severity of Sickle Cell/β-Thalassemia with Hydroxyurea: The Experience of a Single Center in Greece

Cited by 45 publications

References 19 publications

Soluble transferrin receptor for the evaluation of erythropoiesis and iron status

Soluble transferrin receptor for the evaluation of erythropoiesis and iron status

Critical Reviews: How we treat sickle cell patients with leg ulcers

Clinico- haematological Profile of Sickle Cell Disease and Sickle Cell BetaThalassaemia in the State of Odisha

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