Hydroxycarbamine: From an Old Drug Used in Malignant Hemopathies to a Current Standard in Sickle Cell Disease

Cannas, Giovanna; Poutrel, Solène; Thomas, Xavier

doi:10.4084/mjhid.2017.015

Cited by 23 publications

(12 citation statements)

References 129 publications

(135 reference statements)

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“…In fact, results from a protocol suggest minimal genotoxicity or carcinogenicity with long-term hydroxyurea exposure 60. A recent review further lends credence to its safety and efficacy in both pediatric and adult patients as there was no reported increase in the incidence of leukemia and teratogenicity 61. In a longitudinal study of a cohort of sickle cell children in the United States, a group of researchers compared the number of vaso-occlusive pain episodes (including acute chest syndrome/pneumonia episodes) and treatment expenditure in those treated with hydroxyurea and those not treated with it, during a period of 2–3 years 62.…”

Section: Introductionmentioning

confidence: 99%

Vaso-occlusive crisis in sickle cell disease: current paradigm on pain management

Uwaezuoke¹,

Ayuk²,

Ndu³

et al. 2018

JPR

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This narrative review aims to highlight the current paradigm on pain management in sickle cell vaso-occlusive crisis. It specifically examines the pathophysiologic mechanisms of sickle cell pain as well as the pharmacologic and nonpharmacologic methods of pain management. Recurrent painful episodes constitute the major morbidity in sickle cell disease (SCD). While adolescents and young adults experience mostly acute episodic nociceptive pain, it is now recognized that a significant number of adult patients develop chronic neuropathic and centralized pain. In fact, current evidence points to an age-dependent increase in the frequency of SCD patients with chronic pain. Management of disease-related pain should be based on its pathophysiologic mechanisms instead of using recommendations from other non-SCD pain syndromes. Pain management in vaso-occlusive crisis is complex and requires multiple interventions such as pharmacologic, nonpharmacologic, and preventive therapeutic interventions. Pharmacologic treatment involves the use of non-opioid and opioid analgesics, and adjuvants – either singly or in combination – depending on the severity of pain. The basic approach is to treat SCD pain symptomatically with escalating doses of non-opioid and opioid analgesics. Given the moderate-to-severe nature of the pain usually experienced in this form of SCD crisis, opioids form the bedrock of pharmacologic treatment. Multimodal analgesia and structured, individualized analgesic regimen appear more effective in achieving better treatment outcomes. Although the current evidence is still limited on the supportive role of cognitive behavioral therapy in pain management, this nonpharmacologic approach is reportedly effective, but needs further exploration as a possible adjunct in analgesia.

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Section: Introductionmentioning

confidence: 99%

Vaso-occlusive crisis in sickle cell disease: current paradigm on pain management

Uwaezuoke¹,

Ayuk²,

Ndu³

et al. 2018

JPR

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“…The mechanism of this protection is explained by the formation of hybrid polymers (α 2 β s γ) that stop the growth of the phenomenon of polymerization. This property has since been used in the treatment of SCD following empirical evidence that hydroxyurea stimulated the production of FHb 12,13. The protective effect of alpha-thalassemia is related to the reduction of Hb concentration in the erythrocytes, which results in the microcytic anemia 14.…”

Section: Introductionmentioning

confidence: 99%

Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

Mikobi

Lukusa

Muamba

et al. 2019

Mediterr J Hematol Infect Dis

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FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease. Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients. This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has been confirmed by the molecular test. The diagnosis of alpha-thal was made by the multiplex ligation dependent probe amplification (MLPA) technique. Sickle cell pregnancies were followed according to the protocol of care in force in our service. The variables of interest were: hematological variables, sickle cell crises during pregnancy, maternal and fetal complications. Statistical analyzes were performed with SPSS 20.0 software. Means and standard deviations were compared with the Student's t and Annova tests. The value of p <0.05 was considered the significance level. The Hb-SS / alpha-thal and HbSS / HPFH genotypes were observed in 101 and 121 women respectively. Otherwise 758 women had the HbSS genotype. The morbidity related to sickle cell complications in the mother and fetus, were less frequent in the Hb-SS / alpha-thal and HbSS / HPFH groups. The statistical differences were statistically significant. This study showed a significant protective effect of alpa-thal and HPFH during pregnancy in sickle-cell pregnant women. FHb and alpha-thal are two genetic factors that modulate the clinical expression of sickle cell disease. Objective: to determine the beneficial role of FHb and alpha-thal on fetal and maternal morbidity during pregnancy in sickle cell patients. This is a documentary and analytical study that included 960 deliveries of homozygous sickle cell patients. The deliveries were divided into three genotype subgroups: Hb-SS / alpha-thal, HbSS / HPFH and HbSS. The diagnosis of SCD and the quantification of FHb were performed by the capillary electrophoresis technique. The diagnosis of SCD has been confirmed by the molecular test. The diagnosis of alpha-thal was made by the multiplex ligation dependent probe amplification (MLPA) technique. Sickle cell pregnancies were followed according to the protocol of care in force in our service. The variables of interest were: hematological variables, sickle cell crises during pregnancy, maternal and fetal complications. Statistical analyzes were performed with SPSS 20.0 software. Means and standard deviations were compared with the Student's t and Annova tests. The value of p <0.05 was considered the significance level. The Hb-SS / alpha-thal and HbSS / HPFH genotypes were observed in 101 and 121 women respectively. Otherwise 758 women had the HbSS genotype. The morbidity related to sickle cell complications in the mother and fetus, were less frequent in the Hb-SS / alpha-thal and HbSS / HPFH groups. The statistical differences were statistically significant. This study showed a significant protective effect of alpa-thal and HPFH during pregnancy in sickle-cell pregnant women.

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“…For acute, non-life-threatening vaso-occlusive episodes, first-line treatment includes hydration, applying heat, antibiotics, anti-inflammatory agents, and opioids, which are useful in controlling pain and improving oxygen delivery to tissues. 4,5 Hydroxyurea, a crisis-prevention mainstay treatment, is a ribonucleotide reductase inhibitor that has multiple effects in SCD. It increases hemoglobin F concentrations, which decreases HgbS concentration and prevents HgbS polymerization, which in turn decreases RBC sickling and hemolysis.…”

mentioning

confidence: 99%

“…It increases hemoglobin F concentrations, which decreases HgbS concentration and prevents HgbS polymerization, which in turn decreases RBC sickling and hemolysis. 1,5 Additional effects include inhibiting white blood cell and platelet production, which may ameliorate occlusive episodes. Blood transfusion therapy, including exchange and simple transfusion, are indicated for stroke, severe acute chest syndrome, splenic sequestration, and symptomatic anemia.…”

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confidence: 99%

Hyperhemolysis Syndrome in Patients With Sickle Cell Disease

Banks¹,

Shikle

2018

Archives of Pathology &Amp; Laboratory Medicine

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Sickle cell disease is a genetic disease commonly affecting people of African, Indian, and Mediterranean descent. Patients with this chronic disease often require lifelong red blood cell transfusions. Formation of alloantibodies and autoantibodies are well-known complications that can arise with multiple transfusions. Another rare, but serious complication associated with transfusion is hyperhemolysis syndrome. The acquisition of new and/or rare alloantibodies can make it more difficult to find compatible blood products for patients with sickle cell disease. Genotyping and national donor registries are useful tools for finding appropriate blood products for these patients. This review will describe the clinical and laboratory findings of sickle cell disease, including hyperhemolysis syndrome. The challenges associated with locating compatible blood for patients with various red blood cell antibodies will be reviewed.

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Hydroxycarbamine: From an Old Drug Used in Malignant Hemopathies to a Current Standard in Sickle Cell Disease

Cited by 23 publications

References 129 publications

Vaso-occlusive crisis in sickle cell disease: current paradigm on pain management

Vaso-occlusive crisis in sickle cell disease: current paradigm on pain management

Homozygous Deletion Alfa-Thalassemia and Hereditary Persistence of Fetal Hemoglobin, Two Genetic Factors Predictive the Reduction of Morbidity and Mortality During Pregnancy in Sickle Cell Patients . A Report From Democratic Republic of Congo

Hyperhemolysis Syndrome in Patients With Sickle Cell Disease

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