scholarly journals Leg Ulcer in Patient with Sickle Cell Disease on Hydroxyurea

2020 ◽  
Vol 4 (1) ◽  
pp. 13-15
Author(s):  
Jaya Manjunath ◽  
◽  
Fnu Nutan ◽  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Future Study ◽  
Alternative Treatment ◽  
Wound Care ◽  
Leg Ulcers ◽  
Cell Disease ◽  
History Of ◽  
Work Up

Leg ulcers are a painful side-effect commonly seen in patients with long-term use of hydroxyurea. We describe an ulcer in a patient with sickle cell disease (SCD) with an 8-year history of hydroxyurea use. Wound care team used cadexomer-iodine topical and collagenase to aid in healing. There are limited options for the treatment of these ulcers beside the cessation of hydroxyurea, a very effective drug in reducing painful sickle cell episodes. It is imperative to be aware of this complication to prevent expensive tests and further work up. Future study into alternative treatment of hydroxyurea-induced leg ulcers for sickle cell patients is necessary.

scholarly journals Implementation of Escort-HU Extension: European Sickle Cell Disease Cohort - Hydroxyurea - Extension Study : Interests and Methodology

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3098-3098
Author(s):  
Mariane de Montalembert ◽  
Ersi Voskaridou ◽  
Lena Oevermann ◽  
Giovanna Cannas ◽  
Anoosha Habibi ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Sickle Cell ◽  
Research Funding ◽  
Steering Committee ◽  
Leg Ulcers ◽  
Cell Disease ◽  
Advisory Committees ◽  
History Of

Abstract The efficacy and long-term effectiveness of hydroxycarbamide/hydroxyurea (HU) in the prevention of painful crises and in the decrease of mortality and morbidity in sickle cell disease (SCD) patients have been established (Charache et al. 1992; Steinberg et al 2010, Voskaridou et al 2010). From January 2009 to March 2019, the non-interventional prospective cohort study ESCORT-HU was conducted to evaluate the use of HU in real-life conditions and to collect information on the long-term safety of HU when used in current practice for the prevention or treatment of symptomatic complications in patients with sickle cell disease (SCD) (Montalembert et al 2021). A total of 1906 patients, 55% of adults, were enrolled in this study in 62 centres (Germany, Greece, Italy and France). The mean exposure to HU in this cohort was 30 months, for a cumulative exposure of 7310 patient-per year. The main objectives of ESCORT-HU have been fulfilled as regards the collection of data on the most common concerns associated with the use of HU in SCD patients: myelosuppression, child growth, concomitant administration of live vaccines, safety in population with renal and hepatic impairment and frequency of SCD events (painful crises, acute chest syndrome, stroke, acute splenic sequestration, infections, blood transfusions and hospitalizations) (Montalembert et al 2021). In order to better identify potential long-term risks and specific concerns of HU therapy, ESCORT-HU extension is being implemented in Europe with the goal of 2500 patients enrolled. Main risks targeted by the study are leg ulcers, one of the most limiting factors to continue a treatment with HU. Patients will be recruited over a 5-year period. In addition to patients already involved in the first ESCORT-HU study, new at-risk patients might be added such as patients with a history of HU exposure of at least 5 years, to allow a follow-up of patients treated with long-term HU to fully estimate the incidence of potential risk of malignancies prepubescent children aged more than 10 years for girls and more than 13 years for boys in order to document impact or not of HU on puberty and realisation of cryopreservation,patients with a history of leg ulcers, to search for discriminating criteria between leg ulcer caused by the disease and HU-induced leg ulcer,pregnant women without interruption of HU 3 months before the beginning of the pregnancy and males treated with HU whose partner is pregnant and without discontinuation of HU 3 months before the beginning of the pregnancy. To date, there is a limited number of pregnancies exposed to HU with documented outcome. Despite no adverse effects on pregnancy or on the health of the foetus/new-born have been registered, an increase of the number of pregnancies with documented outcomes will allow for meaningful assessment of foetal outcome following exposure to HU during pregnancy, 10 months after the beginning of ESCORT-HU extension, 818 patients have been enrolled in 70 investigational sites in 4 countries (Greece, Italy, Germany and France) (see Graph below). A first steering committee was hold in June 2021. Its conclusions were that distribution of patients (genotype, sex, age) was consistent with the first study and the number of events reported until now was coherent with what was expected per year, with no occurrence of major concern. This extension study involves most of competence centres which manage SCD patients. SC patients have become increasingly well cared for in recent decades and have seen their life expectancy increase. HU treatment is now a chronic treatment possibly for life in many patients with SCD, better its knowledge of its efficiency and tolerance, better patient adherence to treatment will be. Figure 1 Figure 1. Disclosures de Montalembert: Addmedica: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding; Bluebird Bio: Consultancy, Membership on an entity's Board of Directors or advisory committees; Vertex: Consultancy. Voskaridou: ADDMEDICA: Consultancy, Research Funding; BMS: Consultancy, Research Funding; GENESIS: Consultancy, Research Funding; NOVARTIS: Research Funding; PROTAGONIST: Research Funding; IMARA: Research Funding. Oevermann: NOVARTIS: Consultancy; GBT: Consultancy. Joseph: bluebird bio: Consultancy. Colombatti: BlueBirdBio: Membership on an entity's Board of Directors or advisory committees, Research Funding; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees, Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees; Novo Nordisk: Membership on an entity's Board of Directors or advisory committees. Bartolucci: INNOVHEM: Other: Co-founder; Jazz Pharma: Other: Lecture fees; AGIOS: Consultancy; Addmedica: Consultancy, Other: Lecture fees, Research Funding; Fabre Foundation: Research Funding; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees, Other: Lecture fees, Steering committee, Research Funding; Bluebird: Consultancy, Research Funding; F. Hoffmann-La Roche Ltd: Consultancy; GBT: Consultancy; Emmaus: Consultancy; Hemanext: Consultancy. Brousse: BLUEBIRDBIO: Consultancy; ADDMEDICA: Consultancy. Galactéros: Addmedica: Membership on an entity's Board of Directors or advisory committees.

Five years of experience with hydroxyurea in children and young adults with sickle cell disease

Blood ◽  
2001 ◽  
Vol 97 (11) ◽  
pp. 3628-3632 ◽  
Author(s):  
Alina Ferster ◽  
Parvine Tahriri ◽  
Christiane Vermylen ◽  
Geneviève Sturbois ◽  
Francis Corazza ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Laboratory Data ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Significant Difference ◽  
History Of ◽  
Ischemic Attack

The short-term beneficial effect of hydroxyurea (HU) in sickle cell disease (SCD) has been proven by randomized studies in children and adults. The Belgian registry of HU-treated SCD patients was created to evaluate its long-term efficacy and toxicity. The median follow-up of the 93 patients registered is 3.5 years; clinical and laboratory data have been obtained for 82 patients at 1 year, 61 at 2 years, 44 at 3 years, 33 at 4 years, and 22 after 5 years. On HU, the number of hospitalizations and days hospitalized dropped significantly. Analysis of the 22 patients with a minimum of 5 years of follow-up confirm a significant difference in the number of hospitalizations (P = .0002) and days in the hospital (P < .01), throughout the treatment when compared to prior to HU therapy. The probabilities of not experiencing any event or any vaso-occlusive crisis requiring hospitalization during the 5 years of treatment were, respectively, 47% and 55%. On HU, the rate per 100 patient-years of severe events was estimated to be 3.5% for acute chest syndrome, 1.2% for aplastic crisis, 0.4% for splenic sequestration; it was 0% for the 9 patients with a history of stroke or transient ischemic attack followed for an average of 4 years. No important adverse effect occurred. Long-term chronic treatment with HU for patients with SCD appears feasible, effective, and devoid of any major toxicity; in patients with a history of stroke, HU may be a valid alternative to chronic transfusion support.

scholarly journals Hydroxyurea Exposure throughout Pregnancy in Patients with Sickle-Cell Disease : 4 Case Reports from European Non-Interventional, Multicentric, Prospective Escort-HU Study

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 1027-1027
Author(s):  
Justine Gellen-Dautremer ◽  
Sylvain Le Jeune ◽  
Marie-Catherine Receveur ◽  
Elena Foïs
Keyword(s):  
Sickle Cell Disease ◽  
Spontaneous Abortion ◽  
Sickle Cell ◽  
Case Reports ◽  
Oral Contraception ◽  
Obstetric Outcomes ◽  
Leg Ulcers ◽  
Fetal Exposure ◽  
Cell Disease ◽  
History Of

Over the last decades, the number of sickle-cell disease (SCD) women reaching childbearing age and planning to become pregnant is increasing due to improvements in the management of the disease.[1] Physiological changes of pregnancy, such as increased metabolic demand, increased blood viscosity and hyper-coagulability, lead to an increased incidence of complications, such as vaso-occlusive crisis (VOC), acute chest syndrome (ACS), osteonecrosis, hepatic necrosis, leg ulcers, and thromboembolic events. [2] Vaso-occlusion may also involve the placenta, with villi fibrosis, necrosis, and infarction leading to impaired uteroplacental circulation, with consequent chronic fetal hypoxia and adverse fetal outcomes. Therefore, prenatal care is important to evaluate if intensification of treatment as blood transfusion or Hydroxyurea (HU) may be required. HU is approved in EU and USA for VOC including ACS in patients with SCD aged 2 years and over. HU is not recommended for use during pregnancy, primarily because animal studies have suggested potential teratogenic effects on the fetus. In some rare cases, discontinuation of HU treatment to avoid fetal exposure is not possible. A few cases of exposure to HU throughout the pregnancy are published in the literature,[3] but none in SCD patients. We hereby present four cases in which HU was taken during all pregnancy collected in the ESCORT-HU study (European Sickle Cell Disease COhoRT - HydroxyUrea), a multicentric, prospective, non-interventional European study which collected long-term safety information on HU when used in current practice. Patient #1 (GxP2, SS) had a history of VOC, osteonecrosis and leg ulcers and was started on HU at age 18. She had two previous pregnancies without HU exposure. No oral contraception was taken and she spontaneously conceived at age 37. HU was continued at the same dose. Patient #2 (G5P1, Sβ+) had a history of VOC, chronic anemia and heart disease, and was started on HU at age 19. At age 34, she started to receive treatment for fertility (gonadotropin and follitropin). HU which had been started 17 years earlier was continued. Patient #3 (G5P1, Sβ+) had a history of chronic severe anemia and was started on HU at age 37. She had a gynecological history of 1 spontaneous abortion, 2 elective abortions and 1 live birth (pregnancy complicated with preeclampsia). She had been on oral contraception before becoming spontaneously pregnant when aged 38. HU, which had been initiated 11 months earlier, was continued at same dose. Patient #4 (SS, G3P0) female with history of stroke with sequelae and VOC, initiated on HU at the age of 25 following post-transfusion alloimmunization. Gynecological history included one spontaneous abortion and one elective abortion when aged 21 and 23 years (before initiation of HU). Following removal of contraceptive device, she conceived spontaneously at age 27. These four women suffered from severe SCD symptoms which required special management during pregnancies. HU exposure during pregnancy is not recommended but these case reports highlight the difficulty for medical teams to deal with both strong desire to become pregnant and maternal and fetal morbidity and mortality in SCD. Similarly to literature data in non-SCD patients, all four pregnancies in SCD women with HU exposure throughout pregnancy had a favorable outcome and no malformation was reported among the neonates. Details of pregnancy course and outcome are provided in Table 1. Although it is highly recommended to avoid any fetal exposure to HU, it can be difficult in some SCD patients with severe symptoms and a wish to become pregnant, to stop the treatment during pregnancy. In these cases, a strict monitoring of the mother and fetus is necessary. [1] Silva F.A.C., Ferreira A.L.C.G., Hazin-Costa M.F., Dias M.L.G., Araújo A.S., Souza A.I., Adverse clinical and obstetric outcomes among pregnant women with different sickle cell disease genotypes, International Federation of Gynecology and Obstetrics, 2018 [2] Jain D., Atmapoojya P., Colah R., Lodha P. Sickle cell disease and pregnancy. Mediterr J Hematol Infect Dis 2019, 11(1): e2019040 [3] C Thauvin-Robinet, Exposure to hydroxyurea during pregnancy: a case series, Correspondence 2001 Disclosures No relevant conflicts of interest to declare.

Leg Ulcers among Patients with Sickle Cell Disease on Hydroxyurea Therapy.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1676-1676
Author(s):  
Suresh Mendpara ◽  
Betsy Clair ◽  
Mudusar Raza ◽  
Lisa Daitch ◽  
David Smith ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Therapeutic Agent ◽  
Acute Chest Syndrome ◽  
Prior History ◽  
Leg Ulcers ◽  
Cell Disease ◽  
Number Of Patients ◽  
Transfusion Requirements ◽  
History Of

Abstract Leg ulcers remain a debilitating complication of Sickle Cell Disease (SCD): significant pain, tendency to polymicrobial infection, difficult to heal, and tendency to recur in ~ 50% of patients. According to CSSCD data, 2.5% of 2075 patients had leg ulcers at study entry; overall incidence was 9.97 per 100-person years in patients without concomitant alpha thalassemia (thal), and 5.73 per 100 person years in those with alpha thal. Hydroxyurea (HU) is an approved therapeutic agent for adults with SCD; it has been shown to decrease frequency of pain crises and acute chest syndrome and decrease transfusion requirements. Recently, HU was associated with a 40% reduction in mortality. There have been reports of an association between HU therapy and leg ulcers in patients with myeloproliferative disorders (MPD) and more recently with SCD. A retrospective study from the Mayo Clinic (Best et al, Ann. Intern. Med., 128:29, 1998) found that among 115 patients with various forms of MPD, 14 developed leg ulcers after an average HU exposure of 6 years; all ulcers healed after stopping HU, and 2 patients had recurrences of their ulcers after resuming HU. Chaine et al (Arch. Dermatol, 137: 467, 2001) reported that 5/17 (29%) patients with SCD on HU developed leg ulcers. If HU, the most effective available therapeutic agent for SCD is indeed causative of leg ulcers, this should raise serious concerns. In an effort to further clarify this problem, we performed a retrospective analysis. 421 adult SCD patients (age 16–60) formed the subject of this study. 152 were treated with HU for a minimum of 6 months. 269 patients were not exposed to HU. A total of 25 patients (5.9%) had leg ulcers; 17 were treated with HU, 8 were not. Thus, the frequency of leg ulcers was 11.2% among HU treated patients, as opposed to 2.9% among those who did not receive HU therapy (p<0.001). However, of the 17 patients with ulcers, 16(94%) had a history of ulcers prior to HU exposure. Only one patient developed ulcers for the first time after starting HU therapy. 6/17 patients experienced healing of their ulcers despite continuing HU. Among the patients treated with HU, those who had ulcers were more anemic (pre-treatment Hb 7.4 vs 8.2 g/dl, p=0.01) and were older (mean age 40.5 vs 33.0, p<0.001), confirming previous observations. Logistic regression analyses showed that only age and prior history of leg ulcers were significant risk factors for the development of ulcers in patients with SCD under HU therapy. Our data on a large number of patients does not suggest that HU therapy alone is causative of leg ulcers in SCD patients. The supporting evidence for this conclusion comes from our observations that 1) a vast majority of patients (16/17; 94%) who developed leg ulcers after starting HU had a previous history of ulcers. This is also true of the report by Chaine et al, where 4/5 patients with leg ulcers had a prior history; 2) in 6/17 patients (35.3%) ulcers healed with conventional therapy despite continuation of HU. Furthermore, it should be noted that patients with leg ulcers represent a more severe group and are thus more likely candidates for HU therapy. We conclude that HU therapy alone is not causative of leg ulcers in patients with SCD. In addition, HU does not appear to prevent recurrence of leg ulcers in patients with a prior history; nor does it expedite ulcer healing. Other as yet unknown factors, some of which are likely genetic, play an important role in determining the risk for developing leg ulcers.

scholarly journals Intima-media thickness of the common femoral artery as a marker of leg ulceration in sickle cell disease patients

2018 ◽  
Vol 2 (22) ◽  
pp. 3112-3117 ◽  
Author(s):  
Oluwagbemiga O. Ayoola ◽  
Rahman A. Bolarinwa ◽  
Uvie U. Onakpoya ◽  
Tewogbade A. Adedeji ◽  
Chidiogo C. Onwuka ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Femoral Artery ◽  
Intima Media Thickness ◽  
Common Femoral Artery ◽  
Leg Ulcer ◽  
Leg Ulcers ◽  
Cell Disease ◽  
History Of ◽  
The Common

Abstract Leg ulceration is a debilitating chronic complication of sickle cell disease (SCD) the pathogenesis of which is yet to be fully elucidated. We hypothesized that SCD patients with histories of previous leg ulcers would have intima hyperplasia of the common femoral artery (CFA). We enrolled 44 SCD patients and 33 age-matched and sex-matched controls with hemoglobin AA. Anthropometric measurements, biochemical parameters, and sonographic intima-media thickness (IMT) of the CFA were determined. The median CFA IMT in SCD limbs with history of leg ulcers (SWLU) was 1.0 mm, whereas it was 0.7 mm in SCD limbs with no history of leg ulcer (SNLU) and 0.60 mm in controls (P &lt; .001). Among the SNLU, 70.3% had CFA IMT &lt;0.9 mm, whereas only 29.7% had CFA IMT ≥0.9 mm. Conversely, only 20.8% of SWLU had CFA IMT &lt;0.9 mm, whereas the remaining 79.2% had CFA IMT ≥0.9 mm. All the controls had CFA IMT &lt;0.9 mm. Binary logistic regression to determine the odds of having leg ulcer among SCD limbs with CFA IMT of ≥0.9 mm yielded an odds ratio of 9, indicating that SCD limbs with CFA IMT ≥0.9 mm had a 9 times greater risk of having leg ulcer compared with those with CFA IMT &lt;0.9 mm. There is a significant increase in the CFA IMT of SCD limbs with ulcer compared with controls and SCD limbs without ulcer, suggesting that arterial vasculopathy plays a major role in the formation of these ulcers.

scholarly journals SAT-201 Adrenal Myelolipoma in a Patient with Sickle Cell Disease

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Maria Inês Alexandre ◽  
Ana Coelho Gomes ◽  
Diogo Cruz ◽  
Maria João Bugalho
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Adrenal Adenoma ◽  
Malignant Lesion ◽  
Vena Cava ◽  
Diagnostic Study ◽  
Cell Disease ◽  
Adrenal Myelolipoma ◽  
History Of ◽  
Work Up

Abstract Background: Adrenal myelolipoma is a rare benign tumor of the adrenal gland, consisting of adipose tissue and hematopoetic elements. It has been reported to be associated with many chronic diseases such as haematological disorders. The majority of the adrenal myelolipomas are diagnosed in the fourth-sixth decades of life, usually as incidental findings. Despite its benign behavior, it may cause difficulties in the differential diagnosis. Clinical Case: A 33-year-old woman was found to have a right adrenal incidentaloma in a kidney ultrasonography, during work-up for suspected kidney pathology ultimately not confirmed. Her past medical history was significant for sickle cell disease. There was no history of hypertension nor personal or family history of endocrine diseases. The physical examination was unremarkable. The ultrasonography revealed a mass above the right kidney, measuring 5.5 cm in diameter, markedly heterogeneous and hypoechoic in the center. On CT imaging, one month later, this mass measured 6.2x4.3cm. The absolute contrast washout of 52% was indeterminate for adrenal adenoma. The MRI, twelve months later, showed a well-demarcated and heterogeneous tumor with 8x4.7x4cm, with fat areas, suggesting adrenal myelolipoma, but not excluding the possibility of a malignant lesion, such as liposarcoma. The mass was in contact with the liver although not invading it nor the kidney or inferior vena cava. There was no evidence of metastatic disease. Basal biochemical work-up did not disclose hormonal hypersecretion. The ACTH level was 19.6 pg/ml (N7.2-63.3); free urinary cortisol (24 hours) was 101 μg/24h (N36-137) and the overnight 1 mg dexamethasone suppression test was also normal (0.5 μg/dL). Serum metanephrines were within the normal range: metanephrine 7,8 pg/mL (N &lt;65) and normetanephrine 52,3 pg/mL (N&lt;196). Her remaining laboratory values were within normal values including potassium and sodium values. Due to the size, rate of growth and atypical features of the mass, right laparoscopic adrenalectomy was performed. The resected adrenal weighed 66.6g and within it there was an intraparenchymal nodule, measuring 7.5 x 3 x 3.5 cm, which on histologic examination proved to be an adrenal myelolipoma. Conclusion: Adrenal myelolipomas are usually clinically silent. However, their incidental diagnosis should warrant careful diagnostic study. Although the pathogenesis of these tumors remains unclear, theories include retention of embryonic rests, adrenocortical metaplasia and extramedullary hematopoiesis. In this patient with sickle cell disease, bone marrow elements within the myelolipoma may have grown as a result of the persistent anemia. Particular to this case is the patient’s young age, since most cases reported have occurred during the fourth-sixth decades of life. In addition, the association with sickle cell anemia has only been reported in a few cases.

scholarly journals CELL-Derived Microparticles and Sickle CELL Disease Chronic Vasculopathy in Sub-Saharan Africa

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3568-3568
Author(s):  
Abdoul Karim Dembele ◽  
Claudine Lapoumeroulie ◽  
Mor Diaw ◽  
Oumar Tessougué ◽  
Lucile Offredo ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Annexin V ◽  
Sub Saharan Africa ◽  
Leg Ulcers ◽  
Cell Disease ◽  
Cellular Origin ◽  
History Of ◽  
Aseptic Osteonecrosis ◽  
Sub Saharan

Introduction Although most individuals with sickle cell disease (SCD) live in sub-Saharan Africa, the history of the disease on this continent remains largely unknown. SCD is characterized by the association of chronic hemolytic anemia with episodes of acute vaso-occlusive events and progressive vascular organ damage. Several pathophysiological pathways in SCD result in the activation of circulating blood cells and the release of microparticles (MPs). In the present study, we investigated cell-derived MPs in patients with SCD living in Africa and analyzed their relationship with clinical complications. Patients and Methods This cross-sectional case-control study is nested in the CADRE cohort (clinical trials.gov identifier NCTO3114137). We included 232 SS adults in two African centers: Bamako (Mali) and Dakar (Senegal). Patients were chosen depending on the absence or the presence of at least one of the following complications: tricuspid regurgitant jet velocity (TRJV) &gt;3 m/s (which may indicate pulmonary hypertension), macroalbuminuria, leg ulcer, priapism, aseptic osteonecrosis, and retinopathy. Overall, 7 groups of 40 SS patients were constituted (20 in each center). Patients were investigated at steady state (i.e.,at least 15 days after a vaso-occlusive crisis, 8 days after fever or infectious disease, and 3 months after a transfusion). MPs were isolated in the African centers immediately after blood sampling by successive centrifugations at increasing speed: 2,500g x2 and 21,000g x2. MPs pellets were stored at -80 °C. The cellular origin of the MPs, erythrocyte, reticulocyte, endothelial, platelet, and leucocyte, was determined using antibodies directed against CD235a, CD71, CD106, CD41, and CD45, respectively, at the National Institute of Blood TransfusioninParis. To maintain the background at an acceptable level, events of 0.16 µm size were excluded (Fig 1A). Only MPs positive for Annexin V and the cell-type-specific labelling were retained. Potential associations between cell-derived MPs, hematological parameters, and vascular complications were assessed using logistic regression with adjustment for age, sex and country. Results The MP pellets of 106 SS patients from Bamako and 126 from Dakar were analyzed in Paris. In these patients, at a mean age of 29 years (+/- 11), high TRJV was present in 64, microalbuminuria in 84, leg ulcers in 33, priapism in 43, aseptic osteonecrosis in 45, and retinopathy in 31 patients whereas 28 patients had no complication at the time of sampling. As a typical result, Fig 1B shows erythrocyte-derived MPs labelled by Annexin V and CD235a (quarter Q2). The MPs distribution was as follows: erythroid 52% [reticulocytes (CD235a+CD71+) 14%, erythrocytes (CD235a+ CD71-) 38%], leukocyte 18%, platelet 21%, and endothelial 9%. Neither erythrocyte- nor reticulocyte-derived MPs significantly correlated with hemolysis markers (LDH, unconjugated bilirubin or reticulocytes) or hemoglobin levels. Erythrocyte- and reticulocyte-derived MPs were significantly lower in patients with retinopathy (OR=0.48, p=0.003 and OR=0.68, p=0.005, respectively). Reticulocyte-derived MPs were negatively associated with a history of priapism (OR=0.76, p=0.020) and positively associated with a history of leg ulcers (OR=1.23, p=0.040). No correlation was found between MPs of other cellular origin and chronic complications, except for a negative association between endothelial-derived MPs and priapism (OR=0.76, p=0.036). Conclusion In our African patients with SCD, erythroid-derived MPs, although recognized cellular products of hemolysis, were not associated with other markers of hemolysis. We hypothesize that erythroid MPs are not only derived from hemolysis but also probably from the sickling process in this population. They were negatively associated with retinopathy and priapism and positively associated with leg ulcers, but not with the other complications classically associated with the hyperhemolytic sub-phenotype. The search for pertinent biomarkers of SCD complications in Africa is an essential challenge. To our best knowledge, this report is the first illustrating the feasibility of high-technology experiments in an African context. Disclosures No relevant conflicts of interest to declare.

scholarly journals To Give or Not to Give: RhD Immunoglobulin for an RHD*39 Pregnant Woman with Sickle Cell Disease

2021 ◽  
pp. 1-5
Author(s):  
Justin E. Juskewitch ◽  
Craig D. Tauscher ◽  
Sheila K. Moldenhauer ◽  
Jennifer E. Schieber ◽  
Eapen K. Jacob ◽  
...  
Keyword(s):  
Pregnant Woman ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Pregnancy Termination ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Childbearing Age ◽  
Procedural Complications ◽  
History Of ◽  
Chronic Hemolysis

Introduction: Patients with sickle cell disease (SCD) have repeated episodes of red blood cell (RBC) sickling and microvascular occlusion that manifest as pain crises, acute chest syndrome, and chronic hemolysis. These clinical sequelae usually increase during pregnancy. Given the racial distribution of SCD, patients with SCD are also more likely to have rarer RBC antigen genotypes than RBC donor populations. We present the management and clinical outcome of a 21-year-old pregnant woman with SCD and an RHD*39 (RhD[S103P], G-negative) variant. Case Presentation: Ms. S is B positive with a reported history of anti-D, anti-C, and anti-E alloantibodies (anti-G testing unknown). Genetic testing revealed both an RHD*39 and homozygous partial RHCE*ceVS.02 genotype. Absorption/elution testing confirmed the presence of anti-G, anti-C, and anti-E alloantibodies but could not definitively determine the presence/absence of an anti-D alloantibody. Ms. S desired to undergo elective pregnancy termination and the need for postprocedural RhD immunoglobulin (RhIG) was posed. Given that only the G antigen site is changed in an RHD*39 genotype and the potential risk of RhIG triggering a hyperhemolytic episode in an SCD patient, RhIG was not administered. There were no procedural complications. Follow-up testing at 10 weeks showed no increase in RBC alloantibody strength. Discussion/Conclusion: Ms. S represents a rare RHD*39 and partial RHCE*ceVS.02 genotype which did not further alloimmunize in the absence of RhIG administration. Her case also highlights the importance of routine anti-G alloantibody testing in women of childbearing age with apparent anti-D and anti-C alloantibodies.

scholarly journals Concurrent Bilateral Central Retinal Artery Occlusion Secondary to Sickle Cell Crisis

2021 ◽  
Vol 9 ◽  
pp. 232470962110283
Author(s):  
Gowri Renganathan ◽  
Piruthiviraj Natarajan ◽  
Lela Ruck ◽  
Roberto Prieto ◽  
Bharat Ved Prakash ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Vision Loss ◽  
Central Retinal Artery Occlusion ◽  
Retinal Artery Occlusion ◽  
Artery Occlusion ◽  
Cell Disease ◽  
Sickle Cell Crisis ◽  
History Of ◽  
Retinal Artery

Vascular occlusive crisis with a concurrent vision loss on both eyes is one of the most devastating disability for sickle cell disease patients. Reportedly occlusive crisis in the eyes is usually temporary whereas if not appropriately managed can result in permanent vision loss. A carefully managed sickle cell crisis could prevent multiple disabilities including blindness and stroke. We report a case of a 24-year-old female with a history of sickle cell disease who had acute bilateral vision loss during a sickle crisis and recovered significantly with a timely emergent erythrocytapheresis.

Sign in / Sign up

Export Citation Format

Share Document