Priapism with Essential Thrombocythemia Review of Literature, Where We Stand and Where We Are Heading

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 30-30
Author(s):  
Elrazi Awadelkarim A Ali ◽  
Abdulqadir Jeprel Nashwan ◽  
Mohamed A Yassin
Keyword(s):  
Platelet Count ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Essential Thrombocythemia ◽  
Hemoglobin Level ◽  
Medical Attention ◽  
Cell Disease ◽  
Ischemic Priapism ◽  
High Platelet Count ◽  
Stuttering Priapism

Introduction Priapism is a persistent erection of the penis that is not associated with sexual stimulation or desire lasting more than 4 hours. Priapism can be classified as ischemic, non-ischemic, or stuttering. Stuttering priapism is defined as recurrent priapism lasting less than 4 hours. Commonly, no cause is identified, secondary causes include medications specially intracorporal injections or medical conditions such as hematological diseases particularly sickle cell disease, but it is uncommon to be seen with essential thrombocythemia (ET). It adversely affects the quality of life, sexual function, and physical wellness of the affected patients besides the risk of penile fibrosis and permanent erectile dysfunction. The underlying pathophysiology is not clearly understood but the mechanism involves disturbed nitrous oxide, phosphodiesterase enzyme activity, and disturbed auto-regulation of the penile circulation. Method Literature searched in google scholar, PubMed, and Scopus search engines with keywords priapism, essential thrombocythemia, essential thrombocytosis results found from 1960 to 2020. Results Using the keywords above we found 11 cases of ET devolved or presented with priapism. With the case in our center (not published) the total number of cases will be 12. The cases arranged by chronological order. Almost all age groups are involved from 7 years to 71 years. The table shows hematological parameters, type of mutation, and the management of priapism. Discussion In ET the mechanism of priapism is probably related to high platelet count and platelets sludging in the corpora cavernosa, similar to RBC sludging in sickle cell disease and WBC sludging in leukemia. Subsequent stasis and disturbed NO and cGMP signaling. This might be precipitated by increased viscosity or release of free hemoglobin secondary to hemolysis similar to sickle cell anemia as many patients had low hemoglobin levels (table-1). Looking at the table with the available data most hemoglobin levels are in the lower normal limits, but no patient had high hemoglobin levels, opposite to what can be expected that polycythemia might enhance the viscosity of the blood and enhance thrombosis. Observing other MPN who developed priapism, in one study 430 patients with CML 8 patients developed priapism at diagnosis, they had lower hemoglobin levels compared to CML patients who didn't develop priapism. The mean hemoglobin level in patients with priapism was 8.4 ± 2.3 g/dL, compared to 10.8 ± 2.5 g/dL in CML patients control who didn't develop priapism. To the best of our knowledge, there are no reports of cases of adults with polycythemia Vera who devolved priapism which might indicate that anemia has a synergistic effect with leukocytosis in the pathogenesis of priapism in CML patients. But, it is difficult to generalize this observation to ET. However, anemia can enhance thrombosis and the risk of thromboembolism by different mechanisms not related to reactive thrombocytosis. Some patients presented after years from the onset of priapism. This may indicate the complaint of priapism is underreported especially for patients with stuttering priapism who develop erection for a shorter duration of time and subsides spontaneously. While patients with ischemic priapism have prolonged, painful erection usually seeks medical attention from the first time. This can be the reason that CML reports of priapism are relatively more common 1.9 % compared to ET because CML type is predominantly ischemic. At the same time, ET can be ischemic or stuttering. Most of these cases are reported before 2008 WHO criteria for ET, which explains why no genetic studies done for the cases. This case is the first reported case of CLAR mutation associated with priapism. In the table 6/12 had splenomegaly or splenectomy a ratio close to general ET patients. Conclusion Priapism is a rare presentation and complication of ET. It can present as ischemic or stuttering types. ET with stuttering priapism might be underreported. The main risk factors aren't clear due to old and few reported cases. Splenomegaly, age, and type of mutation appear to have little impact. But the characteristic features are the high platelet count and lower hemoglobin level. Disclosures No relevant conflicts of interest to declare.

scholarly journals Men with sickle cell disease experience greater sexual dysfunction when compared with men without sickle cell disease

2020 ◽  
Vol 4 (14) ◽  
pp. 3277-3283
Author(s):  
Ibrahim M. Idris ◽  
Akib Abba ◽  
Jamil A. Galadanci ◽  
Sharfuddeen A. Mashi ◽  
Nafiu Hussaini ◽  
...  
Keyword(s):  
Erectile Dysfunction ◽  
Sexual Dysfunction ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Medical Attention ◽  
Cell Disease ◽  
Cross Sectional Survey ◽  
Cross Sectional ◽  
Ischemic Priapism ◽  
Stuttering Priapism

Abstract Recurrent ischemic priapism is a common complication of sickle cell disease (SCD). We assessed the burden, characteristics, and types of priapism, including sexual dysfunction, in a cohort of men with and those without SCD, to test the hypothesis that sexual dysfunction is more prevalent in men with SCD. In Kano, Nigeria, we conducted a comparative cross-sectional survey that included 500 and 250 men 18 to 40 years of age, with and without SCD, respectively. The survey used the Priapism Questionnaire and the International Index of Erectile Function for sexual function assessment. All eligible participants approached for the study gave informed consent and were enrolled. Stuttering and major priapism were defined based on the average duration of priapism experiences that lasted ≤4 and >4 hours, respectively. The prevalence of priapism was significantly higher in men with SCD than in those without it (32.6% vs 2%; P < .001). Stuttering priapism accounted for 73.6% of the priapism episodes in men with SCD. Nearly 50% of the participants with SCD-related priapism had never sought medical attention for this complication. The majority of the men with SCD-related priapism used exercise as a coping mechanism. Priapism affected the self-image of the men with SCD, causing sadness, embarrassment, and fear. The percentage of the men with SCD who had erectile dysfunction was more than twofold higher than that of those without SCD who had erectile dysfunction (P = .01). The men with SCD had a higher prevalence of priapism and sexual dysfunction than the men without SCD.

Abstract 65: Anemia-related Heart Failure in Sub-saharan African Sickle Cell Disease Patients

2017 ◽  
Vol 121 (suppl_1) ◽  
Author(s):  
Adebayo C Atanda ◽  
Yahya Aliyu ◽  
Oluwafunmilayo Atanda ◽  
Aliyu Babadoko ◽  
Aisha Suleiman ◽  
...  
Keyword(s):  
Heart Failure ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Hemoglobin Level ◽  
Sub Saharan Africa ◽  
Cell Disease ◽  
Jet Velocity ◽  
Sub Saharan

Introduction: Anemia has been implicated in heart failure. Existing literatures, involving predominantly African-Americans, suggests that Sickle Cell Disease (SCD) maybe linked to various cardiovascular complications including pulmonary hypertension and left venticular dysfunction. Peculiarly, our study involves exclusively Sub-Saharan population. Method: We conducted a cross sectional observational study of 208 hydroxyurea-naive consecutive SCD patients aged 10-52 years at steady state and 94 healthy non-matched controls who were studied in an out patient clinic in Sub-Saharan Africa. SCD patients were required to have electrophoretic or liquid chromatography documentation of major sickling phenotypes. Control group was required to have non-sickling phenotypes. Cardiac measurements were performed with TransThoracic Echo according to American Society of Echocardiography guidelines. Hemoglobin level was also obtained. Results: Hemoglobin level in SCD group (8.5+/- 1.5) was significant (P<0.001) compared to control (13.8+/- 1.7). Although SCD group had significantly higher values of left ventricular (LV) size, there was no qualitative evidence of LV dysfunction. SCD group had higher values of Ejection Fraction but not statistically significant. There was no evidence of LV wall stiffening to impair proper filling in SCD group, with the ratio of early to late ventricular filling velocities, E/A ratio elevated (1.7+/-0.4 compared to 1.6+/- 0.4; P=0.010). Right ventricular systolic pressure was determined using the formula of 4x Tricuspid Reugurgitant jet (TRV) square as an indirect measurement of Pulmonary arterial systolic pressure. SCD patients had significantly higher mean±SD values for tricuspid regurgitant jet velocity than did the controls (2.1±0.6 vs. 1.8±0.5; p= 0.001). Within the SCD group, there was no clear pattern of worsening diastolic function with increased TRV. Furthermore, E/A had a significant positive relationship with jet velocity in bivariate analysis (R=0.20; P=0.013). Conclusions: We were unable to demonstrate existence of anemia-associated left ventricular dysfunction in Sub-Saharan African with SCD. Further studies is required to highlight the reason behind this finding.

scholarly journals Clinical and Biomarker Predictors for Avascular Necrosis in Sickle Cell Disease

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3091-3091
Author(s):  
Michael Rabaza ◽  
Maria Armila Ruiz ◽  
Liana Posch ◽  
Faiz Ahmed Hussain ◽  
Franklin Njoku ◽  
...  
Keyword(s):  
Risk Factors ◽  
Sickle Cell Disease ◽  
Board Of Directors ◽  
Avascular Necrosis ◽  
Sickle Cell ◽  
Research Funding ◽  
Medical Attention ◽  
Cell Disease ◽  
Advisory Committees ◽  
Early Management

Abstract Introduction Sickle cell disease (SCD) affects 1 in 365 African Americans and approximately 25 million people world-wide. A common skeletal system complication is avascular necrosis (AVN), which can cause substantial pain and a reduced quality of life. While early management of AVN is focused on increasing range of motion with physical therapy and pain relief, there are no clear predictors for who is more likely to develop AVN and earlier institution of these preventive measure could help decrease disease progression. Vascular endothelial growth factor (VEGF) is a biomarker of endothelial injury and may indicate reduced vascular supply to the femoral or humeral head. Here we describe potential risk factors and biologic pathways for AVN in SCD, as understanding these may lead to improvements in future monitoring, early detection, and early intervention practices. Methods We investigated clinical and laboratory risk factors associated with AVN in a cohort of 435 SCD patients from our center. Blood samples, clinical, and laboratory data were collected at the time of enrollment during a clinic visit. Genotyping for alpha thalassemia was performed by PCR and the serum concentration of VEGF was measured by ELISA. AVN status was confirmed by review of the medical record and available imaging. We conducted a cross-sectional analysis comparing categorical and linear variables by AVN status using the chi-square and Kruskal-Wallis test, respectively. The independent association of the clinical and laboratory variables with AVN status was determined by logistic regression analysis. The initial model included variables with a P-value &lt; 0.1 on univariate analysis and the final model was ascertained by stepwise forward and backward selection. Median values and interquartile range (IQR) are provided. Results The median age of the cohort was 32 (IQR, 24 - 43) years, 57% (250/435) were female, and 46% (198/435) were on hydroxyurea. AVN was observed in 34% (149/435) of SCD patients. SCD patients with AVN were older, had more frequent vaso-occlusive crises requiring medical attention, and had a higher body mass index (Table I) (P ≤ 0.002). We measured VEGF in 241 of the SCD patients with serum samples available at the time of enrolment. Serum VEGF concentrations trended higher in SCD patients with versus without AVN (420 vs. 359 pg/mL, respectively; P = 0.078). In the multivariate analysis model, AVN was independently associated with increased number of vaso-occlusive crises (OR 1.1, 95% CI: 1.0 - 1.14; P = 0.02), AST concentration (natural log OR 0.5, 95% CI: 0.2 - 0.9; P = 0.03), VEGF concentration (natural log OR 1.4, 95% CI: 1.0 - 1.9; P = 0.047), and tobacco use (OR 1.9, 95% CI: 0.9 - 3.7; P = 0.078). Discussion In conclusion, we demonstrate a high prevalence of AVN in an adult cohort of SCD patients. The presence of AVN was independently associated with a greater frequency of vaso-occlusive pain episodes, which may demonstrate a shared pathophysiology between AVN and vaso-occlusion that merits further investigation. We demonstrate that serum VEGF concentrations are higher in SCD patients with AVN and may be a clinical tool to identify those at high-risk and for earlier intervention for this complication. Figure 1 Figure 1. Disclosures Gordeuk: Modus Therapeutics: Consultancy; Novartis: Research Funding; Incyte: Research Funding; Emmaus: Consultancy, Research Funding; Global Blood Therapeutics: Consultancy, Research Funding; CSL Behring: Consultancy. Saraf: Pfizer: 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, Research Funding.

scholarly journals Priapism in Sickle Cell Anemia: Emerging Mechanistic Understanding and Better Preventative Strategies

Anemia ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Genevieve M. Crane ◽  
Nelson E. Bennett
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Treatment Strategies ◽  
Vascular Complications ◽  
Cell Disease ◽  
Nitric Oxide Signaling ◽  
Risk Of Death ◽  
Ischemic Priapism

Sickle cell anemia is a common and disabling disorder profoundly affecting mortality as well as quality of life. Up to 35% of men with sickle cell disease are affected by painful, prolonged erections termed ischemic priapism. A priapic episode may result in fibrosis and permanent erectile dysfunction. The severity of sickle cell disease manifestations is variable dependent on a number of contributing genetic factors; however, priapism tends to cluster with other severe vascular complications including pulmonary hypertension, leg ulceration, and overall risk of death. The mechanisms underlying priapism in sickle cell disease have begun to be elucidated including hemolysis-mediated dysregulation of the nitric oxide signaling pathway and dysregulation of adenosine-mediated vasodilation. A better understanding of these mechanisms is leading toward novel preventative strategies. This paper will focus on the mechanisms underlying development of ischemic priapism in sickle cell disease, current acute and preventative treatment strategies, and future directions for improved management of this disorder.

Red Blood Cell Exchange Transfusion, An Underutilized Effective and Reliable Therapeutic Modality in Sickle Cell Disease, Reduces Number of Admissions and Length of Hospital Stay

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4822-4822
Author(s):  
Aref Agheli ◽  
Kirshma Khemani ◽  
Madhumati Kalavar ◽  
William Steier ◽  
Zili He
Keyword(s):  
Sickle Cell Disease ◽  
Hospital Stay ◽  
Sickle Cell ◽  
Blood Viscosity ◽  
Exchange Transfusion ◽  
Hemoglobin Level ◽  
Therapeutic Modality ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
One Year

Abstract Background: The pathophysiology of sickle cell disease (SCD) is based on increased blood viscosity due to abnormal red blood cells (RBCs), which causes SCD complications, such as chronic hemolytic anemia, vaso-occlusive crisis with tissue hypoxemia, and organ dysfunction. Effective treatment of Sickle Cell Anemia is to reduce the blood concentration of Hemoglobin S (Hb S) RBCs. Exchange transfusion (ET) remains an effective but possibly underutilized therapy for the management of various acute and chronic complications of SCD such as acute chest syndrome, thromboembolic stroke, splenic and hepatic infarction, right upper quadrant syndrome, multi-organ failure syndrome, or in preparation for surgery by reducing HbS to less than 30%. RBC ET quickly replaces abnormal RBCs with normal RBCs, thus improving oxygen transport while reducing overall blood viscosity. Methodology: To determine the effectiveness of ET in SCD in reducing the total number of admissions and total in-hospital Length of Stay (LOS) in patients, admitted with any of acute complications of SCD, we retrospectively reviewed the medical records of 38 patients between June 15, 2007 and June 15, 2008. The eligibility criteria were age above 18 years old and admission to the hospital for any SCD complication. Nineteen patients had ET with Hb A containing RBCs, generally with an average packed RBC exchange volume of 70–80 ml/kg patient’s weight. Nineteen patients were treated with conventional managements. Three patients were excluded from the ET group because of prolonged LOS due to non-SCD- related complications. Four patients in the non-Exchange group signed against medical advice on the first day of admission and were excluded from analysis. Results: Sixteen (42.1%) patients were male and 22 (57.9%) patients were female. Their ages ranged from 19 to 67 years old, mean (SD) 30.2 (10.8). Of eligible patients, 19 (50%) patients received at least one therapeutic ET during the one year period of the study. In an independent-Samples T test analysis, the mean (SD) LOS were 7.5 (0.6) and 4.2 (0.6) days for the groups without ET and with ET respectively (95% CI = −5.2 to 1.5, p=.0011) (Figure 1). In this small studied group, this resulted average 3.3 days shorter in-hospital stay in ET group, could have saved 62 in-hospital days in the group who received conventional treatments. This number could have been easily much greater, since our hospital’s electronically stored data revealed that during year 2007, there had been 278 SCD admissions in all age groups. There was no mortality in the ET group, nor were any transfusion-related complications reported. In another analysis of one year follow up data, the number of admissions for the patients who never received ET ranged from 1 to 14, mean (SD) 1.7 (2.3) times in year 2007, while in patients who received at least one ET, the number of following admissions ranged from 0 to 2, mean (SD) 0.7 (1.3) times during the next year (95% CI = 0.16 to 1.7, p= .020). Hemoglobin level of patients in conventional treatment group on the day of discharge ranged from 6.5 to 10.7, mean (SD) 8.9 (1.9) and in ET group it ranged from 8.4 to 12.4, mean (SD) 10.2 (1.2) gr/dl (p= .045). Conclusion: Patients with SCD are frequently admitted to hospital for vaso-occlusive crisis and other complications. Exchange transfusion is a reliable, safe, and effective therapeutic modality in SCD patients, in particular during a catastrophic event. ET can significantly reduce the number of hospital admissions and in-hospital stay days in these patients. In addition, patients managed with ET have a better hemoglobin level on discharge. Figure Figure

scholarly journals Association of sickle cell disease with nutritional status among under-five children and mediating role of hemoglobin level: secondary analysis of data from 2018 Nigeria Demographic and Health Survey

2020 ◽  
Author(s):  
Mohammad Redwanul Islam ◽  
Md. Moinuddin ◽  
Ayeda Ahmed ◽  
Syed Moshfiqur Rahman
Keyword(s):  
Sickle Cell Disease ◽  
Nutritional Status ◽  
Sickle Cell ◽  
Hemoglobin Level ◽  
Cell Disease ◽  
Anthropometric Indices ◽  
Under Five ◽  
Under Five Children ◽  
Mediating Role

Abstract Background: Malnutrition continues to affect under-five children in Africa to an overwhelming proportion. The situation is further compounded by the burden of sickle cell disease (SCD). However, association of SCD with stunting, wasting, and underweight in a nationally representative sample of under-five children remains unexplored. We aimed to describe prevalence of undernutrition by sickle cell status, to evaluate its association with growth faltering ascertained with anthropometric indices, and to explore mediating role of hemoglobin.Methods: We availed data from the 2018 Nigeria Demographic and Health Survey (DHS) and the sample comprised 11233 children aged 6–59 months who were successfully genotyped for SCD. The DHS employed a two-stage stratified sampling strategy. SickleSCAN rapid diagnostic test was used for SCD genotyping. Z-scores of length/height-for-age (HAZ), weight-for-height (WHZ), and weight-for-age (WAZ) were computed against the 2006 World Health Organization Child Growth Standards. We fitted logistic regression models to evaluate association of SCD with stunting, wasting, and underweight. Mediation analysis was performed to capture the indirect effect of, and proportion of total effect mediated through hemoglobin level in SCD-anthropometric indices association.Results: Prevalences of stunting, wasting, and underweight among children with SCD were 55.4% (54.5–56.4), 9.1% (8.6–9.7), and 38.9% (38.0-39.8), respectively. The odds of stunting were 2.39 times higher (adjusted odds ratio (aOR) 2.39, 95% CI: 1.26–4.54) among sickle children than those with normal hemoglobin. SCD was also significantly associated with underweight (aOR 2.64, 95% CI: 1.25–5.98), but not with wasting (aOR 1.60, 95% CI: 0.85–3.02). Hemoglobin level significantly mediated SCD-HAZ (adjusted indirect effect (aIE) -0.328, 95% CI: -0.387, -0.270), SCD-WHZ (aIE − 0.080, 95% CI: -0.114, -0.050), and WAZ (aIE − 0.245, 95% CI: -0.291, -0.200) associations. The extent of mediation was highest for SCD-HAZ association (adjusted proportion mediated 0.928, 95% CI: 0.535–2.770).Conclusion: We presented compelling evidence of the negative impact of SCD on nutritional status of under-five children. Integration of a nutrition-oriented approach into a definitive SCD care package and its nation-wide implementation could bring promising results by mitigating the nutritional vulnerability of children with SCD.

scholarly journals A Cross Sectional Study of Association of HLA typing with Haemoglobin Level in Sickle Cell Anaemia

2021 ◽  
pp. 523-531
Author(s):  
Raed Alserihi ◽  
Saeed Kabrah ◽  
Hadeel Alsadoun
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Blood Cell ◽  
Sickle Cell ◽  
Blood Groups ◽  
Hemoglobin Level ◽  
Hla Typing ◽  
Control Group ◽  
Allelic Association ◽  
Cell Disease

Background: Sickle-cell Disease (SCD) is the most common blood cell disorder affecting millions of people. In severe cases, regular blood transfusion is an essential practice to relieve clinical symptoms. However, since regular blood transfusion can lead to alloimmunization to foreign human leukocyte antigens (HLA), this may result in severe anemia due to red blood cell destruction. Therefore, this study aimed to determine the association between the hemoglobin level and the presence of HLA genotypes among Sickle Cell Anemia patients.  Methodology: A total of 64 SCD patients and 21 healthy donors seen at King Abdulaziz hospital between November 2019 and February 2021 were recruited for this study. Demographic data including ABO/Rhesus blood groups, hemoglobin concentration, were among the clinical information obtained. HLA genotyping was performed using Polymerase Chain Reaction-Sequence Specific Oligonucleotide (PCR-SSO). The data were cleaned using the Microsoft Excel and analysed using the statistical packages for Social Sciences (SPSS) version 24. Results: The incidence of SCD is not strictly gender-related because of its transmission as an autosomal recessive disorder. Sixty-four individuals (33 females; 31 males) having SCD were analyzed. O blood group recorded the highest prevalence compared to other ABO blood groups in SCD patients. After analysing allelic association, HLA-A*02 was more frequent in SCD patients compared to control. After further allelic combination analysis of patients and compared with the control group, HLA-DQB1*02 was majorly involved in overexpression and decreasing hemoglobin level and significantly different among control and experimental groups. Conclusion: Rhesus-positive blood types were more associated with the SCA. HLA- type II alleles could influence the clinical course of sickle cell disease and HLA-DQB1*02 was significantly different among SCD group and control individuals, which signifies the concept that the allele was overexpressed among patients resulting in low Hb level.

scholarly journals Automated Red Cell Exchange in the Management of Sickle Cell Disease

2021 ◽  
Vol 10 (4) ◽  
pp. 767
Author(s):  
Dimitris A. Tsitsikas ◽  
Saket Badle ◽  
Rhys Hall ◽  
John Meenan ◽  
Oloruntoyin Bello-Sanyaolu ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Clinical Response ◽  
Sickle Cell ◽  
Dramatic Improvement ◽  
Iron Loading ◽  
Red Cell ◽  
Cell Disease ◽  
Echocardiographic Parameters ◽  
Red Cell Transfusion ◽  
Stuttering Priapism

Red cell transfusion represents one of the cornerstones of the chronic management of sickle cell disease, as well as its acute complications. Automated red cell exchange can rapidly lower the number of circulating sickle erythrocytes, without causing iron overload. Here, we describe our experience, having offered this intervention since 2011. A transient reduction in the platelet count by 61% was observed after the procedure. This was not associated with any haemorrhagic complications. Despite exposure to large volumes of blood, the alloimmunisation rate was only 0.027/100 units of red cells. The absence of any iron loading was confirmed by serial Ferriscans, performed over a number of years. However, patients with advanced chronic kidney disease showed evidence of iron loading due to reduced innate haemopoiesis and were subsequently switched to simple transfusions. A total of 59% of patients were on regular automated red cell exchange with a history of recurrent painful crises. A total of 77% responded clinically, as evidenced by at least a 25% reduction in their emergency hospital attendance for pain management. The clinical response was gradual and increased the longer patients stayed on the program. The earliest sign of clinical response was a reduction in the length of stay when these patients were hospitalised, indicating that a reduction in the severity of crises precedes the reduction in their frequency. Automated red cell exchange also appeared to be beneficial for patients with recurrent leg ulcers and severe, drug resistant stuttering priapism, while patients with pulmonary hypertension showed a dramatic improvement in their symptoms as well as echocardiographic parameters.

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