scholarly journals Treatment of recalcitrant chronic leg ulcer in a known sickle cell anaemia patient using honey and fresh hbaa red cell concentrate in a Nigerian secondary healthcare facility

2020 ◽  
Vol 19 (4) ◽  
pp. 278
Author(s):  
MarcellinusUchechukwu Nwagu ◽  
GabriellaIfeoma Omokhua
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Healthcare Facility ◽  
Leg Ulcer ◽  
Red Cell ◽  
Sickle Cell Anaemia Patient

scholarly journals Minimising Transfusion Therapy and Alloimmunisation in Patients with Sickle Cell Disorder in the Era of Hydroxyurea

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5078-5078
Author(s):  
Louise Smith ◽  
Lucy Stead ◽  
Russell D Keenan ◽  
Rekha Thangavelu
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Conflicts Of Interest ◽  
Cell Phenotype ◽  
Acute Chest Syndrome ◽  
Red Cell ◽  
Blood Products ◽  
Related Complication ◽  
Transfusion Therapy ◽  
Historical Presence

Abstract In the UK, 700 patients with sickle cell disease are on a transfusion programme1. Red blood cell (RBC) AI occurs in 4.4-76%2 of regularly transfused sickle patients. Contributing factors include repeated transfusions and ethnic differences between sickle cell patients and their donors. This results in higher rates of mismatch in phenotyped and genotyped blood. There is a continued lack of availability of blood products from more compatible ethnic donors. Sickle patients on transfusion programmes are transfused every 3-6 weeks, creating a large burden on healthcare services in terms of time, labour and resources such as extended red cell typing in order to reduce antibody formation. The presence of RBC antibodies in patients can cause significant delays in obtaining cross matched blood and reductions in phenotype matched blood being transfused. The aims of this project were to a) review transfusion use in relation to the use of hydroxyurea (HU) patients treated at Alder Hey (AH) and b) review rates of AI in these patients. At AH we have changed our practice to offer HU from age 9 months as a disease modifying therapy 20-35mg/kg/day. We retrospectively reviewed the use of blood products in our cohort of 60 patients, age between 0-20 years (mean 11yrs). Patients were observed over their life period treated at AH, mean 10.1 patient years, range 0-20years, total of 439 patient years. We studied retrospectively the number of RBC transfusions and the historical presence of antibodies. We then re-tested all patients to determine current antibody status. Prior to commencement of hydroxyurea, 342 units of bloods were transfused to 21 patients over 243 patient years, which is 1.41 units per patient per year. After commencement of hydroxyurea, 114.5 units were transfused to 17 patients in 187.1 patient years, 0.61 units per patient per year. Indications for transfusion included acute chest syndrome, aplastic crisis, prolonged crisis, increased acute anaemia, hydroxyurea induced anaemia, pre-operatively. 3 patients in our cohort had previous antibodies. Upon repeat antibody screening, no patients had detectable RBC antibodies. All patients have an extended red cell phenotype as part of their initial workup on referral to our service. Elective, non-acute patients (transfusion programme, pre-operative, hydroxycarbamide induced anaemia) all receive fully phenotyped blood. This cannot be guaranteed in acute situations where patients receive the best matched blood available. This practice of minimising blood transfusion use in patients with sickle cell anaemia can reduce blood product usage and as a result reduce the demand for ethnic minority transfusion donors. The subsequent donor exposure would therefore also decrease transfusion related complication of AI. 1 National Haemoglobinopathy Registry Annual Report 2016/17 2G. da Cunha Gomes, E & A. F. Machado, L & C. de Oliveira, L & F. N. Neto, J. (2018). The erythrocyte alloimmunisation in patients with sickle cell anaemia: a systematic review: Erythrocyte alloimmunisation in sickle cell anaemia. Transfusion Medicine. 10.1111/tme.12543. Disclosures No relevant conflicts of interest to declare.

Long Term Regular Automated Red Cell Exchange Transfusion Significantly Reduces Symptoms and Hospital Admissions in Sickle Cell Anaemia.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3746-3746
Author(s):  
Michaela A. Pocock ◽  
Adriaan van Heerden ◽  
Modupe O. Elebute ◽  
Marina Karakantza ◽  
David H. Bevan
Keyword(s):  
Sickle Cell ◽  
Exchange Transfusion ◽  
Sickle Cell Anaemia ◽  
Hospital Admissions ◽  
Femoral Vein ◽  
Globin Gene ◽  
Iron Chelation ◽  
Vascular Trauma ◽  
Red Cell ◽  
Related Hospitalisation

Abstract Introduction Effective therapies for Sickle Cell Anaemia (Hb SS) reduce blood concentration of HbS by transplanting erythroid stem cells or altering globin gene expression with hydroxyurea. A relatively stable reduction in HbS concentration intermediate between these approaches (10–45% HbS) can also be achieved by regular automated red cell exchange transfusion (RBCex), but concerns about cost-effectiveness, alloimmunisation, iron load, pathogen exposure and vascular trauma have limited the use of this treatment modality. Methodology A retrospective study of sixteen fully counselled HbSS individuals with serious complications undergoing regular RBCex with partially phenotype-selected, leucodepleted red cells for a median of 36 (range 11–65) months, for whom accurate pre- and post- RBCex admission data was available. The number of episodes of sickle-related hospitalisation and total inpatient days sustained per year by the group before enrolment in RBCex was compared to the number on RBCex. Excluded from analysis were patients having single RBCex for surgery, pregnancy, or non-recurrent acute complications, having manual exchange transfusions or unable to comply with regular RBCex. Four patients from other units were excluded because complete data on previous admissions was lacking. Target post-procedure haemoglobin was 10–11gm/dL (PCV = 0.31–33) and HbS% <15. Results Between September 1995 and July 2003, 547 RBCex procedures were performed on 56 patients (age: range 16–52 yrs, median 31.5 yrs, mean 29.7 years). Forty patients were excluded from analysis for reasons above. Analysed patients underwent a mean of 7.71 RBCex procedures per year (range 5.12–9.89, median 7.49). A mean of 7.8 (range 4.25–10, median 8) donor red cell units were transfused per procedure. RBCex was performed at a mean of 6.97-week intervals (range 5.26–10.16, median 6.91). Eleven patients (62.5%) required ‘in-out’ femoral vein Vascath insertion. Apheresis nurses placed all catheters in a day-care setting. In the five years before entering the program the patients experienced a mean of 2.03 inpatient episodes per year (range 0.4–5.2, median 1.6): on RBCex this fell to 1.02 per year [range 0–5.3, median 0.33 (p = 0.004)]. Inpatient days fell from a mean of 16.56 per year (range 2–40.6, median 11) to 8.27 [range 0–56.33, median 1.33 (p=0.007)] saving 208.87 in-patient bed days per year. The number of donor red cell units patients received increased from a mean of 15.15 per year (range 0.8–41.1, median 14.1) to 57.9 units per year [range 24–89.7, median 58.0 (p=0.004)]. No patient received any form of iron chelation. No increase in serum ferritin (p=0.796), or loss of vascular access sites, was identified. Two patients acquired new red cell alloantibodies (1-anti Kpa + anti-E; 1- non specific) during RBCex. Three already had alloantibodies (1 anti-e; 1 anti-Kpa + anti-C; 1 non-specific), but did not acquire any more while on RBCex. The presence of antibodies did not preclude continuing RBCex. No transfusion-related infections occurred. One patient died from unrelated causes at home. One novel phenomenon was identified: while on RBCex, ‘rebound’ crises tend to occur when HbS crosses the 45% threshold, emphasising the need for consistency using this therapy. Conclusion We contend that regular RBCex is effective therapy for carefully (self)-selected patients who severely symptomatic. It should be considered in those unresponsive to, or unwilling to take, hydroxyurea. Multidisciplinary support by a skilled team is essential.

Alteration of red cell membrane organization in sickle cell anaemia

1986 ◽  
Vol 63 (4) ◽  
pp. 761-773 ◽  
Author(s):  
Hye-Ryun Choe ◽  
Robert A. Schlegel ◽  
Elizabeth Rubin ◽  
Patrick Williamson ◽  
Maxwell P. Westerman
Keyword(s):  
Cell Membrane ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Red Cell ◽  
Membrane Organization ◽  
Red Cell Membrane

scholarly journals Perioperative Anaesthetic Approach in a Homozygous Sickle Cell Anaemia Patient with Frequent Pain Crises

2014 ◽  
Vol 42 (6) ◽  
pp. 348-351 ◽  
Author(s):  
Kasim Tuzcu ◽  
Murat Karcioglu ◽  
Isil Davarci ◽  
Sedat Hakimoglu ◽  
Seckin Akkucuk
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Sickle Cell Anaemia Patient

scholarly journals Therapeutic Effects of Camel Milk to Sickle Cell Anaemia Patient

2018 ◽  
Vol 8 (1) ◽  
pp. 1-7
Author(s):  
Y Rukkayya ◽  
S Ibrahim ◽  
M Ladan ◽  
R Wasagu ◽  
N Jiya
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Camel Milk ◽  
Therapeutic Effects ◽  
Sickle Cell Anaemia Patient
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