№ lp_1_33370
File format: docx
Character count: 8904
File size: 90 KB
Year:
2019
Region / City:
UK
Topic:
Hematology, Sickle Cell Disease, Blood Transfusion
Document Type:
Guideline
Author:
Dr Elizabeth Rhodes, Dr Julia Sikorska
Target Audience:
Medical staff involved in the care of patients with Sickle Cell Disease
Period of Validity:
2019–2022
Approval Date:
14th February 2019
Review Date:
February 2022
Price: 8 / 10 USD
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Version:
1.0
Review date:
May 2026
Adapted from:
RDTC shared care protocol version 1.1 (October 2023, updated March 2024)
Replaces:
GMMMG Shared Care Protocols for Hydroxycarbamide in adult sickle cell disease and adult myeloproliferative disease (17/8/2017, Version 2.1)
Local approval date:
22.02.2024 (local content added)
CRG approval date:
09.04.2024
GMMMG approval date:
09.05.2024
CEGC approval date:
23.05.2024
Intended users:
NHS healthcare professionals
Target population:
Adults aged 18 and over with myeloproliferative disorders or sickle cell disease
Licensed indications:
Chronic myeloid leukaemia; Essential thrombocythaemia; Polycythaemia vera; Sickle-cell disease
Off-label indications:
Primary myelofibrosis; Unclassified myeloproliferative disorders; Psoriasis
Treatment setting:
Shared care between specialist and primary care (transfer after ≥12 weeks when stable)
Initiation responsibility:
Specialist
Prescribing transfer:
Primary care after stabilisation and satisfactory results
Dose range (initial):
Essential thrombocythaemia 15 mg/kg daily; Polycythaemia vera 15–20 mg/kg daily; Sickle-cell disease 15 mg/kg daily with 5 mg/kg steps
Maintenance dose range (sickle-cell disease):
15–30 mg/kg daily (max 35 mg/kg)
Dose adjustment considerations:
Elderly, renal impairment (CrCl ≤60 mL/min start reduced by 50%), myelotoxicity
Baseline investigations:
FBC, U&Es/eGFR or CrCl, LFTs, viral screening, lung disease screening (clinician discretion), vaccination status
Additional sickle-cell baseline:
Reticulocyte count
Monitoring frequency (primary care):
Every 8–12 weeks (FBC, U&Es, LFTs, reticulocyte count for sickle-cell disease)
Ongoing specialist monitoring:
Usually annually; specialist retains responsibility for treatment response and dose changes
Vaccination note:
Shingles vaccine eligibility for ages 50–79 (refer to Green Book)
Shared care protocol type:
Clinical prescribing and monitoring guideline
Year:
2024
Region / city:
Liverpool
Topic:
Sickle cell crisis, Aplastic crisis
Document type:
Informational leaflet
Author:
Not specified
Target audience:
Parents and carers of children with sickle cell disease
Period of validity:
Until review date
Approval date:
Not specified
Date of changes:
April 2024
Note:
Year
Topic:
Sickle Cell Anemia, Medical History, Treatment
Document Type:
Educational Activity
Year:
Current school year
Revision date:
January 2020
Organization:
Duplin County School System
Department:
Student Health Services
Document type:
School emergency health plan
Medical condition:
Sickle Cell Anemia Disorder
Intended location of use:
School setting
Target individuals:
Student with Sickle Cell Anemia, school staff, parents/guardians
Required signatures:
Parent/Guardian, School Nurse
Parental consent included:
Yes
Medical information release authorization:
Included
Distribution:
Teachers, office personnel, bus driver, emergency responders
Filing instruction:
File original in IHR; copies to appropriate staff and Emergency Action Plan Notebook
Year:
Not specified
Country of origin:
Surinam
Institution:
The Hemoglobin University
Authors:
Bento C; Traeger-Synodinos J; Kountouris P; et al.
Thematic area:
Hematology; Hemoglobinopathies; Molecular genetics
Document type:
Case report submission form
Gene:
HBB
Protein variant:
p.Glu7Val
cDNA variant:
c.20A>T
Variant name:
HbS
Database accession:
HbVar ID 226; IthaID 824
Phenotype:
Abnormal Doppler; organ failure due to vaso-occlusive crisis; skeletal dysplasia
Alpha genotype:
-alpha3.7/-alpha3.7
Beta genotype:
betaS/betaS
Age at evaluation:
56 years
Age at diagnosis:
2 months
Sex at birth:
Female
Family history:
Two sisters carriers of HbS
Clinical history:
Hydroxyurea treatment from childhood until 54 years; uncomplicated pregnancy at 23 years; exchange transfusion started at 54 years
Hematological parameters before transfusion:
Reported at age 53 years (Hb, MCV, MCH, RBC, Hct, Ret, RDW)
Iron status:
Ferritin 35 µg/L
Blood smear:
Target cells at normal iron indicative of alpha-thalassemia trait
Biochemical studies:
Capillary electrophoresis (Sebia); HPLC (Trinity PHR)
Molecular studies:
Sanger sequencing of exon 1 of HBB gene
Transfusion status:
Exchange transfusion from age 54 years
Year:
2026
Field:
Genetics / Molecular Biology
Document Type:
Laboratory Exercise
Institution:
University / Educational Laboratory
Target Audience:
Undergraduate Biology Students
Learning Objectives:
Phenotypic analysis, genotypic analysis, molecular mechanisms, inheritance patterns, treatment planning
Methods:
Microscopy, DNA/RNA modeling, case study analysis
Disorder Focus:
Sickle Cell Anemia
End Product:
Lab report with drawings and answers to guided questions
Duration:
One laboratory session
Assessment:
Comparison of phenotypes, explanation of gene expression, transcription modeling
Year:
2026
Organization:
Sickle Cell Society
Document Type:
Job Application Form
Position Applied For:
[To be filled by applicant]
Applicant Information:
Name, Address, Date of Birth, Contact Details
Education:
Schools, Colleges, Universities, Qualifications
Employment History:
Current and Previous Employment Details
Health Information:
Medical History, Days Absent
References:
Two Professional References Required
Declaration:
Applicant Signature and Date
Equal Opportunities Monitoring:
Sex, Ethnic Origin, Disability Status, Recruitment Source
Year:
2026
Organization:
Sickle Cell Society
Document Type:
Job Application Form
Position Applied For:
[to be filled by applicant]
Applicant Name:
[to be filled by applicant]
Date of Birth:
[to be filled by applicant]
Contact Information:
[to be filled by applicant]
Education:
[to be filled by applicant]
Employment History:
[to be filled by applicant]
Health Information:
[to be filled by applicant]
References:
[to be filled by applicant]
Declaration Date:
[to be filled by applicant]
Jurisdiction:
United Kingdom
Organisation:
Sickle Cell Society
Document Type:
Job Application Form
Confidentiality:
Confidential
Country:
United Kingdom
Related Legislation:
Asylum & Immigration Act 1996
Purpose:
Application for employment position within the organisation
Sections Included:
Personal Information; Education; Employment History; Supporting Information; Health Declaration; References; Applicant Declaration; Equal Opportunities Monitoring
Equal Opportunities Data:
Sex; Ethnic Origin; Disability Status
Medical Requirement:
Possible medical examination by an approved doctor prior to employment
Applicant Declaration:
Confirmation of truthfulness of provided information and right to work in the UK
Required Attachments:
Additional sheets for supporting information if necessary
Reference Requirement:
Two referees with professional relationship to the applicant
Note:
Year
Topic:
Medical assessment
Document type:
Questionnaire
Target audience:
Medical professionals
Year:
2026
Organization:
Sickle Cell Society
Document Type:
Job Application Form
Position Applied For:
[Blank field]
Target Audience:
Job applicants
Region:
United Kingdom
Required Documents:
Proof of right to work in the UK
Health Information:
Applicant to declare serious illnesses and absences
References:
Two referees required, one current employer
Date:
[Date field]
Year:
2026
Region / City:
United Kingdom
Subject:
Employment Application
Document Type:
Job Application Form
Organization:
Sickle Cell Society
Position Applied For:
[Not specified]
Applicant Name:
[To be filled by applicant]
Education:
[To be filled by applicant]
Employment History:
[To be filled by applicant]
Health Information:
[To be filled by applicant]
References:
[To be provided by applicant]
Declaration:
Applicant confirms truthfulness of provided information and compliance with UK work eligibility requirements
Year:
2025
Region / City:
Washington, D.C.
Topic:
Drug Development, Clinical Trials, Sickle Cell Disease
Document Type:
Press Release
Organization / Institution:
Sickle Cell Medical Advocacy Inc., Sickle Cell Foundation of Georgia, Inc., Sickle Cell Disease Association of America Inc., Crescent Foundation, SC RED, Sickle Cell 101, Sickle Cell Community Consortium
Author:
Sickle Cell Advocates
Target Audience:
Sickle Cell Disease Community, Medical Researchers, Pharmaceutical Industry
Effective Period:
August 15, 2025
Approval Date:
August 15, 2025
Date of Changes:
N/A
Note:
Contextual Description
Year:
2024
Region / City:
Liverpool
Topic:
Sickle Cell Disease, Respiratory Care
Document Type:
Information Leaflet
Author:
Not specified
Target Audience:
Parents and Carers of Children with Sickle Cell Disease
Effective Period:
Indefinite, subject to review
Approval Date:
June 2028
Modification Date:
Not specified
Year:
2023
Region:
Asia
Topic:
Oncology, Rare Renal Tumors
Document Type:
Case Report
Institution:
Unspecified Medical Institute
Author:
Unspecified
Patient Age:
36
Patient Sex:
Male
Clinical Presentation:
Hematuria, Flank Pain
Diagnosis:
Renal Medullary Carcinoma, SMARCB1 mutation, Sickle Cell Trait
Treatment:
Nephrectomy, Chemo-immunotherapy (Gemcitabine, Carboplatin, Pembrolizumab), Palliative Radiation
Outcome:
Initial complete metabolic response, progression after 1 year, patient deceased
Keywords:
Renal Medullary Carcinoma, Sickle Cell Trait, Clinical Trials, Young Males, Aggressive
Clinical Trial Eligibility:
Recommended
Histopathology:
Loss of INI1 expression, Central renal medulla infiltration, Metastatic disease
Imaging:
PET-CT confirmed renal lesion, lymph node enlargement, pulmonary and bone metastases
Year:
2023
Region / City:
Colchester
Topic:
Haematology, Blood Transfusion, Phlebotomy
Document Type:
User Guide
Organization / Institution:
East Suffolk and North Essex NHS Foundation Trust
Author:
Kathryn Hurst
Target Audience:
Hospital and community users of laboratory services
Effective Period:
From 1st July 2019
Approval Date:
Not specified
Date of Changes:
Not specified
Year:
2022
Region / City:
United Kingdom & Ireland
Theme:
Clinical decision-making, Blood transfusion
Document Type:
Framework
Organization / Institution:
United Kingdom & Ireland Blood Transfusion Network Education Working Group
Author:
Midlands NMA Working Group
Target Audience:
Healthcare professionals, Nurses, Midwives
Period of Validity:
Not specified
Approval Date:
Not specified
Review Date:
2022
Year:
2023
Region / City:
East of England
Topic:
Transfusion management, Haematology
Document type:
Toolkit
Institution:
East of England Regional Transfusion Committee
Author:
Nicola Jones
Target audience:
Haematology Consultants, Transfusion Leads, Hospital Transfusion Committees
Action period:
Not specified
Approval date:
Not specified
Date of amendments:
Not specified
Region:
East of England
Thematic area:
Blood transfusion in obstetrics
Document type:
Resource compilation
Issuing body:
East of England Regional Transfusion Committee
Target audience:
Midwives and obstetric healthcare staff
Healthcare sector:
Maternity and obstetrics
Related organizations:
NHS Blood and Transplant; Serious Hazards of Transfusion; British Committee for Standards in Haematology
Resource formats:
Patient information leaflets, clinical guidelines, posters, factsheets
Access conditions:
Free of charge resources; some materials restricted to contracted NHS Trusts
Time references:
Materials published up to at least 2016
Source type:
Professional and patient educational resources
Year:
2026
Region / City:
Not specified
Topic:
Blood transfusion and specialist treatment requirements
Document Type:
Medical Form
Organization / Institution:
Not specified
Author:
Not specified
Target Audience:
Clinical Team, Transfusion Laboratories
Period of validity:
Not specified
Approval Date:
Not specified
Date of Changes:
Not specified