Guideline:Sickle cell disease

At Diagnosis (prior to first transfusion)
 * 1) Complete Blood Count and Red cell morphology
 * 2) Reticulocyte count
 * 3) Number of nucleated re cells per 100 leukocytes in the peripheral blood
 * 4) Hb pattem analysis (Electophoresis or HPLC) - repeat HbF levels at intervals in patients taking an HbF modifying substance
 * 5) Extended Red Cell Antigen typing, including at least C, c, E, e, and kell.
 * 6) Complete genotype - α and β globin mutations
 * 7) G-6PD
 * 8) HbsAg, Anti-Hbc, Anti-HBs, Anti-HCV, Anti-HIV, Anti-HAV, Anti-CMV, Anti-EBV; Toxoplasma, Rugeolla, Coxcackie, Parvo Β19, H.Pylori, Yersinia (seroprevalence maybe high after multiple transfusions)
 * 9) Serum Homocysteine - in case of increased values repeat 2 months after initiating treatment
 * 10) Lipoprotein α

Before Each Transfusion
 * 1) Complete blood count
 * 2) Complete cross-match
 * 3) Screen for new antibodies
 * 4) Number of nucleated red blood cells per 100 leukocytes in peripheral blood in splenectomised patients
 * 5) Indirect Coomb's test before and 8-15 days after each transfusion. Every month during pregnancy

Every 3-4 months
 * 1) Routine biochemical indices including:                                                         liver enzymes, s. bilirubin, LDH, Proteins (Total, Albumin & globulin), γ-GT, glucose, urea, creatinine, uric acid, cholesterol, HDL & LDL, triglycerides, Calcium, Phosphorus, Sodium, Magnesium, Zinc and S. Iron and TIBC.                                                     Repeat at each admission for vaso-occlusive crisis
 * 2) Complete Blood Count - in steady state and witii infections or complications

Every 6 months
 * 1) Prothrombin time, PTT, INR, Fibrinogen, D-DIMERS, Fibrin Degratation Products (FDP), - and at each admission for vasculo-occlusive crisis and complications.
 * 2) Protein C, Protein S, APCR on clinical indications.
 * 3) Factor VIII
 * 4) Lupus anticoagulant - when prolonged PTT or upon indication
 * 5) Direct Coombs (more often in auto and/or allo-immunised patients)
 * 6) Indirect Coombs (every month during pregnancy)
 * 7) S. Ferritin
 * 8) Endocrinology tests - Prolactin, FSH, LH, estradiol, progesterone, testosterone, ACTH, cortisol, BGP (osteocalcin), iPTH, CEA), FT3, FT4, TSH (if high then Anti-microsormal antibodies and anti-thyroglobulin antibodies)
 * 9) a-fetoprotein
 * 10) CA-15-3, CA 125 in adult women
 * 11) CA-19
 * 12) PSA-in men over 50 years old
 * 13) Erythropoietin

Every 1-3 years or on clinical indication

1) 2- Hour Urine Collection (fasting)

Hydroxyproline, pyridium cross-hnks and creatinine and calcium (especially for patients with renal calculi and to investigate bone turnover and parathyroid disorders)

24-hour urine collection For urea creatinine, calcium or protein (rarely needed)

2) Autoantibodies e.g. ΑΜΑ (anti-mitochondrial), ASMA (anti-smooth muscle), APCA (anti-parietal cell), ANCA (anti-neutrophil cytoplasmic). Such auto-antibodies are commonly found among patients with chronic HCV1 infection and in chronic GVHD2. Also anti-cardiolipin antibodies and anti-β2-GPl

ΕΝΑ (extractable nuclear antigens) -

3) Immunology - C3, C4, CIC (Circulating Immune Complex), Immunoglobulins (quantitative) and Immune precipitation (upon clinical indication)

4) Vitamin Β12 and Folic Acid - rarely an indication

5) SpO2 measurement - upon indication

6) Oral glucose tolerance test - 2-5 samples - rarely, on indication

7) Hblc

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1 Ghonaim Μ et. al. Autoantibodies in chronic liver disease, Egypt J. Immunol. 2005,12(2): 101-111

2 Patriarca F et.al. Autoantibodies after allogeneic stem cell transplantation, Exp. Hematol, 2006,34(3):389-96

Cardiac Monitoring


 * 1) ECG -1-2 times a year: more often if cardiac rhythm abnormalities have been detected
 * 2) Echocardiography - 1-2 times per year
 * 3) Holter - on indication of arrhythmias
 * 4) Cardiac MRI-Every 1-2 years
 * 5) MRA - upon indication

Radiography


 * 1) Chest X-ray every 1-3 years
 * 2) Bone age - if growth retardation suspected
 * 3) Hip, Lumbar spine and sinuses upon clinical indication
 * 4) DEXA - this is low dose radiation and can be performed annually. However in children or anyone less than 20 years, use the criteria set by the Intemational Society for Clinical Densitometry (www.iscd.org) using Z-scores instead of T-scores etc. For adults use the WHO criteria.

Ultra Sound


 * 1) Abdomen-every 2-5 years and upon clinical indication
 * 2) Breast in adult women- every 6 months - 2 years
 * 3) Thyroid - upon indication

Radioiabelled Scan Of thyroid, bones or parathyroid -only on clinical indication

CT


 * 1) Abdomen and retro-peritoneum - every 2-3 years in patients not on regular transflisons
 * 2) Spine - upon indication

MRI


 * 1) Heart and liver - every 2-3 years
 * 2) Pituitary - every 2-4 years
 * 3) Pancreas - ?