and T cells, plus B and NK cells as applicable in NMD or PIDMixed chimerism undesirable for DBA and WAS (risk AML, MDS)
Iron overload
Ferritin, transferrin saturation (TS)
Check at day 80–100, 1 year, then annually until normalBe mindful of ferritin as an acute phase reactantConsider HFE gene testing if a family member has been diagnosed with HH or TS >45% in a patient of Northern or Western European ethnicityFA, DC, DBA, hemoglobinopathies require more aggressive management beginning at 6 months
T2*MRI
Gold standard to quantify tissue iron‐burden, annual for DBA until resolved
cGVHD
Screen monthly while on IST, then q3 month x 2 years after IST stops
Screening includes GVHD‐focused history and physical [122] directed at skin, mouth, eyes, GI tract, genitalia, lungs (PFT frequency – see below), LFTs and P‐ROM
Infection and immunity
IgG, IgA, IgM
If history of chronic conjunctivitis, sinopulmonary infections, other recurrent, unusual, or severe infectionsNormalization of IgA, IgM plus increasing trough IgG levels before next IgG‐replacement dose due tracks with humoral recovery – especially in PIDs where replacement is routine
Peripheral blood T‐ and B‐lymphocyte counts
Some centers use arbitrary cut‐offs (CD4 >200 per microliter, CD19 >20 per microliter) for early vaccination beginning at Day 180 [38]
Routine vaccinations
DTaP, IPV, Hib, PCV13, PPSV23, MCV4, Hepatitis A and B, seasonal influenza, MMR, and risk‐based GpB meningococcus and HPV9 [38]. HPV vaccination from age 9–11 years up to age 26 (consider up to age 45 through shared clinical decision‐making in some cases) [123, 124]
Serum viral PCR testing
As clinically indicated and may include serum PCR for CMV, EBV, HHV6, hepatitis B, C, HIV (HIV, HCV if exposed to blood products prior to universal testing)
Pitted RBC score and liver‐spleen scan (periodic)
SCD: ECO prophylaxis until splenic regeneration proven by liver‐spleen scan, pitted RBC score <1.5% and PCV13 and PPSV23 vaccinations completeThalassemia: If splenectomized need ECO prophylaxis life‐long [15]
Ocular
Annual full eye examination
Ask about vision, dry or gritty eyes, diplopia, halosFull eye exam focuses on cataracts (TBI, steroids, infection), retinal exam essential for late CMV staging or other infectionsFA: screen for vision, cataracts. DC: screen for vision, lacrimal duct stenosis, retinal pathology, and cataracts
Hearing
Annual audiology or per audiologist recommendations
If platinum exposure or had >30 Gy cranial radiation: testing advised through age 10 y or 5 y post‐HCT whichever occurs laterIf underlying HCT indication is FA or DC
Oral
Dental examinations and cleanings every 6 months. Educate on routine dental hygiene.
Ask about xerostomia, chewing difficulties, swallowing and speaking.Antimicrobial endocarditis prophylaxis per AHA guidelines
Baseline panorex
Special pediatric risks in the developing mandible, especially below age 6 years, include hypodontia, microdontia, enamel hypoplasia and root malformation
SMN screening Avoid oral tobacco
Minimum annual (see SMN below for more details)Increased risk for oral cancers if received TBI, had oral GVHD, or underlying FA or DC
HPV vaccination
See under “Infection and Immunity” above
Pulmonary
Full PFTs at 3, 6 months, for 5 years annually or until adult (whichever is later)
Spirometry alone is usually feasible age >6 yearsPFTs at cGVHD diagnosis then every 3 months for 1 year, then at least annually while on ISTHigh‐resolution chest CT with inspiration and expiration to confirm air‐trapping and BOS (consider non‐parametric response mapping for age <6 years if unable to do PFTs)DC: lifelong lung symptom screening and annual PFTs because high‐risk for pulmonary fibrosis and pulmonary arteriovenous malformation (see also Cardiovascular)
Cardiovascular or metabolic
Blood pressure each visit, fasting blood glucose (or HbA1c) fasting cholesterol (LDL, HDL) and triglycerides, annually while on IST, then at least every 5 years
Early treatment of CVS risk factors like diabetes hypertension, dyslipidemiaIf patient had cranial TBI then screen neurocognitivelyCardiovascular risk assessment tools: https://ccss.stjude.org/cvcalc
Risk‐based ECG with echocardiogram Thalassemia, SCD: 1‐year check TRJV to rule out pulmonary hypertension and measure pulmonary arterial pressure if TRJV >3 m/s to confirm pulmonary hypertension
Use COG risk assessment tool to determine frequency: http://www.survivorshipguidelines.org/pdf/2018/COG_LTFU_Guidelines_v5.pdfReferral to cardiology for abnormal or declining cardiac function. FA and DBA may also have congenital cardiac anomalies and cardiac iron‐overload (may need T2*MRI)Patients with pulmonary hypertension should be referred to a pulmonologist
Body composition by DEXA
Can also be done by anthropometry (arm‐muscle‐area, arm‐fat‐area)
GI or hepatic
ALT, bilirubin (direct and indirect), alkaline phosphatase, albumin PT/INR, albumin to assess liver synthesis as needed Liver biopsy (selective)
DC: surveillance for liver fibrosis and GI bleeding, ulceration, telangiectasias, varices as clinically indicatedThalassemia or SCD: Consider liver biopsy at 2 years if bridging fibrosis present before HCTConsider liver biopsy at 2 years for patients who had hepatitis B or HCV before HCTRefer patients who have HCV or HBV infection to gastroenterologist or infectious disease specialist for consideration of antiviral therapy
Liver ultrasound
If high‐risk for hepatocellular carcinoma (HBV, HCV, obesity, diabetes, low platelets)
Renal or genitourinary
Blood pressure Serum creatinine, BUN
FA, DC, DBA: follow‐up congenital genitourinary anomalies with a urologist and renal abnormalities and renal function with a nephrologistDC: exam for urethral stenosis
Urine microalbumin: creatinine ratio
If >30 mg/gm and <300 mg/gm, repeat with at least two tests in 3–6 monthsIf >300 mg/gm repeat every 3–6 monthsIf >300 mg/gm on one occasion or if >30 mg on 3 occasions within 6 months and has hypertension, consider treatment with an ACEI or ARB
Renal biopsy
If suspect TMA or unexplained kidney disease.
Musculoskeletal
Serum CPK, aldolase, CRP, ESR
If investigating cGVHD‐associated myositis/myopathy, or following myopathy/myositis while on tapered IST
