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C were iron overload may accelerate cirrhosis. Patients with thalassemia, SCD, DBA, HH require aggressive evaluation and management beginning ideally 6 months post‐HCT.
Table 8.1 LTFU recommendations for survivors
| Organ System or Late Effect | Recommendations: | Qualifiers and other comments: |
|---|---|---|
| Engraftment | Annual CBC, MCV ± reticulocytes | More frequently, if persistent or progressive abnormalities that may be due to medications, infection, GVHD, relapse |
| Flow cytometry‐sorted lineage specific donor chimerisms | Minimum of annual in NMDs where long‐term graft stability is unclear (especially after NMT or RIC)SCD: check myeloid chimerism q3–6 months for 2 years, then yearly with HbS levelThalassemia: if microcytic anemia recurs check chimerism as for SCDUsually unnecessary in malignant diseases unless as a baseline before DLIUsually check chimerism in granulocytes 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 |
