Table 6.

Poikiloderma with Neutropenia: Recommended Surveillance

System/ConcernEvaluationFrequency
General Exam by physician familiar w/PNAnnually
Skin Assessment for calcinosis cutis, cellulitis, palmar/plantar hyperkeratosis & evidence of non-healing ulcersAnnually or as needed
Exam by dermatologist to assess for skin cancerAnnually beginning at age 10 yrs
Dental Dental cleaning & eval for gingivitis/cariesEvery 3-6 mos
Pulmonology Eval by pulmonologistAnnually in persons w/bronchiectasis, chronic cough, &/or reactive airway disease
Hematology CBC w/differential & platelet count for evidence of anemia &/or thrombocytopenia (signs of possible myelodysplastic syndrome)Annually
Eval by hematologist/oncologist for consideration of bone marrow examAs needed
Endocrine Assessment of growth & pubertal development in childrenAt each visit throughout childhood
Consider DXA scan to eval for low bone density.As needed in adults
Development Assessment for developmental & educational progressAt each visit throughout childhood

CBC = complete blood count; DXA = dual-energy x-ray absorptiometry; PN = poikiloderma with neutropenia

From: Poikiloderma with Neutropenia

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