PMC8144492_01
Bone marrow biopsy of the patient. (A) Hematoxylin and eosin section at onset showing a hypocellular marrow with trilineage hypoplasia (original magnification x40). (B) Silver staining section at onset showing increased diffuse coarse reticulin (arrow) (original magnification x100). (C) Hematoxylin and eosin section following pharmacological treatment for hyperparathyroidism showing normal trilineage hematopoiesis and complete reversal of fibrosis (original magnification x100).
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F1
A bone marrow biopsy revealed a hypocellular marrow with trilineage hypoplasia (Figure 1A). Increased bone remodeling with fibrosis and increased diffuse coarse reticulin was confirmed by silver staining (Figure 1B). Bone marrow biopsy showed normal trilineage hematopoiesis and a complete reversal of fibrosis (Figure 1C).
Renal biopsy histopathology. (A) Masson staining section showing irregular tubular dilatation with diverticular outpouchings (white arrow). (B) Periodic acid silver staining section showing thickening (white arrow) and thinning (black arrow) of tubular basement membrane and chronic interstitial inflammation.
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F2
His renal biopsy specimen was suggestive of NPHP (Figure 2). Several tubules showed irregular tubular profiles with diverticular outpouchings (Figure 2A). Furthermore, thickening and thinning of the tubular basement membrane and chronic interstitial inflammation were observed (Figure 2B).
Clinical course of the patient. Parathyroid hormone levels (open square) are shown to fall and neutrophils (filled square), erythrocyte counts (open circle) and platelets (filled circle) are shown to rise gradually following HD and pharmacological therapy with Epo, phosphate binder and active vitamin D.
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F3
HPT in a patient with ESRD caused by NPHP. Hemodialysis (HD) and erythropoietin (dosage 75 U/kg/dose x 3 times/week) were initiated, and combined therapy with a phosphate binder and an active vitamin D agent achieved reduction of PTH levels, along with improvement of pancytopenia (Figure 3).