PMC6983319_01
a AP spine film demonstrating severe scoliosis and spinal fusion for correction. b AP film of lower extremities with intramedullary rods in the right femur and bilateral tibiae to correct deformities. Note the typical radiographic findings of fibrous dysplasia (FD), including ground glass appearance and thin cortices (black arrow). c Bone marrow obtained as surgical waste 2 years prior to pancytopenia. d Areas of fibrous tissue (FT), woven bone (WB), and osteoid (o), Masson stain. e Evidence of normal hematopoiesis. f Iliac crest bone marrow biopsy at the time of presentation with pancytopenia, notable for hypocellularity, Masson stain, x 400. g Liver biopsy, H & E stain, erythroid focus (black arrow) demonstrating extramedullary hematopoiesis. h Liver biopsy, anti-CD71 immunostain (black arrow) confirms erythroid precursors. i Spleen, postsplenectomy, H & E stain. Encircled are erythroid precursors, indicating extramedullary hematopoiesis
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F1
She underwent multiple orthopedic surgeries, including spinal fusion and intramedullary rod placement in both femora and tibiae (Fig. 1a, b). On histology, bone marrow obtained from the femur during an osteotomy as surgical waste 2 years prior revealed multiple areas of normal hematopoietic marrow (Fig. 1c, d, e). In contrast, the results of an iliac crest bone marrow biopsy at presentation was hypocellular with only 10% cellularity (Fig. 1f). Liver biopsy revealed significant trilineage EMH (Fig. 1g, h). Postsurgical pathology of the spleen confirmed the presence of EMH (Fig. 1i).
Hematology laboratory trend. The patient was noted to have normal values of leukocytes, hemoglobin, and platelets prior to her acute presentation with pancytopenia. She required multiple red blood cell transfusions (double headed arrow) between days 750 and 1100. She underwent splenectomy (black arrow), followed by normalization of her counts. Please note that the patient received packed red blood cell transfusion right before splenectomy in preparation for the surgery; hence the acute increase in her hemoglobin level before splenectomy
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F2
Following splenectomy, all cell counts started to recover (Fig.2).