Extramedullary Hematopoiesis Assessment in a Patient With Osteopetrosis

Enhanced bone formation in lipodystrophic PPARγhyp/hyp mice relocates haematopoiesis to the spleen

“…The haematopoietic defect is offset by extramedullary haematopoiesis in the PPARγ hyp/hyp spleen. Extramedullary haematopoiesis is also reported in humans, in which bone marrow space is reduced due to osteopetrosis (Freedman & Saunders 1981; Caluser et al , 1995).…”

“…Extramedullary haematopoiesis has previously been described in osteopetrotic mice (Tagaya et al , 2000) and humans (Freedman & Saunders 1981; Caluser et al , 1995). Our study suggests that an enlarged spleen observed in other lipodystrophic mouse models and patients with lipodystrophy (Moitra et al , 1998; Shimomura et al , 1998; Misra & Garg, 2003) cannot be attributed to infiltrative processes or steatotic liver dysfunction and portal hypertension alone, but also to bone abnormalities.…”

Computed tomography of intrathoracic extramedullary haematopoiesis occurring as a complication of osteopetrosis

“…The liver, spleen and lymph nodes are the usual sites of extramedullary haematopoiesis, which can also occur in the adrenals, kidneys, gastrointestinal tract, testis, spinal cord and thorax. Despite the extensive loss of marrow cavity associated with osteopetrosis, extramedullary haematopoiesis appears to be an uncommon complication of this condition 2,4 , 8,9 .…”