Intrathoracic extramedullary hematopoiesis arising in marble bone disease

Abstract

We report a man with intrathoracic extramedullary hematopoiesis accompanied by marble bone disease who underwent thoracoscopic resection safely and definitively, followed by a radionuclide scan. Although intrathoracic extramedullary hematopoiesis is almost always accompanied by hemolytic anemia, this man's hematological data normalized. A consensus on the tumor genesis mechanism has yet to be reached, but the existence of marble bone disease in our case throws new light on the genetic mechanism in intrathoracic extramedullary hematopoiesis.

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

“…Although a diagnosis using CT‐guided FNA had been previously made in this patient, haemorrhage following biopsy is a potential risk 8 . The classic radiographic findings of osteopetrosis along with the CT findings of bilateral paravertebral soft tissue masses should be sufficient to suggest the diagnosis, which may be non‐invasively confirmed using nuclear medicine techniques.…”

“…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 .…”

Synovial tumefactive extramedullary hematopoiesis associated to polycythemia vera

“…It is thought that in these disorders, the stroma of the bone marrow reacts and thus generates reticulin and collagen fibrosis, angiogenesis, and osteosclerosis as a result of the stimulus produced by the diverse cytokines locally secreted by megakaryocytes, monocytes, and platelets [11,15]. Other entities producing medullar fibrosis include certain lymphoid and myeloid hemopathies, not only neoplastic but also nonneoplastic, metastatic carcinomas, chronic granulomatous infections, connectivepathies, renal osteodystrophy, and some treatments (colony stimulating factor) [3,[8][9][10]13]. In general, the resulting extramedullary hematopoiesis affects those organs containing a reticulum endothelial system (spleen, liver, and lymph nodes), although any organ or structure may occasionally be affected, in which case there is a certain predisposition for the serous membranes.…”