Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Extramedullary hematopoiesis
- Radiologic Findings
- The PA radiograph showed a smoothly outlined mass posterior to the heart on the both side with paravertebral stripes obliteration. It also demonstrated marked hepatic and splenic enlargement (Fig. 1). The chest CT revealed homogeneously enhancing bilateral paraspinal masses from T8 to T10 vertebral level (Fig. 2, 3). There were multiple GB stones with hepatosplenomegaly (Fig. 4, 5). She was a hereditary spherocytosis patient and underwent splenectomy.
- Brief Review
- Extramedullary hematopoiesis (EMH) is the proliferation of hematopoietic cells outside of the bone marrow in response to production of too few blood cells to satisfy the body’s demand. This insufficient production is caused by either bone marrow replacement disease (myelofibrosis or chronic myelogenous leukemia) or hemolytic anemia (thalassemia, sickle cell anemia, or hereditary spherocytosis). The liver, spleen, kidney, lymph nodes, and posterior mediastinum are the most common sites of EMH.
Intrathoracic EMH may manifest as a paraosseous mass, interstitial pulmonary abnormality, pleural mass, or hemothorax, either alone or in combination. The paraspinal masses and rib expansion are the most common manifestation of intrathoracic EMH. The paraspinal masses have to be differentiated from lymphadenopathy and neurogenic tumors. Cases of EMH involving the pulmonary interstitium mimick an inflammatory or neoplastic diffuse interstitial process and have occasionally resulted in cardiopulmonary insufficiency. EMH usually regresses or disappears after treatment with blood transfusion and hydroxyurea, splenectomy in cases of spherocytosis, or radiation therapy.
In conclusion, the combination of homogeneously enhancing bilateral paraspinal masses with hepatosplenomegaly and gallstones, which occur frequently in hereditary spherocytosis patient makes EMH the most likely diagnosis.
- References
- 1.Yahya M. Berkmen and Benjamin A. Zalta. Case 126: Extramedullary Hematopoiesis Radiology 2007; 245: 905-908
2.Christos S. Georgiades, Edward G. Neyman, Isaac R. Francis, Michael B. Sneider, and Elliot K. Fishman Typical and Atypical Presentations of Extramedullary Hemopoiesis Am. J. Roentgenol. 2002; 179: 1239 - 1243
- Keywords
- Paravertebral, Multiple organ,