Open AccessExtramedullary paraspinal hematopoiesis in hereditary spherocytosis
Author(s) -
Pratibha Gogia,
R Goel,
Sandeep Nayar
Publication year - 2008
Publication title -
annals of thoracic medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.639
H-Index - 33
eISSN - 1817-1737
pISSN - 1998-3557
DOI - 10.4103/1817-1737.39640
Subject(s) - hereditary spherocytosis , medicine , extramedullary hematopoiesis , spherocytosis , bone marrow , hemolytic anemia , haematopoiesis , mediastinum , pathology , respiratory distress , anemia , spleen , radiology , splenectomy , stem cell , biology , genetics
Hereditary spherocytosis (HS) is a common inherited hemolytic anemia due to red cell membrane defects. Extramedullary hematopoiesis is a compensatory response to insufficient bone marrow blood cell production. The preferred sites of extramedullary hematopoietic involvement are the spleen, liver and lymph nodes; but in HS, the posterior paravertebral mediastinum is also commonly involved. We report a case of a 50-year-old male who presented to us in respiratory distress and with bilateral paravertebral posterior mediastinal masses, which on trucut biopsy were found to be extra-hematopoietic masses; and the patient was found to have hereditary spherocytosis.