INTRODUCTION
Beta thalassemia major is the severe form of beta thalassemia, which is
caused by mutations in beta globin gene, either reduced
(β+) or absent (β0) in hemoglobin A
resulting in unbound α globin chains that accumulate in erythroid
precursors in the bone marrow and in the mature erythrocytes leading to
ineffective erythropoiesis and peripheral hemolysis. About 1.5 % of the
world population are carriers of β thalassemia [1].
In severe disease, extramedullary hematopoiesis (EMH) -which is
production of blood elements outside the bone marrow- occurs mostly in
the liver and the spleen but in rare cases can manifest as bony masses
that behave clinically like tumors, leading to optic nerve atrophy,
spinal cord compression and other clinical scenarios [2,3].
Here we present a case of a 31-year old Pakistani woman with
transfusion-dependent beta thalassemia (TDBT), who complained of low
back pain due to a mass reflecting extra medullary hematopoiesis which
very rarely occurs in the sacrum as there was only one case reported in
the literature and this is the second one.