Paraspinal extramedullary hematopoiesis in alpha-thalassemia with concurrent monoclonal gammopathy of undetermined significance: an uncommon case report

Abstract

Extramedullary hematopoiesis (EMH) functions as a compensatory phenomenon in cases of insufficient bone marrow function and refers to the hematopoiesis that takes place outside the medulla of the bone. It is usually associated with various hematologic disorders, including thalassemia.1–4 Hemoglobin H disease is a type of a-thalassemia consisting of the loss of 3 genes (--/-a) and is often manifested as a mild anemia with thalassemic morphological changes and the existence of erythrocyte inclusions. Hemoglobin H is a tetramer composed of four beta globin chains and a great decrease in alpha chain availability.5 In this hemolytic disorder, splenomegaly may occur, as well as mandibular and bone marrow hyperplasia and pathological fractures. Reported herein is the case of a 64-year-old male who suddenly developed a mild anemia, splenomegaly, monoclonal hypergammaglobulinemia and a paraspinal mass and was admitted to our hospital for further investigation. The patient was eventually diagnosed with hemoglobin H disease with concurrent MGUS. The present case report discusses the diagnostic methods, problems and treatment strategies that were applied and seeks to further examine this uncommon clinical manifestation. Case presentation