Bone marrow production of the cellular elements of blood may be supplemented by foci of extramedullary hematopoiesis. This compensatory mechanism takes place when curtailed production or increased demand interferes with normal hematopoiesis. Extramedullary hematopoiesis has occurred in anemias associated with thalassemia, pernicious anemia, hereditary spherocytosis, osteosclerosis where the bone marrow has been replaced with tumor cells (i.e., carcinoma, Hodgkin's disease, lymphoma, and leukemia), hyperparathyroidism, and erythremia (9). Extramedullary hematopoiesis has been reported in the liver, spleen, hilus of kidney, thymus, adrenal, appendix, lymph node, dura mater, broad ligament, prostate, sciatic nerve, breast, and in the paraspinal region of the thorax (9). Nine cases of intrathoracic extramedullary hematopoiesis have been proved at autopsy (1–5, 7, 8, 10, 12) and 3 at biopsy (6, 9, 11). This is the tenth autopsy-proved case and the first in a patient with sickle-cell anemia. Case Report A 31-year-old Negro male was admitted to Temple University Hospital on Oct. 3, 1963, with a chief complaint of increasing hoarseness during the three months prior to admission. He had homozygous sickle-cell anemia. The review of symptoms disclosed an increasing dyspnea for the past several years associated with intermittent ankle edema. Physical examination showed an underdeveloped Negro male in no acute distress. The sclerae were not icteric. A positive hepatojugular reflex was elicited. The heart was enlarged. A Grade IV pansystolic murmur was heard. The pertinent laboratory findings were an initial hemoglobin of 3.8 g per 100 ml, hematocrit 12 per cent, and a white count of 7,500 with a normal differential. There were 151 nucleated red blood cells per 100 white cells. The thrombocyte count was 81,000. The red blood cell count was 1.3 million, and the peripheral smear showed target cells, a moderate number of sickle cells, and Howell-Jolly bodies. The blood urea nitrogen was 43 mg per 100 ml. Hemoglobin rose to 8.1 g per 100 ml following 3 units of blood, but dropped again to 4.2 g within a short time. Bone marrow aspiration revealed an erythroid hyperplasia with mild maturation arrest and sickle-cell phenomenon. The paper electrophoresis of a blood sample showed a 97.8 per cent hemoglobin S and 2.2 per cent hemoglobin A2 (fetal hemoglobin). Ten per cent of the fetal hemoglobin was alkali-denaturated. At cardiac catheterization, no intracardiac defects were found. The murmur, therefore, was attributed to the anemia. Roentgen examination of the chest revealed generalized cardiomegaly with no specific chamber enlargement. There was a smooth, right, posterior paraspinal soft-tissue density from T-8 to T-11 with a small extension to the left of the spine (Fig. 1), but without calcification or evidence of an associated osseous lesion. The density did not change in the lateral decubitus position.