SPLEEN - Hereditary spherocytosis

Description of Specimen
The specimen is a very large spleen; the normal outline is preserved; there is some artefactual tearing of the capsule on the anterior surface. The cut surface at the upper pole shows a rim of darker tissue; this is an artefact of fixation.
The patient was 15 years old when the spleen was removed. At birth she was found to be anaemic and the diagnosis of hereditary spherocytosis was made; a top-up transfusion was given at five weeks. Her haemoglobin had fallen from 16 to 6 grams. At the age of four her haemoglobin was 12 grams; the spleen was firm and palpable some 3 cm below the left costal margin. At the age of 15 an elective cholecystectomy and splenectomy were performed; the gallbladder was thickened and contained numerous pigment stones. The spleen weighed 1074 grams. The child's father had suffered spherocytosis and had his spleen removed at the age of 11 years.