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Spleen and Thymus D S O’Briain, March 2009 Spleen • 150 g, receives 10% of cardiac output • Splenic artery branches surrounded by lymphoid tissue (white pulp); end in cords and sinuses of red pulp Functions – – – – Haemopoietic Reservoir Immunological Filtration Splenomegaly • Infections • Collagen disease • Rheumatoid arthritis, Felty syndrome, SLE • Haemodynamic • Congestive failure, portal hypertension, portal vein thrombosis • Lympho- and myelo- proliferative diseases • Haemolytic anaemias • Miscellaneous • cysts, neoplasms, storage disease, amyloid Hypersplenism • Cytopenia • isolated (red cell, white cell, platelet); combined; pancytopenia • Splenomegaly • Normal or increased marrow production • Reversal of cytopenia following splenectomy Splenic atrophy or absence • Congenital • Traumatic (including surgery) • Vascular • sickle cell, embolus, thrombus • GI disease • Coeliac disease, inflammatory bowel disease • Miscellaneous • radiation, collagen disease, amyloid Hyposplenism • Abnormal peripheral blood (Howell Jolly bodies, target cells, pitted red cells) • Immune defects – Encapsulated bacteria (Str pneumoniae, H influenza); may require vaccination, antibiotic cover Splenic Lesions • • • • • • • • Rupture: immediate or delayed; splenosis Fibrocongestive changes Infarcts Infections, granulomas Immune thrombocytopenia Leukaemia, CML, hairy cell leukaemia, myelosclerosis Lymphomas Cysts, tumours Thymus • • • • Bilobed organ 50 g at puberty, 10 g in age Epithelial cells and lymphocytes Absent in some immune deficiency states Enlargement: childhood, myasthenia gravis, autoimmune diseases • Tumours: • thymic carcinoid • germ cell tumours • lymphomas (Hodgkin, lymphoblastic lymphoma, large cell lymphoma) • thymoma Thymoma • Adults, mixture of epithelial cells and lymphocytes • Most (80%) encapsulated and histologically benign (benign thymoma) • Some (10%) similar histology but locally invasive (invasive thymoma; 75% 10-year survival) • Malignant thymoma (10%); histologically malignant, usually invasive, 25% 5-year survival – One-third of thymomas have myasthenia gravis; – hypogammaglobulinemia, erythroid hypoplasia may occur Myastenia gravis • Myopathy with antibodies to • acetylcholine receptor molecule (and a similar thymic epitope) • skeletal muscle cells (cells which also occur in the thymus) • Other autoimmune disease in 10% • Most (65%) have lymphoid follicular hyperplasia (these respond best to thymectomy) • Thymoma in 10% and normal thymus in 25%