Hypersplenism inWilson's disease

SUMMARY Thirteen patients withWilson's disease were compared withsevencirrhotic and13 normal controls todefine better thehaematological abnormalities inthis condition. Hypersplenism (anaemia, leukopenia, thrombocytopenia, andreduced redcell survival) commonly occurred in patients withbothWilson's disease andcirrhosis. Theseabnormalities correlated withsplenic enlargement. Despite reduced haematocrits, redcell mass was greater inthese twogroupsthanin normal controls. Plasma volumeandthebodyhaematocrit/peripheral haematocrit ratios were also greater inpatients withWilson's disease andcirrhosis. Increased splenic sequestration of51Cr-tagged redblood cells was notdemonstrated inany subjects. Thehypersplenism inpatients withWilson's disease issimilar tothat foundinpatients withcirrhosis fromother causes. Splenomegaly andpancytopenia are common in Wilson's disease, a rarehereditary illness withan excessive burden ofcopperinthebody.Patients with Wilson's disease havecirrhosis oftheliver, a condition inwhichhypersplenism isfrequent. Haemolytic anaemia hasalsobeenreported in Wilson's disease (Mclntyre, Clink, Levi, Cumings, andSherlock, 1967). To gainfurther insight intotheassociation of pancytopenia andsplenomegaly, routine haematological tests, redcell mass andsurvival with5"Crtagged redbloodcells, andplasmavolumewith "2'I-tagged albumin were performed on 13patients withWilson's disease, andthefindings were compared withthose ofsevenpatients withcirrhosis of theliver and13normal controls. External monitoring wasperformed overthespleen after theinjection of 51Cr-tagged RBCstodetect anysplenic sequestration ofRBCs.Results ofallthese studies werecorrelated withsplenic size asestimated bypalpation andpercussion.