Hyposplenism (excluding that induced by medical or surgical intervention) occurs in a wide range of conditions.

In some disorders such as sickle cell disease, gluten-induced enteropathy (coeliac syndrome) and dermatitis herpetiformis, hyposplenism occurs frequently; it is seen less frequently in Crohn's disease, ulcerative colitis and essential thrombocy-thaemia, and it occurs only occasionally in the other conditions listed in Table 21.2. Congenital absence of the spleen is rare and may be associated with organ transposition and with severe malformations of the heart and lungs.

After the age of 65 -70 years, there is evidence of a decrease in splenic function. In old age, there is a rapid decrease in the weight of the spleen, together with increasing atherosclerotic vascular obstruction and fibrosis.

Patients with functional hyposplenism have impaired immunity to blood-borne bacterial and protozoal infections, and persistent thrombocytosis. Management is similar to that required post-splenectomy. It includes prophylactic antibiotics and vaccines (see above) and advice to the patients to seek

Figure 21.4 Demonstration of residual splenunculus by a scan of heat-damaged isotope-labelled red cells: (a) anterior view; (b) posterior view. Uptake is predominantly in the liver.

medical attention immediately in the event of illness or fever. Antiplatelet therapy is advisable when the platelet count is high.

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