Numerical Abnormalities of the Spleen: Immunohematological and Anatomic Consequences

Congenital asplenia is a rare disease which occurs predominantly in boys and is associated with severe infections that can influence the long-term survival. Polysplenia disorder, defined as the presence of multiple spleens of almost equal volume, is also a rare disorder, but it mainly affects girls and the long-term survival is often possible. The case report presents the diagnostic difficulties of a 2 years old boy who presented leukocytosis and persistent thrombocytosis, together with the methods of diagnosis which led to the identification of the isolated congenital asplenia and the case of a 4 months-old girl who presented for the evaluation of a tuberous hemangioma at tight, which was later diagnosed with polysplenia syndrome, knowing that these two syndromes are associated with plurimalformative organic disorders. In terms of morphogenesis, the splenic abnormalities are the result of an unknown teratogenic factor which occurs in the embryonic development in the first 5 weeks of gestation. These rare affections represent the result of an early unknown teratogenic insult which came about during the embryogenesis and complex investigations are needed in order to exclude pluriorganic affection. The cases presented here are relevant for the identification of the haematological, immunological and imaging methods and aim to evaluate the function of the spleen, the splenic hypofunction being also encountered in other chronic disorders in children.