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SUMMARY The alkaline phosphatase-antialkaline phosphatase (APAAP)

Four patients with congenital dyserythropoiesis characterized by marked macrocytosis, little or no anaemia, and vitamin B12- and folate-independent megaloblastic erythropoiesis are reported. Their erythroblasts also showed various dysplastic changes but not those diagnostic for congenital dyserythropoietic anaemia (CDA) types I or III. The haematological features of the four patients, who included two siblings, resemble those of a previously reported patient and together these patients form a recognizable subgroup within those cases of CDA not belonging to CDA types I-III. In two of the cases studied, and possibly a third, the inheritance was as an autosomal recessive character.

A peripheral blood film reflecting hyposplenism is an important finding. Not only does it indicate the risk to the patient of fulminant sepsis requiring preventative action but it may also direct further investigations towards an underlying medical condition. The occurrence of hyposplenism in systemic amyloidosis has been described. We present three cases which demonstrate this association and highlight the value of the peripheral blood film.