Congenital dyserythropoietic anaemias

In addition to the non-specific phenomenon of dyserythro-poiesis described above, there is a group of disorders of variable severity with recessive inheritance, known as congenital dyserythropoietic anaemias (CDA). They are characterized by chronic anaemia with a relatively low reticulocyte count, jaundice and haemosiderosis. Peripheral blood films show anisocytosis, poikilocytosis and irregularly crenated and contracted cells. The marrow shows one of three patterns:

Type I. Megaloblastic changes, macrocytosis, internuclear chromatin bridges, but binuclearity not prominent; proerythroblasts and basophilic erythroblasts especially affected (Figure 13.5). Type II. Binuclearity, especially of late erythroblasts, multinucle-arity, pluripolar mitoses, karyorrhexis (Figure 13.6).

Internuclear Chromatin Bridging
Figure 13.5 CDA type I. Bone marrow aspirate showing internucleur bridging in normoblasts.
Congenital Dyserythropoietic Anemias
Figure 13.6 CDA type II (HEMPAS). Bone marrow aspirate showing typical multinuclearity.
Internuclear Chromatin Bridging
Figure 13.7 CDA type III. Giant multinucleated erythroblast from the marrow.

Type III. Multinuclearity with up to 12 nuclei, gigantoblasts, macrocytosis (Figure 13.7).

There is an overlap between types I and II and there are variants of each type. There are also a number of reports of congenital ineffective erythropoiesis, which do not fit into any of the three categories given.

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