Congenital dyserythropoietic anemia with ultrastructural features of type I and II.

Blut
J SchupplerB Speck

Abstract

The autopsy and electron microscopic findings in a pair of brothers with congenital dyserythropoietic anemia (CDA) are presented. In both patients autopsy revealed severe secondary hemochromatosis, including cirrhosis of the liver and fatal heart involvement. According to current ultrastructural criteria, a mixture of CDA type I (interchromatin bridges, wide euchromatin-cytoplasmic connections) and of type II (marginal cisternae, nuclear protrusions, multinuclearity, karyorrhexis) was found in erythroblasts of one patient. In the second patient electron microscopy of bone marrow stored in formalin for several years allowed the diagnosis of CDA with marginal cisternae in retrospect. These findings illustrate the usefulness of electron microscopy for the diagnosis of CDA in unsolved cases of chronic ineffective erythropoiesis, even from formalin fixed material. For subtyping CDA into type I and II, however, other than morphological parameters should be used for definition. In the clinical management splenectomy and a drastic phlebotomy programme have been found favourable.

References

May 27, 1974·Virchows Archiv. A, Pathological Anatomy and Histology·P KerkhovenG Hug
Jul 1, 1972·British Journal of Haematology·S M LewisC S Pitcher
Jul 1, 1972·British Journal of Haematology·R GoudsmitE Reynierse
Apr 1, 1962·The Journal of Cell Biology·R C BUCK, J M TISDALE

❮ Previous
Next ❯

Citations

Jan 1, 1978·Progress in Histochemistry and Cytochemistry·A F Morselt
Jan 1, 1996·American Journal of Hematology·P W Marks, A J Mitus
Jan 1, 1984·Graefe's Archive for Clinical and Experimental Ophthalmology = Albrecht Von Graefes Archiv Für Klinische Und Experimentelle Ophthalmologie·J DavidowitzG M Breinin
Nov 1, 1975·Blut·H Heimpel

❮ Previous
Next ❯

Related Concepts

Related Feeds

Anemia

Anemia develops when your blood lacks enough healthy red blood cells. Anemia of inflammation (AI, also called anemia of chronic disease) is a common, typically normocytic, normochromic anemia that is caused by an underlying inflammatory disease. Here is the latest research on anemia.