It is believed that there is a definite disease syndrome characterized clinically by fever, marked anemia, purpura, thrombocytopenia, central nervous system involvement, and a progressive fatal course of a few weeks’ duration. Histologically the presence of platelet thrombi in the capillaries, arterioles, and venules is pathognomonic. Only by histologic demonstration of the platelet thrombi can the disease be definitely differentiated from idiopathic thrombocytopenic purpura.
The data of 9 previously reported cases of this rare disease are summarized and 2 additional cases reported.
Diagnosis of the disease probably can be made by bone marrow biopsy or, possibly, by skin biopsy. If a case be diagnosed and the patient is not responding to conservative treatment it is suggested that splenectomy be considered.
Spleens removed for thrombopenic diseases should be examined for platelet thrombi in the possibility that unrecognized cases of the disease have been treated by this procedure. Proper evaluation of splenectomy in this disease might be aided by follow-up study of any cases showing platelet thrombi.
The name "thrombocytic acroangiothrombosis" is suggested for this disease.