| The association of thrombocytopenia, macrothrombocytopathia, nephritis and deafness is rare. Reported here is a new case of this triple association. The clinical course, the nephropathologic findings and the bilateral neurologic hearing loss were similar to those already reported, with a slowly progressive impairment of renal function accompanied by a persistent proteinuria. The platelet diameters were increased. These macroplatelets contained granules of normal structure but with an irregular distribution in the cytoplasm. In other areas the cytoplasm was rich in surface connected system. The survival of these platelets and their contraction were normal. Their aggregation and excretion in response to collagen, adenosine diphosphate and thrombin, and the values of platelet factor 3 activity were all decreased. The degranulation defect, also present, was observed in the absence of a decrease in intracellular cyclic adenosine 5'-monophosphate (AMP) suggesting a relationship between these two findings. ( info) |
| A case of type IV ehlers-danlos syndrome with a partial platelet delta-storage pool disease is reported. The diagnosis of Ehlers-Danlos was clinical. The platelet-dense granule deficiency was determined by ultrastructural platelet morphology. Dense bodies were decreased in number, and most showed loss or fragmentation of electron-dense material. Aggregation studies revealed a retarded response to ristocetin and arachidonic acid, which was corrected with desmopressin acetate-DDAVP. ( info) |
| Percutaneous transfemoral arterial balloon occlusion or gelatin sponge embolization of the splenic artery or both were carried out in three high-risk patients with hepatic cirrhosis to reduce splenic hyperfunction and improve severe thrombocytopenia. Although this maneuver raised peripheral platelet counts in each patient, in one patient left upper quadrant pain with splinting of the lower chest cage led to hypostatic lower lobe pneumonia, while in another septic splentitis with gas-forming organisms necessitated splecectomy. Transfemoral occlusion of the splenic artery, although an effective, noninvasive approach to control splenic hyperfunction, has at the same time potential dangers that should be viewed with extreme caution in therapeutic application. ( info) |