delta-reservoir disease
Introduction
Introduction to -storage disease -storage pool disease (-SPD) was first described by Weiss in 1969. This disease is a heterogeneous disease with mild bleeding tendency in patients, platelet second phase aggregation wave abnormalities, and platelet compaction. The body can be characterized by varying degrees of reduction. basic knowledge The proportion of sickness: 0.00001% Susceptible people: no specific people Mode of infection: non-infectious complication:
Cause
-storage disease
(1) Causes of the disease
Currently no relevant information
(two) pathogenesis
The basic defect is that the platelet dense particle contents ADP, ATP, serotonin, calcium ions, etc. are all reduced.
Prevention
-preservation disease prevention
The genetic pattern is unknown, but some patients have been found to be autosomal dominant.
Complication
-storage disease complications Complication
No special complications
Symptom
-Storage disease symptoms Common symptoms Nasal bleeding Bleeding bleeding Menstrual volume Multiple skin bleeding spots
The patient's bleeding was mild, with mild to moderate bleeding quality, manifested as skin ecchymosis, bleeding gums, nosebleeds, menorrhagia, and bleeding during childbirth, but generally no joint and gastrointestinal bleeding.
Examine
-storage check
1. The number and morphology of platelets are normal.
2. The bleeding time is prolonged.
3. Platelets are normal for ADP and adrenaline-induced platelet first phase aggregation, while second phase aggregation waves have different degrees of abnormality.
4. The contents of platelet dense granules ATP, ADP, 5-HT, Ca2, etc. were significantly reduced, and the whole platelet ATP/ADP 3.0.
Diagnosis
Diagnosis and identification of -storage disease
The disease may exist alone or as part of other hereditary diseases, such as Hermanky-Pudlak syndrome, Chediak-Higashi syndrome, Wiskott-Aldrich syndrome, etc. The diagnosis of this disease depends mainly on clinical manifestations and laboratory tests.
