Hamostaseologie 2019; 39(S 01): S1-S92
DOI: 10.1055/s-0039-1680253
Poster
P11 Thrombocytopenia and Dysfunction
Georg Thieme Verlag KG Stuttgart · New York

Combined Acquired Alpha- and Delta Storage Pool Disease in Endstage Renal Failure

K. Althaus
1   Center for Clinical Transfusion Medicine, University Hospital of Tuebingen, Tuebingen, Germany
,
S. Bosher
1   Center for Clinical Transfusion Medicine, University Hospital of Tuebingen, Tuebingen, Germany
,
M. Gessner
2   Department of Child Nephrology, University Children's Hospital, Tuebingen, Germany
,
H. Billing
2   Department of Child Nephrology, University Children's Hospital, Tuebingen, Germany
,
S. Nadalin
3   Visceral and Transplant Surgery, Department of General, University of Tuebingen, Tuebingen, Germany
,
T. Bakchoul
1   Center for Clinical Transfusion Medicine, University Hospital of Tuebingen, Tuebingen, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
13 February 2019 (online)

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    Background: Bleeding due to platelet dysfunction can result from congenital or acquired defects. The laboratory approach to identify the pathogeneses of acquired platelet dysfunctions is challenging, especially in pediatric patients. A combination of immunofluorescent microscopy (IFM), flow cytometry (FC) and luminoaggregometry (LA) may help establishing the right diagnosis.

    Case Report: We report on a 20 month old boy with endstage renal failure due to congenital anomalies of kidney and urinary tract. The patient was scheduled for living-kidney transplantation. The family reported that he suffered from several bleeding episodes including bleeding after Tenckhoff-catheter insertion and Hb-relevant bleeding under hemodialysis.

    Results: While routine testing for plasmatic coagulation (aPTT, Quick, FXIIIa and Fibrinogen) revealed normal results, platelet function assays showed pathological findings. In the IFM, reduced and disrupted expression was observed for thrombospondin and P-Selectin. Weak signal was also documented for the delta granule markers Lamp 2 and CD63. Granule mobilization was investigated using LA and FC showing reduced ATP (0.05, normal range 0.8-3.8) and CD63 (fold increase: 0.5, normal > 1.2) release from delta-granula and weak increase in P-Selectin expression after activation with TRAP indicating a combined alpha- and delta granule disorder. Kidney transplantation was performed under tranexamic acid therapy without major bleeding complication. Platelet transfusion was necessary once for double J-stent removal. Laboratory investigations for platelet function three months after kidney transplantation and normalization of renal function showed significant improvement with normal expression of CD62p and CD63 expression in microscopy.

    Conclusions: Renal insufficiency may be associated with combined acquired alpha- and delta storage pool disease leading to hemorrhagic diathesis in uremic patients.


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    No conflict of interest has been declared by the author(s).