International Journal of Medical and Dental Sciences

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Amyloidosis Presenting as Severe Bleeding Diathesis


Affiliations
1 Department of Hematology, All India Institute of Medical Sciences, New Delhi, India
2 Dept of Pathology, Army Hospital Research and Referral, New Delhi, India
 

Bleeding is one of the rare presentations of Amyloidosis. The mechanism behind spontaneous or peri- interventional bleeding in patients of amyloidosis is complex and involves multiple co-existing factors like coagulation factor deficiency, abnormal synthesis of coagulation factors due to advanced liver dysfunction, acquired Von Willebrand disease, platelet dysfunction, amyloid angiopathy and other unknown mechanisms. We present a case of middle aged female, presenting with spontaneous retroperitoneal haemorrhage, on further investigations was found to have systemic amyloidosis and secondary severe factor X deficiency (2.7 % of normal by one stage factor assay method). Factor X deficiency (both inherited and acquired) is known to present with the most severe bleeding phenotype. The management option for such acute spontaneous haemorrhage is limited and mostly supportive in nature. Definitive treatment is directed towards the primary pathology and requires chemotherapy and hematopoietic stem cell transplantation.

Keywords

Amyloidosis, Bleeding, Factor X Deficiency, Platelet Dysfunction.
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  • Amyloidosis Presenting as Severe Bleeding Diathesis

Abstract Views: 229  |  PDF Views: 133

Authors

Preeti Tripathi
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India
H. P. Pati
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India
Seema Tyagi
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India
M. Mahapatra
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India
Ankur Ahuja
Dept of Pathology, Army Hospital Research and Referral, New Delhi, India
Renu Saxena
Department of Hematology, All India Institute of Medical Sciences, New Delhi, India

Abstract


Bleeding is one of the rare presentations of Amyloidosis. The mechanism behind spontaneous or peri- interventional bleeding in patients of amyloidosis is complex and involves multiple co-existing factors like coagulation factor deficiency, abnormal synthesis of coagulation factors due to advanced liver dysfunction, acquired Von Willebrand disease, platelet dysfunction, amyloid angiopathy and other unknown mechanisms. We present a case of middle aged female, presenting with spontaneous retroperitoneal haemorrhage, on further investigations was found to have systemic amyloidosis and secondary severe factor X deficiency (2.7 % of normal by one stage factor assay method). Factor X deficiency (both inherited and acquired) is known to present with the most severe bleeding phenotype. The management option for such acute spontaneous haemorrhage is limited and mostly supportive in nature. Definitive treatment is directed towards the primary pathology and requires chemotherapy and hematopoietic stem cell transplantation.

Keywords


Amyloidosis, Bleeding, Factor X Deficiency, Platelet Dysfunction.

References





DOI: https://doi.org/10.18311/ijmds%2F2017%2F149915