Amyloidosis Presenting as Severe Bleeding Diathesis

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dc.contributor.authorTripathi, Pen_US
dc.contributor.authorPati, H Pen_US
dc.contributor.authorTyagi, Sen_US
dc.contributor.authorMahapatra, Men_US
dc.contributor.authorAhuja, Aen_US
dc.contributor.authorSaxena, Ren_US
dc.date.accessioned2019-11-04T04:57:56Z
dc.date.available2019-11-04T04:57:56Z
dc.date.issued2017-02
dc.description.abstractBleeding 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.en_US
dc.identifier.affiliationsSenior Resident, DM Hematopathology, Department of Hematology All India Institute of Medical Sciences New Delhi, Indiaen_US
dc.identifier.affiliationsProfessor, Department of Hematology All India Institute of Medical Sciences New Delhi, harappati@yahoo.co.inen_US
dc.identifier.affiliationsProfessor, Department of Hematology All India Institute of Medical Sciences New Delhi, drseematyagi@hotmail.comen_US
dc.identifier.affiliationsProfessor, Department of Hematology All India Institute of Medical Sciences New Delhi, mrmahapatra@hotmail.comen_US
dc.identifier.affiliationsReader, Dept of pathology Army Hospital research and Referral New Delhi, ankurahuja74@gmail.comen_US
dc.identifier.affiliationsProfessor, Department of Hematology All India Institute of Medical Sciences New Delhi,renusaxena@outlook.comen_US
dc.identifier.citationTripathi P, Pati H P, Tyagi S, Mahapatra M, Ahuja A, Saxena R. Amyloidosis Presenting as Severe Bleeding Diathesis. International Journal of Medical and Dental Sciences. 2017 Jul; 6(2): 1535-1538en_US
dc.identifier.issn2454-8952
dc.identifier.issn2320-1118
dc.identifier.placeIndiaen_US
dc.identifier.urihttps://imsear.searo.who.int/handle/123456789/183603
dc.languageenen_US
dc.publisherDr. J.S. Sodhi Memorial Educational Societyen_US
dc.relation.issuenumber2en_US
dc.relation.volume6en_US
dc.source.urihttps://doi.org/10.18311/ijmds/2017/18846en_US
dc.subjectAmyloidosisen_US
dc.subjectBleedingen_US
dc.subjectFactor X Deficiencyen_US
dc.subjectPlatelet Dysfunctionen_US
dc.subjectPulmonologyen_US
dc.titleAmyloidosis Presenting as Severe Bleeding Diathesisen_US
dc.typeJournal Articleen_US
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