Abstract
Systemic amyloidosis with hepatic involvement is a rare disorder, which is characterized by the deposits of amyloid fibrils in the liver. The prognosis is poor and the median survival is 13 months. Bleeding problems resulting from coagulopathy frequently complicates systemic amyloidosis. We present two patients with a severe factor X deficiency and hepatomegaly as the presenting abnormalities of systemic amyloidosis. One of the patients was treated with high dose melphalan chemotherapy and autologous stem cell reinfusion, resulting in a normalization of the liver enzyme tests and the factor X level. The diagnosis and treatment of systemic amyloidosis with hepatic involvement and the management of the multifactorial coagulopathy in these cases is discussed.
MeSH terms
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Alkaline Phosphatase / blood
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Amyloidosis / complications*
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Amyloidosis / drug therapy
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Amyloidosis / metabolism
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Amyloidosis / therapy*
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Antineoplastic Combined Chemotherapy Protocols / therapeutic use
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Blood Coagulation Disorders / etiology
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Blood Coagulation Tests
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Dexamethasone / administration & dosage
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Doxorubicin / administration & dosage
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Factor X / metabolism
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Factor X Deficiency / blood
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Factor X Deficiency / drug therapy
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Factor X Deficiency / etiology
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Factor X Deficiency / therapy*
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Hepatomegaly / blood
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Hepatomegaly / etiology
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Hepatomegaly / therapy
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Humans
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Male
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Melphalan / administration & dosage*
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Melphalan / therapeutic use*
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Middle Aged
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Stem Cell Transplantation / methods*
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Transplantation, Autologous
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Treatment Outcome
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gamma-Glutamyltransferase / blood
Substances
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Dexamethasone
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Doxorubicin
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Factor X
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gamma-Glutamyltransferase
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Alkaline Phosphatase
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Melphalan