Abstract
The clinical presentation of AL amyloidosis is highly variable. In this series, we describe five cases of AL amyloidosis with vertebral compression fractures as initial presentation. All five patients had evidence of bone marrow replacement on magnetic resonance imaging and bone marrow biopsies demonstrating diffuse interstitial amyloid deposition. Hepatomegaly and elevated liver enzymes, consistent with liver involvement with amyloidosis, were also seen in each case. All five patients responded well to anti-plasma cell chemotherapy, with normalization of serum free light chain levels, reduction in alkaline phosphatase and improvement in pain and functional status. Although rare, AL amyloidosis should be considered in the differential diagnosis of selected patients with spontaneous vertebral compression fractures. Moreover, there seems to be an association of vertebral compression fractures with liver involvement in AL amyloidosis.
Keywords:
AL amyloidosis; bone; vertebral compression fractures.
MeSH terms
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Alkaline Phosphatase / metabolism
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Amyloidosis / diagnosis*
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Amyloidosis / metabolism
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Amyloidosis / pathology
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Amyloidosis / therapy
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Antineoplastic Agents / therapeutic use
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Bone Marrow / drug effects
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Bone Marrow / metabolism
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Bone Marrow / pathology
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Bortezomib / therapeutic use
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Dexamethasone / therapeutic use
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Diagnosis, Differential
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Female
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Fractures, Compression / diagnosis*
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Fractures, Compression / metabolism
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Fractures, Compression / pathology
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Fractures, Compression / therapy
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Hematopoietic Stem Cell Transplantation
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Hepatomegaly / diagnosis*
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Hepatomegaly / drug therapy
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Hepatomegaly / enzymology
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Hepatomegaly / pathology
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Humans
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Immunoglobulin Light Chains / biosynthesis
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Immunoglobulin Light-chain Amyloidosis
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Liver / drug effects
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Liver / enzymology
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Liver / pathology
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Magnetic Resonance Imaging
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Male
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Melphalan / therapeutic use
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Middle Aged
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Plasma Cells / drug effects
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Plasma Cells / metabolism
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Plasma Cells / pathology
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Spinal Fractures / diagnosis*
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Spinal Fractures / metabolism
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Spinal Fractures / pathology
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Spinal Fractures / therapy
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gamma-Glutamyltransferase / metabolism
Substances
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Antineoplastic Agents
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Immunoglobulin Light Chains
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Bortezomib
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Dexamethasone
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gamma-Glutamyltransferase
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Alkaline Phosphatase
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Melphalan