Case Reports in Rheumatology

Case Reports in Rheumatology / 2014 / Article

Case Report | Open Access

Volume 2014 |Article ID 424056 | https://doi.org/10.1155/2014/424056

Frank Verhoeven, Clément Prati, Daniel Wendling, "Amyloidoma, an Unusual Cause of Fracture", Case Reports in Rheumatology, vol. 2014, Article ID 424056, 3 pages, 2014. https://doi.org/10.1155/2014/424056

Amyloidoma, an Unusual Cause of Fracture

Academic Editor: G. J. Tsay
Received22 Dec 2013
Accepted23 Feb 2014
Published16 Mar 2014

Abstract

We report a case of a spontaneous hip fracture in a context of dysglobulinemia. The bone histologic examination found amyloidoma. Amyloidoma is an overload pathology and an unusual cause of fracture. In most of the cases, it is associated with myeloma and the difference between bone invasion of myeloma and amyloidoma in an osteolytic radiographic picture is not easy but is of importance because prognosis and treatment may be totally different. Thus, in the context of dysglobulinemia, one must keep in mind that spontaneous bone fracture may be due to amyloidoma with another prognosis.

1. Introduction

Amyloidoma is an uncommon cause of bone fragility. It occurs in the context of dysglobulinemia and the difference between amyloidoma and myeloma is difficult but is important.

2. Case

We report the clinical story of an 87-year-old man who was hospitalized for a right hip fracture without high energy trauma. He was known, but not followed up, for a kappa light chain monoclonal gammopathy diagnosed 7 years ago. He had no inflammatory syndrome (CRP 10 mg/dL and ESR 25 mm) and no hypercalcemia (2, 57 mmol/L). The electrophoresis was characterized by an hypogammaglobulinemia and the presence of kappa light chain. The myelogram showed 9% of kappa light chain monoclonal plasmocytes. The Bence Jones proteinuria was positive. He underwent a hip arthroplasty and the histological examination of the femoral head found multifocal non-AA amyloid deposits, with osteolysis and a pseudotumoral aspect (amyloidoma). A biopsy of accessory salivary glands found non-AA amyloid deposits too. The radiographic exam (Figure 1) at the time of the fracture diagnosis showed geodes surrounded by area of osteosclerosis corresponding to focal deposits of amyloid. The extension statement showed only other geodes in the left hip (Figure 2). Six months later, he suffered from a contralateral hip fracture probably secondary to amyloidosis too.

3. Discussion

This is the second observation of hip fracture secondary to amyloidoma [1]. Amyloidoma is an unusual cause of fracture. Amyloidosis is an overload pathology characterized by extra cellular deposits of amyloid substance. The osteoarticular involvement represented by amyloidoma, amyloid arthropathy [2], and amyloid polyarthritis [3] is rare. Few types of amyloidosis are responsible for these manifestations [4]: AL amyloidosis which is primary or associated with myeloma [5], β 2-microglobulin amyloidosis which is associated with chronic dialysis [6], and ATTR amyloidosis including the senile and the hereditary amyloidosis.

Amyloidoma is most frequently associated with the AL amyloidosis which is linked with myeloma by the kappa or lambda light chain. These light chains share the same amino acid sequence with the AL amyloid substance [4]. Among these associations, amyloidoma can be a primary pathology, but it is less frequent. In the literature, 35 cases of bone locations (Table 1) are described to date and mainly in the spine. There are 23 vertebral involvements, and 6 are located at the skull. Peripheral involvements are more exceptional. There are just two humeral locations and one femoral [1] location described and finally 3 locations in the belts. This distribution is the same as for myeloma, in the trabecular bone. These locations might be explained by the possible immunoglobulin origin of amyloid proteins and the similarity of the amino acid sequence. The difference between bone invasion of myeloma and amyloidoma in front of an osteolytic lesion is not clear in a patient suffering from myeloma. In case of amyloidoma, we can observe a geode surrounded by osteosclerosis and this osteosclerosis may differentiate between the two conditions. This detail may be interesting in the absence of histologic data. This is important since the treatment and the prognosis are totally different between myeloma lesions and amyloidoma.


AuthorsLocationsContext

Factor RE et al.; Diagn Cytopathol. (2012)Iliac boneMyeloma
Farrell K et al.; J Clin Oncol. (2011)Cervical spineMyeloma
Parmar H et al.; AJNR Am J Neuroradiol. (2010)1 skull
1 lumbar spine
1 thoracic spine
1 iliac bone
Primary
Oruckaptan H et al.; Turk Neurosurg. (2009)Skull and cervical spineDialysis
Abbas N et al.; Br J Neurosurg. (2008)Thoracic spinePrimary
Iplikcioglu AC et al.; Spine. (2007)Cervical spinePrimary
Volkan Aydin M et al.; J Spinal Disord Tech. (2006)Thoracic spinePrimary
Iguchi T et al.; Rinsho Ketsueki. (2005)Humerus Dysglobulinemia
Mulleman D et al.; Eur Spine J. (2004)Cervical spinePrimary (evolution to systemic amyloidosis)
Manucha V et al.; Diagn Cytopathol. (2003)Thoracic spinePrimary
Rachbauer F et al.; AJR Am J Roentgenol. (2003)ClavicleDysglobulinemia
Unal A et al.; Clin Neurol Neurosurg. (2003)Thoracic spinePrimary
Bruninx G et al.; J Radiol. (2001)SacrumPrimary
Hsu CW et al.; Ren Fail. (2001)Cervical spineDialysis
Simoens WA et al.; AJNR Am J Neuroradiol. (2000)Skull Primary
Hwang SS et al.; AJNR Am J Neuroradiol. (2000)Cervical spinePrimary
Miossec P et al.; Diabetes Metab. (1999)Lumbar spinePrimary
Sancho JM et al.; Med Clin (Barc). (1999)Lumbar spinePrimary
Mathew JM et al.; Br J Neurosurg. (1998)Thoracic spinePrimary
Dee CH et al.; Spine. (1998)Thoracic spinePrimary
Porchet F et al.; Spine. (1998)Cervical spinePrimary
Pambuccian SE et al.; Am J Surg Pathol. (1997)1 cervical spine
1 scapula
1 humeral head
Dysglobulinemia and
lymphoplasmacytic infiltrate
Hidalgo F et al.; Neuroradiology. (1996)Skull
Cloft HJ et al.; AJNR Am J Neuroradiol. (1995)Thoracic spinePrimary
Chang YS et al.; Zhonghua Yi Xue Za Zhi (Taipei). (1993)Thoracic and lumbar spinePrimary
Unal F et al.; J Neurosurg. (1992)SkullPrimary
Dickman CA et al.; Neurosurgery. (1988)Cervical spine
Leeson MC et al.; Spine. (1985)Thoracic spinePrimary
Lai KN et al.; Am J Med. (1984)Femoral neckMyeloma
Giordano A et al.; Otolaryngol Head Neck Surg. (1983)Skull
FADELL EJ et al.; Am J Surg. (1964)Sternum

Thus, in case of fracture in a patient with dysglobulinemia, one must keep in mind the possibility of an amyloidoma, especially in front of a geode surrounded by osteosclerosis.

Disclosure

Frank Verhoeven is the first author.

Conflict of Interests

The authors declare that there is no conflict of interests regarding the publication of this paper.

References

  1. K. N. Lai, K. W. Chan, and D. L. Siu, “Pathologic hip fractures secondary to amyloidoma. Case report and review of the literature,” American Journal of Medicine, vol. 77, no. 5, pp. 937–943, 1984. View at: Google Scholar
  2. B. Fautrel, J.-P. Fermand, J. Sibilia, D. Nochy, B. Rousselin, and P. Ravaud, “Amyloid arthropathy in the course of multiple myeloma,” Journal of Rheumatology, vol. 29, no. 7, pp. 1473–1481, 2002. View at: Google Scholar
  3. T. Prokaeva, B. Spencer, M. Kaut et al., “Soft tissue, joint, and bone manifestations of AL amyloidosis: clinical presentation, molecular features, and survival,” Arthritis and Rheumatism, vol. 56, no. 11, pp. 3858–3868, 2007. View at: Publisher Site | Google Scholar
  4. P. M'bappé and G. Grateau, “Osteo-articular manifestations of amyloidosis,” Best practice & research: Clinical rheumatology, vol. 26, no. 4, pp. 459–475, 2012. View at: Google Scholar
  5. J. D. Sipe and A. S. Cohen, “Review: history of the amyloid fibril,” Journal of Structural Biology, vol. 130, no. 2-3, pp. 88–98, 2000. View at: Publisher Site | Google Scholar
  6. J. D. Sipe, M. D. Benson, J. N. Buxbaum et al., “Amyloid fibril protein nomenclature: 2012 recommendations from the Nomenclature Committee of the International Society of Amyloidosis,” Amyloid, vol. 19, no. 4, pp. 167–170, 2012. View at: Publisher Site | Google Scholar

Copyright © 2014 Frank Verhoeven et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


More related articles

1274 Views | 441 Downloads | 2 Citations
 PDF Download Citation Citation
 Download other formatsMore
 Order printed copiesOrder

Related articles

We are committed to sharing findings related to COVID-19 as quickly as possible. We will be providing unlimited waivers of publication charges for accepted research articles as well as case reports and case series related to COVID-19. Review articles are excluded from this waiver policy. Sign up here as a reviewer to help fast-track new submissions.