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 Table of Contents 
Year : 2017  |  Volume : 6  |  Issue : 1  |  Page : 146-147  

Macroglossia as a presenting feature of multiple myeloma

1 Department of Dermatology and Venereology, Faculty of Medicine, Kirikkale University, Yahsihan, Kirikkale, Turkey
2 Department of Dermatology and Venereology, Faculty of Medicine, Adnan Menderes University, Aydın, Turkey
3 Department of Nephrology, Tepecik Research and Training Hospital, İzmir, Turkey
4 Department of Pathology, Faculty of Medicine, Adnan Menderes University, Aydın, Turkey
5 Department of Hematology, Kent Hospital, İzmir, Turkey

Date of Web Publication18-Sep-2017

Correspondence Address:
Serkan Demirkan
Department of Dermatology and Venereology, Faculty of Medicine, Kirikkale University, No. 14, Tahsin Duru Avenue, Yenisehir, Yahsihan, Kirikkale
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2249-4863.214968

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Macroglossia has been very rarely reported as a first clinical sign of multiple myeloma.

Keywords: Amyloidosis, multiple myeloma, tongue growth

How to cite this article:
Demirkan S, Şavk E, Alp A, Doger FK, Kadikoylu G, Gunduz O. Macroglossia as a presenting feature of multiple myeloma. J Family Med Prim Care 2017;6:146-7

How to cite this URL:
Demirkan S, Şavk E, Alp A, Doger FK, Kadikoylu G, Gunduz O. Macroglossia as a presenting feature of multiple myeloma. J Family Med Prim Care [serial online] 2017 [cited 2021 Aug 3];6:146-7. Available from: https://www.jfmpc.com/text.asp?2017/6/1/146/214968

  Background Top

The term “macroglossia” defines a painless and long-term tongue enlargement, which can be recognized by observation of a resting tongue protruding over the dentoalveolar structures.[1]

Macroglossia is classified into two major categories: true- and pseudo-macroglossia. True macroglossia is associated with definitive histological changes in the tongue and it is frequently observed due to hypertrophy of the tongue muscles or due to infiltration of normal tissue with abnormal proteins or glycogen.[2] Pseudomacroglossia refers to a normal-sized but large-appearing tongue. This condition is usually a result of the anatomical abnormalities of the oral cavity.[3],[4],[5]

  Case Report Top

A 54-year-old female presented with tongue enlargement. She had first recognized enlargement of her tongue about 6 months ago. Soon after that, red-purple spots emerged on both her arms, and she began to feel fatigued all the time. Her physical examination revealed an enlarged tongue with teeth indentation marks on both lateral borders [Figure 1]. Her arms were covered with numerous erythematous macules with a diameter of 1 mm [Figure 1]. Bilateral pretibial three positive pitting edema was also observed. Her liver and renal function tests were in normal range, but her complete blood count was consistent with anemia of chronic disease. Her urinalysis revealed proteinuria consistent with nephrotic syndrome. The patient was consulted to internal medicine department with preliminary diagnosis of amyloidosis, hypothyroidism, and lipoid proteinosis. Multiple biopsies from kidneys and bone marrow were performed. Biopsy specimens were stained positive with Congo red and crystal violet in vascular walls and interstitium. According to histopathological findings, a diagnosis of amyloid light type amyloidosis was established. Subsequent bone marrow biopsy revealed an increased percentage (30%) of plasma cells [Figure 2]. The patient was diagnosed with multiple myeloma and underwent chemotherapy treatment. However, she was lost due to multiorgan involvement and loss of function in 6 months.
Figure 1: (a) Dental indentations located on lateral surfaces of the tongue as the result of macroglossia (b) Petechiae on the antecubital fossa (pinch purpura)

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Figure 2: (a) Plasma cells in the bone marrow (H and E, ×400) (b) bone marrow (Congo red, ×100) (c) plasma cells in the bone marrow (CD138, ×100)

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  Conclusion Top

Amyloidoses are rare diseases characterized by extracellular aggregation of at least 31 different amyloid proteins.[6] Diagnosis of amyloidosis depends on histopathological examination. In routine hematoxylin-eosin staining, homogeneous eosinophilia is observed. Amyloid protiens also react with Congo red stain and yield reflection under polarized microscopy.[7] Amyloid-proteinaceous materials have various effects on cellular level. They may aggregate between cells and cause atrophy. Furthermore, it has a direct toxic effect on cells leading to eventual cell death.[8] Amyloid proteins may accumulate in various internal organs, particularly in the heart and kidneys and also in the tongue.

Macroglossia has been very rarely reported as a first clinical sign of multiple myeloma. In making a diagnosis of multiple myeloma-associated amyloidosis, it is possible for dermatological and venereal diseases' specialist to contribute to early diagnosis by considering the condition seriously when a patient applies with a complaint of tongue enlargement.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Murthy P, Laing MR. Macroglossia. BMJ 1994;309:1386-7.  Back to cited text no. 1
Topouzelis N, Iliopoulos C, Kolokitha OE. Macroglossia. Int Dent J 2011;61:63-9.  Back to cited text no. 2
García de Guilarte RF, Frönher BB, Urcelay PR, Nájera RC, Meli BG, Enríquez de Salamanca Celada J. An idiopathic case of macroglossia. J Plast Reconstr Aesthet Surg 2009;62:e41-3.  Back to cited text no. 3
Renehan A, Morton M. Acute enlargement of the tongue. Br J Oral Maxillofac Surg 1993;31:321-4.  Back to cited text no. 4
Renehan AG, Morton ME. Macroglossia. Classify the pathology as normal or abnormal. BMJ 1995;310:329.  Back to cited text no. 5
Xavier SD, Bussoloti IF, Müller H. Macroglossia secondary to systemic amyloidosis: Case report and literature review. Ear Nose Throat J 2005;84:358-61.  Back to cited text no. 6
Mollee P, Renaut P, Gottlieb D, Goodman H. How to diagnose amyloidosis. Intern Med J 2014;44:7-17.  Back to cited text no. 7
Muchtar E, Buadi FK, Dispenzieri A, Gertz MA. Immunoglobulin light-chain amyloidosis: From basics to new developments in diagnosis, prognosis and therapy. Acta Haematol 2016;135:172-90.  Back to cited text no. 8


  [Figure 1], [Figure 2]

This article has been cited by
1 Macroglossia, the first manifestation of systemic amyloidosis associated with multiple myeloma: Case report
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Journal of Stomatology, Oral and Maxillofacial Surgery. 2018;
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