Issue: 2017 > September > photo quiz

Answer to Photo Quiz: Advanced Waldenström’s macroglobulinaemia presenting as tongue swelling

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The past medical history was suggestive for oral manifestation of a lymphoproliferative disorder, with the first hypothesis a deposit of amyloid.1 Plain macroglossia is a common sign of primary amyloidosis,1 while an increased volume with multiple nodules may be found in dialysis-related amyloidosis.2 In fact, macroglossia has been claimed to be a potential paraneoplastic sign of plasma cell dyscrasia. Nevertheless, in the present case bone marrow biopsy failed to identify any specific haematological disorder. When addressing other potential diagnoses, the absence of symptoms and the persistency of the relapsingremitting tongue swelling over several weeks lead to the exclusion of an acute inflammatory/infective disorder (e.g. tongue abscess). Other disorders to be excluded in case of relapsing-remitting tongue swelling over time were a false lingual artery, tuberculosis, syphilitic gumma, actinomycosis and infiltrating carcinoma.
The pathological assessment of the oral biopsy showed mature B-cells (CD20+, CD3-, CD4-, CD5-, CD7-, CD8-, CD138-) with secretory capacity with stacks formed of kappa chains (figure 2). The joint assessment of such results, bone marrow biopsy and serum protein electrophoresis resulted in the diagnosis of Waldenstrom’s macroglobulinaemia with oral secondary manifestations. Two weeks after the diagnosis the patient died of a cardiac arrest. Death occurred because of direct damage to the heart and lung tissue due to massive deposition of M-protein and lymphocyte invasion, with a pathological appearance similar to what was observed in the tongue biopsy.
Oral manifestations of Waldenstrom’s macroglobulinaemia have been reported in just two patients, both of whom had gingival hyperplasia, in one case preceding the onset of massive oral ulcers.3,4
The present case reinforces the previously reported importance of an exhaustive assessment of macroglossia as a potential sign of haematological disorders.1


  1. Van der Waal RI, van de Scheur MR, Huijgens PC, Starink TM, van der Waal I. Amyloidosis of the tongue as a paraneoplastic marker of plasma cell dyscrasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2002;94:444-7. 
  2. Matsuo K, Nakamoto M, Yasunaga C, Goya T, Sugimachi K. Dialysis-related amyloidosis of the tongue in long-term hemodialysis patients. Kidney Int. 1997;52:832-8. 
  3. Gamble JW DE. Oral manifestations of macroglobulinemia of Waldenstrom. Report of a case. Oral Surg Oral Med Oral Pathol. 1960;13:104-10. 
  4. Hjorting-Hansen EPH, Drivsholm A. [Oral manifestations in Waldenstrom’s macroglobulinemia]. Ugeskr Laeger. 1962;124:133-7.