To describe unusual vascular lesions in the nasal mucosa of a patient with multiple myeloma (MM), presenting with recurrent epistaxis.
Materials and Methods
An extensive literature review was done looking for previous reports describing epistaxis secondary to nasal mucosa abnormalities in patients with MM. Medical records of a patient with unusual mucosal vascular lesions, including her presenting symptoms, history and nasoendoscopic examination were reviewed. Nasoendoscopic images showing changes in the mucosal vascular lesions, before and after MM treatment, were compared.
To date, there are no reports describing nasal vascular lesions in MM patients, that is not otherwise attributable to another pathology. Our patient presented with vascular lesions in her nasal mucosa, causing recurrent bloodstained nasal secretions. With definitive treatment of MM with chemotherapy and stem-cell transplant, her nasal vascular lesions and epistaxis resolved alongside resolution of her MM.
While vascular lesions in MM are described in other areas (eg. skin, retina), there are no reports describing any local nasal lesions not otherwise attributable to other pathophysiologies. Whether these lesions developed as part of the natural disease progress of MM is currently unclear. In patients with nasoendoscopy showing widespread raw granular mucosa or petechiae, and negative investigations (especially non-specific histology), we propose that MM be considered as a rare but possible differential diagnosis.
Epistaxis is one of the commonest reasons for presentation to the ENT clinic. In a study by Mors et al., approximately 12.7% of ENT consults were for epistaxis [ ]. Anatomical causes include bleeding from Little’s area, trauma, and benign or malignant neoplasms (such as nasopharyngeal carcinoma, especially in our local South-East Asia context). There are also systemic causes of epistaxis, such as bleeding diathesis (eg. coagulopathy), uncontrolled hypertension and hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease). While epistaxis may sometimes be nothing more than an inconvenience, it can occasionally be a harbinger for an underlying sinister disease, therefore warranting further investigation if there are atypical patient and disease factors.
Multiple myeloma (MM) is a subtype of hematological malignancy, specifically of plasma cells. MM typically presents with the classical ‘CRAB’ symptoms: hyper C alcemia, R enal failure, A nemia, B one pain. However, a significant proportion of patients do present with a variety of other signs and symptoms. A study by Talamo et al. found that among patients with symptomatic MM, 20% presented with non-CRAB symptoms [ ]. One of the ENT manifestations of MM that has been described is recurrent epistaxis [ ].
Bleeding and epistaxis in multiple myeloma
Creston et al. proposed that the bleeding tendency in MM, where epistaxis was one of the bleeding manifestations, was due to the presence of pathologic proteins which interfere with plasma clotting factors, as well as the precipitation of these proteins on the walls of small blood vessels [ ]. Ishimura et al. described a patient with MM presenting with epistaxis, who was subsequently diagnosed with nasopharyngeal amyloidosis on histopathology [ ]. It was noted that systemic amyloidosis is described in 12–15% of patients with MM, 3% of which have nasopharyngeal involvement. Momin and Aluri presented a case report where a patient initially presenting with isolated epistaxis was eventually diagnosed with MM [ ]. Notably, this patient had a normal diagnostic nasoendoscopy. They suggested that hemorrhage in MM patients could be due to the interference of circulating monoclonal proteins with platelet function and coagulation factors, in particular the polymerization of fibrin monomers, resulting in an acquired dysfibrinogenemia.
Despite the existence of case reports describing MM patients with epistaxis, none have attributed the epistaxis to any gross visible lesion in the nasal cavity, that is not otherwise attributable to any other pathophysiology that may suggest a related but separate diagnosis (eg. localised plasmacytoma, amyloidosis, infections or granulomatous diseases of the nasal cavity). In this case report, we present a case of MM who presented with recurrent epistaxis, and was found to have unusual nasal mucosal abnormalities on nasoendoscopy. Of significance, this patient had resolution of said lesions with successful treatment of her MM.
Patient A is a 51-year-old Chinese Female, presenting to ENT in March 2018 with two years history of occasional blood-stained nasal secretions, approximately one spoonful per day. She was known to have smoldering MM since 2014, which had recently progressed to active MM with anemia in January 2018.
Initial nasoendoscopy revealed widespread areas of raw, granular mucosa ( Fig. 1 ), especially over her left nasal septum and right lateral nasal wall.