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134:Severe Oral Ulcerations Associated with Myeloid Neoplasm[AAOM2021]
EP36057
Poster Title: 134:Severe Oral Ulcerations Associated with Myeloid Neoplasm[AAOM2021]
Submitted on 29 Mar 2021
Author(s): Brittany A Klein1, Eli Miloslavsky2, Philip C Amrein3, Nathaniel S Treister1
Affiliations: 1: Brigham and Women's Hospital, Division of Oral Medicine and Dentistry; 2: Massachusetts General Hospital, Division of Rheumatology, Allergy, and Immunology; 3: Massachusetts General Hospital Cancer Center
This poster was presented at 2021 American Academy of Oral Medicine Virtual Conference
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Poster Information
Abstract: Background:

Acute onset severe oral ulcerations can be associated with a variety of systemic diseases, including hematologic malignancies. Thorough workup with attention to other sites of involvement, relevant laboratory studies, and histopathology are essential to elucidate a diagnosis.

Case summary:

A 68-year-old male with a history of gastroesophageal reflux disease and hyperlipidemia was referred for urgent evaluation of acute onset severely painful oral ulcerations present for one month associated with 20-lb weight loss, frequent epistaxis, and rash. Laboratory findings included mild eosinophilia; elevated WBC, CRP, ESR, and vitamin B12; positive ANA; and positive HLA-B51. Extensive infectious disease and rheumatologic workup were negative. Skin biopsy revealed perifollicular neutrophilic granulomatous inflammation and superficial perivascular lymphohistiocytic infiltrate with neutrophils and occasional eosinophils. Oral ulcers worsened and eosinophilia persisted (reaching a maximum value of 1.77 K/uL) despite regimen of colchicine 0.6 mg BID and methylprednisolone up to 96 mg daily. On exam, extensive ulceration of the upper and lower labial mucosa and bilateral ventral tongue and focal ulcerations of the soft palate were noted. Biopsy of the lower lip mucosa revealed mild non-specific chronic inflammation. The patient was admitted for pain management and multi-disciplinary workup. Ultimately, he was found to have a FIP1L1-PDGFRA gene fusion and a RUNX1 gene variant. Bone marrow biopsy revealed left-shifted myeloid-predominant maturing trilineage hematopoiesis without evidence of dysplasia. A diagnosis of myeloid neoplasm associated with eosinophilia and rearrangement of PDGFRA was made and the patient was started on imatinib 400 mg daily. Complete molecular remission was achieved within two months and oral ulcerations have not recurred.

Conclusions:

Myeloid neoplasm associated with eosinophilia and PDGFRA rearrangement is a rare malignancy with a striking male predilection and exquisite responsiveness to imatinib. Painful oral ulcerations with non-specific clinical and histopathologic features that are refractory to high-dose glucocorticoids can be a presenting feature. Though rare, oral medicine specialists should be aware of this oral manifestation of underlying malignancy.
Summary: Acute onset severe oral ulcerations can be associated with a variety of systemic diseases, including hematologic malignancies. Thorough workup with attention to other sites of involvement, relevant laboratory studies, and histopathology are essential to elucidate a diagnosis.

Ask the author questions about this poster:bklein@bwh.harvard.edu
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