Plummer-Vinson syndrome complicated by Schatzki ring, non-Hodgkin lymphoma, and myeloproliferative disorder: A rare case report from Pakistan

Abstract

We report a rare case of Plummer-Vinson syndrome (PVS) in a 55-year-old female with no known premorbid condition who presented to the hospital with a chief complaint of dysphagia for 1 month, weight loss, and a history of fever (high grade up to 40°C) associated with occasional rigors and chills. Laboratory testing showed iron-deficiency anemia, together with dysphagia, raising clinical suspicion for PVS. She was further examined with upper gastrointestinal endoscopy to evaluate the cause of dysphagia, which revealed a Schatzki ring in the esophagus at 38 cm from the incisors, near the gastroesophageal junction. The ring was dilated endoscopically, and the rest of the stomach and duodenum were normal. The computed tomography scan presented hepatosplenomegaly and lymphadenopathy. Findings were consistent with a myeloproliferative disorder and non-Hodgkin lymphoma. Oral iron supplementation (150-200 mg) was recommended, and dysphagia should be evaluated with gastroduodenal endoscopy as indicated.

Keywords

Plummer-Vinson syndrome / Esophageal webs / Iron deficiency / Postcricoid dysphagia

Funding

This case study was part of a PhD research study financially supported by the Pakistan Science Foundation (Project # PSF/Res/C-PINSTECH/Med531).

Conflict of interest

The authors declare that they have no competing interests.

References

[1] Paterson DR. A clinical type of dysphagia. Proc R Soc Med. 1919;12(Laryngol Sect):235-239. doi: 10.1177/0035915719012006110

[2] Wynder EL, Hultberg S, Jacobsson F, Bross IJ. Environmental factors in cancer of the upper alimentary tract; A swedish study with special reference to plummer-vinson (paterson-kelly) syndrome. Cancer. 1957; 10(3):470-487. doi: 10.1002/1097-0142(195705/06)10:3<470:aid-cncr28201 00309>3.0.co;2-7

[3] Lawoyin D, Reid E, Sam F, Obayomi T, Bonnick A. Plummer-vinson syndrome. a case report and literature review. Int J Dent Sci. 2006; 5(2):10-5580.

[4] Adeyemo TA, Adeyemo WL, Adediran A, Akinbami AJA, Akanmu AS. Orofacial manifestations of hematological disorders: Anemia and hemostatic disorders. Indian J Dent Res. 2011; 22(3):454-461. doi: 10.4103/0970-9290.87070

[5] Richards SH, Kilby D, Shaw JD. Post-cricoid carcinoma and the paterson-kelly syndrome. J Laryngol Otol. 1971; 85(2):141-152. doi: 10.1017/S0022215100073254

[6] Suzman MM. Syndrome of anemia, glossitis, and dysphagia: Report of eight cases, with special reference to observations at autopsy in one instance. Arch Intern Med. 1933; 51(1):1-21. doi: 10.1001/archinte.1933.00150200004001

[7] Atmatzidis K, Papaziogas B, Pavlidis T, Mirelis C, Papaziogas T. Plummer-vinson syndrome. Dis Esophagus. 2003; 16(2):154-157. doi: 10.1046/j.1442-2050.2003.00316

[8] Entwistle CC, Jacobs A. Histological findings in the Paterson-kelly syndrome. J Clin Pathol. 1965; 18(4):408-413. doi: 10.1136/jcp.18.4.408

[9] Hefaiedh R, Boutreaa Y, Ouakaa-Kchaou A, et al. Plummer vinson syndrome association with coeliac disease. Arab J Gastroenterol. 2013; 14(4):183-185. doi: 10.1016/j.ajg.2013.10.003

[10] Medrano M. Dysphagia in a patient with rheumatoid arthritis and iron deficiency anemia. MedGenMed. 2002; 4(3):10.

[11] Park JM, Kim KO, Park CS, Jang BI. A case of plummer-vinson syndrome associated with Crohn’s disease. Korean J Gastroenterol. 2014; 63(4):244-247. doi: 10.4166/kjg.2014.63.4.244

[12] Elwood PC, Jacobs A, Pitman RG, Entwistle CC. Epidemiology of the paterson-kelly syndrome. Lancet. 1964; 2(7362):716-720. doi: 10.1016/S0140-6736(64)92541-3.

[13] Spivak JL. Myeloproliferative neoplasms. N Engl J Med. 2017; 376(22):2168-2181. doi: 10.1056/NEJMra1406186

[14] Tefferi A, Barbui T. Polycythemia vera and essential thrombocythemia: 2021 update on diagnosis, risk-stratification and management. Am J Hematol. 2020; 95(12):1599-1613. doi: 10.1002/ajh.26008

[15] Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016; 127(20):2375-2390. doi: 10.1182/blood-2016-01-643569

[16] Campo E, Swerdlow SH, Harris NL, Pileri S, Stein H, Jaffe ES. The 2008 WHO classification of lymphoid neoplasms and beyond: Evolving concepts and practical applications. Blood. 2011; 117(19):5019-5032. doi: 10.1182/blood-2011-01-293050

[17] Goel A, Lakshmi CP, Bakshi SS, Soni N, Koshy S. Single-center prospective study of Plummer-Vinson syndrome. Diseases of the Esophagus. 2016; 29(7):837-841.

[18] Ben Gamra O, Mbarek C, Mouna C, et al. Syndrome de plummer-vinson [plummer vinson syndrome]. Tunis Med. 2007; 85(5):402-404.

[19] Novacek G. Plummer-vinson syndrome. Orphanet J Rare Dis. 2006; 1:36. doi: 10.1186/1750-1172-1-36

[20] Mansell NJ, Jani P, Bailey CM. Plummer-vinson syndrome--a rare presentation in a child. J Laryngol Otol. 1999; 113(5): 475-476. doi: 10.1017/S0022215100144263

[21] López Rodríguez MJ, Robledo Andrés P, Amarilla Jiménez A, Roncero Maíllo M, López Lafuente A, Arroyo Carrera I. Sideropenic dysphagia in an adolescent. J Pediatr Gastroenterol Nutr. 2002; 34(1):87-90. doi: 10.1097/00005176-200201000-00021

[22] Jameson JL, Fauci AS, Kasper DL, Hauser SL, Longo DL, Loscalzo J. Harrison’s Principles of Internal Medicine. 20^th ed. United States: McGraw-Hill Education; 2018.

[23] Beyler AR, Yurdaydin C, Bahar K, Gören A, Soykan I, Uzunalimoğlu O. Dilation therapy of upper esophageal webs in two cases of plummer-vinson syndrome. Endoscopy. 1996; 28(2):266-267. doi: 10.1055/s-2007-1005446

[24] Huynh PT, De Lange EE, Shaffer HA Jr. Symptomatic webs of the upper oesophagus: Treatment with fluoroscopically guided balloon dilation. Radiology. 1995; 196(3):789-792. doi: 10.1148/radiology.196.3.7644644

[25] Pinto J, Nobre De Jesus G, Palma Anselmo M, et al. Iron refractory iron deficiency anemia in dizygotic twins due to a novel TMPRSS6gene mutation in addition to polymorphisms associated with high susceptibility to develop ferropenic anemia. J Investig Med High Impact Case Rep. 2017; 5(2):2324709617701776. doi: 10.1177/2324709617701776

[26] Ribeiro U Jr., Posner MC, Safetle-Ribeiro AV, et al. Risk factors for squamous cell carcinoma of the esophagus. Br J Surg. 1996; 83:1174-1185. doi: 10.1002/bjs.1800830903

[27] Rashid Z, Kumar A, Komar M. Plummer-vinson syndrome and postcricoid carcinoma: Late complications of unrecognized celiac disease. Am J Gastroenterol. 1999; 94(7):1991-1993. doi: 10.1111/j.1572-0241.1999.01242

[28] Bakari G, Benelbarhdadi I, Bahije L, El Feydi Essaid A. Endoscopic treatment of 135 cases of plummer-vinson web: A pilot experience. Gastrointest Endosc. 2014; 80(4):738-741. doi: 10.1016/j.gie.2014.05.005

[29] Yasawy MI. Treatment of plummer-vinson syndrome with savary-gillard dilatation. Saudi Med J. 2004; 25(4):524-526. doi: 10.15537/smj.2004.4.9025