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Table of Contents
CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 1  |  Page : 30-32

Plummer–Vinson syndrome


1 Department of General Medicine, Meenakshi Medical College, Hospital and Research Institute, Kanchipuram, Tamil Nadu, India
2 Department of Medical Gastroenterology, Meenakshi Medical College, Hospital and Research Institute, Kanchipuram, Tamil Nadu, India

Date of Web Publication8-Jan-2019

Correspondence Address:
M S Sridhar
Department of General Medicine, Meenakshi Medical College, Hospital and Research Institute, Enathur, Kanchipuram, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/JCSR.JCSR_5_18

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  Abstract 


The clinical findings of a 36-year-old female who presented to Medicine out-patient service with the triad of dysphagia, iron deficiency anaemia, splenomegaly and oesophageal web are reported. Clinical pathology of anaemia is diagnostic of iron deficiency anaemia. Dysphagia was treated with endoscopic dilation of the web and was prescribed iron therapy. This relatively rare condition is reported as the physician should be alert to its possibility because of its association with increased risk of hypopharyngeal carcinoma.

Keywords: Dysphagia, iron-deficiency anaemia, oesophageal web


How to cite this article:
Gopalakrishna P, Prasad M R, Joyes P T, Sridhar M S. Plummer–Vinson syndrome. J Clin Sci Res 2018;7:30-2

How to cite this URL:
Gopalakrishna P, Prasad M R, Joyes P T, Sridhar M S. Plummer–Vinson syndrome. J Clin Sci Res [serial online] 2018 [cited 2019 Mar 22];7:30-2. Available from: http://www.jcsr.co.in/text.asp?2018/7/1/30/249627




  Introduction Top


Plummer–Vinson syndrome or Paterson–Kelly syndrome which is a well-known clinical entity was not considered a strictly defined condition as late as in 1975.[1] This condition was defined as simple, painless, permanent stricture in the cervical oesophagus, not associated with inflammation, swelling or ulceration, which causes obstructive dysphagia.[2] The classic triad of dysphagia, iron-deficiency anaemia and oesophageal webs describes Plummer–Vinson syndrome.[3] Although it is a relatively rare condition, the physician should be alert to its possibility because of its association with increased risk of hypopharyngeal carcinoma.


  Case Report Top


A 35-year-old female presented herself with gradual onset of difficulty in swallowing, mainly for solids, of 6-month duration. She complained of generalised weakness and tiredness of 2-month duration. There was no history of pain during swallowing, weight loss or abdominal pain. She attained menarche at 13 years of age and has regular menstrual cycle of 30-day duration with flow for 3 days.

The patient was moderately built and nourished, and the patient's height was 1.55 m, weight was 44 kg and body mass index was 18.31 kg/m.[2] There was pallor of palpebral conjunctivae and nail bed. Koilonychias was absent; there was no icterus, cyanosis, lymphadenopathy or pedal oedema. Her pulse was 84/min, blood pressure was 120/70 mmHg, respiratory rate was 16/min and oral temperature was 36.9°C. Systemic examination was normal except for the fact that the spleen was palpable.

Her laboratory results were as follows: haemoglobin 6.8 g/dL, packed cell volume 23%, red blood cells 2.42 million/mm,[3] mean corpuscular volume 51fl, mean corpuscular volume 15 pg, mean corpuscular haemoglobin concentration 31%, total leucocyte count 5600/mm,[3] differential count showed neutrophils 65%, lymphocytes 32%, eosinophils 1%, monocytes 2%, platelets 283,000/mm3, erythrocyte sedimentation rate 8 mm at the end of 1st h, random blood glucose 77 mg/dL and serum ferritin <1.0 ng/mL. Peripheral smear showed microcytic hypochromic anaemia.

Ultrasonography of the abdomen was normal, and splenic size was 10.8 cm and 11.5 cm on two different occasions.

She was suspected to be having oesophageal growth for which she underwent upper gastrointestinal random endoscopy which revealed a typical circumferential membranous cricopharyngeal web [Figure 1] which was treated by endoscopic dilatation [Figure 2]. She improved symptomatically. She was transfused one unit of packed red blood cells for anaemia correction. She was prescribed iron supplementation and discharged.
Figure 1: Oesophageal web before dilatation

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Figure 2: Photograph obtained post-endoscopic dilatation

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  Discussion Top


Plummer–Vinson syndrome is easy to diagnose because of easy availability of endoscopy even in smaller centres. The dysphagia and iron-deficiency anaemia can be identified without any difficulty, and the web is detected by endoscopy. The oesophageal web is usually formed during long-standing iron deficiency, and since iron-deficiency anaemia is common among women, this syndrome is said to be much more common in women. Onset is said to be very gradual. There is danger of mistaking it for 'globus hystericus'. The incidence and prevalence of the condition are largely unknown due to uncertain level of ascertainment.[2]

A study by Lundholm was quoted by Larson-Gunnar et al.,[1] wherein 19% of persons with primary hypochromic anaemia were found to be having Plummer–Vinson syndrome.[1] Among 353 patients with iron-deficiency anaemia, this condition was found in 1.7%.[4] Based on endoscopy studies on 15,000 participants, the relative frequency of this condition was 1/1000; cervical oesophageal mucous diaphragms were found in 25/100,000 and women-to-men ratio was 3:1.[5] Among those who had webs in hypopharynx and oesophagus, there was a higher number of persons with iron-deficiency anaemia.[6] A review of patients with Plummer–Vinson syndrome in English literature was quoted to highlight female predominance and the mean haemoglobin was 8.2 g/dL.[3] As a part of clinical picture, enlargement of spleen and thyroid has been described.[7] In the patient described in this report, the spleen was clinically palpable; however, by ultrasonography, it measured 10.8 cm and 11.5 cm on two different occasions, and the haemoglobin level was 6.8 g/L. While correction of iron deficiency did not change the structure,[2] there is an opinion that correction of anaemia with iron supplementation is, in fact, beneficial.[3],[7] Recommended treatment was surgery or by dilatation, and early recurrence was expected.[2] At present, iron supplementation coupled with mechanical dilatation appears to be the definitive treatment for the condition.[3] In addition, endoscopic surveillance is suggested for detecting recurrence as well as post-cricoid carcinoma at an early stage.[7]

A high degree of suspicion for Plummer–Vinson syndrome in all patients with iron-deficiency anaemia with dysphagia would allow recognition of the rare disorder and enable endoscopic surveillance which is recommended for early detection and treatment of carcinoma hypopharynx. The correction of anaemia by iron supplementation and health education would be beneficial.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgement

We sincerely thank Professor and Head, Department of Medical Gastroenterology, Professor and Head, Department of Medicine and the Dean, Meenakshi Medical College, Hospital and Research Institute, Enathur, Kanchipuram, for their help and permission to publish this case.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Larsson LG, Sandström A, Westling P. Relationship of Plummer-Vinson disease to cancer of the upper alimentary tract in Sweden. Cancer Res 1975;35:3308-16.  Back to cited text no. 1
    
2.
Logan JS. A review of the Plummer-Vinson stricture of the cervical oesophagus. Ulster Med J 1978;47 Suppl 2:1-14.  Back to cited text no. 2
    
3.
Novacek G. Plummer-Vinson syndrome. Orphanet J Rare Dis 2006;1:36.  Back to cited text no. 3
    
4.
Uchida T, Matsuno M, Ide M, Kawachi Y. The frequency and development of tissue iron deficiency in 6 iron deficiency anemia patients with Plummer-Vinson syndrome. Rinsho Ketsueki 1998;39:1099-102.  Back to cited text no. 4
    
5.
Peghini M, Barabe P, Jean P, Griffet P, Eynard JP, Mbaye PS, et al. Mucoid webs of the cervical esophagus. Apropos of 38 cases discovered during upper endoscopy at the hôpital principal of Dakar. Relationship to Plummer-Vinson syndrome. Dakar Med 1989;34:93-101.  Back to cited text no. 5
    
6.
Nosher JL, Campbel WL, Seaman WB. The clinical significance of cervical esophageal and hypopharyngeal webs. Radiology 1975;117:45-7.  Back to cited text no. 6
    
7.
Hoffman RM, Jaffe PE. Plummer-Vinson syndrome. A case report and literature review. Arch Intern Med 1995;155:2008-11.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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