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CASE REPORT
Year : 2016  |  Volume : 29  |  Issue : 1  |  Page : 179-180

Kayser-Fleischer-like rings in a patient with vitamin-B 12 deficiency


Department of Medicine, Military Hospital, Ranchi, Jharkhand, India

Date of Submission25-May-2014
Date of Acceptance06-Aug-2014
Date of Web Publication18-Mar-2016

Correspondence Address:
Sanjay Singhal
MD, PDCC, Department of Medicine, Military Hospital, Namkum, Ranchi, Jharkhand
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-2098.179012

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  Abstract 

An 18-year-old man presented with recurrent jaundice over the past 2 years. Physical examination indicated severe pallor, icterus, and glossitis. Systemic evaluation indicated Kayser-Fleischer rings on slit-lamp examination. Laboratory parameters indicated severe anemia and indirect hyperbilirubinemia with normal liver enzymes. Further evaluation indicated a diagnosis of severe nutritional vitamin-B 12 deficiency. Here, we report the unusual case of the presence of Kayser-Fleischer-like rings in a patient with severe vitamin-B 12 deficiency.

Keywords: Kayser-Fleischer ring, recurrent jaundice, vitamin-B 12 deficiency


How to cite this article:
Vardhan V, Pawah AK, Chopra M, Singhal S. Kayser-Fleischer-like rings in a patient with vitamin-B 12 deficiency. Menoufia Med J 2016;29:179-80

How to cite this URL:
Vardhan V, Pawah AK, Chopra M, Singhal S. Kayser-Fleischer-like rings in a patient with vitamin-B 12 deficiency. Menoufia Med J [serial online] 2016 [cited 2019 Sep 21];29:179-80. Available from: http://www.mmj.eg.net/text.asp?2016/29/1/179/179012


  Introduction Top


Kayser-Fleischer (KF) rings have traditionally been considered pathognomonic of Wilson's disease. However, they have been reported in many other conditions. Here, we report a case of KF-like rings occurring in a patient with vitamin-B 12 deficiency, a previously unreported association.

Case summary

An 18-year-old man was admitted to our hospital with a history of fatigue, anorexia, and jaundice. There was no history of fever, abdominal discomfort, and pruritus. His medical history indicated three episodes of jaundice in the last 2 years. In view of recurrent jaundice, and negative virological and immunological work-up, he was evaluated by a private practitioner for Wilson's disease. Ophthalmology consult confirmed the presence of corneal pigmentation rings indistinguishable from KF rings on slit-lamp examination. Serum ceruloplasmin level was 0.14 g/l (normal 0.15-0.50). A diagnosis of Wilson's disease was considered and he was treated by chelation with d-penicillamine. Subsequently, the patient presented to this hospital. Informed consent was taken from the patient for publication.

In our hospital, a general evaluation indicated a thin boy with severe pallor, glossitis, and icterus. Systemic evaluation was unremarkable, except KF rings on slit-lamp examination. A detailed review of a previous document showed that during previous episodes of jaundice, there was indirect hyperbilirubinemia with normal liver enzymes. Investigations in our hospital indicated hemoglobin of 4.9 g/dl, a total leukocyte count of 3000 cells/mm 3 (low), and a differential count of 44% neutrophils, 48% lymphocytes, 2% monocytes, and 6% eosinophils. His total bilirubin was 5.2 mg/dl (indirect 4.2 mg/dl and direct 1.0 mg/dl), with normal liver enzymes, serum albumin, and prothrombin time. Serum haptoglobin, the Coombs test, the osmotic fragility test, and glucose-6 phosphate dehydrogenase were normal. Red blood cell indices indicated increased mean corpuscular volume (108.5 fl). Peripheral blood smear showed a reticulocyte count of 1.5%, with marked anisopoikilocytosis, microcytic hypochromic, and macrocytic picture with marked polychromasia. On further work-up, serum vitamin-B 12 levels were found to be low (69 pg/ml), with normal folate levels (8.8 ng/ml) and a low to normal serum ferritin level (30 ng/ml). A diagnosis of indirect hyperbilirubinemia secondary to vitamin-B 12 deficiency-induced ineffective erythropoiesis was made. Subsequently, he was started on parenteral vitamin-B 12 supplementation with folic acid and iron, on which the patient showed rapid improvement in appetite, disappearance of KF rings, reticulocytosis, and increase in hemoglobin to 11.4 g/dl (≥1 g/dl/week).


  Discussion Top


Nutritional vitamin-B 12 deficiency is very common in India [1]. Vitamin-B 12 deficiency can manifest with a wide range of signs and symptoms. These clinical presentations can be classified into hematologic and neurologic disease. The principal hematologic complication is megaloblastic anemia [2]. Other blood cell lines may also be involved, producing pancytopenia in the peripheral blood smear [3] (as in our case). The features of hemolysis can occur because of ineffective erythropoiesis in association with overt anemia [1]. Neurological abnormality can range from a subtle personality change to reversible dementia and noncompressive myelopathy to peripheral neuropathy. Patients with vitamin-B 12 deficiency with neurologic disease may have only minor hematologic involvement [4], whereas patients with severe hematologic disease may have no neurologic disease [5].

Broadly, KF rings seen in Wilson's disease are because of copper deposition in Descemet's membrane in the sclera-corneal junction. Although, KF rings are believed to be pathognomonic of Wilson's disease, it has been reported in many other conditions such as cryptogenic cirrhosis, chronic active hepatitis, neonatal hepatitis, primary biliary cirrhosis, alcoholic liver disease, multiple myeloma, galactosialidosis, and intraocular foreign body containing copper [6],[7]. The exact nature of these rings is not known. KF rings secondary to vitamin-B 12 deficiency have not been reported to date. Hence, it is imperative that the medical community consider KF rings in the differential diagnosis of diseases other than Wilson's disease.


  Acknowledgements Top


Conflicts of interest

None declared.

 
  References Top

1.
Dasari S, Naha K, Prabhu M. An unusual cause for recurrent jaundice in an otherwise healthy male. Australas Med J 2012; 5 :541-543.  Back to cited text no. 1
    
2.
Koury MJ, Ponka P. New insights into erythropoiesis; the roles of folate, vitamin B 12 and iron. Annu Rev Nutr 2004; 24 :105-131.  Back to cited text no. 2
    
3.
Ishtiaq O, Baqai HZ, Anwer F, Hussain N. Patterns of pancytopenia patients in a general medical ward and a proposed diagnostic approach. J Ayub Med Coll Abbottabad 2004; 16 :8-13.  Back to cited text no. 3
    
4.
Bottiglieri T, Crellin R, Reynolds EH. Folate and neuropsychiatry. In: Bailey LB, editor. Folate in health and disease. New York: Marcel Dekker; 1995. 435-462.  Back to cited text no. 4
    
5.
Shorvon SD, Carney MWP, Chanarin I, Reynolds EH. The neuropsychiatry of megaloblastic anaemia. Br Med J 1980; 281 :1036-1038.  Back to cited text no. 5
    
6.
Williams EJ, Gleeson D, Burton JL, Stephenson TJ. Kayser-Fleischer like rings in alcoholic liver disease: a case report. Eur J Gastroenterol Hepatol 2003; 15 :91-93.  Back to cited text no. 6
    
7.
Suvarna JC. Kayser-Fleischer ring. J Postgrad Med 2008; 54:238-240.  Back to cited text no. 7
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