|Year : 2016 | Volume
| Issue : 1 | Page : 179-180
Kayser-Fleischer-like rings in a patient with vitamin-B 12 deficiency
Vasu Vardhan, Anil Kumar Pawah, Manu Chopra, Sanjay Singhal
Department of Medicine, Military Hospital, Ranchi, Jharkhand, India
|Date of Submission||25-May-2014|
|Date of Acceptance||06-Aug-2014|
|Date of Web Publication||18-Mar-2016|
MD, PDCC, Department of Medicine, Military Hospital, Namkum, Ranchi, Jharkhand
Source of Support: None, Conflict of Interest: None
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 Jun 26];29:179-80. Available from: http://www.mmj.eg.net/text.asp?2016/29/1/179/179012
| Introduction|| |
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.
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|| |
Nutritional vitamin-B 12 deficiency is very common in India . 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 . Other blood cell lines may also be involved, producing pancytopenia in the peripheral blood smear  (as in our case). The features of hemolysis can occur because of ineffective erythropoiesis in association with overt anemia . 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 , whereas patients with severe hematologic disease may have no neurologic disease .
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 ,. 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|| |
Conflicts of interest
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