Hematological manifestation of scurvy

Scurvy is caused by the deficiency of vitamin C. It is thought to be uncommon in the 21st century; however, recent researches show that micronutrient deficiency, especially poor dietary vitamin C intake, is one of the growing public health concerns even in developed countries [1, 2]. Despite this trend, nutrition is still frequently overlooked by healthcare professionals because of the nonspecific nature of the relevant symptoms. Given that scurvy is frequently associated with anemia [3], little is known for the clinical picture of the defective erythropoiesis, especially in children. A 4-year-old male with autism spectrum disorder presented with progressive wasting for the last 6 months. His developmental milestone was normal until 2 years of age, but the following growth curve was severely suppressed with unbalanced diet for preference for fried potato and white bread and with less intake of other essential nutrients. Inspection of the oral cavity showed gingival hyperplasia and bleeding. He developed mild megaloblastic anemia; hemoglobin of 9.6 g/dL (normal range: 11.0–14.2) and mean corpuscular volume of 105 fL (normal range: 73–86). Both platelet count and coagulation profiles were normal. Peripheral blood film was characterized with oral shaped macrocytes intermixed with hyperchromic spherocytes (red arrows). Roentgenogram showed a transverse radiolucent Trümmerfeld zone (white arrowhead) and a dense Frankel line (yellow arrows) at the end of metaphysis. Serum levels of copper, zinc, iron and vitamin B12 were maintained within normal ranges, but both folate and vitamin C were significantly suppressed. Based on the series of classical clinical findings and compromised dietary condition, he was finally diagnosed as having scurvy and treated with ascorbic acid and food fortification, which successfully reverted the concurrent growth failure and hematological manifestations. Vitamin C is involved not only in the absorption of non-heme iron and folate from the gut, but also in the stabilization of cytoskeletal protein of collagen and beta-spectrin to ensure the deformability of red blood cells that protect them from intravascular hemolysis [4]. Although the outer clinical manifestations of scurvy are often overlooked due to the non-specific nature of the symptoms, we would like to suggest that the presence of hyperchromic spherocytes as a result of loss of membrane stability coupled with ineffective erythropoiesis is one of the hematological manifestations of the malnutrition associated with vitamin C depletion. We therefore believe that vigilant surveillance for both nutritional status and blood film evaluation to uncover the underlying nutritional status might be helpful to make an early diagnosis of scurvy and to employ an appropriate prevention measure for high-risk population, such as children with eating disorder, adults with substance abuse and isolated elderly persons with poor nutrition [2, 4].

AUTHOR CONTRIBUTIONS

KK conceptualized the research scheme, performed data curation and the formal analysis and wrote the paper. TT, SI, IU, TM and TH analyzed the data. All authors approved the final revision of the manuscript to be published.

CONFLICT OF INTEREST STATEMENT

On behalf of all the authors, the corresponding author states that there is no conflict of interest to declare.

FUNDING INFORMATION

The authors received no specific funding for this work.

ETHICS STATEMENT

Written informed consent was obtained from legal guardians for publication of this case report and any accompanying images. The authors affirm that no identifiable images/data of the patients have been utilized in the preparation of the paper.

DATA AVAILABILITY STATEMENT

The data that support the findings of this study are available from the corresponding author upon reasonable request.