Case Report: Multisystem Langerhans Cell Histiocytosis (LCH) and Myelodysplastic Syndrome (MDS) in a 13-Month-Old Female

Authors

DOI:

https://doi.org/10.21776/ub.jkb.2021.031.03.10

Keywords:

angerhans cell histiocytosis, myelodysplastic syndrome

Abstract

Langerhans Cell Histiocytosis or LCH and Myelodysplastic syndrome or MDS in children are rare diseases. It is estimated to be 1 to 4 cases per 1 million population and less than 5% of hematologic cases. MDS in LCH can occur due to genetic predisposition, impaired cytokine production, or secondary to chemotherapy.  The article reported a patient case of a 13-month-old female who came to hospital with paleness since two weeks before admission. The patient also experienced skin redness, abdominal distention, and weight loss. From the physical examination, anemia, maculopapular rash, and hepatosplenomegaly were obtained. From the laboratory test, anemia of hypochromic anisopoikilocytosis, monocytosis, thrombocytopenia, and hypoalbuminemia were obtained. On examination of bone marrow aspiration, MDS with Refractory Cytopenia of Childhood type or RCC was obtained. Positive results confirmed the diagnosis of Langerhans Cell Histiocytosis on CD68 and S100 in histopathological examinations.

Downloads

Download data is not yet available.

Author Biography

Dian Sukma Hanggara, Brawijaya University

Clinical Pathology

References

Kaushansky K, Lichtman MA, Prchal JT, et al. Williams Hematology. 9th edition. New York: McGraw-Hill Education; 2016.

Jezierska M, Stefanowicz J, Romanowicz G, Kosiak W, and Lange M. Langerhans Cell Histiocytosis in

Children – A Disease with Many Faces. Recent Advances in Pathogenesis, Diagnostic Examinations and Treatment. Advances in Dermatology and Allergology. 2018; 35(1): 6-17.

Yi X, Han T, Zai H, Long X, Wang X, and Li W. Liver Involvement of Langerhans cell histiocytosis in Children. International Journal of Clinical and Experimental Medicine. 2015; 8(5): 7098-7106.

Malempati S and Nicholson HS. Langerhans Cell Histiocytosis. In: Greer JP, Foerster J, Rodgers GM, et al. (Eds). Wintrobe's Clinical Hematology, 12th Edition. Philadelphia: Lippincott Williams & Wilkins; 2009: p. 1307-1316.

Donadieu J, Chalard F, and Jeziorski E. Medical Management of Langerhans Cell Histiocytosis From Diagnosis to Treatment. Expert Opinion on Pharmacotherapy. 2012; 13(9): 1309-1322.

Locatelli F and Strahm B. How I Treat Myelodysplastic Syndrome of Childhood. Blood. 2018;131(13):1406-1414.

Parmawati NKA, Herawati S, and Wande IN. Myelodysplastic Syndrome(MDS) in a Child Who Transformed to Acute Myeloid Leukaemia with Myelodysplasia-Related Changes(AML-MRC). Bali Medical Journal. 2019; 8(2): 358-362.

Haupt R, Astigarraga I, Scaefer E, et al. Langerhans Cell Histiocytosis(LCH): Guidelines for Diagnosis, Clinical Work-Up and Treatment During Childhood. (Online) 2011. https://www.eurohistio.net/ e1623/e1554/e1856/index_eng.html

Gaspar BL, Sharma P, and Das R. Anemia in Malignancies: Pathogenetic and Diagnostic Considerations. Hematology. 2015; 20(1): 18-25.

Maccio A, Madeddu C, Gramignano G, et al. The Role of Inflammation, Iron, and Nutritional Status in Cancer-Related Anemia: Results of a Large Prospective Observational Study. Haematologica. 2015;100(1): 124-132.

Murakami J and Shimizu Y. Hepatic Manifestations in Hematological Disorders. International Journal of Hepatology. 2013; 2013: 1-13.

Hung J and Chang AM. Langerhans Cell Histiocytosis of the External Auditory Canal in an Adult Patient with Myelodysplastic Syndrome: A Case Report. Philippine Journal of Pathology. 2017; 2(1): 34-37.

Slade JM, Korman S, Khan B, Jakate SM, Reddy VB, and Miller IJ. A Unique Case of a Myelodysplastic/ Myeloproliferative Neoplasm with Distinct Histiocytic and Dendritic Cell Outgrowths. Journal of Hematopathology. 2015; 8(2); 85-93.

Edelbroek JR, Vermeer MH, Jansen PM, et al. Langerhans Cell Histiocytosis First Presenting In The Skin In Adults: Frequent Association With A Second Haematological Malignancy. The British Journal of Dermatology. 2012; 167(6): 1287-1294.

Resende C, Marques H, Pereira T, Pardal F, Torres F, and Paiva A. Langerhans Cell Histiocytosis and Myelodysplastic Syndrome: A Casual Association or Pathogenetic Correlation? Journal of Dermatology. 2014; 1(6): 1-5

Downloads

Published

2021-02-27

Issue

Section

Case Report

Most read articles by the same author(s)