Email this article Langerhans cell histiocytosis: a rare cause of exudative enteropathy in a young child
- Authors: Movsisyan G.B.1, Komarova A.D.1, Kulikov K.A.1, Kalinina I.I.2, Lokhmatov M.M.1, Oldakovskiy V.I.1, Tepaev R.F.1, Roslavtseva E.A.1, Potapov A.S.1, Shchigoleva N.E.1, Materikin A.I.1, Savostyanov K.V.1
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Affiliations:
- National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
- Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthсare of the Russian Federation
- Issue: Vol 22, No 1 (2023)
- Pages: 110-121
- Section: CLINICAL OBSERVATIONS
- Submitted: 12.09.2022
- Accepted: 28.02.2023
- Published: 14.02.2023
- URL: https://hemoncim.com/jour/article/view/700
- DOI: https://doi.org/10.24287/1726-1708-2023-22-1-110-121
- ID: 700
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Abstract
Exudative enteropathy is a clinical syndrome characterized by excessive loss of proteins through the gastrointestinal tract and is a rare complication of various gastrointestinal diseases. One of the rarest causes of protein malabsorption is Langerhans cell histiocytosis, which occurs as a result of inflammatory neoplasia of myeloid precursor cells caused by mutations in the mitogenactivated protein kinase pathway. Abnormal proliferation and accumulation of Langerhans cells in the intestinal wall leads to a violation of the outflow of lymph, and causes clinical manifestations characteristic of enteropathy. Given that the lesion of the gastrointestinal tract with histiocytosis from Langerhans cells occurs in 2–3% of cases, and the clinical signs are not highly specific, timely diagnosis is difficult. Delayed verification of the diagnosis and late initiation of adequate treatment are risk factors for multisystem lesions and lead to an unfavorable outcome. The literature describes a few observations of the onset or manifestation of the disease with symptoms of protein malabsorption. We present a rare clinical case of diagnosing histiocytosis from Langerhans cells in a young child with severe manifestations of exudative enteropathy at the onset. The patient's parents gave their consent to the use of their child's data, including photographs, for research purposes and in publications.
Refractory gastrointestinal symptoms require mandatory endoscopic and histological examination to identify rare causes of malabsorption. Timely initiation of targeted therapy with vemurafenib in combination with subsequent chemotherapy provided the child with a favorable prognosis and stable remission of the disease.
About the authors
G. B. Movsisyan
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
Author for correspondence.
Email: Movsisyan@nczd.ru
ORCID iD: 0000-0003-2881-4703
Goar B. Movsisyan, Cand. Med. Sci., a senior researcher at the Laboratory of Rare Hereditary Diseases in Children, a pediatrician, a gastroenterologist of the Gastroenterology Department with Hepatology Group
2, building 1, Lomonosovskiy Avenue, 119991, Moscow
Russian FederationA. D. Komarova
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0001-9064-6443
Moscow
Russian FederationK. A. Kulikov
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0002-1107-8693
Moscow
Russian FederationI. I. Kalinina
Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0002-0813-5626
Moscow
Russian FederationM. M. Lokhmatov
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0002-8305-7592
Moscow
Russian FederationV. I. Oldakovskiy
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0002-8805-8164
Moscow
Russian FederationR. F. Tepaev
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0001-6667-9472
Moscow
Russian FederationE. A. Roslavtseva
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0002-3993-1246
Moscow
Russian FederationA. S. Potapov
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0003-4905-2373
Moscow
Russian FederationN. E. Shchigoleva
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
Moscow
Russian FederationA. I. Materikin
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0002-6034-8231
Moscow
Russian FederationK. V. Savostyanov
National Medical Research Center for Children’s Health of Ministry of Healthсare of the Russian Federation
ORCID iD: 0000-0003-4885-4171
Moscow
Russian FederationReferences
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