ISSN (print) 1997-6933     ISSN (online) 2500-2139
Vol. 17 No. 3 (2024), CASE REPORTS
Vol. 17 No. 3 (2024)
CASE REPORTS

Cladribine in the Treatment of Multifocal Multisystem Langerhans Cell Histiocytosis in a Patient with a Poor Prognosis. A Case Report

Published 01.07.2024
Vsevolod Gennadevich Potapenko
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
https://orcid.org/0000-0003-2985-0503
E.P. Burlachenko
SM Clinic Medical Center, 17-1 Vyborgskoe sh., Saint Petersburg, Russian Federation, 194355
J.-F. Emile
Ambroise Pare Hospital, 9 Charles de Gaulle Avenue, Boulogne-Billancourt, France, 92100; University of Versailles Saint-Quentin-en-Yvelines, 55 Avenue de Paris, Versailles, France, 78000
https://orcid.org/0000-0002-6073-4466
E.V. Zabutova
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
E.N. Imyanitov
NN Petrov National Medical Cancer Research Center, 68 Leningradskaya ul., pos. Pesochnyi, Saint Petersburg, Russian Federation, 197758
https://orcid.org/0000-0003-4529-7891
O.V. Nebelitskaia
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
https://orcid.org/0009-0009-7592-4957
N.A. Kotova
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
https://orcid.org/0009-0006-9297-2991
V.V. Kozyro
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
I.A. Samorodova
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
https://orcid.org/0000-0001-9321-9084
A.V. Serkov
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
https://orcid.org/0009-0003-4473-6546
K.A. Skoriukova
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
https://orcid.org/0000-0003-4329-5622
E.A. Turkevich
NN Petrov National Medical Cancer Research Center, 68 Leningradskaya ul., pos. Pesochnyi, Saint Petersburg, Russian Federation, 197758
N.V. Medvedeva
City Clinical Hospital No. 31, 3 Dinamo pr-t, Saint Petersburg, Russian Federation, 197110
https://orcid.org/0000-0002-4350-4204
2024-3
PDF_2024-17-3_273-279 (Russian)

Keywords

Langerhans cell histiocytosis
cladribine
diabetes insipidus
V600E mutation in the BRAF gene

How to Cite

Potapenko V.G., Burlachenko E.P., Emile J..-F., Zabutova E.V., Imyanitov E.N., Nebelitskaia O.V., Kotova N.A., Kozyro V.V., Samorodova I.A., Serkov A.V., Skoriukova K.A., Turkevich E.A., Medvedeva N.V. Cladribine in the Treatment of Multifocal Multisystem Langerhans Cell Histiocytosis in a Patient with a Poor Prognosis. A Case Report. Clinical Oncohematology. 2024;(3):273–279. doi:10.21320/2500-2139-2024-17-3-273-279.
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Abstract

Langerhans cell histiocytosis is a hematologic clonal disease. The treatment of localized histiocytosis includes local methods such as radiotherapy or surgery. In cases of disseminated lesions with multiple system damage, chemotherapy is administered, which provides recovery in some patients. The present paper is a case report of multifocal multisystem Langerhans cell histiocytosis with bone, liver, spleen, abdominal lymph node and pituitary lesions in a 40-year-old patient. The diagnosis was verified by histology and immunohistochemistry of bone biopsy specimens. Polymerase chain reaction revealed no V600E mutation in the BRAF gene in tumor tissue. Persistent antitumor response was achieved after 6 chemotherapy cycles with cladribine monoregimen. This drug was well tolerated by the patient. By the time of this publication, the patient had preserved the persistent response for 38 months.

PDF_2024-17-3_273-279 (Russian)

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