Langerhans Cells Histiocytosis: Features of Clinical and Laboratory Manifestations and Course of the Disease

O.I. Dorosh, I.P. Tsymbalyuk-Voloshyn, R.S. Polishchuk, L.Ya. Dubey, O.I. Vorobel, O.I. Kozlova, O.O. Troyanovska, O.I. Stepanyuk, L.L. Skoropad, N.I. Kitsera, L.P. Seredych, A.M. Mykh, N.B. Gryshchuk, A.I. Kuzmenko

Abstract


The aim of the research. To specify the features of clinical symptoms, course of the disease and the efficacy of treatment for Langerhans cells histiocytosis (LCH) in children.
Methods of the Study. Clinacal, haemotological, biochemical, histological, immunohistochemical, radiological ones.
Results of the Study. An analysis of 25 cases of LCH in children was presented. Monosystem LCH most often affects the skeletal system. Multisystem LCH is characterized by diversity of clinical manifestations, more severe course and high risk of death. One third of patients with multisystem LCH are infants. In children with monosystem LCH we observed complete clinical response to first-line therapy. At the same time, complete response to polychemotherapy is observed only in 30 % of children with multisystem LCH. Prognosis of the disease depends on the initial affection of risk organs (bone marrow, liver, lungs, spleen), their dysfunction and the child’s age at the time of diagnosis. Process reactivation in children with multisystem LCH occurs in the first 12 months from the onset of the disease.


Keywords


children; Langerhans cells histiocytosis

References


Akkari V. Hematopoietic stem cell transplantation in patients with severe Langerhans cell histiocytosis and hematological dysfunction: experience of the French Langerhans Cell Study Group // Bone Marrow Transplant. — 2003. — 31 (12). — 1097-1103.

Arceci R., Brenner M., Pritchard J. Controversies and new approaches to treatment of Langerhans cell histiocytosis // Hematol. Oncol. Clin. North. Am. — 1998. — 12 (2). — 339-357.

Bhatia S., Nesbit M., Egeler R. et al. Epidemiologic study of Langerhans cell histiocytosis in children // J. Pediatr. — 1997. — 130. — 774-784.

Broadbent V., Gadner H. Current therapy for Langerhans cell histiocytosis // Hematol. Oncol. Clin. North. Am. — 1998. — 12. — 327-338.

Caselli D., Arico M. The role of BMT in childhood histiocytoses // Bone Marrow Transplant. — 2008. — 41 (2). — 8-13.

Conter V., Reciputo A., Arrigo C. et al. Bone marrow transplantation for refractory Langerhans’ cell histiocytosis // Haematologica. — 1996. — 81 (5). — 468-471.

Danger D., Broadbent В., Veoman E. et al. The frequency and natural history of diabetes insipidus in children with Langerhans cell histiocytosis // N. Engl. J. Med. — 1989. — 321. — 1157.

Gadner H., Grois N., Arico M. et al. A randomized trial of treatment for multisystem Langerhans’ cell histiocytosis // J. Pediatr. — 2001. — 138 (5). — 728-734.

Gadner H., Grois N., Potschger U. et al. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification // Blood. — 2008. — 111 (5). — 2556-2562.

Gadner H., Minkov M., Grois N. et al. Therapy prolongation improves outcome in multisystem Langerhans cell histiocytosis // Blood. — 2013. — 121 (25). — 5006-5014.

Greinix H.T., Storb R., Sanders J.E. Marrow transplantation for treatment of multisystem progressive Langerhans cell histiocytosis // Bone Marrow Transplant. — 1992. — 10 (1). — 39-44.

Grois N. Central nervous system disease in Langerhans cell histiocytosis // Hematol. Oncol. Clin. North Am. — 1998. — 12 (2). — 287-305.

Haupt R., Nanduri V., Calevo M.G. et al. Permanent consequences in Langerhans cell histiocytosis patients: a pilot study from the Histiocyte Society-Late Effects Study Group // Pediatr. Blood Cancer. — 2004. — 42 (5). — 438-444.

Imamura T., Sato T., Shiota Y. et al. Outcome of pediatric patients with Langerhans cell histiocytosis treated with 2 chlorodeoxyadenosine: a nationwide survey in Japan // Int. J. Hematol. — 2010. — 91 (4). — 646-651.

Jakobson A., Kreuger A., Hagberg H. et al. Sundstrom C. Treatment of Langerhans cell histiocytosis with alpha-interferon // Lancet. — 1987. — 2 (8574). — 1520-1521.

Kanavi M., Javadi F, Javadi M. et al. Unifocal Langerhans cell histiocytosis simulating a limbal papilloma // J. Ophtalmic Vis. Res. — 2012. — 7 (3). — 240-243.

Kesik V., Citak C., Kismet E., Koseoglu V., Akyuz C. Hematopoietic stem cell transplantation in Langerhans cell histiocytosis: case report and review of the literature // Pediatr. Transplant. — 2009 May. — 13(3). — 371-374.

Krstovski N., Janic D., Docmanovic L. et al. Clinical characteristics and survival of children with Langerhans cell histiocytosis // Spr. Arh. Celok. Lek. — 2008. — 136 (9–10). — 514-518.

McCowage G., Frush D., Kurtzberg J. Successful treatment of two children with Langerhans’ cell histiocytosis with 2’-deoxycoformycin // J. Pediatr. Hematol. Oncol. — 1996. — 18 (2). — 154-158.

Melanud A., Efrat M., Sova Y. Epibular Module as a Symptom of Langerhans cell histiocytosis // Arch. Ophtalmol. — 2002. — 120 (10). — 1400-1401.

Minguez I., Minguez J., Bonet J. et al. Oral manifestations of chronic disseminated histiocytosis. A report of 10 cases // Med. Oral. — 2004. — 9. — 149-154.

Minkov M. Treatment of multisys tem Langerhans cell histiocytosis. Results of the DALHX 83 and DALHX 90 studies. DAL HX Study Group // Klin. Padiatr. — 2000. — 212 (4). — 139-144.

Minkov M., Grois N., Broadbent V. et al. Cyclosporine A therapy for multisystem langerhans cell histiocytosis // Med. Pediatr. Oncol. — 1999. — 33 (5). — 482-485.

Minkov M., Grois N., Heitger A. et al. Response to initial treatment of multisystem Langerhans cell histiocytosis: an important prognostic indicator // Med. Pediatr. Oncol. — 2002. — 39 (6). — 581-585.

Mosterd K., van Marion A., van Steensel M. Neonatal Langerhans cell histiocytosis: a rare and potentially life-threatening disease // Int. J. Dermatol. — 2008. — 47 (1). — 10-12.

Perek D. Histiocytosis X u dzieci w świetle własnych obserwacji // Ped. Pol. — 1982. — 7–8. — 555-564.

Pollono D., Rey G., Latella A. et al. Reactivation and risk of sequelae in Langerhans cell histiocytosis // Pediatr. Blood. Cancer. — 2007. — 48 (7). — 696-699.

Rapp G., Motta A Periodontal Disease Associated with Langerhans’ Cell. Histiocytosis: Case Report // Braz. Dent. J. — 2000. — 11 (1). — 59-66.

Saven A., Foon K., Piro L. 2-Chlorodeoxyadenosine-induced complete remissions in Langerhans-cell histiocytosis // Ann. Intern. Med. — 1994. — 121 (6). — 430-437.

Skierski T., Welfel L., Grzybowski W. Problematyka histiocytosis X w laryngologii // Otolaryngol. Pol. — 1994. — 48 (18). — 347-351.

Stine K., Saylors R., Williams L. et al. 2-Chlorodeoxyadenosine (2-CDA) for the treatment of refractory or recurrent Langerhans cell histiocytosis (LCH) in pediatric patients // Med. Pediatr. Oncol. — 1997. — 29 (4). — 288-292.

Vosoghi H., Rodriguez-Galindo C., Wilson M. Orbital involutment in Langerhans cell histiocytosis // Ophthal. Plast. Reconstr. Surg. — 2009. — 25. — 430-433.

Weitzman S., Braier J., Donadieu J. et al. 2’-Chlorodeoxyadenosine (2-CdA) as salvage therapy for Langerhans cell histiocytosis (LCH). Results of the LCH-S-98 protocol of the Histiocyte Society // Pediatr. Blood. Cancer. — 2009. — 53 (7). — 1271-610.

Weitzman S., Wayne A., Arceci R. et al. Nucleoside analogues in the therapy of Langerhans cell histiocytosis: a survey of members of the histiocyte society and review of the literature // Med. Pediatr. Oncol. — 1999. — 33 (5). — 476-481.

Валиев Т., Махонова Л., Ковригина А. и др. Случай врожденного лангергансоклеточного гистиоцитоза у ребенка раннего возраста // Онкогематология. — 2011. — 2. — 19-22.

Коколина В., Румянцев А. Практическое руководство по детским болезням. — М.: Медпрактика-М, 2004. — Т. 4. — 578, 581, 586.

Луговская С., Лукина Е., Цветаева Н.В. Морфофункциональная характеристика мононуклеарных фагоцитов лейкоконцентрата венозной крови больных гистиоцитозами // Тер. архив. — 1994. — 4. — 49-53.

Лукина Е. Гистиоцитозы как заболевания макрофагальной системы // Тер. архив. — 1996. — 7. — 82-88.

Махаонова Л., Дурнов Л. Гистиоцитарные заболевания у детей. — М.: Медицинское информационное агентство, 2004. — 103 с.

Махонова Л., Морозова О., Попа А. Современные проблемы терапии гистиоцитоза из клеток Лангерганса у детей (обзор литературы) // Детская онкология. — 2005. — 4. — 41-48.

Минков М. Лечение незлокачественных гистиоцитозов у детей: Автореф. дис... д-ра мед. наук. — М., 2005.

Минков М., Гаднер Х. Гистиоцитоз из клеток Лангерганса: результаты кооперированных исследований // Вопр. гематологии/онкологии и иммунопатологии в педиатрии. — 2004. — 3. — 7-10.

Минков М., Новичкова Г., Цельгер Г. и др. Гистиоцитозы детского возраста. — Москва — Вена: Макс-Пресс, 2005. — 30-36.

Солопова Г., Байдильдина Д., Жарикова Л. и др. Применение 2-хлордезоксиаденозида в терапии гистиоцитоза из клеток Лангерганса у детей // Онкогематология. — 2010. — 3. — 8-15.




DOI: https://doi.org/10.22141/2224-0551.5.56.2014.76242

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