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1.
Chinese Journal of Pediatrics ; (12): 1118-1123, 2023.
Article in Chinese | WPRIM | ID: wpr-1013233

ABSTRACT

Objective: To analyze the efficacy, safety, and long-term prognosis of intermediate-dose cytarabine (Ara-c) regimen in the treatment of children with refractory risk organ involvement Langerhans cell histiocytosis (LCH). Methods: Clinical data of 17 children with multisystem and risk organ involvement LCH who failed the first-line therapy and were treated with intermediate-dose Ara-c (250 mg/m2, twice daily) regimen in the Hematology Center, Beijing Children's Hospital from January 2013 to December 2016 were analyzed retrospectively. In addition to the basic treatment of vindesine and dexamethasone, the patients received two regimens: regimen A: the intermediate-dose Ara-c combined with cladribine and regimen B: the intermediate-dose Ara-c alone. The efficacy, safety and prognosis of the two regimens were analyzed. Results: Among all 17 patients, there were 11 males and 6 females, with the diagnosis age of 2.1 (1.6, 2.7) years. Ten children received regimen A, all of them achieved active disease-better (AD-B) after 8 courses of induction therapy. The disease activity scores (DAS) decreased from 5.5 (3.0, 9.0) to 1.0 (0, 2.3). Seven children received regimen B, and 6 of them achieved AD-B after 8 courses of induction therapy. The DAS decreased from 4.0 (2.0, 4.0) to 1.0 (0, 2.0). The follow-up time was 6.2 (4.9,7.2) and 5.2 (3.7,5.8) years in group A and B. The 5-year overall survival rate was 100.0% in both groups, and the 5-year event free survival rate was (88.9±10.5)% and (85.7±13.2)% in group A and B. Grade 3 or 4 myelosuppression was observed in 8 patients in group A and 2 patients in group B. Conclusions: The intermediate-dose Ara-c regimen (with or without cladribine) is effective and safe for patients with refractory high-risk LCH, with a good long-term prognosis.


Subject(s)
Male , Female , Child , Humans , Cytarabine/adverse effects , Cladribine/adverse effects , Retrospective Studies , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Histiocytosis, Langerhans-Cell/drug therapy , Prognosis
2.
Article in Chinese | WPRIM | ID: wpr-939714

ABSTRACT

OBJECTIVE@#To observe the efficacy and prognosis of cladribine (2-CdA) combined with cytarabine (Ara-C) regimen in the treatment of relapsed refractory Langerhans cell histiocytosis (LCH) in children.@*METHODS@#Nine patients with relapsed refractory LCH treated with the 2-CdA combined with Ara-C regimen in the Department of Hematology and Oncology of Wuhan Children's Hospital from July 2014 to February 2020 were retrospectively analyzed, and the efficacy and disease status were evaluated according to the Histiocyte Society Evaluation and Treatment Guidelines (2009) and the Disease Activity Score (DAS), the drug toxicity were evaluated according to the World Health Organization(WHO) grading criteria for chemotherapy. All patients were followed up for survival status and disease-related sequelae.@*RESULTS@#Before the treatment combining 2-CdA and Ara-C, 7 of 9 patients were evaluated as active disease worse (ADW), and 2 as active disease stable (ADS) with a median disease activity score of 8 (4-15). Of 9 patients, 6 cases achieved non active disease (NAD) and 3 achieved active disease better (ADB) with a median disease activity score of 0 (0 to 5) after 2-6 courses of therapy. All 9 patients experienced WHO grade IV hematologic toxicity and 3 patients had hepatobiliary adverse effects (WHO grade I~II) after treatment. The median follow-up time was 31(1 to 50) months with all 9 patients survived, 3 of the 9 patients experienced sequelae to the disease with 2 combined liver cirrhosis as well as cholestatic hepatitis and 1 with oral desmopressin acetate tablets for diabetes insipidus.@*CONCLUSION@#2-CdA combined with Ara-C is an effective regimen for the treatment of recurrent refractory LCH in children, and the main adverse effect is hematologic toxicity, which is mostly tolerated in children. Early treatment with this regimen may be considered for patients with multisystem LCH with risky organ involvement who have failed first-line therapy and for patients with relapse.


Subject(s)
Child , Humans , Cladribine/adverse effects , Cytarabine , Histiocytosis, Langerhans-Cell/drug therapy , Recurrence , Retrospective Studies
3.
Int. j. morphol ; 31(3): 1137-1145, set. 2013. ilus
Article in Spanish | LILACS | ID: lil-695013

ABSTRACT

La Histiocitosis de células de Langerhans (HCL) corresponde a una proliferación anormal de células dendríticas, de tipo clonal, cuyo espectro clínico general incluye compromiso de la piel y las mucosas, las uñas, el hueso, la médula ósea, el hígado, el bazo, linfonodos, el pulmón, el tracto gastrointestinal inferior, el sistema endocrino y el sistema nervioso central. En este trabajo presentamos tres casos de la enfermedad, con manifestaciones orales y craneofaciales, analizadas desde el punto de vista clínico (examen extra e intra oral), imagenológico (tomografías computadas) e histopatológico (expresión de marcador específico CD1a). Dos casos fueron clasificados como HCL de presentación aguda diseminada y uno como presentación crónica. Los pacientes fueron tratados oportunamente con quimioterapia según el protocolo del Programa Infantil Nacional de Drogas Antineoplásicas.


The Langerhans cell histiocytosis (LCH) corresponds to an abnormal proliferation of dendritic cells, clonal type, which usually involves compromise of skin and mucous membranes, nails, bone, bone marrow, liver, spleen, lymph nodes, lung, lower gastrointestinal tract, endocrine system and the central nervous system. We present three cases of the disease, with oral and craniofacial manifestations, analyzed from the clinical perspective (intra and extra oral exam), imaging (CT scans) and histopathological (specific marker CD1a expression). Two cases were classified as acute disseminated LCH presentation and one as a chronic disease. Patients were treated with chemotherapy timely according to the protocol of the National Child Program of Antineoplastic Drugs.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Bone Diseases/pathology , Mouth Diseases/pathology , Histiocytosis, Langerhans-Cell/pathology , Clinical Protocols , Face/pathology , Skull/pathology , Bone Diseases/diagnosis , Bone Diseases/drug therapy , Mouth Diseases/diagnosis , Mouth Diseases/drug therapy , Histiocytosis, Langerhans-Cell/drug therapy , Immunohistochemistry , Tomography, X-Ray Computed
4.
An. bras. dermatol ; 86(4,supl.1): 78-81, jul,-ago. 2011. ilus
Article in Portuguese | LILACS | ID: lil-604127

ABSTRACT

A histiocitose de células de Langerhans é representante de um raro grupo de síndromes histiocitárias, sendo caracterizada pela proliferação das células de Langerhans. Suas manifestações variam de lesão solitária a envolvimento multissistêmico, sendo o acometimento vulvar incomum. Segue-se o relato de caso refratário da doença limitada à pele, em mulher de 57 anos. A paciente apresentava história de pápulas eritematosas ulceradas em couro cabeludo, face, vulva, tronco e axila há seis anos. O diagnóstico da doença é difícil, sendo confirmado neste caso através de estudo imuno-histoquímico e se obteve resposta terapêutica e eficaz, com a administração de talidomida.


Langerhans cell histiocytosis is a member of a group of rare histiocytic syndromes and is characterized for the proliferation of histiocytes called Langerhans'cells. Its manifestations vary from a solitary injury to systemic involvement, and vulvar lesions are uncommon. We describe a refractory case of cutaneous limited disease in a 57-year-old woman. She presented with a 6-year history of an erythematous papular eruption of the scalp, face, vulva, trunk and axillae. The diagnosis is difficult and in this case it was confirmed through immunohistochemical study and clinical improvement was achieved with thalidomide.


Subject(s)
Female , Humans , Middle Aged , Histiocytosis, Langerhans-Cell/drug therapy , Immunosuppressive Agents/therapeutic use , Skin Diseases/drug therapy , Thalidomide/therapeutic use , Vulvar Diseases/drug therapy , Facial Dermatoses/drug therapy , Facial Dermatoses/pathology , Histiocytosis, Langerhans-Cell/pathology , Scalp Dermatoses/drug therapy , Scalp Dermatoses/pathology , Skin Diseases/pathology , Vulvar Diseases/pathology
5.
Arq. bras. neurocir ; 29(1): 32-36, mar. 2010. ilus
Article in Portuguese | LILACS | ID: lil-585501

ABSTRACT

Histiocitose de células de Langerhans é uma doença uni ou multifocal que acomete osso, tecido mole ou ambos. Relativamente incomum, abrange entidades clínicas anteriormente conhecidas como doença de Hand-Schüller-Christian, Abt-Letterer-Siwe, Hashimoto-Pritzker, granuloma eosinofílico e histiocitose X. Responsável por menos de 1% dos tumores ósseos, muito raramente leva a acometimento neurológico. Sua etiologia é desconhecida e seu tratamento, controverso. A ocorrência de um caso com comprometimento neurológico nos motivou a este relato. Paciente do sexo feminino, 4 anos deidade, com processo expansivo de C7 a T3 e paraparesia crural; após a cirurgia apresentou melhora importante já no segundo pós-operatório. Encontra-se em terapia complementar com corticoide e quimioterapia estando no nono mês de tratamento. Na revisão da literatura foram encontrados poucos relatos de manejo cirúrgico desse tipo de lesão, em razão de sua característica de doença autolimitada. O tratamento adequado pode variar dependendo das características do paciente e de seu comprometimento neurológico.


Langerhans cell histiocytosis is a uni- or multifocal disease that affects bone, soft tissue, or both. Relatively uncommon, covers clinical entities previously known as Hand-Schüller-Christian disease,Abt-Letterer-Siwe, Hashimoto-Pritzker, eosinophilic granuloma and histiocytosis X. Responsible forless than 1% of bone tumors, rarely causes neurological impairment. Its etiology is unknown and the treatment controversial. We report a rare case with neurological impairment. A 4 year-old female children, presented with paraparesis due to expansive process at C7 to T3; soon after surgery showed significant improvement in the second postoperative day; she is in complementary therapy with corticosteroids and chemotherapy in the ninth month of treatment. In the review of the literature we found few reports ofsurgical management of this type of injury, due to its characteristic of self-limited disease. The appropriate treatment may vary depending on the characteristics of patients and their neurological impairment.


Subject(s)
Humans , Female , Child, Preschool , Spinal Cord Diseases/etiology , Histiocytosis, Langerhans-Cell/surgery , Histiocytosis, Langerhans-Cell/complications , Histiocytosis, Langerhans-Cell/drug therapy
7.
Odontol. pediatr. (Lima) ; 8(1): 26-30, ene.-jun. 2009. ilus
Article in Spanish | LILACS, LIPECS | ID: lil-565335

ABSTRACT

La histiocitosis de células de Langerhans es una entidad de etiología no bien establecida, antes conocida como Histiocitosis X. Engloba la triada de enfermedades: granuloma eosinófilo, la enfermedad de Hand-Schüller-Christian y la enfermedad de Letterer-Siwe. Su comportamiento clínico va desde una forma localizada hasta formas generalizadas afectando huesos, hígado, bazo, médula ósea y piel, entre otros. El presente reporte describe el caso de una niña de 3 años de edad que presenta nodulaciones en paladar duro, hiperplasia gingival en ambos maxilares, movilidad dentaria, gingivorragia y halitosis. A la cual le realizan examen de imágenes observando lesiones líticas, alteración de la densidad ósea, dientes flotantes, ausencia de gérmenes dentarios. No hubo evidencia de enfermedad en otros huesos, órganos o tejidos. La biopsia confirma el diagnóstico de Histiocitosis de células de Langerhans, iniciando tratamiento con quimioterapia y conjuntamente odontológico. Actualmente la niña ha completado su tratamiento oncológico con respuesta completa y es portadora de prótesis parcial removible en ambos maxilares.


Langerhans Cell Histiocytocis (LCH) is an entity of uncertain etiology, known previously as Histiocytosis X. It includes the following diseases: eosinophilic granuloma, Hand-Schuller-Christian disease and Letterer-Siwe disease. The clinical behavior goes from a localized lesion to a generalized involvement affecting bones, liver, spleen, bone narrow and skin. Ths report describes the case of a 3-year-old girl who presented with nodulesin the heart palate, gum hyperplasia of both maxilary bones, loose teeth, gum bleeding and halitosis. X-ray exams revealed lytic lesions, abnormal bone density, floating teeth, absence of dental germs. There was no evidence of disease in other bones, organs or tissues. A surgical biopsy was performed and confirmed the diagnosis of LCH. Chemotherapy treatment was begun as well as dental care. The patient has completed chemotherapy at this time with a complete response. She is wearing a partial removable prosthesis for both maxilary bones.


Subject(s)
Humans , Female , Child, Preschool , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/etiology , Histiocytosis, Langerhans-Cell/drug therapy , Chediak-Higashi Syndrome
9.
Rev. venez. oncol ; 20(2): 91-97, abr.-jun. 2008. ilus
Article in Spanish | LILACS | ID: lil-549503

ABSTRACT

La histiocitosis de Langerhans, es un espectro de trastornos poco común y mal comprendido. Se desconoce la etiología de la enfermedad a pesar los estudios. El cuadro clínico depende de la extensión de la enfermedad y del tejido u órgano comprometido. El compromiso gastrointestinal es excesivamente raro y cambia el rango de severidad de la enfermedad. Se presenta el caso de un niño con encefalopatía crónica no progresiva con tumoración abdominal y síntomas digestivos de mes y medio de evolución en quien se plantea diagnóstico de tumor gástrico vs., linfoma, los hallazgos anatomopatológicos e inmunohistoquímica dan cuenta de histiocitosis gástrica. Se indica tratamiento con quimioterapia según protocolo LCH-III segundo para enfermedad de alto riesgo con prednisona y vinblastina con respuesta favorable al finalizar el primer ciclo de 6 semanas de tratamiento. Se reportan pocos casos con compromiso de intestino delgado y ocasionalmente colon, no se encontró reportes de compromiso gástrico.


Langerhans cell histiocytosis is a spectrum of infrequent and poorly understood diseases. Etiology remains essentially unknown despite of number studies. Clinical manifestation depends of the extension of disease and organs compromised. The gastrointestinal involvement is extremely rare and changes the staging of the disease, its presence is indicative of multisystemic disease and aggressive treatment should be considered. The case reported is of a seven years old child with chronic non progressive encephalopathy and epigastric mass, gastrointestinal symptoms for one and half months, diagnostics proposed where gastric tumor, vs. lymphoma, however the anatomophatologic and immunohistochemical evidence concluded in gastric histiocytosis. Treatment with quimioteraphy was according to Protocol LCH-III second for high risk disease with prednisone and vinblastin during six weeks, toward the end of the first cycle patient showed good response Literature review reports few cases with small intestine and colon involvement, no reports with gastric involvement where found.


Subject(s)
Humans , Male , Child , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/drug therapy , Stomach Neoplasms/surgery , Stomach Neoplasms/pathology , Tomography/methods , Brain Damage, Chronic/pathology , Abdominal Pain/diagnosis , Medical Oncology
10.
Indian J Cancer ; 2007 Oct-Dec; 44(4): 137-41
Article in English | IMSEAR | ID: sea-51310

ABSTRACT

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disorder characterized by clonal proliferation of immature and abnormal bone marrow derived langerhans cells. Treatment is usually multimodal. Potent anti-monocyte as well as immunomodulatory activity of 2-CDA and its proven efficacy in many lymphoproliferative disorders has made 2-CDA a rational choice in treatment of LCH. AIM: To evaluate the efficacy and toxicity profile of 2-CDA in children with relapsed or refractory LCH. SETTING AND DESIGN: This is a pilot study and we present the initial data of the first seven patients treated at our institution. MATERIALS AND METHODS: Seven patients of relapsed and refractory LCH were enrolled from July 2000 to June 2004. The cohort of seven patients included six males and one female with a median age at initiation of cladribine was 2.25 years (range, 1.67 to 7.0 years). Three patients had received one prior chemotherapy regimen while the rest were heavily pretreated. Cladribine was administered over two hours IV daily for five days and repeated every four weeks. RESULTS: After a median of six courses of cladribine (range, 2 to 9), two (33%) patients achieved PR and two (33%) patients have SD on imaging but are clinically better. None experienced grade 3 or 4 hematologic toxicity. At a median follow-up of 19 months (range, 8 to 52 months), five patients remain alive and one patient has died. CONCLUSION: Our study shows that single agent 2-CDA is active and well-tolerated in children with relapsed or refractory LCH.


Subject(s)
2-Chloroadenosine/adverse effects , Antimetabolites, Antineoplastic/adverse effects , Antineoplastic Agents/adverse effects , Child, Preschool , Cladribine/adverse effects , Deoxyadenosines/adverse effects , Drug-Related Side Effects and Adverse Reactions , Female , Histiocytosis, Langerhans-Cell/drug therapy , Humans , Infant , Male , Pilot Projects , Prospective Studies , Time Factors
11.
J. pediatr. (Rio J.) ; 83(1): 79-86, Jan.-Feb. 2007. tab, graf
Article in English | LILACS | ID: lil-444532

ABSTRACT

OBJETIVOS: Descrever a apresentação clínica da histiocitose das células de Langerhans e comparar sua evolução de acordo com a idade, estadiamento e resposta ao tratamento. MÉTODOS: Análise retrospectiva dos dados referentes a 33 crianças com histiocitose das células de Langerhans acompanhadas no Hospital das Clínicas da Universidade Federal de Minas Gerais no período de 1988 a 2004. RESULTADOS: A idade ao diagnóstico variou de 2 meses a 16 anos (mediana: 2,5 anos). Dezessete crianças eram do sexo masculino. O tempo de seguimento variou de 21 dias a 16,2 anos (mediana: 3,4 anos). As manifestações clínicas mais comuns ao diagnóstico foram lesões osteolíticas, linfadenomegalia e lesões cutâneas. A sobrevida global para todo o grupo foi de 86,1 por cento aos 16 anos (IC95 por cento 66,6-94,6). Os óbitos ocorreram em pacientes com doença multissistêmica e disfunção orgânica ao diagnóstico. Os pacientes que apresentaram resposta "melhor" à sexta semana de tratamento apresentaram uma probabilidade estimada de sobrevida global significativamente maior em relação aos que apresentaram progressão da doença. A sobrevida global foi significativamente maior para os pacientes com doença em um único sistema. A probabilidade de sobrevida livre de eventos para todo o grupo foi de 30,9 por cento aos 16 anos (IC95 por cento 15,6-47,5), sendo significativamente maior para os portadores de doença em um único sistema. A idade não se associou com a sobrevida livre de eventos. A seqüela mais comum foi o diabetes insipidus. Não foram observados casos de neoplasias secundárias. CONCLUSÃO: A histiocitose das células de Langerhans apresenta uma grande variedade de manifestações clínicas, com alta taxa de recidivas e baixa taxa de mortalidade.


OBJECTIVES: To describe the clinical course of Langerhans cell histiocytosis and to compare its outcome according to age, staging of the disease and treatment response. METHODS: Retrospective analysis of data on 33 children with Langerhans cell histiocytosis followed at Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 1988 and 2004. RESULTS: Age at diagnosis ranged from 2 months to 16 years (median: 2.5 years). Seventeen children were male. The follow-up period varied from 21 days to 16.2 years (median: 3.4 years). The most common clinical manifestations at diagnosis were osteolytic lesions, enlarged lymph nodes and skin lesions. The overall survival rate for the whole group was 86.1 percent at 16 years (95 percentCI 66.6-94.6 percent). Deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Those patients who had a "better" response to treatment in the sixth week were likely to have a significantly higher overall survival rate than those who showed disease progression. Overall survival rate was significantly higher for patients with single-system disease. The disease-free survival rate for the whole group was 30.9 percent at 16 years (95 percentCI 15.6-47.5 percent), and was significantly higher for those with single-system disease. Age groups were not associated with different disease-free survival rates. Diabetes insipidus was the most common sequela. No cases of secondary neoplasms were observed. CONCLUSION: The clinical manifestations of Langerhans cell histiocytosis vary widely, with a high relapse rate and low mortality rate.


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Histiocytosis, Langerhans-Cell/diagnosis , Antibodies, Monoclonal , Antigens, CD1 , Disease-Free Survival , Histiocytosis, Langerhans-Cell/drug therapy , Histiocytosis, Langerhans-Cell/mortality , Immunologic Factors , Prognosis , Recurrence , Retrospective Studies , Survival Rate , Treatment Outcome
12.
Indian J Pediatr ; 2006 Nov; 73(11): 1036-8
Article in English | IMSEAR | ID: sea-84051

ABSTRACT

We describe ultrasonographic and computed tomographic features of hepatic lesions in two cases of disseminated Langerhans' cell histiocytosis affecting children. In the first case, hyperechoic band like periportal lesions were observed at ultrasonography, which on computed tomography was found to be hypodense admixed with fatty attenuation (HU@23 to - 57) at places. In addition, the caudate lobe was very prominent. In the second case, the hepatic parenchyma showed predominantly hyperechoic diffusely heterogeneous echogenicity. There were features of cirrhosis of liver with portal hypertension in the form of atrophy of right lobe with hypertrophy of left lobe of liver with lobulated outline, prominent main portal vein and splenoportal axis, splenomegaly and gastroesophageal varices. Both the patients were put on chemotherapy as per schedule (Protocol: DAL HX - 83) and are on follow up.


Subject(s)
Child, Preschool , Histiocytosis, Langerhans-Cell/drug therapy , Humans , Liver Diseases/drug therapy , Male
13.
Col. med. estado Táchira ; 14(1): 17-23, ene.-mar. 2005. tab, graf
Article in Spanish | LILACS | ID: lil-530730

ABSTRACT

La Histiocitosis de Células de Langerhans, anteriormente denominadas histiocitosis X son un grupo de enfermedades caracterizadas por la proliferación localizada o generalizada de células del sistema monocito-macrofágico y/o dendríticas. Dicha patología es infrecuente y son pocos los casos reportados en nuestro medio. Se realizó revisión de las historias clínicas de los pacientes que durante los años 1989 a 2004 egresaron de los hospitales "Central y Dr. Patrocinio Peñuela Ruíz" de la ciudad de San Cristóbal, Estado Táchira, con el diagnóstico de "Histiocitosis de células de Langerhans". Se evaluaron los datos relacionados con las manifestaciones clínicas, diagnóstico y tratamiento recibidos, encontrando: Un total de 10 pacientes en 15 años, con un grupo promedio de edad de 7,8 años; con un rango de 2 a 21 años. Con mayor incidencia en el sexo femenino con una relación de masculinidad de 0,66 el 100 por ciento de los pacientes tenían compromiso del sistema óseo; y éste mayor predominio en los huesos del cráneo. La adenopatías y la otitis media se encontraron en el 50 por ciento de los pacientes. Solo se encontró esplenomegalia en un paciente, lesiones cutáneas en dos pacientes y compromiso pulmonar en un paciente. Infiltración de médula ósea en dos pacientes y exoftalmos en un paciente. A todos los pacientes se les realizó biopsia de la lesión que permitió la carroboración diagnóstico. El tratamiento recibido por los pacientes fue: solo radioterapia tres pacientes; solo quimioterapia tres pacientes y quimioterapia más radioterapia cuatro pacientes. Se concluye que los casos revisados en el Estado Táchira pertenecen a la presentación de buen pronóstico.


Subject(s)
Humans , Male , Adult , Female , Child , Langerhans Cells , Histiocytosis, Langerhans-Cell/diagnosis , Data Interpretation, Statistical , Histiocytosis, Langerhans-Cell/drug therapy , Venezuela/epidemiology
14.
Rev. odonto ciênc ; 19(43): 69-78, jan.-mar. 2003. tab
Article in Portuguese | LILACS, BBO | ID: lil-397018

ABSTRACT

A doença da célula da Langerhans é uma desordem de etiologia incerta, caracterizada por um amplo aspecto clínico e comportamento bastante variado. Neste estudo foram analisados 33 casos de pacientes com diagnóstico de Doenca de célula de Langerhans no Hospital Infantil Varela Santiago em Natal, RN-Brasil, entre 1997 e 1999. Deste total, 42,4 por cento dos pacientes manifestaram o Granuloma Eosinofílico Solitário, 33 por cento dos pacientes tinham a Doença de Hand-Schüller- Christian e 24,3 por cento destes pertenciam a forma clínica da Doença de Letterer-Siwe. Do total, 54,5 por cento dos pacientes eram do sexo masculino e 45,5 por cento do feminino, com uma média de idade de 3 anos e 7 meses (variando de 06 meses a 15 anos). Setenta por cento dos pacientes apresentavam envolvimento extra-oral, sendo os ossos do crânio e extremidades inferiores, os sítios mais comuns da doença. O tratamento consistiu primariamente da excisão cirúrgica para pacientes com lesões localizadas e terapia com drogas para aquelas com a Doença de Letterer-Siwe. Nosso estudo demonstrou que a forma clínica mais freqüente foi o Granuloma Eosinofílico Solitário e que pacientes mais jovens com disfunção orgânica têm prognóstico pouco favóravel


Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Mouth , Histiocytosis, Langerhans-Cell/surgery , Histiocytosis, Langerhans-Cell/epidemiology , Histiocytosis, Langerhans-Cell/drug therapy
15.
Tanaffos. 2002; 1 (3): 57-61
in English | IMEMR | ID: emr-61060

ABSTRACT

Langerhans cell histiocytosis [LCH] is a disease of unknown etiology that presents in three forms: Letterer- Siwe disease, Hand-Schuller-Christian disease, and Eosinophilic Granuloma. It is a multifocal disease that usually affects several organs and unifocal forms are rare. Pulmonary involvement occurs in approximately 40% of cases and almost always in young adults. Lungs are rarely affected in infant patients. This report presents a 15-month-old male infant that was admitted for cyanosis and respiratory distress. Diagnostic work up revealed a primary pulmonary histiocytosis and the treatment made a significant improvement of signs and symptoms by the time of discharge


Subject(s)
Humans , Male , Histiocytosis, Langerhans-Cell/drug therapy , Infant
17.
Indian Pediatr ; 1999 Jan; 36(1): 29-36
Article in English | IMSEAR | ID: sea-7052

ABSTRACT

OBJECTIVE: To study the clinical profile and outcome of langerhans cell histiocytosis in children upto 2 years of life. DESIGN: Retrospective analysis. METHODS: Medical records of Children upto 2 years of age with a diagnosis of langerhans cell histiocytosis (LCH) were analyzed. Their clinical pattern, treatment modalities and outcome were studied. The patients Were categorized into 2 groups according to their clinical presentation: (i) Subject without organ dysfunction; and (ii) cases with organ dysfunction. Treatment considered of surgical intervention, radiotherapy, chemotherapy or combination of all these modalities depending upto the extent of disease. RESULTS:There were 20 children upto 2 years of age with histiocytosis during the 12 year period (January 1983 - December 1994). The median age at diagnosis was 18 months (range 52 days - 24 months). Of the twenty patients,13 patients didn't have organ dysfunction and 7 had organ dysfunction. Out of the 13 children without organ dysfunction eleven patients received treatment and all of them are alive free of disease with a median follow up of 62 months. But all children with organ dysfunction succumbed to disease within a few weeks. CONCLUSION: Children under 2 years of age with localised and or multifocal LCH without organ dysfunction have a good prognosis and they should not be exposed to aggressive form of treatment. All children with organ dysfunction require multi-agent chemotherapy.


Subject(s)
Age Factors , Antineoplastic Agents, Phytogenic/therapeutic use , Bone Marrow Diseases/physiopathology , Child, Preschool , Combined Modality Therapy , Cyclophosphamide/therapeutic use , Disease-Free Survival , Female , Follow-Up Studies , Glucocorticoids/therapeutic use , Histiocytosis, Langerhans-Cell/drug therapy , Humans , Immunosuppressive Agents/therapeutic use , Infant , Liver Diseases/physiopathology , Lung Diseases/physiopathology , Male , Prednisolone/therapeutic use , Prognosis , Recurrence , Remission Induction , Retrospective Studies , Survival Rate , Treatment Outcome , Vinblastine/therapeutic use
19.
Saudi Medical Journal. 1999; 20 (9): 722-723
in English | IMEMR | ID: emr-114938

ABSTRACT

Both Caroli's Syndrome and Langerhan's cell histiocytosis can be associated with a variety of congenital anomalies. However, the association of the 2 diseases together has not been reported. We report a case of a 3 year old boy who had Caroli's disease as diagnosed radiologically and Langerhan's cell histiocytosis as diagnosed histologically


Subject(s)
Humans , Male , Caroli Disease/diagnosis , Histiocytosis, Langerhans-Cell/drug therapy , Syndrome
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