Clinico-biologic profile of Langerhans cell histiocytosis: A single institutional study

Narula, Gaurav; Bhagwat, R; Arora, Brijesh; Banavali, Sripad; Pai, Swati; Nair, C. N.; Seth, Tanuja; Laskar, Siddharth; Muckaden, Maryann; Pa, Kurkure; Parikh, PM

doi:10.4103/0019-509x.38939

Cited by 6 publications

(2 citation statements)

References 19 publications

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“…Although the histiocytic disorders are considered rare conditions, they can cause many challenges for pediatricians 1,3,9,10 . The etiology and pathogenesis of such diseases are not fully understood; they have different clinical manifestations, which complicates diagnosis of the disease.…”

Section: Discussionmentioning

confidence: 99%

“…This disorder may occur at any age, most commonly below 15 years [3][4][5][6][7][8] . Although histiocytic disorders are relatively rare, 1,3,9,10 , they have presented great challenges for pediatric pathologists and hematologists/oncologists, because of unknown etiologies and pathogenesis, the clonality theory, variable classifications and subtypes, diagnostic difficulties, spontaneous remission to chronic active courses, poor therapeutic responses with high mortality, recurrences, and some poorly understood complications after different types of therapies 1,8,11,12 . According to the World Health Organization (WHO) classification, histiocytic disorders are classified into three major groups, characterized by the central role occupation of one cell type 1,9,13 : LCs in Langerhans cell histiocytosis (LCH), as class 1; hemophagocytic lymphohistiocytosis (HLH), including infection-associated hemophagocytic lymphohistiocytosis (IAHL) and familial hemophagocytic lymphohistiocytosis (FHL), as class 2; and malignant histiocytosis, as class 3.…”

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confidence: 99%

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A 10-year single center survey of pediatric patients with histiocytic disorders in Iran

Shamsian,

Nikoufar,

Esfahani

et al. 2011

Turk J Pediatr

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Childhood histiocytosis is a rare and diverse group of proliferative disorders, characterized by accumulation and infiltration of antigen-presenting cells or antigen-processing cells, which can affect any tissue or organ. This study was performed in order to investigate the clinical characteristics of Iranian children with different types of histiocytic disorders. Thirty-five patients, with a median age of 3.5 years, who were referred and diagnosed with histiocytic disorders in a referral Children's Hospital in Iran from 1997-2006, were investigated in this study. According to the World Health Organization classification, 27 patients were in class 1, followed by 6 patients in class 2, and 1 patient in class 3. Moreover, 1 patient was diagnosed with sinus histiocytosis with massive lymphadenopathy. Bone lesions were the most common manifestation, which were detected in 15 cases, followed by skin lesions (11 cases) and fever (10 cases). Nonspecific findings like hepatomegaly and splenomegaly were found in 15 cases. Different types of treatment protocols were used according to the diverse groups of histiocytic disorders and different stages of disease, including surgical excision, radiotherapy, chemotherapy, and stem cell transplantation. Twelve patients did not respond well to the treatment and subsequently died due to complications of their disease. Although histiocytosis is considered a rare condition, it can be problematic for pediatric hematologists because of the unknown etiologies and pathogenesis, variable classifications and subtypes, diagnostic difficulties, poor therapeutic responses with high mortality, and some complications after different therapeutic protocols.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

A 10-year single center survey of pediatric patients with histiocytic disorders in Iran

Shamsian,

Nikoufar,

Esfahani

et al. 2011

Turk J Pediatr

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Treatment of Langerhans cell histiocytosis with a modified risk‐adapted protocol—experience from a tertiary cancer institute in India

Narula

Pradhan

Arora

et al. 2018

Pediatric Blood & Cancer

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The 5-Year EFS of Multisystem LCH With Risk-Organ Involvement Is Suboptimal

Totadri¹,

Bansal²,

Trehan³

et al. 2016

Journal of Pediatric Hematology/Oncology

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The study describes an 8-year experience of a single center in managing patients with langerhans cell histiocytosis on the basis of the langerhans cell histiocytosis-III platform. A retrospective case-file review of children diagnosed during 2006 to 2013 was performed. Group 1 (multisystem with risk-organ involvement) patients received an initial treatment of 6 to 12 weeks, followed by continuation treatment to complete 12 months. Drugs included vinblastine, prednisolone, and 6-mercaptopurine. Group 2 (multisystem without risk-organ involvement) patients received a similar treatment, except for 6-mercaptopurine. Group 3 (single-system/multifocal bone disease) patients were treated for a duration of 6 months. Forty-nine patients were treated: 24 (49%), 14 (28.6%), and 11 (22.4%) in groups 1, 2, and 3, respectively. The mean age at diagnosis was 31.6 ± 28.4 months (range, 4 to 120 mo). Five patients abandoned treatment. There were 7 deaths, all in group 1. All patients who died had either a partial response or progressive disease after induction (P=0.000). Among patients with liver involvement, those with sclerosing cholangitis had a greater mortality (P=0.007). A relapse was observed in 12 (24.5%) patients. The frequency of relapse was not different in the 3 groups (P=0.833). The 5-year event-free survival in groups 1, 2, and 3 was 29.3 ± 10%, 58.9 ± 14.6%, and 69.3 ± 15%, respectively (P=0.019). The 5-year overall survival was 100% in groups 2/3 and 68.9 ± 9.8% in group 1 (P=0.011).

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Clinico-biologic profile of Langerhans cell histiocytosis: A single institutional study

Cited by 6 publications

References 19 publications

A 10-year single center survey of pediatric patients with histiocytic disorders in Iran

A 10-year single center survey of pediatric patients with histiocytic disorders in Iran

Treatment of Langerhans cell histiocytosis with a modified risk‐adapted protocol—experience from a tertiary cancer institute in India

The 5-Year EFS of Multisystem LCH With Risk-Organ Involvement Is Suboptimal

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