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Clinical outcomes and screening for organ involvement in pediatric Langerhans cell histiocytosis in Thailand: multicenter study on behalf of the Thai Pediatric Oncology Group

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Abstract

Langerhans cell histiocytosis (LCH) is a rare disease across all age groups and is characterized by various degrees of severity and organ system involvement. A multi-institutional retrospective study of pediatric patients with LCH treated between 1999 and 2018 at five pediatric oncology centers was conducted to describe the clinical characteristics, prognostic factors, and outcomes of LCH and to validate screening tools for organ system involvement in pediatric LCH in Thailand. A total of 127 patients with a median age of 2.7 years were studied. The single-to-multisystem (MS) LCH ratio was 1:1. Forty-seven patients (71%) with MS-LCH had risk-organ involvement (RO +), whereas 19 (29%) patients had no risk-organ involvement (RO −). The 5-year overall and event-free survival rates were 91.3% and 73.6%, respectively, which were comparable to those in developed countries. Prognostic factors included age < 2 years, RO + MS-LCH, and number of RO + . Abnormal complete blood count was a highly sensitive indicator of bone marrow involvement. Plain radiography is an appropriate screening tool to detect bone involvement.

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Abbreviations

6-MP:

6-Mercaptopurine

Ara-C:

Cytarabine

BM:

Bone marrow

CBC:

Complete blood count

CD:

Cluster of differentiation antigens

CI:

Confidence interval

CNS:

Central nervous system

DI:

Diabetes insipidus

EFS:

Event-free survival

Hb:

Hemoglobin

HR:

Hazard ratio

ITP:

Individualized treatment protocol

JLSG:

The Japan Langerhans cell histiocytosis Study Group

LCH:

Langerhans cell histiocytosis

MFB:

Multifocal bones

MP:

Mercaptopurine

MS:

Multisystem

MTX:

Methotrexate

NA:

Not available

ND:

Neurodegeneration

NPV:

Negative predictive value

OS:

Overall survival

PPV:

Positive predictive value

Pred:

Prednisolone

RO −:

No risk-organ involvement

RO +:

Risk-organ involvement

SD:

Standard deviation

SS:

Single system

ThaiPOG:

Thai Pediatric Oncology Group

VBL:

Vinblastine

VCR:

Vincristine

WBC:

White blood cells

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Acknowledgements

The authors gratefully acknowledged Prof. Surapon Wiangnon, Faculty of Medicine, Mahasarakham University for the initial development of ThaiPOG-LCH protocols and research support, Mrs. Supak Cae-Ngow, Office of Research and Development, Phramongkutklao College of Medicine for statistical analysis, English Editing Service, Research Affairs, Faculty of Medicine, Chulalongkorn University for English language editing, and ThaiPOG for supporting this study.

Funding

The funding to conduct the study was obtained from the Phramongkutklao College of Medicine, Royal Thai Army.

Author information

Authors and Affiliations

  1. Division of Hematology-Oncology, Department of Pediatrics, Phramongkutklao Hospital and Phramongkutklao College of Medicine, 315 Ratchawithi Road, Ratchathewi, Bangkok, 10400, Thailand

    Chalinee Monsereenusorn & Piya Rujkijyanont

  2. Division of Hematology-Oncology, Department of Pediatrics, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand

    Kunanya Suwannaying & Patcharee Komvilaisak

  3. Clinical Research for Holistic Management in Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, King Chulalongkorn Memorial Hospital, Chulalongkorn University, Bangkok, Thailand

    Piti Techavichit & Panya Seksarn

  4. Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand

    Lalita Sathitsamitphong & Pimlak Charoenkwan

  5. Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi, Bangkok, 10400, Thailand

    Samart Pakakasama

Authors
  1. Chalinee Monsereenusorn
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Contributions

CM conceptualized and designed the study, collected, analyzed, and interpreted data, and drafted and edited the manuscript. PC assisted in conceptualizing the study. SP contributed to the conception and design of the work, overall management of the program, interpreting data, and writing the manuscript. All authors contributed to patients' care, collected data, and critically reviewed and approved the final manuscript.

Corresponding authors

Correspondence to Chalinee Monsereenusorn or Samart Pakakasama.

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Conflict of interest

The authors declare that they have no conflict of interest.

Data sharing statement

The datasets generated or analyzed during the current study are not publicly available due to privacy or ethics restriction. The data are available from the corresponding author upon reasonable request.

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Monsereenusorn, C., Suwannaying, K., Techavichit, P. et al. Clinical outcomes and screening for organ involvement in pediatric Langerhans cell histiocytosis in Thailand: multicenter study on behalf of the Thai Pediatric Oncology Group. Int J Hematol 115, 563–574 (2022). https://doi.org/10.1007/s12185-022-03293-0

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