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Fetal and neonatal histiocytoses
1Department of Pathology, Children's Hospital San Diego, San Diego, California 92123, USA. hisaacs@ucsd.edu
Pediatric Blood & Cancer
|January 18, 2006
Summary
Neonatal histiocytoses like Langerhans' cell histiocytosis (LCH) and juvenile xanthogranuloma (JXG) show higher spontaneous regression rates than in older individuals. Cutaneous forms exhibit the best prognosis.
Area of Science:
- Pediatric Pathology
- Dermatology
- Hematology
Background:
- Histiocytoses are disorders of the monophagocytic system with diverse clinical and pathological presentations.
- These conditions are less common in the perinatal period compared to later life.
- Biologic behavior, therapeutic response, and histology vary among histiocytic disorders.
Purpose of the Study:
- To analyze the incidence and outcomes of histiocytoses in fetuses and neonates.
- To compare the characteristics of different histiocytic disorders in the perinatal period.
- To investigate the potential for spontaneous regression in neonatal histiocytoses.
Main Methods:
- A comprehensive review of 221 fetuses and neonates from literature and personal records.
- Classification of histiocytic disorders based on established diagnostic criteria.
- Analysis of mortality rates and treatment outcomes for each histiocytic type.
Main Results:
- Langerhans' cell histiocytosis (LCH), hemophagocytic lymphohistiocytoses (HLH), and juvenile xanthogranuloma (JXG) were the predominant perinatal histiocytoses.
- HLH exhibited the highest mortality (74%), followed by disseminated LCH (52%) and JXG (11%).
- Neonates with skin/subcutaneous LCH or JXG achieved complete survival, irrespective of treatment.
Conclusions:
- Neonatal histiocytic lesions demonstrate a notable incidence of spontaneous regression, exceeding that in older patients.
- Cutaneous forms of JXG and LCH showed the highest rates of regression.
- Infection-associated HLH also displayed regression, though with higher associated mortality.
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