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Cutaneous hematologic disorders in children
Olivia Boccara1, Stéphane Blanche, Yves de Prost
1Department of Pathology, Hôpital Necker-Enfants Malades, AP-HP, Université René Descartes, Paris V, France.
Insights
Childhood cutaneous hematologic diseases, primarily lymphomatoid papulosis (LyP), differ from adult presentations. Pityriasis lichenoides chronica (PLC) often precedes LyP and mycosis fungoides (MF).
Area of Science:
- Pediatric Hematology
- Dermatology
- Oncology
Background:
- Cutaneous hematologic diseases in children present unique clinical and pathological features.
- Understanding these features is crucial for accurate diagnosis and management in pediatric patients.
Purpose of the Study:
- To determine and list the clinical and pathological features of cutaneous hematologic diseases in children under 15 years old.
- To compare childhood presentations with those typically seen in adults.
Main Methods:
- Retrospective analysis of 51 pediatric patients (age ≤15) with primary cutaneous hematologic disorders.
- Classification based on WHO-EORTC criteria, including lymphomatoid papulosis (LyP), lymphoblastic lymphoma (LL), acute leukemias (AL), mycosis fungoides (MF), and EBV-related lymphoproliferative disorders (EBV-related LPD).
Main Results:
- Lymphomatoid papulosis (LyP) was the most common diagnosis.
- Pityriasis lichenoides chronica (PLC) preceded disease onset in 29% of LyP and 80% of MF cases.
- Acute leukemias (AL) and lymphoblastic lymphoma (LL) showed stereotypical features aiding prompt diagnosis, while mycosis fungoides (MF) was often initially underdiagnosed.
Conclusions:
- Cutaneous hematologic disease distribution differs between pediatric and adult populations.
- Pityriasis lichenoides chronica (PLC) is strongly associated with mycosis fungoides (MF) and LyP.
- Awareness of stereotypical presentations of LL and AL is vital for timely pediatric diagnosis and treatment.
Background:
To determine and list the clinical and pathological features of cutaneous hematologic diseases in childhood.
Procedure:
We retrospectively analyzed the data for 51 patients up to 15 years of age, who presented with primary cutaneous hematologic disorders according to the WHO-EORTC classification, at Necker-Enfants Malades Hospital, Paris, France, over a 17-year period. The cases were classified into the following diagnostic categories: CD30+ T-cell lymphoproliferative disorders (24) all consisting of lymphomatoid papulosis (LyP, 24), lymphoblastic lymphoma (LL, 7), acute leukemias (AL, 7), mycosis fungoides (MF, 5), Epstein-Barr virus-related lymphoproliferative disorders (EBV-related LPD, 5), T/NK-cell lymphoma, nasal type (1), γ/δ T-cell lymphoma (1), and panniculitis-like T-cell lymphoma (1).
Results:
We encountered a majority of LyP. No secondary lymphoma was found in LyP patients with a median follow-up of 8 years. 29% and 80% of LyP and MF patients, respectively, presented with pityriasis lichenoides chronica (PLC) before onset of disease. Owing to a frequently misleading clinicopathological presentation, MF patients were first underdiagnosed. Clinicopathological features of LL and AL were highly stereotypical, leading to the diagnosis being suspected and confirmed more promptly. In the latter patients and in EBV-related LPD patients, skin lesions usually led to the discovery of systemic disease.
Conclusion:
Distribution of cutaneous hematologic diseases seems to be different in adults and in children aged <15-year old. PLC was strongly correlated with MF and LyP. Physicians must be made aware of the stereotypical clinical presentations of LL and AL to allow prompt diagnosis and treatment.
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