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Langerhans’ cell histiocytosis: diagnosis and therapy. The point of view of pediatric hematologist
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Langerhans’ cell histiocytosis: diagnosis and therapy. The point of view of pediatric hematologistVII EAFO Hematology Forum
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Langerhans’ cell histiocytosis (LCH)is a rare disease that arises due to abnormal accumulation of myeloid
cells that phenotypically resemble Langerhans cells, which leads to
compromised organs lesions
Single system disease – skin, bones (uni- and multifocal lesions)
Multisystem disease – with or without risk organs lesions (spleen, liver, bone marrow)
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Standard of care (LCH-III or LCH-IV)LCH Registry & Stratification
Multisystem, multifocal bone,
and special single system LCH
Isolated tumorous
LCH of the brain
STRATUM VI:
Natural history and
Management of
“other” SS-LCH
STRATUM I:
1st-line Therapy
(Group 1 & 2)
Lack of response,
Progression,
in risk organs
STRATUM III:
Salvage Therapy for
Risk LCH
NAD
Progression,
Reactivation,
in non-risk organs
Progression,
Reactivation
STRATUM II:
2nd-line Therapy for
Non-risk LCH
Lack of response,
Progression,
in risk organs
STRATUM IV:
LCH-HSCT
Other single system
LCH
STRATUM V:
Monitoring &
Treatment of
CNS-LCH
STRATUM VII:
Long-term Follow up
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New therapeutic approaches and a hope for MRD6.
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