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← Langerhans Cell Histiocytosis

HEALTH PROFESSIONAL · SOURCE READING

Cladribine and cytarabine

Source: Langerhans Cell Histiocytosis Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: January 6, 2025 · Captured 2026-09-09.

Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.

Context: Childhood LCH / Recurrent, Refractory, or Progressive Childhood LCH / Treatment of recurrent, refractory, or progressive high-risk disease: multisystem LCH / Chemotherapy

Evidence (cladribine and cytarabine):

Ten patients with refractory high–risk-organ involvement (liver, spleen, or bone marrow) and resistant multisystem low–risk-organ involvement were treated with an intensive acute myeloid leukemia–like protocol consisting of cladribine and cytarabine.[143][Level of evidence C3] The follow-up HISTSOC-LCH-S-2005 trial accrued 27 patients and demonstrated the following results:[144]

Source links and citations

Ten patients with refractory high–risk-organ involvement (liver, spleen, or bone marrow) and resistant multisystem low–risk-organ involvement were treated with an intensive acute myeloid leukemia–like protocol consisting of cladribine and cytarabine.[143][Level of evidence C3] The follow-up HISTSOC-LCH-S-2005 trial accrued 27 patients and demonstrated the following results:[144]

The progression-free survival rate was 63%, and the 5-year OS rate was 85% in this refractory, high-risk patient population.

Ten patients with refractory high–risk-organ involvement (liver, spleen, or bone marrow) and resistant multisystem low–risk-organ involvement were treated with an intensive acute myeloid leukemia–like protocol consisting of cladribine and cytarabine.[143][Level of evidence C3] The follow-up HISTSOC-LCH-S-2005 trial accrued 27 patients and demonstrated the following results:[144]

All patients developed grade 4 hematologic toxicity, and five of these patients had severe sepsis.

For centers that cannot provide the intensive supportive care needed for this protocol, an alternative protocol using lower doses of cladribine (5 mg/m2/day × 5 days) and cytarabine (100 mg/m2/day × 4 days) was published.[145][Level of evidence C2]

Source links and citations

For centers that cannot provide the intensive supportive care needed for this protocol, an alternative protocol using lower doses of cladribine (5 mg/m2/day × 5 days) and cytarabine (100 mg/m2/day × 4 days) was published.[145][Level of evidence C2]

Six of nine patients achieved NAD status, and one patient had improved status after six courses.

For centers that cannot provide the intensive supportive care needed for this protocol, an alternative protocol using lower doses of cladribine (5 mg/m2/day × 5 days) and cytarabine (100 mg/m2/day × 4 days) was published.[145][Level of evidence C2]

Some patients received maintenance therapy.

For centers that cannot provide the intensive supportive care needed for this protocol, an alternative protocol using lower doses of cladribine (5 mg/m2/day × 5 days) and cytarabine (100 mg/m2/day × 4 days) was published.[145][Level of evidence C2]

Seven of nine patients remained in complete remission, with a median follow-up of 6.5 years.

Publication references

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Preserved source evidence · Not medical advice