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랑게르한스세포 조직구증: 국가별 치료 선택지
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선택지 정리됨희귀·유전 질환최종 확인 2026.06
국가별 선택지
국가별 치료 선택지
공식 규제·평가 기관 출처를 바탕으로 한 국가별 승인·접근 상태입니다. 무엇이 어디에 존재하는지를 보여줄 뿐, 추천이 아닙니다.
United States
- Cobimetinib (Cotellic)[1]FDA-approvedAny mutational status in the FDA label's histiocytic neoplasm trial; BRAF V600E patients were allowed only under specified prior-access/toxicity circumstances in the trial description; Adult histiocytic neoplasms; trial context included multisystem, recurrent/refractory, or selected single-system disease unlikely to benefit from conventional therapies. · This is an adult histiocytic-neoplasm label, not a pediatric LCH-specific approval. Label safety warnings and monitoring requirements apply; this entry does not imply eligibility or superiority over other MAPK-targeted approaches. Confidence/conflicts: High for adult FDA label; LCH subgroup was small, so disease-specific generalizability should be discussed with a histiocytosis specialist.
- Observation, local therapy, surgery/curettage, radiation therapy, topical/oral/IV therapies, vinblastine/prednisone, methotrexate, cytarabine, cladribine, clofarabine, bisphosphonates, hydroxyurea with or without methotrexate, MAPK inhibitors, hematopoietic stem cell transplant, long-term follow-up[2]Standard option (per NCI PDQ)BRAF V600E, MAP2K1 and other RAS-MAPK pathway alterations; CD1a/CD207 diagnostic confirmation; risk-organ involvement of liver, spleen, and hematopoietic system; response at 6-12 weeks; Low-risk single-system or multisystem disease; high-risk multisystem disease; recurrent/refractory/progressive disease; long-term monitoring for reactivation and permanent consequences. · NCI states optimal therapy for recurrent/refractory/progressive LCH is not fully determined, and treatment depends on site, risk organs, molecular features, and early response. Do not generalize targeted therapy to all LCH without mutation and clinical context. Confidence/conflicts: High for U.S. evidence-summary framework; no conflict recorded.
출처
- U.S. Food and Drug Administration — official drug label · official drug label
- National Cancer Institute — national cancer agency evidence summary · national cancer agency evidence summary
위 내용은 공식 규제·접근 상태일 뿐, 의학적 조언이나 추천이 아니고, 적격성을 판단하지도 않습니다. 어떤 선택지가 적합한지는 환자의 상황과 종양내과 팀에 달려 있습니다. 규제 상태는 바뀔 수 있으니 표시된 출처에서 확인하세요. 임상 세부 내용은 영문이 정본입니다. 최종 확인 2026.06.
승인된 치료 너머
임상시험 및 신흥 선택지
환자가 거주하는 국가에서 아직 승인된 표준 치료가 아닌 선택지입니다 — 연구, 임상시험, 허가 외 사용, 담당 종양내과 의사가 먼저 언급하지 않을 수 있는 초기 근거입니다. 각 항목은 근거의 강도에 따라 구분됩니다. 여기에 실린 항목은 조사하고 의료진과 상의할 정보일 뿐, 효과가 입증되었거나 안전하거나 현재 이용 가능하다는 의미가 아닙니다. 임상 세부 내용은 영문이 정본입니다.
임상시험 진행 중
- Vinblastine/prednisolone, methotrexate/leucovorin, cytarabine, vinblastine/prednisone, clofarabine임상시험 · NCT00276757임상시험Trial only (registry)United States · Histopathologic LCH confirmation; CD1a/Langerin/CD207 confirmation in protocol contexts; recurrent/refractory disease after prior systemic therapy for clofarabine; Newly diagnosed pediatric LCH; frontline systemic therapy; recurrent/refractory LCH after prior systemic therapy. · LCH-III is completed and older; current treatment should be checked against contemporary histiocytosis-center practice. Clofarabine record requires prior systemic therapy failure for LCH stratum. Confidence/conflicts: High for registry-listed interventions and geographies; no approval claim is made for each drug in LCH. ClinicalTrials.gov — clinical-trial registry
- Dabrafenib plus trametinib임상시험 · NCT07440290임상시험Trial only (registry)United Kingdom · BRAF V600 mutation-positive cancer, including LCH in the arm 07 condition and eligibility text; Rare BRAF V600 mutation-positive cancer basket setting, including LCH; patients aged at least 1 year and meeting DETERMINE master protocol criteria. · Not-yet-recruiting in the fetched registry record. This is a molecular basket trial, not an LCH-specific standard-care recommendation. Confidence/conflicts: High for UK registry cell and BRAF V600 trial framing; no approval/reimbursement claim is made. trial-registry listing only — does not establish approval, reimbursement, or eligibility ClinicalTrials.gov — clinical-trial registry
- Prednisone, cytarabine, vincristine, mercaptopurine, local therapy, dabrafenib or trametinib with clofarabine, luvometinib alone or with modified LCH-III regimen, cytarabine with thalidomide, CD207 CAR-T cells임상시험 · NCT04773366임상시험Trial only (registry)China · CD1a/CD207/Langerin diagnostic confirmation; BRAF/MAPK pathway context; high-risk liver/spleen/hematopoietic involvement; special-site bone lesions; relapsed/refractory criteria; Newly diagnosed pediatric LCH; high-risk/recurrent/refractory pediatric LCH; multisystem pediatric LCH; single-system special-site/multifocal bone pediatric LCH; untreated adult multisystem or multifocal LCH; relapsed/refractory LCH. · Registry status varies, including recruiting and unknown status. Luvometinib and CD207 CAR-T remain investigational trial-framed options. Confidence/conflicts: High for China registry cells; no NMPA approval claim is made. ClinicalTrials.gov — clinical-trial registry
- Vemurafenib with cytarabine and 2-chlorodeoxyadenosine / cladribine임상시험 · NCT03585686임상시험Trial only (registry)Russia · BRAF V600E mutation; CD1a/CD207 histologic diagnosis; Children aged 0-18 with histologically verified LCH and BRAF V600E mutation, including life-threatening cases where urgent vemurafenib use before mutation confirmation is described in the protocol criteria. · The registry record is unknown status and should be locally confirmed. Vemurafenib is trial-framed in this pediatric LCH combination context. Confidence/conflicts: Medium because registry status is unknown; high that the fetched record supports the Russia BRAF V600E pediatric LCH trial cell. trial-registry listing only — does not establish approval, reimbursement, or eligibility ClinicalTrials.gov — clinical-trial registry
임상시험이나 초기 보고에 실렸다는 것은 해당 선택지가 연구되고 있다는 의미일 뿐, 효과가 있거나 특정 환자에게 안전하거나 현재 등록 가능하다는 뜻은 아닙니다. 어떤 선택지가 적합한지는 담당 종양내과 팀과 임상시험 팀이 함께 상의할 문제입니다. 최종 확인 2026.06.
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모든 선택지는 규제·평가·지침 등 공신력 있는 출처에 연결되며, 새 출처가 검증되면 계속 확장됩니다.
진료 전 정리할 정보
- Is this single-system, multisystem risk-organ negative, or multisystem risk-organ positive LCH?
- Have CD1a/CD207 pathology and BRAF/MAPK pathway testing been performed?
- What response checkpoint is being used to decide whether to continue or change therapy?
- Is the patient an adult with a histiocytic neoplasm covered by the label?