TABLE 4.
Cause of death in patients with Langerhans cell histiocytosis included in the study.
| No. | Age | Sex | Disease classification | BRAF V600E | Organ involvement (all) | Initial treatment | Response | Additional malignancy | Cause of death | |
|---|---|---|---|---|---|---|---|---|---|---|
| Tissue | Plasma cfDNA | |||||||||
| IHC | PCR | |||||||||
| 1 | 45 | F | SSm‐LCH | − | + | PG, CNS | Specia‐C | PD | 39y PB | LCH |
| 2 | 61 | F | MS‐LCH | + | + | B, L, PG, LN, LV, ST, SP, DO, CA | 2CdA | PD | − | LCH |
| 3 | 79 | M | MS‐LCH | ND | ND | B, L, SP, BM | Special‐C | PR | 82y AML | Therapy‐related (2nd AML) |
| 4 | 72 | M | MS‐LCH | ND | ND | LN, SP, BM | Ara‐C | PD | − | LCH |
| 5 | 81 | M | MS‐LCH | ND | ND | B, LN | 2CdA | PD | 75y CRC | LCH |
| 6 | 34 | M | MS‐LCH | ND | ND | B, LN, LV, ST, SP, BM | Ara‐C | PR | − | Therapy‐related (GVHD) |
Abbreviations: 2CdA, cladribine; 2nd AML, secondary acute myeloid leukemia; Ara‐C, cytarabine; B, bone; BM, bone marrow; CA, cardiovascular; cfDNA, cell‐free DNA; CNS, central nervous system; CRC, colorectal cancer; DO, digestive organs excluding the liver; GVHD, graft‐versus‐host‐disease; IHC, immunohistochemistry; L, lung; LN, lymph nodes; LV, liver; MS‐LCH, multisystem LCH; ND, no data; PB, phyllodes tumor of the breast; PD, progressive disease; PG, pituitary gland; PR, partial response; SP, spleen; Special‐C, the Japan LCH Study Group Speial‐C regimen, comprising of vinblastine, prednisolone, methotrexate and 6‐mercaptopurine; SSm‐LCH, single system with multi‐site LCH; ST, soft tissue.