Characteristics of relapsed/refractory LCH patients treated with HU/MTX
| Pt no. . | Sex . | Age at diagnosis . | Age at start of HU . | Historical sites of disease . | HU target lesion . | Risk status . | Prior therapies . | Duration of HU therapy, mo . | Grade 3-4 toxicities . | Best response . | Progressed/ relapse . | Concurrent chemotherapy . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 45 y 5 mo | 48 y 7 mo | Bone Oral Skin | Skin Oral | LR | 1. Radiation therapy 2. Cytarabine | 3 | No | PD | Yes | N/A |
| 2 | F | 33 y 10 mo | 34 y 2 mo | Pulmonary Skin Oral Lymph nodes | Skin Oral Lymph nodes | LR | 1. Prednisone, azathioprine 2. MTX, 6-MP | 1 | No | SD | No | N/A |
| 3 | F | 48 y 7 mo | 58 y 2 mo | Pulmonary Skin Bone Pituitary | Skin Mastoid | LR | 1. Cytarabine 2. MTX 3. Etoposide 4. Cytarabine 5. Campath | 10 | No | SD | No | N/A |
| 4 | M | 12 y | 28 y 5 mo | Skin Oral Pituitary | Skin Oral | LR | 1. MTX 2. Narrow band UV 3. IV MTX | 6 | No | PR | No | N/A |
| 5 | M | 27 y 8 mo | 33 y 2 mo | Skin | Skin | LR | 1. MTX 2. Cytarabine, MTX 3. Etoposide | 6 | No | PR | Yes | MTX |
| 6 | F | 1 mo | 26 y 8 mo | Spleen Liver Bone marrow Skin Oral CNS (Pit/ND) | Oral | HR | 1. Vinblastine, prednisone 2. Repeat vinblastine, prednisone | 12 | No | PR | Yes | N/A |
| 7 | F | 42 y 8 mo | 50 y 6 mo | Bone Pulmonary Skin Oral | Bone Skin Oral Vaginal | LR | 1. Prednisone, vinblastine 2. Prednisone, vinblastine, 6-MP, MTX 3. Cladribine 4. Cytarabine 5. MTX | 13 | No | PR | Yes | MTX |
| 8 | M | 47 y | 73 y 2 mo | Pulmonary Skin Pituitary | Skin | LR | 1. Prednisone 2. Cytoxan 3. Etoposide | 4 | No | CR | No | N/A |
| 9 | M | 8 y | 9 y 2 mo | Skin | Skin | LR | 1. MTX + prednisone 2. 6-MP + MTX | 6 | Thrombo-cytopenia Neutropenia | CR | No | N/A |
| 10 | M | 1 y 4 mo | 2 y 6 mo | Skull Sternum Spleen (by PET) | Bone | HR | 1. Cytarabine 2. Local injection | 8 | No | CR | No | N/A |
| 11 | M | 66 y 2 mo | 69 y 8 mo | Pulmonary Skin Pituitary | Skin | LR | 1. MTX 2. Prednisone | 10 | No | CR | Yes | MTX |
| 12 | F | 15 y 11 mo | 19 y 1 mo | Bone Pituitary | Bone | LR | 1. Cytarabine 2. Clofarabine 3. 6-MP/MTX | 12 | No | CR | No | N/A |
| 13 | F | 44 y | 57 y 10 mo | Skin Otic Oral Pulmonary | Skin Oral Vaginal Otic | LR | 1. MTX/6-MP 2. MTX | 17 | No | CR | No | N/A |
| 14 | F | 33 y 1 mo | 41 y 3 mo | Pulmonary Skin Oral | Skin Oral Vaginal | LR | 1. Cytarabine 2. Cladribine | 22 | No | CR | Yes | MTX |
| 15 | F | 58 y 6 mo | 59 y 6 mo | Orbital Skin Oral | Skin Oral | LR | 1. MTX/6-MP | 24 | Anemia Neutropenia | CR | No | MTX |
| Pt no. . | Sex . | Age at diagnosis . | Age at start of HU . | Historical sites of disease . | HU target lesion . | Risk status . | Prior therapies . | Duration of HU therapy, mo . | Grade 3-4 toxicities . | Best response . | Progressed/ relapse . | Concurrent chemotherapy . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | F | 45 y 5 mo | 48 y 7 mo | Bone Oral Skin | Skin Oral | LR | 1. Radiation therapy 2. Cytarabine | 3 | No | PD | Yes | N/A |
| 2 | F | 33 y 10 mo | 34 y 2 mo | Pulmonary Skin Oral Lymph nodes | Skin Oral Lymph nodes | LR | 1. Prednisone, azathioprine 2. MTX, 6-MP | 1 | No | SD | No | N/A |
| 3 | F | 48 y 7 mo | 58 y 2 mo | Pulmonary Skin Bone Pituitary | Skin Mastoid | LR | 1. Cytarabine 2. MTX 3. Etoposide 4. Cytarabine 5. Campath | 10 | No | SD | No | N/A |
| 4 | M | 12 y | 28 y 5 mo | Skin Oral Pituitary | Skin Oral | LR | 1. MTX 2. Narrow band UV 3. IV MTX | 6 | No | PR | No | N/A |
| 5 | M | 27 y 8 mo | 33 y 2 mo | Skin | Skin | LR | 1. MTX 2. Cytarabine, MTX 3. Etoposide | 6 | No | PR | Yes | MTX |
| 6 | F | 1 mo | 26 y 8 mo | Spleen Liver Bone marrow Skin Oral CNS (Pit/ND) | Oral | HR | 1. Vinblastine, prednisone 2. Repeat vinblastine, prednisone | 12 | No | PR | Yes | N/A |
| 7 | F | 42 y 8 mo | 50 y 6 mo | Bone Pulmonary Skin Oral | Bone Skin Oral Vaginal | LR | 1. Prednisone, vinblastine 2. Prednisone, vinblastine, 6-MP, MTX 3. Cladribine 4. Cytarabine 5. MTX | 13 | No | PR | Yes | MTX |
| 8 | M | 47 y | 73 y 2 mo | Pulmonary Skin Pituitary | Skin | LR | 1. Prednisone 2. Cytoxan 3. Etoposide | 4 | No | CR | No | N/A |
| 9 | M | 8 y | 9 y 2 mo | Skin | Skin | LR | 1. MTX + prednisone 2. 6-MP + MTX | 6 | Thrombo-cytopenia Neutropenia | CR | No | N/A |
| 10 | M | 1 y 4 mo | 2 y 6 mo | Skull Sternum Spleen (by PET) | Bone | HR | 1. Cytarabine 2. Local injection | 8 | No | CR | No | N/A |
| 11 | M | 66 y 2 mo | 69 y 8 mo | Pulmonary Skin Pituitary | Skin | LR | 1. MTX 2. Prednisone | 10 | No | CR | Yes | MTX |
| 12 | F | 15 y 11 mo | 19 y 1 mo | Bone Pituitary | Bone | LR | 1. Cytarabine 2. Clofarabine 3. 6-MP/MTX | 12 | No | CR | No | N/A |
| 13 | F | 44 y | 57 y 10 mo | Skin Otic Oral Pulmonary | Skin Oral Vaginal Otic | LR | 1. MTX/6-MP 2. MTX | 17 | No | CR | No | N/A |
| 14 | F | 33 y 1 mo | 41 y 3 mo | Pulmonary Skin Oral | Skin Oral Vaginal | LR | 1. Cytarabine 2. Cladribine | 22 | No | CR | Yes | MTX |
| 15 | F | 58 y 6 mo | 59 y 6 mo | Orbital Skin Oral | Skin Oral | LR | 1. MTX/6-MP | 24 | Anemia Neutropenia | CR | No | MTX |
6-MP, 6-mercaptopurine; CNS, central nervous system; F, female; HR, high risk; LR, low risk; M, male; mo, months; N/A, not applicable; ND, neurodegenerative LCH; Pit, pituitary; Pt, patient; y, years.