Skip to main content

Table 1 Patient descriptions

From: Intermediate-dose cytarabine is an effective therapy for adults with non-Langerhans cell histiocytosis

Patient # Diagnosis Age at cytarabine initiation (years) Gender Sites of disease Gene mutations Previous therapies Total number of cytarabine cycles Response Progression or disease recurrence? (time from start of therapy to relapse) Follow-up duration (months) Maintenance or recurrent treatment
1 ECD 45 F CNS, bones, pituitary None-detected None 4 PR No 48 Interferon-α
2 ECD 24 F CNS, bones, obit, lung, retroperitoneum BRAF V600E None 4 PR No 39 Interferon-α
3 ECD 56 M bones, vasculature, orbit, lung, nasal sinus, retroperitoneum BRAF V600E Interferon-α 6 PR No 13 Interferon-α
4 ECD 36 F bones, vasculature, orbit, thyroid, pericardium, retroperitoneum None-detected Interferon-α 4 PD Yes (5 months) 12 Sirolimus/prednisone
5 ECD 50 M CNS, bones, pituitary, thyroid, vasculature, nerve root NA None 6 PR No 17 Interferon-α
6 ECD 51 F CNS, bones BRAF V600E None 6 PR Yes (19 months) 25 Interferon-α → vemurafenib
7 ECD 53 F Bones, heart, pericardium BRAF V600E Interferon-α 2 PD (died) Yes (6 months) 6 NA
8 ECD 51 F CNS, bones, skin, thyroid, retroperitoneum, pancreas None-detected None 4 PR No 9 Interferon-α
9 ECD 52 M CNS, Bone, pericardium, orbit, retroperitoneum, vasculature, lung None-detected Interferon-α 5 PR No 68 Interferon-α
10 ICH 22 M Bones, CNS (Spinal cord) NA None 6 PR No 14 NA
11 RDD 34 M CNS None-detected None 6 PR No 23 Lenalidomide/dexamethasone
12 RDD 37 M CNS, dura NA Surgery 6 CR No 12 NA
13 RDD 70 M Bones, orbit, nasal sinus, glottis BRAF R188G MAP2K1D147G1 Methylprednisolone 4 PR No 8 Lenalidomide/dexamethasone
14 RDD 18 M CNS, pituitary BRAF V600E wild type None 6 PR No 61 Interferon-α
15 RDD 50 M CNS, dura NA Methylprednisolone/Rituximab 6 PR No 60 Interferon-α
16 Unclassified histiocytosis 22 M Bones, skin, gingiva NA COP, CHOP, lenalidomide 3 PR No 6 NA
  1. COP: Cyclophosphamide/vincristine/prednisone; CHOP: Cyclophosphamide/epirubicin/vincristine/predisone
Back to article page

Orphanet Journal of Rare Diseases

ISSN: 1750-1172