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Clinical characteristics and outcomes of Langerhans cell histiocytosis at a single institution in Thailand: a 20-year retrospective study

Ponrachet Kitticharoenjit
Ponrachet Kitticharoenjit
, 
Nucharin Supakul
Nucharin Supakul
, 
Piya Rujkijyanont
Piya Rujkijyanont
, 
Chanchai Traivaree
Chanchai Traivaree
, 
Apichat Photia
Apichat Photia
 and 
Chalinee Monsereenusorn
Chalinee Monsereenusorn
   | Aug 20, 2021
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Article Category: Original article
Published Online: Aug 20, 2021
Page range: 171 - 181
DOI: https://doi.org/10.2478/abm-2021-0022
Keywords
© 2021 Ponrachet Kitticharoenjit et al., published by Sciendo
This work is licensed under the Creative Commons Attribution 4.0 International License.

Figure 1

Five-year reactivation EFS for patients with LCH and SS (n = 6, solid line) and MS (n = 8, dashed line) involvement (P = 0.085). Survival was calculated using a Cox proportional hazard model. EFS, event-free survival; LCH, Langerhans cell histiocytosis; MS, multisystem; SS, single system.
Five-year reactivation EFS for patients with LCH and SS (n = 6, solid line) and MS (n = 8, dashed line) involvement (P = 0.085). Survival was calculated using a Cox proportional hazard model. EFS, event-free survival; LCH, Langerhans cell histiocytosis; MS, multisystem; SS, single system.

Figure 2

Five-year reactivation EFS for patients with LCH and RO+ (n = 5, solid line) and RO− (n = 9, dashed line) (P = 0.005). Survival was calculated using a Cox proportional hazard model. EFS, event-free survival; LCH, Langerhans cell histiocytosis; MS, multisystem; RO−, no risk-organ involvement; RO+, risk-organ involvement; SS, single system.
Five-year reactivation EFS for patients with LCH and RO+ (n = 5, solid line) and RO− (n = 9, dashed line) (P = 0.005). Survival was calculated using a Cox proportional hazard model. EFS, event-free survival; LCH, Langerhans cell histiocytosis; MS, multisystem; RO−, no risk-organ involvement; RO+, risk-organ involvement; SS, single system.

Figure 3

Comparison of CT/MRI and FDG-PET-CT scan from patient No. 4 in Table 2. (A) Sagittal T1 postcontrast MRI demonstrating abnormal marrow signal intensity involving L2, L3, S1–S2 vertebral bodies (B) paralleled with the FDG-PET-CT image. (C) Contrast enhanced CT image of the abdomen demonstrating hepatosplenomegaly with heterogenous contrast enhancement and scattered hypodense lesions in the spleen and a lytic lesion at the L3 vertebral body (D) corresponding FDG-PET-CT image confirming hypermetabolic activities at L2–L3 vertebral bodies and the spleen. Additional hypermetabolic activities are noted at the right sacral alar, right hilar lymph node, right middle lobe, and right-sided of second rib, which is suggestive of disease involvement. CT, computed tomography; FDG, fluorodeoxyglucose F18; MRI, magnetic resonance imaging; PET, positron emission tomography.
Comparison of CT/MRI and FDG-PET-CT scan from patient No. 4 in Table 2. (A) Sagittal T1 postcontrast MRI demonstrating abnormal marrow signal intensity involving L2, L3, S1–S2 vertebral bodies (B) paralleled with the FDG-PET-CT image. (C) Contrast enhanced CT image of the abdomen demonstrating hepatosplenomegaly with heterogenous contrast enhancement and scattered hypodense lesions in the spleen and a lytic lesion at the L3 vertebral body (D) corresponding FDG-PET-CT image confirming hypermetabolic activities at L2–L3 vertebral bodies and the spleen. Additional hypermetabolic activities are noted at the right sacral alar, right hilar lymph node, right middle lobe, and right-sided of second rib, which is suggestive of disease involvement. CT, computed tomography; FDG, fluorodeoxyglucose F18; MRI, magnetic resonance imaging; PET, positron emission tomography.

Treatment and outcomes of patients with LCH (n = 14)

Treatment n = 14 (%)
Surgery 3 (21)
Curettage 1 (33)
Partial resection 2 (67)
CMT 12 (86)
SS 4 (33)
Single bone 2 (50)
MFB 2 (50)
MS 8 (67)
Low risk 3 (38)
High risk 5 (62)
RT 1 (7)
Disease status
Remission 10 (71)
Reactivation/progressive 4 (29)
On therapy 3 (75)
Off therapy 1 (25)
Status at last follow-up
ANED 10 (72)
AWD 2 (14)
DWD 2 (14)

Characteristics and outcomes of patients with LCH (n = 14)

No Age at diagnosis (years) Sex System involvement (SS vs. MS) Organ/system involvement Organ/system of risk involvement DI at diagnosis Treatment CMT protocols Disease status Status at last follow-up Follow-up (years)
1 1.7 Female MS Skin, bone Liver, spleen, hematopoietic No CMT Ara-C, Pred Progressive DWD 1
2 0.25 Female MS Lung, bone Liver, spleen, hematopoietic No CMT DAL-Hx83 Remission ANED 16
3 1.2 Female MS Bone, LN Liver, spleen No CMT LCH-III Reactivation MFB AWD 8.6
4 15.6 Male MS Lung, MFB (vertebrae) Liver, spleen No CMT LCH-IV Reactivation AWD 2.7
5 1.3 Female MS Skin, bone Liver, spleen, hematopoietic No CMT LCH-IV Progressive DWD 0.6
6 9 Female SS Bone – No RT, CMT CTX, VCR, Pred Remission ANED 12
7 0.5 Female SS Skin – No Observation – Remission ANED 12
8 3 Female SS Bone (CNS risk) – No Curettage, CMT LCH-III Remission ANED 15
9 8 Female MS MFB, pituitary – Yes CMT LCH-III Remission ANED 3
10 7 Female SS Bone – No Surgery – Remission ANED 1
11 10 Male SS MFB – No CMT LCH-III Remission ANED 6
12 2 Female MS Skin, pituitary, bone – Yes CMT LCH-III Remission ANED 9
13 10.25 Male MS Bone (vertebrae), LN – No Surgery, CMT LCH-III Remission ANED 8
14 1.4 Female SS MFB – No CMT LCH-IV Remission ANED 0.5

MS “risk” LCH (RO+ MS-LCH) characteristics of 5 patients

No Age at diagnosis (years) Sex Abnormal LFT Organ/system of risk involvement Other organ involvement CMT regimen Disease status Status last follow-up Follow-up (years) BRAFV600E mutation
1 1.7 Female TP 4.8 Alb2.4 TB 13.7DB 12.9AST 56ALT 47ALP 451 Liver, spleen, hematopoietic Skin, bone Ara-C, Pred Progressive DWD 1 Not done
2 0.25 Female TP 8 Alb4.8 TB 14DB 0.5 AST30ALT 16ALP 254 Liver, spleen, hematopoietic Lung, bone DAL-Hx83: VBL, VP-16, 6-MP, MTX, Pred Remission ANED 16 Not done
3 1.2 Female – Liver, spleen Bone, LN LCH-III (High risk): MTX, VBL, Pred, 6-MP Reactivation MFB AWD 8.6 Not done
4 15.6 Male – Liver, spleen Lung, bone (special site) LCH-IV adapted: (High risk): VBL, Pred, 6-MP Reactivation AWD 2.7 Negative
5 1.3 Female TP 6.27Alb 2.73TB 0.65DB 0.28AST 29.8ALT 19.2ALP 80 Liver, spleen, hematopoietic Skin, bone LCH-IV adapted: (High risk): VBL, Pred, 6-MP Progressive DWD 0.6 Positive

Radiology of patients with LCH (n = 14)

Imaging n = 14 (%)
Skull X-ray
Normal 3 (21)
Abnormal 11 (79)
MR brain 8 (73)
Normal 1 (13)
Abnormal 7 (88)
MR brain not done 3 (27)
Tc-99m bone
Done 9 (64)
Not done 5 (36)
FDG-PET-CT
Done 4 (29)
Not done 10 (71)

Patient demographic and clinical characteristics

Demographic n = 14 (%)
Age at diagnosis (years)
  Median (range) 2.5 (0.25–15.6)
  Mean ± SD 5.1 ± 4.8
Sex
  Female 11 (79)
  Male 3 (21)
DI at diagnosis 2 (14)
System involvement
  SS 6 (43)
  Skin only 1 (17)
  Single bone 3 (50)
  MFB 2 (33)
  MS 8 (57)
  Low risk 3 (38)
  High risk 5 (63)
Follow-up (years)
  Median (range) 6.8 (0.5–16.2)
  Mean ± SD 6.9 ± 5.7
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