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

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

Asian Biomedicine
Volume 15 (2021): Issue 4 (August 2021)
By: Ponrachet Kitticharoenjit,  Nucharin Supakul,  Piya Rujkijyanont,  Chanchai Traivaree,  Apichat Photia and  Chalinee Monsereenusorn  
Open Access
|Aug 2021

Figures & Tables

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)

Treatmentn = 14 (%)
Surgery3 (21)
Curettage1 (33)
Partial resection2 (67)
CMT12 (86)
SS4 (33)
Single bone2 (50)
MFB2 (50)
MS8 (67)
Low risk3 (38)
High risk5 (62)
RT1 (7)
Disease status
Remission10 (71)
Reactivation/progressive4 (29)
On therapy3 (75)
Off therapy1 (25)
Status at last follow-up
ANED10 (72)
AWD2 (14)
DWD2 (14)

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

NoAge at diagnosis (years)SexSystem involvement (SS vs. MS)Organ/system involvementOrgan/system of risk involvementDI at diagnosisTreatmentCMT protocolsDisease statusStatus at last follow-upFollow-up (years)
11.7FemaleMSSkin, boneLiver, spleen, hematopoieticNoCMTAra-C, PredProgressiveDWD1
20.25FemaleMSLung, boneLiver, spleen, hematopoieticNoCMTDAL-Hx83RemissionANED16
31.2FemaleMSBone, LNLiver, spleenNoCMTLCH-IIIReactivation MFBAWD8.6
415.6MaleMSLung, MFB (vertebrae)Liver, spleenNoCMTLCH-IVReactivationAWD2.7
51.3FemaleMSSkin, boneLiver, spleen, hematopoieticNoCMTLCH-IVProgressiveDWD0.6
69FemaleSSBone–NoRT, CMTCTX, VCR, PredRemissionANED12
70.5FemaleSSSkin–NoObservation–RemissionANED12
83FemaleSSBone (CNS risk)–NoCurettage, CMTLCH-IIIRemissionANED15
98FemaleMSMFB, pituitary–YesCMTLCH-IIIRemissionANED3
107FemaleSSBone–NoSurgery–RemissionANED1
1110MaleSSMFB–NoCMTLCH-IIIRemissionANED6
122FemaleMSSkin, pituitary, bone–YesCMTLCH-IIIRemissionANED9
1310.25MaleMSBone (vertebrae), LN–NoSurgery, CMTLCH-IIIRemissionANED8
141.4FemaleSSMFB–NoCMTLCH-IVRemissionANED0.5

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

NoAge at diagnosis (years)SexAbnormal LFTOrgan/system of risk involvementOther organ involvementCMT regimenDisease statusStatus last follow-upFollow-up (years)BRAFV600E mutation
11.7FemaleTP 4.8 Alb2.4 TB 13.7DB 12.9AST 56ALT 47ALP 451Liver, spleen, hematopoieticSkin, boneAra-C, PredProgressiveDWD1Not done
20.25FemaleTP 8 Alb4.8 TB 14DB 0.5 AST30ALT 16ALP 254Liver, spleen, hematopoieticLung, boneDAL-Hx83: VBL, VP-16, 6-MP, MTX, PredRemissionANED16Not done
31.2Female–Liver, spleenBone, LNLCH-III (High risk): MTX, VBL, Pred, 6-MPReactivation MFBAWD8.6Not done
415.6Male–Liver, spleenLung, bone (special site)LCH-IV adapted: (High risk): VBL, Pred, 6-MPReactivationAWD2.7Negative
51.3FemaleTP 6.27Alb 2.73TB 0.65DB 0.28AST 29.8ALT 19.2ALP 80Liver, spleen, hematopoieticSkin, boneLCH-IV adapted: (High risk): VBL, Pred, 6-MPProgressiveDWD0.6Positive

Radiology of patients with LCH (n = 14)

Imagingn = 14 (%)
Skull X-ray
Normal3 (21)
Abnormal11 (79)
MR brain8 (73)
Normal1 (13)
Abnormal7 (88)
MR brain not done3 (27)
Tc-99m bone
Done9 (64)
Not done5 (36)
FDG-PET-CT
Done4 (29)
Not done10 (71)

Patient demographic and clinical characteristics

Demographicn = 14 (%)
Age at diagnosis (years)
  Median (range)2.5 (0.25–15.6)
  Mean ± SD5.1 ± 4.8
Sex
  Female11 (79)
  Male3 (21)
DI at diagnosis2 (14)
System involvement
  SS6 (43)
  Skin only1 (17)
  Single bone3 (50)
  MFB2 (33)
  MS8 (57)
  Low risk3 (38)
  High risk5 (63)
Follow-up (years)
  Median (range)6.8 (0.5–16.2)
  Mean ± SD6.9 ± 5.7
DOI: https://doi.org/10.2478/abm-2021-0022 | Journal eISSN: 1875-855X | Journal ISSN: 1905-7415
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Language: English
Page range: 171 - 181
Published on: Aug 20, 2021
Published by: Chulalongkorn University
In partnership with: Paradigm Publishing Services
Publication frequency: 6 issues per year
Keywords:
fluorodeoxyglucose F18,
hyperbilirubinemia,
conjugated,
hematopoietic,
hypoalbuminemia,
organs at risk,
positron emission tomography computed tomography
Related subjects:
Medicine,
Assistive professions, nursing,
Basic medical science,
Basic medical science, other,
Clinical medicine,
Clinical medicine, other

© 2021 Ponrachet Kitticharoenjit, Nucharin Supakul, Piya Rujkijyanont, Chanchai Traivaree, Apichat Photia, Chalinee Monsereenusorn, published by Chulalongkorn University
This work is licensed under the Creative Commons Attribution 4.0 License.

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