Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc
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Case Report

Volume 3, Number 4, December 2014, pages 107-111

A Case of Langerhans Cell Histiocytosis With Risk Organ Involvement in a Young Adult

Figures

Figure 1.
Figure 1. Axillary lymph node biopsy with Langerhans cell histiocytosis. A (× 100, hematoxylin and eosin). The lymph node shows paracortical and sinus Langerhans cells that spare the follicles; B. (× 400, hematoxylin and eosin): The Langerhans cells show irregularly grooved and folded nuclei. Admixed lymphocytes, eosinophils, plasma cells, and multinucleated giant cells are present; C. (× 200, CD1a): The LCH cells are strongly CD1a positive; D. (× 200, S100): The LCH cells are strongly S100 positive. LCH: Langerhans cell histiocytosis; CD 1a: cluster of differentiation 1a.
Figure 2.
Figure 2. MRI of the brain demonstrated hyperdense lesions around the optic nerves, lesions appearing hypointense to the cerebral cortex on T2-weighted imaging, suggestive of Langerhans cell involvement.

Tables

Table 1. Summary of Studies for Management of MS-LCH in the Pediatric and Adult Populations
 
StudyNo. of patientsRegimen usedResponse rateToxicityReactivationSurvival
Ara-C: cytosine arabinoside; MTX: methotrexate; 6MCP: 6-mercaptopurine; MS: multisystem; RO: risk organ; SS: single system; VCR: vincristine.
Summary of pediatric studies for management of MS-LCH
LCH-I: Gadner et al 2001 [4]14324 weeks:
Vinblastine (V) or etoposide (E)
plus single initial dose of corticosteroids		At 6 weeks
V: 57%
E: 49%		V: 47%
E: 58%
3 years
V: 61%
E: 55%		3 years
V: 76%
E: 83%
LCH-II: Gadner et al 2008 [5]193Arm A: prednisone and vinblastine × 6 weeks + vinblastine/prednisone + 6MCP × 18 weeks
Arm B: arm A + etoposide (for 24 weeks)		At 6 weeks
Arm A: 63%
Arm B: 71%		NA3 years
Arm A: 46%
Arm B: 46%		5 years
Arm A: 74%
Arm B: 79%
LCH-III: Gadner et al 2013 [6]235
RO+		6 weeks × 1 - 2 cycles:
Arm A: vinblastine + prednisone
Arm B: vinblastine + prednisone + MTX
12 months:
Arm A: vinblastine + prednisone + 6MCP
Arm B: vinblastine + prednisone + MTX + 6MCP		At 6 weeks
Arm A: 65%
Arm B: 66%		Arm A: 30%
Arm B: 46%		3 years
Arm A: 25%
Arm B: 29%		5 years
Arm A: 87%
Arm B: 82%
Japan LCH Study Group (JLSG-96): Morimoto et al 2006 [7]596 weeks:
Ara-C + VCR + prednisolone
Response: 6 months maintenance tx
Poor response: salvage therapy		76.30%NA45.30%5 years
94.40%
Summary of adult studies for management of MS-LCH
Morimoto et al 2013 [8]14 (10 MS)Vinblastine/prednisolone + MTX + 6MCP × 36 weeks60%NA80%
Von Stebut et al 2008 [9]1Prednisolone + vinblastine + 6MCP × 12 months100%NANA
Matsuki et al 2011 [10]2Prednisone + vinblastine + 6-MCP × 6 - 9 months100%NANA
Adam et al 2012 [11]10 (8 MS)Cladribine × 4 - 6 cycles
No response → cladribine + cyclophosphamide + dexamethasone		90%NANA
Pardanani et al 2003 [12]5Cladribine × 4 cycles100%NANA
Derenzini et al 2010 [13]7 (3MS, 4 SS)MACOP-B: methotrexate, doxorubicin, cyclophosphamide, vincristine, prednisone and bleomycin100%67%33%

 

Table 2. Summary of Studies for Refractory/Relapsed MS-LCH in Pediatrics and Adults
 
StudyNo. of PatientsRegimenResponse rateReactivationSurvival
Ara-C: cytosine arabinoside; ATG: anti-thymocyte globulin; CSA: cyclosporine; ECD: Erdheim-Chester disease; HSCT: hematopoietic stem cell transplant; LCH: Langerhans cell histiocytosis; RIC: reduced intensity; RO: risk organ.
Summary of pediatric studies for refractory/relapsed MS-LCH
Minkov et al 1999 [15]26Cyclosporine or CSA/steroid + one or more of vinblastine/etoposide/ATG15% (total)NANA
Weitzman et al 2009 [16]46 RO+
37 RO-		CladribineRO+ 26%
RO- 62%		NA2 years
RO+ 48%
RO- 97%
Overall 68%
Biswas et al 2007 [17]6Cladribine67%NA83%
Bernard et al 2005 [18]9Cladribine + Ara-C × 2 cycles67%NA78%
Apollonsky and lipton 2009 [19]5Cladribine + Ara-C × 3 - 5 cycles100%0% (2.5 - 6 years)NA
Steiner et al 2005 [20]9RIC allogeneic HSCT78%NA78%
Kudo et al 2010 [21]15Myeloablative regimen (10 patients) and RIC (5 patients), followed by allogeneic cord blood73%NA73.30%
Simko et al 2013 [22]11Clofarabine × 6 cycles73%18%2 years
91%
Summary of adult studies for refractory/relapsed MS-LCH
Pardanani et al 2003 [12]5Cladribine × 5 days, median of 4 cycles60%NA
Ingram et al 2006 [23]1RIC allogeneic HSCT100%NA
Ichikawa et al 2007 [24]1Autologous HSCT100%NA
Konno et al 2007 [25]1Etoposide100%NA
Janku et al 2010 [26]3 (2 LCH, 1 ECD)Imatinib100%NA
Haroche et al 2013 [27]3 ECD (2 with concurrent LCH)Vemurafenib100%NA