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 Table of Contents  
Year : 2019  |  Volume : 6  |  Issue : 4  |  Page : 472-474

Langerhans cell histiocytosis: know before you go

Department of Anesthesiology, AIIMS Rishikesh, Uttarakhand, India

Date of Submission15-May-2019
Date of Acceptance16-Sep-2019
Date of Web Publication06-Jan-2020

Correspondence Address:
MD Vijay Adabala
Department of Anesthesiology, AIIMS Rishikesh, Uttarakhand 249201
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/roaic.roaic_108_18

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How to cite this article:
Adabala V, Govil N. Langerhans cell histiocytosis: know before you go. Res Opin Anesth Intensive Care 2019;6:472-4

How to cite this URL:
Adabala V, Govil N. Langerhans cell histiocytosis: know before you go. Res Opin Anesth Intensive Care [serial online] 2019 [cited 2020 Apr 6];6:472-4. Available from: http://www.roaic.eg.net/text.asp?2019/6/4/472/275133

  Introduction Top

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of bone marrow-derived histiocytes (Langerhans cells) and granuloma in various organs. LCH is a rare disease of unknown pathogenesis [1]. Incidence of LCH is approximately five cases per one million population, with most cases occurring in childhood. The male: female sex ratio was 2 : 1. Spinal involvement of Langerhans is though a known phenomenon, with skull and spine being the most common sites of involvement. Anesthetic management of these cases under regional anesthesia was rarely reported. We report a case regarding the anesthetic management of a 24-year-old male patient with Langerhans histiocytosis posted for internal fixation of femur because of pathological fracture.

  Case report Top

A 24-year-old male patient came to our institute with pain and palpable mass over the right thigh. The lesion was diagnosed as a case of Langerhans histiocytosis causing pathological fracture. He underwent three cycles of chemotherapy and 15 cycles of radiotherapy. He was posted for open reduction and internal fixation with plating of femur. Radiological screening (Tc-99m MDP whole-body scan) of the patient was done to rule out the involvement of spine. After taking written informed consent regarding regional anesthesia, combined spinal-epidural anesthesia was given for patient-controlled analgesia intraoperatively and postoperatively. Perioperative period was uneventful ([Figure 1] and [Figure 2]).
Figure 1 Tc-99m MDP whole-body scan.

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Figure 2 Radiography showing pathological fracture of left femur.

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  Discussion Top

LCH is a rare disease, associated with clonal proliferation of histiocytes and granuloma formation in various organs. However, its etiology remains unknown until now. It is thought to be a reactive hyperplastic disease related to chromosomal instability and gene mutation. It can occur as a solitary lesion or with multiorgan involvement. Organs like bones, lungs, skin, liver, and spleen can get affected [2]. LCH is associated with diabetes insipidus following cranial involvement. It is also associated with leukemia [3].

LCH is mostly seen in between ages 5 and 10 years in approximately 80% of the cases. Anatomic sites commonly involved are skull (26%), vertebra (7%), ribs (12%), upper and lower jaw (9%), and bones of extremities (11%). In the spine, LCH mainly involves the vertebral bodies, with a predilection for the thoracic spine (54%) followed by the lumbar (35%) and cervical spine (11%) [4].

One of the variant of LCH is eosinophilic granuloma which refers to a single bone lesion in older children, teenagers, or adults with symptoms of pain, swelling and, eventually, pathologic fracture, with a very good prognosis. It typically has no extraskeletal involvement, but rarely an identical lesion can be found in the skin, lungs, or stomach. When found in the lungs, it should be distinguished from pulmonary LCH − a special category of disease most commonly seen in adult smokers. Solitary eosinophilic granuloma involvement of the spine is rare. The incidence varies between approximately 6.5 and 25% of all skeletal cases [5]. Involvement of the spine in LCH can be multifocal with uneventful clinical course [6].

Ishii et al. [7] had reported a case where a pregnant patient with a pulmonary LCH underwent cesarean section under spinal anesthesia successfully. She had repeated pneumothorax with bilateral pulmonary cysts rapidly becoming worse during pregnancy.

LCH can convert from a focal lesion to multisystem involvement easily and may accompany with malignant tumor. As spine is commonly involved in LCH affecting both vertebrae and the surrounding soft tissue, working knowledge of these kind of rare diseases is necessary for an anesthesiologists for the successful management in the perioperative period.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Crooks B, Grenier D. Langerhans cell histiocytosis: a complex recurrent disease. Paediatr Child Health 2010; 15:69–70.  Back to cited text no. 1
Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A et al. Histiocyte society. A randomized trial of treatment for multisystem Langerhans’ cell histiocytosis. J Pediatr 2001; 138:728–734.  Back to cited text no. 2
Swerdlow SH. WHO classification of tumours of haematopoietic and lymphoid tissues. WHO classification of tumours. 4th edition. WHO; 2008:439.  Back to cited text no. 3
Ippolito E, Farsetti P, Tudisco C. Vertebra plana. Long-term follow-up in five patients. J Bone Joint Surg Am 1984; 66:1364–1368.  Back to cited text no. 4
Bertram C, Madert J, Eggers C. Eosinophilic granuloma of the cervical spine. Spine 2002; 27:1408–1413.  Back to cited text no. 5
Vadivelu S, Mangano FT, Miller CR, Leonard JR. Multifocal Langerhans cell histiocytosis of the pediatric spine: a case report and literature review. Childs Nerv Syst 2007; 23:127–131.  Back to cited text no. 6
Ishii E, Takaenoki Y, Shizukuishi M, Fukuda I, Kazama T. Anesthetic management of a patient with langerhans cell histiocytosis during cesarean section. Masui 2015; 64:444–448.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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