|LETTER TO EDITOR
|Year : 2019 | Volume
| Issue : 4 | Page : 472-474
Langerhans cell histiocytosis: know before you go
Vijay Adabala, Nishith Govil
Department of Anesthesiology, AIIMS Rishikesh, Uttarakhand, India
|Date of Submission||15-May-2019|
|Date of Acceptance||16-Sep-2019|
|Date of Web Publication||06-Jan-2020|
MD Vijay Adabala
Department of Anesthesiology, AIIMS Rishikesh, Uttarakhand 249201
Source of Support: None, Conflict of Interest: None
|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
| Introduction|| |
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 . 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|| |
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]).
| Discussion|| |
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 . LCH is associated with diabetes insipidus following cranial involvement. It is also associated with leukemia .
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%) .
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 . Involvement of the spine in LCH can be multifocal with uneventful clinical course .
Ishii et al.  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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[Figure 1], [Figure 2]