|Year : 2013 | Volume
| Issue : 4 | Page : 194-195
Langerhans cell histiocytosis
Arpita J Nishal, Jigna P Modi, Hasmukh R Balar
Department of Pathology, Government Medical College, New Civil Hospital, Majura Gate, Surat, Gujarat, India
|Date of Web Publication||7-Jan-2014|
Hasmukh R Balar
74, Shree Ganesh Raw House, Sarthana Jakatnaka, Surat - 395 006, Gujarat
Source of Support: None, Conflict of Interest: None
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder in which Langerhans's cells accumulate in a variety of organs. LCH affects mostly children and adolescents, involves one or many body systems or tissues such as bone, lung, hypothalamus, posterior pituitary gland, skin, lymph node, liver, and various soft tissues. The clinical features of LCH depend on the extent of involvement. Here in, we report a case of 2-year-old girl presented with fever, swelling in front and behind of right ear and ear discharge. She had anaemia and lytic lesions on skull x-ray and high resolution computed tomography. Biopsy was performed and histopathology and immunohistochemistry findings suggested diagnosis of LCH.
Keywords: Eosinophilic granuloma, Hand-Sculler-Christian disease, Langerhan cell histiocytosis, Letterer-Siwe disease
|How to cite this article:|
Nishal AJ, Modi JP, Balar HR. Langerhans cell histiocytosis. Indian J Otol 2013;19:194-5
| Introduction|| |
Langerhans's cell histiocytosis (LCH) is a proliferative disorder of Langerhans' cells, but its nature whether reactive, benign, or malignant is still controversial. Historically, the nomenclature regarding LCH has been confusing because the disease had been subcategorized simply based upon different clinical manifestations  . As histiocytosis X, eosinophilic granuloma if solitary lesion, Hand-Schuller Christian disease if multiple lesion, Letterer-Siwe disease if disseminated or visceral involvement  . Ordinarily, patients with solitary disease have localized pain. Patients with disseminated disease may have lymphadenopathy, skin lesions, or diabetes insipidus  .
| Case Report|| |
A 2-year-old girl reported to the outpatient department of tertiary care hospital with swelling in front and behind right ear of 3-month duration [Figure 1]. She gave a history of right ear discharge 2 months back. Examination revealed diff use and soft swelling. No punctum was noticed. The swelling was nontender. The overlying skin was normal. Systemic examination revealed no abnormality. A laboratory finding suggests anaemia with normal liver and renal function test.
High-resolution computed tomography findings were suggestive of centrally necrotic malignant lesion in right mastoid temporal and orbital regions showing frank bony destruction and extension with right middle cranial fossa, temporal bone and right orbit did not show any lesion elsewhere in the body.
On microscopy examination [Figure 2], the section from biopsy right temporal region shows histiocyte like cells in sheets along with intermixed eosinophils and few lymphocytes. Cells are round having indented, lobated, vesicular nuclei along with moderate to abundant delicate pale cytoplasm. Few cells show nuclear folding (grooving). Few foamy cells and multinucleated giant cells were also seen. Areas of necrosis haemorrhage along with bits of bony tissue noted.
On Immunohistochemistry, histiocytic-like cells were reactive for S-100 [Figure 3] and CD 1a. and were immunonegative for CD-68 and CD-30, whereas multinucleated giant cell express CD-68.
Final diagnosis was given LCH.
| Discussion|| |
LCH is a disease of unknown cause and diverse manifestations. In 1953, Lichtenstein proposed that the disease entities known as eosinophilic granuloma, Hand-Schuller-Christian disease and Letterer-Siwe disease all share a common pathologic appearance. This view was challenged by Lieberman et al. , . They suggested that Letterer-Siwe disease is not a specific entity but may involve several pathologic processes. Evidence, however, suggests that at least some examples of disseminated disease with features of Letterer-Siwe syndrome are examples of LCH  . It was once thought that LCH most likely represented a reactive disorder rather than a neoplastic process. More recent studies, however, have demonstrated clonality in LCH, supporting a neoplastic origin  . The differential diagnosis includes osteomyelitis on one hand and malignant lymphoma on the other. Rarely special stains may help in separating an unusual form of osteomyelitis from LCH. LCH cells are usually positive for S-100 protein. However, the most specific marker of LCH is CD1a and langerin. CD1a expression can be detected in frozen or in paraffin embedded tissue by use of the monoclonal antibody O10  .In malignant lymphoma, the characteristic nuclear features of LCH are absent.
| Conclusion|| |
LCH represents a disease with a diverse spectrum of clinical manifestations. Herein through this case, we summarize the current recommendations for diagnosis LCH. A definitive diagnosis of LCH can be made by obtaining a biopsy that yields cells that are morphologically and immunohistochemically compatible with Langerhans' cells. The prognosis depends chiefly upon the involvement of multiple organ systems, organ dysfunction, and the patient's response to chemotherapy during the initial 6 weeks of treatment.
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[Figure 1], [Figure 2], [Figure 3]