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• Re: Acute ataxia complicating Langherans cell histiocytosis
  Jon Pritchard
  Published on: 22 March 2016
• Spelling difficulties
  Ulrich Heininger
  Published on: 22 March 2016

• Published on: 22 March 2016

  Re: Acute ataxia complicating Langherans cell histiocytosis

  • Jon Pritchard, Consultant Paediatric Oncologist

  Dear Editor

  Some of the statements in this interesting "Short Report" by A Polizzi et al.[1] can be challenged. It is incorrect to suggest that cerebellar ataxia has been reported "only occasionally" in children and that it is commoner in adults with Langerhans Cell Histiocytosis (LCH). Diabetes insipidus is the only CNS complication that is more common than cerebellar disease and though the precise relative...

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  Dear Editor

  Some of the statements in this interesting "Short Report" by A Polizzi et al.[1] can be challenged. It is incorrect to suggest that cerebellar ataxia has been reported "only occasionally" in children and that it is commoner in adults with Langerhans Cell Histiocytosis (LCH). Diabetes insipidus is the only CNS complication that is more common than cerebellar disease and though the precise relative incidence of cerebellar ataxia in children and in adults is unknown, because all published series are institution-based, there is no reason to suspect that proportionately more adults suffer this complication. It is also misleading to suggest that the patient described by Polizzi et al. represents a "unique" occurrence. Cerebellar ataxia may be present at diagnosis or appear during follow-up and may be progressive or static. More details of the clinical and pathological spectrum of CNS involvement by LCH can be found in a recent review.[2]

  As the authors point out, pituitary-hypothalamic axis involvement is caused by direct infiltration of the structures by pathological Langerhans Cells ("LCH cells") and accompanying inflammatory cells. In patients who develop ataxia, cerebellar biopsy usually reveals only gliosis and demyelination, but CD1a-positive cells have been demonstrated in a few instances. Therefore, it is likely that cerebellar involvement follows the same pattern, with "LCH cell" infiltration followed by cytokine- and chemokine-mediated neural damage. The same sequence, with fibrosis as the end point, occurs in the liver and lungs of other LCH patients. Immune mechanisms may also be involved, as suggested by Polizzi et al., because CD8-positive T cells are also found in the cerebellar biopsies (Grois NG, personal communication). It is unlikely, however, that they represent the primary pathogenetic process. In other words, it is improbable that cerebellar involvement represents a "paraneoplastic syndrome" (ie an autoimmune disorder), as suggested by Polizzi and colleagues. Cerebrospinal fluid (CSF) "anti-neural" antibodies have not been detected in two studies (Grois N et al. and Donadieu J et al., unpublished observations).

  The combination of reticulo-nodular pulmonary shadowing and ataxia, as in this case, makes the diagnosis of LCH a real possibility. Given the onset of fatal pneumothorax soon after the child's discharge from hospital, a lung CT scan would almost certainly have shown cystic change and led to tissue diagnosis via lung biopsy or bronchial lavage. With chemotherapy, the prognosis for pulmonary LCH is usually favourable, even if complicated by pneumothorax.[3] It is generally recommended that patients with suspected LCH are referred to Paediatric Oncologists and/or managed on the international evidence-based trials of the Histiocyte Society, which include recommendations for the management of CNS complications.[2,4] More details of the Histiocyte Society's activities and their contact address can be found on the Society's website: www.histio.org-society.

  References

  (1) Polizzi A, Coghill S, McShane MA, and Squier W. Acute ataxia complicating Langherans cell histiocytosis. Arch Dis Child 2002;86:130-131.

  (2) Grois NG, Favara BE, Mostbeck GH, Prayer D. Central Nervous System Disease in Langerhans Cell Histiocytosis. Hem/Oncology Clinics of North America 1998;12:287-305.

  (3) Ha SY, Helms P, Fletcher M, Broadbent V, Pritchard J. Lung involvement in Langerhans Cell Histiocytosis: Prevalence, clinical features and outcome. Pediatrics 1992;89:466-469.

  (4) Broadbent V, Gadner H. Current Therapy for Langerhans Cell Histiocytosis. Hem/Oncology Clinics of North America 1998;12:327-338.

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  Conflict of Interest:
  None declared.

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• Published on: 22 March 2016

  Spelling difficulties

  • Ulrich Heininger

  Dear Editor

  Of course, spelling of names from a language different from your own is sometimes difficult and typographic errors happen easily. Just to clarify: Paul's last name is spelt "Langerhans", not "Langherans", as it is done erroneously constantly in this paper. Paul Langerhans lived from 1847 to 1888 and did most of his famous work in Berlin. He not only discovered the dendritic cells (named after him) associa...

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  Dear Editor

  Of course, spelling of names from a language different from your own is sometimes difficult and typographic errors happen easily. Just to clarify: Paul's last name is spelt "Langerhans", not "Langherans", as it is done erroneously constantly in this paper. Paul Langerhans lived from 1847 to 1888 and did most of his famous work in Berlin. He not only discovered the dendritic cells (named after him) associated with histiocytosis, but also the insulin producing islet cells in the pancreas.

  By the way, Polizzi and collaborators are in good company. When you search for "Langherans" in Medline, this paper and another 16 show up - quite a few of them from Italy, by the way, probably because "gh" is a frequent sequence in the Italian language. If you search for "Langerhans", however, 33227 citations are identified. So, if you want to be complete in your search for papers dealing with "Langerhans", don't forget "Langherans". Never mind, nobody is perfect (surely I am not...).

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  Conflict of Interest:
  None declared.

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