Histiocytosis Syndromes of Childhood: A report of four cases

Authors

  • M Ramesh Resident, Dept of Paediatrics, Armed Forces Medical College, Pune, Maharashtra
  • V Singh Resident, Dept of Paediatrics, Armed Forces Medical College, Pune, Maharashtra
  • R Ghuliani Sr.Advisor, Dept of Paediatrics, Command Hospital, Southern Command, Pune, Maharashtra, India
  • BN Kapur Sr. Advisor, Dept of Medicine & Oncology, Command Hospital, Southern Command, Pune, Maharashtra, India
  • J Singh Classified Specialist, Dept of Medicine, Clinical Hematology & Oncology, Command Hospital, Southern Command, Pune, Maharashtra, India
  • S Shankar Immunology, Reader, Dept of Medicine, Armed Forces Medical College, Pune, Maharashtra, India.

DOI:

https://doi.org/10.3126/jnps.v30i3.3923

Keywords:

Histiocytosis Syndromes of Childhood, Langerhan’s cell Histiocytosis (LCH), Hemophagocytic Lymphohistiocytosis (HLH), Macrophage Activation Syndrome (MAS)

Abstract

Histiocytosis Syndromes of Childhood (HSC) are a group of rare and diverse disorders characterized by aggressive proliferation or accumulation of cells of monocyte - macrophage system of bone marrow. The clinical spectrum of this syndrome is distinctly varied. The exact pathophysiology of HSC is yet to be determined; however, evidence suggests that one of the subtypes, Hemophagocytic Lymphohistiocytosis, is due to decreased Natural Killer cell activity, resulting in increased activation of other T cell subtypes and production of cytokines. We present four cases of HSC managed at our center between October 2008 & February 2010.

Key words: Histiocytosis Syndromes of Childhood; Langerhan’s cell Histiocytosis (LCH); Hemophagocytic Lymphohistiocytosis (HLH); Macrophage Activation Syndrome (MAS).

DOI: 10.3126/jnps.v30i3.3923

J Nep Paedtr Soc 2010;30(3):171-174

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How to Cite

Ramesh, M., Singh, V., Ghuliani, R., Kapur, B., Singh, J., & Shankar, S. (2010). Histiocytosis Syndromes of Childhood: A report of four cases. Journal of Nepal Paediatric Society, 30(3), 171–174. https://doi.org/10.3126/jnps.v30i3.3923

Issue

Vol. 30 No. 3 (2010)

Section

Brief Reports/Case Reports/Case Series

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