Article: Histiocytosis Syndromes of Childhood: A report of four cases
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.
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.
Keywords
Histiocytosis Syndromes of Childhood, Langerhan’s cell Histiocytosis (LCH), Hemophagocytic Lymphohistiocytosis (HLH), Macrophage Activation Syndrome (MAS)
Identifier
https://doi.org/10.3126/jnps.v30i3.3923
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