Indian Journal of Pathology and Microbiology


Year
: 2007  |  Volume : 50  |  Issue : 4  |  Page : 766--8

Gaucher«SQ»s disease : report of 4 cases.


Meenal Vitthal Jadhav, Meenal P Landge, S Surana, Satyakam K Sawaimoon 
 Department of Pathology, B. J. Medical College, Pune

Correspondence Address:
Meenal Vitthal Jadhav
Department of Pathology, B. J. Medical College, Pune

Review of records for last 5 years has shown 4 cases of Type I Gaucher«SQ»s disease in our institute. The cases were diagnosed on bone marrow aspiration, examination of splenectomy specimen, liver biopsy and post mortem in one case. The age range was 2 years to 22 years. Male to female ratio was 3:1.Splenectomy was performed in one case and one case received enzyme replacement therapy with high dose, low frequency regimen for six months without any favorable effect. All were Hindus. Family history of similarly affected and treated twin brother was available in one case. The predominant clinical presentation was pancytopenia and splenohepatomegaly with splenomegaly greater than hepatomegaly. Remarkable constitutional inferiority was noted in one case, which succumbed to death following acute illness and bleeding diathesis. Post mortem performed showed infiltration of spleen and liver with Gaucher cells, fibrosis and myeloid metaplasia in liver and lung.


How to cite this article:
Jadhav MV, Landge MP, Surana S, Sawaimoon SK. Gaucher's disease : report of 4 cases. Indian J Pathol Microbiol 2007;50:766-8


How to cite this URL:
Jadhav MV, Landge MP, Surana S, Sawaimoon SK. Gaucher's disease : report of 4 cases. Indian J Pathol Microbiol [serial online] 2007 [cited 2022 Aug 8 ];50:766-8
Available from: https://www.ijpmonline.org/article.asp?issn=0377-4929;year=2007;volume=50;issue=4;spage=766;epage=8;aulast=Jadhav;type=0