Mendelian susceptibility to mycobacterial disease masquerading as lymphoproliferative disorder

Journal of Applied Hematology

article

Thulasi Raman Ramalingam¹  Deenadayalan Munirathnam² 

[1] Department of Hematology, Apollo Cancer Centre;Department of Pediatric Hematology, Kanchi Kamakoti CHILDS Trust Hospital

关键词: Granuloma;    interferon-gamma;    lymphoproliferative disorder;    Mendelian susceptibility to mycobacterial diseases;   

DOI  :  10.4103/joah.joah_45_20

学科分类：社会科学、人文和艺术（综合）

来源: Medknow

【 摘 要 】

Mendelian susceptibility to mycobacterial diseases (MSMDs) is a rare disorder where histiocytes lack the interferon-gamma receptor and are not activated by the cytokine secreted by T-lymphocyte. The lymph node biopsy in these cases lack granuloma but show sheets of histiocytes with effacement of architecture, which may mimic a lymphoproliferative disorder. The predominance of a histiocytic population in immunodeficient children should alert the pathologist, and ancillary techniques such as immunohistochemistry and flow cytometry should be used to establish the correct diagnosis. Here, we report a case of MSMD which mimicked as a lymphoproliferative disorder.

【 授权许可】

CC BY-NC-SA