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Year : 2020  |  Volume : 11  |  Issue : 3  |  Page : 145-148

Mendelian susceptibility to mycobacterial disease masquerading as lymphoproliferative disorder

1 Department of Hematology, Apollo Cancer Centre, Chennai, Tamil Nadu, India
2 Department of Pediatric Hematology, Kanchi Kamakoti CHILDS Trust Hospital, Chennai, Tamil Nadu, India

Correspondence Address:
Dr. Thulasi Raman Ramalingam
Department of Hematology, Apollo Cancer Centre, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joah.joah_45_20

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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.

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