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Indian J Dermatol Venereol Leprol. 2018 Oct 3. doi: 10.4103/ijdvl.IJDVL_277_17. [Epub ahead of print]

Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma.

Author information

1
Department of Dermatology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
2
Department of Pathology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
3
Department of Radiotherapy, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.

Abstract

Subcutaneous panniculitis-like T cell lymphoma is a rare subtype of cutaneous lymphomas with distinct clinical, histological and immunophenotypic characteristics, as well as an indolent clinical course. Rarely, it may be complicated with hemophagocytic lymphohistiocytosis: a hyperinflammatory syndrome which, if not diagnosed early, carries a dismal outcome. In this article, we describe a case of subcutaneous panniculitis-like T cell lymphoma in a middle-aged female patient which was complicated with secondary hemophagocytic lymphohistiocytosis with a favorable outcome following etoposide-based therapy. The various histological mimics of subcutaneous panniculitis-like T cell lymphoma and the management options are also briefly discussed.

KEYWORDS:

Hemophagocytic lymphohistiocytosis; prognosis; subcutaneous panniculitis like T cell lymphoma

PMID:
30289114
DOI:
10.4103/ijdvl.IJDVL_277_17
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