TY - JOUR
T1 - Subcutaneous panniculitis-like T cell lymphoma with hemophagocytic syndrome
AU - Kim, So Young
AU - Bang, Sook Hyun
AU - Kim, Myung Hwa
AU - Cho, Min Sun
AU - Choi, Hae Young
AU - Myung, Ki Bum
PY - 2005/4
Y1 - 2005/4
N2 - Subcutaneous pnniculitis-like T cell lymphoma (SPTCL) is a rare subtype of cutaneous T cell lymphoma which clinically and histopathologically mimics benign panniculitis. It presents as tender erythematous, subcutaneous nodules or plaques on the extremities and trunk, and displays systemic symptoms such as weight loss, fever, chills, fatigue or myaligia. The association of hemophagocytic syndrome (HPS) portends a poor prognosis. We report a case of SPTCL that occurred in a 20-month-old girl. She presented with multiple erythematous subcutaneous nodules on the face, right thigh, buttocks and trunk, plus fever and vomiting. Histopathological examination of the subcutaneous nodules revealed a dense infiltration of atypical T-lymphoid cells in the subcutaneous fat layer, with rimming of fat cells, numerous karyorrhetic bodies and histiocytes, phagocytosed apoptotic cells and Bean bag cells In spite of combination chemotherapy, she died of disseminated intravascular coagulation due to HPS.
AB - Subcutaneous pnniculitis-like T cell lymphoma (SPTCL) is a rare subtype of cutaneous T cell lymphoma which clinically and histopathologically mimics benign panniculitis. It presents as tender erythematous, subcutaneous nodules or plaques on the extremities and trunk, and displays systemic symptoms such as weight loss, fever, chills, fatigue or myaligia. The association of hemophagocytic syndrome (HPS) portends a poor prognosis. We report a case of SPTCL that occurred in a 20-month-old girl. She presented with multiple erythematous subcutaneous nodules on the face, right thigh, buttocks and trunk, plus fever and vomiting. Histopathological examination of the subcutaneous nodules revealed a dense infiltration of atypical T-lymphoid cells in the subcutaneous fat layer, with rimming of fat cells, numerous karyorrhetic bodies and histiocytes, phagocytosed apoptotic cells and Bean bag cells In spite of combination chemotherapy, she died of disseminated intravascular coagulation due to HPS.
KW - Hemophagocytic syndrome (HPS)
KW - Subcutaneous panniculitis-like T cell lymphoma (SPTCL)
UR - http://www.scopus.com/inward/record.url?scp=20544473257&partnerID=8YFLogxK
M3 - Article
AN - SCOPUS:20544473257
SN - 0494-4739
VL - 43
SP - 507
EP - 511
JO - Korean Journal of Dermatology
JF - Korean Journal of Dermatology
IS - 4
ER -