■细胞吞噬性组织细胞性脂膜炎(CHP)是一种罕见的结节性脂膜炎,以皮肤红斑等临床表现为特征,结节,发烧,全血细胞减少症,肝功能衰竭,浆细胞增多,和肝脾肿大.我们报告了一例CHP,最初被误诊为皮下脂膜炎样T细胞淋巴瘤(SPTCL),但完全缓解,预后良好。
■一名38岁女性到皮肤科就诊,有15天皮下结节病史,全身性水肿,持续发烧。
■患者经典型临床表现诊断为CHP合并噬血细胞综合征,正电子发射断层扫描/计算机断层扫描(PET/CT)中SUVmax的低值,良性分化T细胞,负TCR基因重排,全血细胞减少症,凝血异常,高甘油三酯血症,减少NK细胞计数,肝功能受损,骨活检涂片中观察到噬血细胞的存在。
■在我们的例子中,患者表现为血液动力学不稳定的噬血细胞综合征,表明需要强化治疗。SPTCL的诊断需要一个细致的鉴别诊断过程,以及谨慎的积极化疗方案。延长的后续行动对于确定长期结果至关重要。
UNASSIGNED: Cytophagic histiocytic panniculitis (CHP) is a rare form of nodular panniculitis characterized by clinical manifestations such as skin erythema, nodules, fever, pancytopenia, liver failure, plasmacytosis, and hepatosplenomegaly. We report a
case of CHP that was initially misdiagnosed as subcutaneous panniculitis-like T-cell lymphoma (SPTCL) but achieved complete remission with a favorable prognosis.
UNASSIGNED: A 38-year-old female presented to the dermatology department with a 15-day history of subcutaneous nodules, generalized edema, and continuous fever.
UNASSIGNED: The patient was diagnosed as CHP combined with hemophagocytic syndrome by typical clinical manifestations, low value of SUVmax in positron emission tomography/computed tomography (PET/CT), benign differentiated T cells, negative TCR gene rearrangement, pancytopenia, abnormal coagulation, hypertriglyceridemia, decreased NK cell count, impaired liver function, and the presence of hemophagocytic cells observed in bone biopsy smears.
UNASSIGNED: In our
case, the patient presented with hemophagocytic syndrome with hemodynamic instability, indicating an intensive treatment is needed. The diagnosis of SPTCL necessitates a meticulous process of differential diagnosis, along with the cautious administration of an aggressive chemotherapy regimen. Extended follow-up is imperative to ascertain the long-term outcomes.