SUBCUTANEOUS PANNICULITIS LIKE T-CELL LYMPHOMA PRESENTING WITH HLH C Sullivan, MD MSc, A Loghmani, DO, A Abbas MD, K Thomas, MD MSc, H Hall MD, G Destin DO, S Guillory MD, S Walvekar MD, R Chowdry, MD Department of Internal Medicine, LSU Health Sciences Center, New Orleans, Louisiana CASE: A 34-year-old man with no past medical history presented with two months of a rash on his lower back and daily fevers up to 103°F. Additionally he had fatigue and loss of appetite resulting in an 18-pound weight loss. He had previously been treated with sulfamethoxazole-trimethoprim and clindamycin for presumed cellulitis. He had erythematous indurated dusky plaques on his right neck, bilateral lower back and hips, right upper abdomen and groin, which were not painful, or pruritic. He also had faint erythematous pink macules scattered on his trunk and bilateral upper extremities. His lab work revealed elevated liver enzymes, cytopenia, ferritinemia, hypertriglyceridemia, and increased levels of soluble IL-2 receptors. These findings along with fevers and hemophagocytosis seen on skin biopsy met criteria for hemophagocytic lymphohistiocytosis (HLH). Further evaluation of the skin biopsy led to a diagnosis of subcutaneous panniculitis like T-cell lymphoma (SPTCL) alpha/beta subtype. He was started on cyclosporine 5mg/kg/day and dexamethasone 20mg/day. The patient noted almost immediate improvement is his skin lesions and laboratory abnormalities trended towards patient’s baseline within two weeks. DISCUSSION: SPTCL typically presentswith erythematous painless plaques and nodules on the trunk and extremities. Systemic symptoms include fever, chills and weight loss. Cytopenia and elevated liver enzymes are common. The two types of SPTCL are alpha/ beta and gamma/delta with the former being the less aggressive. STPCL presenting with HLH portends worse outcomes. Historically, many of these cases were treated with aggressive cytotoxic chemotherapy including cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate and prednisone (CHOP) based regimens; however, patients frequently relapse. There is no standardized treatment for SPTCL but previous studies suggest successful treatment with systemic steroids and cyclosporine. CONCLUSION: Our findings suggest that HLH secondary to SPTCL may be treated with cyclosporine and dexamethasone to achieve rapid clinical and symptom management of this rare malignancy.
8
Made with FlippingBook Digital Publishing Software