PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV

Poster Tumore der Haut
While histologic features of hemophagocytosis were not observed in the skin, they
were clearly present in a bone marrow biopsy. T-cells obtained from ascites puncture
showed an α/β T-cell phenotype, a T-cell-receptor rearrangement and a positive
staining for CD8, CD3, CD7, CD5 and CD2. Before admittion to our hospital a lupus
panniculitis was primarily suspected, and the patient was placed on a treatment with
oral prednisone at a dose of 1mg/kg per day at a local hospital, resulting in no clinical
improvement. After the diagnosis of SPTL-AB with HPS was made, we referred the
patient to our hematology department where a CHOP 14 regime was started.
Meanwhile, the patient received 3 cycles of chemotherapy, leading to a dramatic
decrease of LDH and to a complete resolution of the B-symptoms and ascites, as well.
Further follow up is pending.
Discussion: Currently, there is no standardized therapy for SPTL. Recent studies suggest
that sustained complete remission can be achieved with less aggressive treatment such
as systemic steroids, cyclosporine, methotrexate, bexaroten or romidepsin instead of
chemotherapy regimens in patients with a relatively indolent or localized presentation.
However, SPTL complicated by HPS or refractory and resistant SPTL require more
aggressive treatments with multiagent chemotherapy and bone marrow
transplantation.
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