PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV
PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV
PROGRAMM JAHRESTAGUNG 2012 30. Nov. – 2. Dez ... - ÖGDV
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Poster Tumore der Haut<br />
While histologic features of hemophagocytosis were not observed in the skin, they<br />
were clearly present in a bone marrow biopsy. T-cells obtained from ascites puncture<br />
showed an α/β T-cell phenotype, a T-cell-receptor rearrangement and a positive<br />
staining for CD8, CD3, CD7, CD5 and CD<strong>2.</strong> Before admittion to our hospital a lupus<br />
panniculitis was primarily suspected, and the patient was placed on a treatment with<br />
oral prednisone at a dose of 1mg/kg per day at a local hospital, resulting in no clinical<br />
improvement. After the diagnosis of SPTL-AB with HPS was made, we referred the<br />
patient to our hematology department where a CHOP 14 regime was started.<br />
Meanwhile, the patient received 3 cycles of chemotherapy, leading to a dramatic<br />
decrease of LDH and to a complete resolution of the B-symptoms and ascites, as well.<br />
Further follow up is pending.<br />
Discussion: Currently, there is no standardized therapy for SPTL. Recent studies suggest<br />
that sustained complete remission can be achieved with less aggressive treatment such<br />
as systemic steroids, cyclosporine, methotrexate, bexaroten or romidepsin instead of<br />
chemotherapy regimens in patients with a relatively indolent or localized presentation.<br />
However, SPTL complicated by HPS or refractory and resistant SPTL require more<br />
aggressive treatments with multiagent chemotherapy and bone marrow<br />
transplantation.<br />
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