Lymph node 8a as a prognostic marker for poorer prognosis in pancreatic and periampullary carcinoma

Objectives: An investigation of patients with pancreatic carcinoma aims to identify those who will benefit from surgery. Physical examination, radiology and laboratory findings are helpful. Most prognostic markers, such as lymph node status, micro metastasis and tumour differentiation, are not preoperatively accessible. Metastatic disease in lymph node 8a (Ln8a) in patients operated for pancreatic carcinoma has been reported to be a predictor of shorter overall survival (OS). This lymph node can be assessed preoperatively through resection (possibly even with laparoscopy) and subsequent histopathology. The value of the procedure is disputed. The aim of this study is to investigate whether metastatic disease in Ln8a is a predictor of decreased OS.

Materials and methods: In patients with suspected pancreatic or periampullary carcinoma, who were operated with pancreatoduodenectomy (PD), Ln8a was separately resected and analysed with standard and immuno-histochemical methods. Patients with or without metastasis in Ln8a were compared regarding OS.

Results: Between 2008 and 2011, 122 consecutive patients were eligible and 87 were resected and had LN8a analysed separately. Sixteen patients were Ln8a?+?and 71 were Ln8a-. Patients with Ln8a?+?had a significantly reduced median OS as compared to patients with Ln8a– (0.74 (95% CI 0.26–1.26) versus 5.91 years (95% CI 2.91–), p?Conclusion: Ln8a?+?was associated with a marked reduction of OS, indicating a possible role in the future preoperative workup in patients with a suspicion of pancreatic cancer.     

Congenital thrombotic thrombocytopenic purpura caused by new compound heterozygous mutations of the ADAMTS13 gene

Upshaw–Schulman syndrome (USS) is due to severe congenital deficiency of von Willebrand factor (VWF)‐cleaving protease ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 domains, nr 13) activity resulting in the presence of unusually large forms of VWF in the circulation, causing intravascular platelet clumping and thrombotic microangiopathy. Our patient, a 26‐year‐old man, had attacks of thrombotic thrombocytopenic purpura (TTP) with thrombocytopenia and a urine dipstick positive for hemoglobin (4+), often as the only sign of hemolytic activity. He had ADAMTS13 activity of <1% of normal plasma without the presence of inhibitors of ADAMTS13. ADAMTS13 deficiency was caused by two new mutations of the ADAMTS13 gene: a deletion of a single nucleotide in exon17 (c. 2042 delA) leading to a frameshift (K681C fs X16), and a missense mutation in exon 25 (c.3368G>A) leading to p.R1123H. This case report confirms the importance of the analysis of the ADAMTS13 activity and its inhibitor in patients who have episodes of TTP, with a very low platelet count and sometimes without the classic biochemical signs of hemolysis.     

Corticosteroid treatment exacerbates nephrotic syndrome in a zebrafish model of magi2a knockout

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