Thrombotic microangiopathies encompass a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia associated with hyaline thrombi (comprised primarily of platelet aggregates in the microcirculation), and varying degrees of end-organ failure. Many primary (genetic) and secondary etiological predisposing factors have been described—namely pregnancy, autoimmune disorders, cancer, drugs and antineoplastic therapy, bone marrow transplantation/solid organ transplantation, and infections. In the setting of infectious diseases, the association with shiga or shiga-like exotoxin of Escherichia coli 0157:H7 or shigella dysenteriae type 1-induced typical hemolytic uremic syndrome is well known. Recently however, an increasing body of evidence suggests that viruses may also play an important role as trigger factors in the pathogenesis of thrombotic microangiopathies. This is a comprehensive review focusing on the current understanding of viral associated/induced endothelial stimulation and damage that ultimately leads to the development of this life-threatening multisystemic disorder.

Extramedullary plasmacytoma (EMP) of the small intestine is an unusual plasma cell neoplasm in this anatomic region with only 61 cases described so far. Clinical suspicion is infrequent owing to its location and nonspecific manifestations such as abdominal pain, obstructive symptoms or even bleeding. Diagnosis is reached through histopathological examination coupled with immunohistochemistry of the endoscopic biopsy or surgical resection specimens. Nevertheless, the differential diagnosis between EMP, lymphoma and other kinds of tumors can sometimes be troublesome. The managements include surgery, radiotherapy or chemotherapy. Generally, the prognosis of EMP is favorable, but occasionally it may relapse, or progress to a plasma cell myeloma. EMP should therefore be followed-up for a long period after treatment. In this comprehensive review of the current literature, the patients' characteristics, clinical manifestations, diagnosis, differential diagnosis, treatment and outcome were discussed.

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Introduction Pancreatic involvement by plasma cell neoplasms is an extremely rare event, with only 50 cases described in the literature. They can present as a primary solitary extramedullary plasmacytoma or plasmacytoma secondary to a plasma cell myeloma. Clinical manifestations are due to the presence of a pancreatic mass usually in the pancreas head, which causes extra-biliary obstruction and abdominal pain. Methods Abdominal imaging including CT scan or endoscopic ultrasound with fine-needle aspiration tissue sampling is essential for the initial diagnostic procedure. However, immunohistochemical analysis of the biopsy specimen or flow cytometry of the aspirated material is crucial to prove the monoclonality and the final diagnosis of a plasma cell neoplasm. Discussion Management of these situations include radiotherapy, chemotherapy, surgery or combined therapy. Novel medications including the immunomodulatory drugs or the proteasome inhibitors followed by consolidation with intensive chemotherapy and haematopoietic stem cell transplantation are nowadays used as upfront treatment in the cases associated to a plasma cell myeloma. Conclusion Despite the rarity, plasma cell neoplasms should be considered in the differential diagnosis of obstructive jaundice and pancreatic neoplasms since they are potentially treatable situations.

We present a rare case of Stewart-Treves syndrome characterized by a diffuse angiosarcoma of the leg in a 22-year-old man with a history of chronic lymphedema due to Klippel-Trénaunay-Weber syndrome. He underwent limb disarticulation and medical treatment with cycles of doxorubicin, oral thalidomide and sunitinib with a very good response after 12 months of follow-up.