Faculty Bibliography

Paraneoplastic cerebellar degeneration (PCD) is a rare disorder that is associated with lung or gynecological malignancies and Hodgkin lymphoma. Neurologic symptoms are commonly the initial presenting sign leading to the diagnosis of an underlying malignancy. We are presenting an Asian male with progressive lower extremity weakness with EBV-positive nasopharyngeal carcinoma (NPC) and anti-Yo antibodies. Peculiarly, transient diffuse leptomeningeal enhancement is seen on MR imaging. This is the first report of PCD associated with NPC and thus illustrates that PCD embodies a boarder set of disease than previously described.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a serious disorder with arteriolar and capillary thrombosis for which the treatment usually requires plasma exchange with plasma as the replacement fluid. Management of patients who do not accept blood products is a serious challenge. We present the case of a Jehovah's Witness patient who achieved clinical response after treatment with plasma exchange using human albumin solution as the replacing fluid, high dose corticosteroids, and rituximab. The patient also received ADAMTS13 containing plasma cryoprecipitate and von Willebrand factor VIII concentrates. She had an exacerbation of her TTP in less than 3 weeks. She was treated with further plasma exchange with human albumin solution as the replacement fluid, high dose steroids, and rituximab. Bortezomib and N-acetylcysteine were added. The patient eventually improved clinically and achieved remission that is ongoing for more than 7 months. A review of the literature shows that all five previously reported cases of aTTP in Jehovah's Witnesses survived although none received plasma. Two were not even treated with plasma exchange. The experience of this case and those in the literature demonstrates that remission of aTTP may be achieved without using plasma or plasma exchange.