PPCL were associated with a very poor
PPCL were associated with a very poor prognosis. The median survival duration according to published small trials was short, ranging from 7 to 13 months, with less than 10% of patients having a median survival of more than 5 years. In the largest recently published epidemiological study, the median survival in 231 patients was just four months. The best survival data were achieved in patients undergoing autologous stem cell transplantation (ASCT), where survival was longer than 3 years in 64% of patients. The discouraging survival results in PPCL might be due to its aggressive presentation with complications leading to early mortality within the first month after diagnosis. In the French cohort, 11 of 40 (27.5%) PPCL patients died within the first month after diagnosis. Although the introduction of so-called novel agents (thalidomide, lenalidomide, and bortezomib) contributed significant benefit to older patients in whom the early mortality rate decreased from 26% to 15%, the cause of early mortality had not been studied in depth. This case was consistent with the literature showing dismal outcomes, in that our patient unfortunately suddenly succumbed to this aggressive condition within two weeks of admission, and was too late to benefit from the update anti-MM treatment. In regard to the prognostic factors and hospital course of the presenting case, the patient was relatively older and had a relatively higher tumor load, evidenced by elevated s1p receptor cell counts in the blood and LDH in the serum, and extramedullary manifestations with massive ascites and omental cake. On the last day of admission, the bilateral cardiophrenic angle blunting as compared with the image on the admission (Fig. 6) might indicate a progressive change of extramedullary involvement and/or hospital acquired pneumonia, without strong clues of fluid overload such as large amount fluid supplement or body weight gain. On the other hand, such circumstance was also somehow similar to the case of systemic capillary leak syndrome (SCLS) preceding plasma cell leukemia presented Ghosh et al., but our patient was too severe to receive further treatment for plasma cell leukemia. SCLS, a rare disease of reversible plasma extravasation and vascular collapse, was believed to be a manifestation of transient endothelial dysfunction and would result in shock, massive edema, often after a nonspecific prodrome of weakness, fatigue, and myalgia followed by flash pulmonary edema during rapid fluid remobilization. It was also reported with high concomitant presence of monoclonal gammopathy in an estimated 79%–82% of patient with SCLS and was reported with postulated pathogenesis including nonimmunoglobulin humoral factors such as vascular endothelial growth factor and angiopoietin 2. It was shown that systemic capillary leak syndrome in MM patients might be improved by anti- MM treatment.
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Introduction Nasopharyngeal carcinoma (NPC) is endemic in Southeast Asia and is highly associated with Epstein-Barr virus (EBV). Radiotherapy (RT) is the primary and only curative treatment for NPC, achieving a 5-year overall survival of 90% for early stage I disease. For intermediate or advanced disease, i.e. stage II-IVB disease, concurrent chemoradiotherapy (CRT) is the standard, with or without induction or adjuvant chemotherapy. Despite advancement in combined modality treatment, 5–15% of patients will develop local failure, and 15–30% will experience failure at distant sites. Only a small subset of these patients can be successfully salvaged by surgery or re-irradiation. Most patients are only amenable to palliative systemic chemotherapy. Regarding systemic chemotherapy for recurrent or metastatic NPC, platinum-containing doublet regimens are generally considered to be standard first-line treatment. 5-fluorouracil, gemcitabine, capecitabine, paclitaxel, and docetaxel have been combined with cisplatin, yielding response rates of 60–70%. For patients who have become cisplatin-refractory, capecitabine, irinotecan, docetaxel, gemcitabine-vinorelbine, pemetrexed may be considered. However, treatment-related toxicities are frequently of great concern, especially for heavily-pretreated patients or patients with advanced age and/or poor performance status. Metronomic chemotherapy may be an option for these patients. We report here a patient with recurrent NPC with bone metastases, who experienced a remarkable durable disease control under metronomic chemotherapy.