Trends in survival of patients with primary plasma cell leukemia: a population-based analysis.

Primary plasma cell leukemia (pPCL) is a rare malignancy with an aggressive course and poor outcome. There has been significant improvement in the survival of multiple myeloma patients over the past decade as a result of incorporating autologous stem cell transplantation (ASCT) and novel agents into treatment regimens. However, it is unknown whether these therapies have had a similar impact on the survival of patients with pPCL. We conducted an analysis of the Surveillance, Epidemiology, and End Results database to evaluate the trends in survival of 445 patients with pPCL between 1973 and 2009. The widespread availability of ASCT and use of novel agents in the upfront setting of multiple myeloma and pPCL began after 1995 and 2006, respectively. The median overall survival based on periods of diagnosis were 5, 6, 4, and 12 months for those diagnosed during 1973-1995, 1996-2000, 2001-2005, and 2006-2009, respectively (P = .001). Thus, the current study confirms the recent survival improvement in pPCL within a large US population that may be associated with the use of better therapeutic strategies.

Implications of continued response after autologous stem cell transplantation for multiple myeloma.

Patients undergoing autologous stem cell transplantation (ASCT) for multiple myeloma (MM) undergo disease assessment approximately 100 days later. Some patients continue to have a decline in their serum or urine monoclonal protein after day 100 in the absence of additional therapy. We evaluated 430 MM patients who underwent ASCT within 12 months of their diagnosis and had not achieved a complete remission at day 100. Of these patients, 167 (39%) had a continued response after day 100 without additional therapy. When compared with patients who did not (n = 263), those who had a continued response had a longer progression-free survival (35 vs 13 months, P < .001), time to next therapy (43 vs 16 months, P < .001), and overall survival (96 vs 57 months, P < .001). This phenomenon of a continued response maintained prognostic value in a multivariable analysis and should be considered when interpreting posttransplant responses.

Quantification of clonal circulating plasma cells in relapsed multiple myeloma.

The presence of clonal circulating plasma cells (cPCs) remains a marker of high-risk disease in newly diagnosed multiple myeloma (MM) patients. However, its prognostic utility in MM patients with previously treated disease is unknown. We studied 647 consecutive patients with previously treated MM seen at the Mayo Clinic, Rochester who had their peripheral blood evaluated for cPCs by multi-parameter flow cytometry. Of these patients, 145 had actively relapsing disease while the remaining 502 had disease that was in a plateau and included 68 patients in complete remission (CR) and 434 patients with stable disease. Patients with actively relapsing disease were more likely to have clonal cPCs than those in a plateau (P < 0·001). None of the patients in CR had any clonal cPCs detected. Among patients whose disease was in a plateau, the presence of clonal cPCs predicted for a worse median survival (22 months vs. not reached; P = 0·004). Among actively relapsing patients, the presence of ≥100 cPCs predicted for a worse survival after flow cytometry analysis (12 months vs. 33 months; P < 0·001). Future studies are needed to determine the role of these findings in developing a risk-adapted treatment approach in MM patients with actively relapsing disease.

Gallbladder cancer-associated thrombotic microangiopathy.

Cancer-associated thrombotic microangiopathy (TMA) is a rare but serious condition seen in patients diagnosed with malignancy. Certain tumor characteristics highlight this entity, such as large tumor burden, adenocarcinoma histology with mucinous features and bone marrow infiltration. Although these tumors may originate from any site, the majority are of stomach, breast or prostate origin. The optimal therapy is unknown but there is evidence that immediate initiation of an effective antineoplastic regimen is important. However, it is difficult to differentiate cancer-associated TMA from primary thrombotic thrombocytopenic purpura in a timely manner. We present the first case of cancer-associated TMA in a patient secondary to a locally advanced gallbladder adenocarcinoma that lacked mucinous features and bone marrow involvement. The clinical presentation closely mimicked primary thrombocytopenic purpura and led to the ineffective use of plasma exchange. Nonetheless, the patient eventually received systemic chemotherapy and had a remarkable response by the resolution of her TMA.

Obesity is Independently Associated With Increased Risk of Hepatocellular Cancer-related Mortality: A Systematic Review and Meta-Analysis.

OBJECTIVE: Excess body weight is associated with increased risk of developing hepatocellular cancer (HCC), but its effect on HCC-related mortality remains unclear. We performed a systematic review and meta-analysis to assess the association between premorbid obesity and HCC-related mortality. MATERIALS AND METHODS: Through a systematic literature search-up to March 2016, we identified 9 observational studies (1,599,453 individuals, 5705 HCC-related deaths) reporting the association between premorbid body mass index (BMI), and HCC-related mortality. We estimated summary adjusted hazard ratio (aHR) with 95% confidence intervals (CIs), comparing obese (BMI>30 kg/m(2)) and overweight (BMI, 25 to 29.9 kg/m(2)) individuals with normal BMI individuals using random-effects model. RESULTS: On meta-analysis, compared with individuals with normal BMI, obese (aHR, 1.95; 95% CI, 1.46-2.46), but not overweight individuals (aHR, 1.08; 95% CI, 0.97-1.21), had higher HCC-related mortality, with moderate heterogeneity. On subgroup analysis, magnitude of increased mortality was higher in obese men (aHR, 2.50; 95% CI, 2.02-3.09; 3 studies) as compared with obese women (aHR, 1.45; 95% CI, 1.08-1.97; 2 studies). The impact of premorbid obesity on HCC-related mortality was observed only in western populations (aHR, 2.10; 95% CI, 1.77-2.48; 4 studies), but not Asian populations (aHR, 1.10; 95% CI, 0.63-1.92; 1 study). There was limited assessment of competing risk because of advanced liver disease. CONCLUSIONS: On the basis of this meta-analysis, premorbid obesity may be independently associated with a 2-fold risk of HCC-related mortality. This association was more pronounced in men and western populations. Strategies targeting obesity-associated metabolic abnormalities may provide novel pathways for HCC therapy.

Secondary Hemophagocytic Syndrome Associated with Richter's Transformation in Chronic Lymphocytic Leukemia.

Hemophagocytic syndrome (HPS) is an extremely rare condition arising from the overactivation of one's own immune system. It results in excessive inflammation and tissue destruction. Prompt initiation of treatment is warranted in either scenario in order to decrease mortality. Most cases are triggered by infectious agents, malignancy, or drugs. We describe the first case of a CLL patient presenting with HPS due to acquisition of EBV-related large cell lymphoma in the setting of profound immunodeficiency.

Recurrent acute inflammatory demyelinating polyradiculoneuropathy following R-CHOP treatment for non-Hodgkin lymphoma.

Acute flaccid paralysis following chemotherapy has a wide differential diagnosis, including drug toxicity, acute inflammatory demyelinating polyradiculoneuropathy (AIDP), and malignant nerve infiltration. We present a case of recurrent acute quadriparesis due to AIDP following chemotherapy for non-Hodgkin lymphoma, which resolved each time following administration of intravenous immunoglobulin. Although many chemotherapeutic agents can cause neurologic side effects, such as peripheral neuropathy, drug toxicity as a cause is a diagnosis of exclusion.