Summary: The incidence of a malignant disease reflects the genetic and cumulative exposure to the environment of a population. Therefore, evaluation of the incidence and trends of a disease in different populations may provide insights into its aetiology and pathogenesis. To evaluate the incidence of haematological malignancies according to specific subtypes, we used population‐based registry data in Japan (N = 125 148) and the United States (US; N = 172 925) from 1993 to 2008. The age‐adjusted incidence of haematological malignancies in Japan was approximately one‐half that in the US but has been increasing significantly, whereas no significant change was seen in the US [annual percent change (95% C confidence interval): Japan, +2·4% (1·7, 3·1); US, +0·1% (−0·1, 0·2)]. Hodgkin lymphoma (HL) and non‐Hodgkin lymphoma (NHL) showed the largest differences in incidence, with the most remarkable differences observed for chronic lymphocytic leukaemia, HL‐nodular sclerosis, mycosis fungoides and cutaneous T‐cell lymphoma. HL and NHL are increasing substantially in Japan but not in the US, suggesting that environmental exposures, such as Westernization of the life style may be causing this increase. Differences in the incidence and trends for specific subtypes also showed a marked contrast across subtypes, which, in turn, may provide significant new insights into disease aetiology in the future.

Although ibrutinib is highly effective in patients with relapsed/refractory mantle cell lymphoma (MCL), a substantial proportion of patients have resistant disease. The subsequent outcomes of such patients are unknown. We carried out a retrospective review of all patients with MCL treated with ibrutinib at MD Anderson Cancer Center between January 2011 and January 2014 using pharmacy and clinical databases. Patients who had discontinued ibrutinib for any reason were included in the study. We identified 42 patients with MCL who discontinued therapy due to disease progression on treatment (n = 28), toxicity (n = 6), elective stem-cell transplant in remission (n = 4) or withdrawn consent (n = 4). The median age was 69 years, 35 (83%) were male; the median number of prior treatments was 2 (range 1–8) and the median time from initial diagnosis of MCL to commencing ibrutinib was 3.0 (range 0.5–15.5) years. Patients had received a median of 6.5 (range 1–43) cycles of ibrutinib. Among 31 patients who experienced disease progression following ibrutinib and underwent salvage therapy, the overall and complete response rates were 32% and 19%, respectively. After a median follow-up of 10.7 (range 2.4–38.9) months from discontinuation of ibrutinib, the median overall survival (OS) among patients with disease progression was 8.4 months. By univariate analysis, elevated serum lactate dehydrogenase at progression was associated with inferior OS. The outcome of patients with MCL who experience disease progression following ibrutinib therapy is poor, with both low response rates to salvage therapy and short duration of responses. Further studies to better understand and overcome ibrutinib resistance are urgently needed.

Although we usually report 5‐year cancer survival using population‐based cancer registry data, nowadays many cancer patients survive longer and need to be followed‐up for more than 5 years. Long‐term cancer survival figures are scarce in Japan. Here we report 10‐year cancer survival and conditional survival using an established statistical approach. We received data on 1 387 489 cancer cases from six prefectural population‐based cancer registries in Japan, diagnosed between 1993 and 2009 and followed‐up for at least 5 years. We estimated the 10‐year relative survival of patients who were followed‐up between 2002 and 2006 using period analysis. Using this 10‐year survival, we also calculated the conditional 5‐year survival for cancer survivors who lived for some years after diagnosis. We reported 10‐year survival and conditional survival of 23 types of cancer for 15–99‐year‐old patients and four types of cancer for children (0–14 years old) and adolescent and young adults (15–29 years old) patients by sex. Variation in 10‐year cancer survival by site was wide, from 5% for pancreatic cancer to 95% for female thyroid cancer. Approximately 70–80% of children and adolescent and young adult cancer patients survived for more than 10 years. Conditional 5‐year survival for most cancer sites increased according to years, whereas those for liver cancer and multiple myeloma did not increase. We reported 10‐year cancer survival and conditional survival using population‐based cancer registries in Japan. It is important for patients and clinicians to report these relevant figures using population‐based data.

Angioimmunoblastic T-cell lymphoma (AITL) is a major type of peripheral T-cell lymphoma (PTCL). To elucidate the clinicopathologic characteristics and prognosis of AITL in Japan, we retrospectively analyzed 207 patients with AITL. The median patient age was 67 years (range, 34-91 years), with 73% of patients older than 60 years. With a median follow-up of 42 months in surviving patients, 3-year overall survival (OS) was 54% and progression-free survival (PFS) was 38%. The International Prognostic Index (IPI) and the prognostic index for PTCL, not otherwise specified (PIT) were predictive for OS in this analysis. Multivariate analysis found that age older than 60 years, elevated white blood cell (WBC) and IgA levels, the presence of anemia and thrombocytopenia, and extranodal involvement at > 1 site were significant prognostic factors for OS, and IgA, anemia, and mediastinal lymphadenopathy were significant prognostic factors for PFS. A novel prognostic model consisting of the prognostic factors for OS was successfully constructed. In conclusion, IPI and PIT were still useful for prognostication of AITL, and other factors, including those not used in IPI, such as IgA, anemia, WBC count, thrombocytopenia, and mediastinal lymphadenopathy, also significantly affected prognosis. Future investigations for IgA as a unique prognostic factor are warranted.

BACKGROUND: Malignancy‐associated hemophagocytic lymphohistiocytosis (HLH) in adults is a highly lethal disorder. Knowledge gaps have resulted in under diagnosis or delayed diagnosis.METHODS: The University of Texas MD Anderson Cancer Center pathology database (1991‐2014) was retrospectively interrogated for the keywords “hemophagocytosis” and/or “lymphohistiocytosis.” Seventy‐seven adult patients were identified. All had an underlying malignancy. Sixteen patients who had insufficient documentation were excluded.RESULTS: The majority of patients who had pathologic evidence of hemophagocytosis/lymphohistiocytosis had an incomplete workup to confirm or refute HLH using the 2004 HLH criteria (HLH‐2004; n = 8 variables), which is a common problem in adult HLH. Only 13 of 61 patients (21%) met the HLH‐2004 diagnostic criteria based on available retrospective data. To identify potentially missed cases of HLH, the published literature was reviewed, and selected additional variables known to be associated with adult HLH were selected, resulting in extended diagnostic criteria of 18 variables. Thirty‐five patients met the extended criteria, and 33 had follow‐up data available. The median overall survival of the 13 patients who met both the extended criteria and the HLH‐2004 criteria was similar to that of the 20 patients who met the extended criteria but NOT the HLH‐2004 criteria (1.43 vs 1.76 months, respectively; P = .34) indicating a similar underlying, aggressive, systemic process. Twenty‐six patients did not meet either criteria, and 17 had follow‐up data available. The median overall survival of the 17 patients who had pathologic hemophagocytosis or lymphohistiocytosis but met neither criteria was significantly superior to the survival of those who met both the extended criteria and the HLH‐2004 criteria and those who met the extended criteria but not the HLH‐2004 criteria (17.27 vs 1.43 vs 1.76, respectively; P = .002).CONCLUSIONS: The addition of diagnostic laboratory variables that are more easily and rapidly available in smaller institutions and primary care settings than the HLH‐2004 variables may be a good surrogate to raise early suspicion of malignancy‐associated HLH. Prospective validation is warranted. Cancer 2016. © 2016 American Cancer Society. Cancer 2016;122:2857–2866. © 2016 American Cancer Society

Summary: Survival outcome of patients with peripheral T‐cell lymphoma‐not otherwise specified (PTCL‐NOS) and angioimmunoblastic T‐cell lymphoma (AITL) who experience disease progression/relapse remains very poor. A total of 321 patients, newly diagnosed with PTCL‐NOS (n = 180) or AITL (n = 141) between 1999 and 2015, were analysed. Failure‐free survival (FFS) and overall survival (OS) were calculated from the time of first disease progression (FFS1, OS1), from second disease progression (FFS2, OS2) and from third progression (FFS3, OS3). With a median follow‐up duration of 52 months, 240 patients (135 PTCL‐NOS, 105 AITL) experienced progression/relapse. In patients with PTCL‐NOS, the median durations of FFS1, FFS2 and FFS3 were 3·1, 2·5 and 2·1 months, respectively. In patients with AITL, they were 5·5, 2·9 and 2·3 months, respectively. There was no improvement in FFS1 and OS1 by the time of recurrence during this period (1999–2004, 2005–2009 and 2010–2015). The median FFS after pralatrexate and romidepsin was only 3·0 and 2·5 months, respectively. The 5‐year OS rates after salvage autologous and allogeneic transplant were 32% and 52%, respectively; while the 5‐year OS rates for patients who did not undergo transplant was 10%. Further research for novel therapeutic approaches with higher efficacy and better safety profile are needed.