Outcome of patients with concurrent chronic lymphocytic leukemia and Hodgkin lymphoma

Abstract Introduction/Objective Concurrent diagnosis of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) and Hodgkin lymphoma (HL) is rare. CLL/SLL can rarely advance into Hodgkin-variant of Richter transformation, or there can be a simultaneous presence of separate CLL/SLL and HL from different clonal origins. Due to its rarity, the epidemiological features and outcome of concurrent CLL and HL are not well-known. Here we have used the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database to identify concurrent CLL/SLL and HL cases and analyzed overall and disease-specific survival across various epidemiological factors. Methods/Case Report We identified all patients diagnosed with CLL/SLL and HL between the period of 1975 to 2017. Next, we identified the patients with a simultaneous CLL/SLL and HL diagnosis by matching the patient identification number. Overall survival and disease-specific survival were calculated using Kaplan-Meier curves and Cox proportional hazards models. Results (if a Case Study enter NA) We identified 166 cases with a concurrent diagnosis of CLL, and HL. 4 cases were excluded from analysis as the diagnosis of CLL and HL were not simultaneous. The age distribution of the patient showed a unimodal distribution, with most patients being diagnosed between the age of 50 and 79. 67% of patients were male, and 92% of patients were Caucasian. The majority of the CLL was diagnosed in bone marrow or lymph nodes, while almost all HL were diagnosed in lymph nodes. Both disease-specific and overall survival were worse for patients with the advanced age of diagnosis. Race or sex did not significantly affect patients’ survival. Conclusion Our comprehensive review of clinical and epidemiological features of concurrent CLL and HL cases shows that the age of diagnosis is the most significant factor in determining the survival of these patients.

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Outcome of primary pulmonary salivary gland-type carcinoma

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqab191.319 ◽

2021 ◽

Vol 156 (Supplement_1) ◽

pp. S150-S150

Author(s):

P Q Deb ◽

D Suster

Keyword(s):

Overall Survival ◽

Salivary Gland ◽

Lung Carcinoma ◽

Minor Salivary Gland ◽

Age Distribution ◽

Distribution Analysis ◽

Epidemiological Features ◽

Age Of Diagnosis ◽

Gland Type ◽

Disease Specific

Abstract Introduction/Objective Primary pulmonary salivary gland-type carcinomas are rare malignancies that arise from minor salivary gland tissue present within seromucinous submucosal glands within the lower respiratory tract. Due to their rarity (~ <1% of all primary lung malignancies), the epidemiological features and outcome of these malignancies are not well documented. Data from the National Cancer Institute’s Surveillance, Epidemiology, and End Results (SEER) database was analyzed to identify cases of primary pulmonary salivary gland carcinoma. The most common tumor types included mucoepidermoid carcinoma (MEC), adenoid cystic carcinoma (ADCC), and epithelial-myoepithelial carcinoma (EMEC). The tumors were analyzed for overall survival across various epidemiological factors. Methods/Case Report All patients diagnosed with MEC, ADCC, and EMEC with the lung designated as the primary site between the period of 1975 to 2017 were analyzed. We calculated overall survival and disease-specific survival with Kaplan-Meier curves and Cox proportional hazards models using SPSS v25. Results (if a Case Study enter NA) 323 cases of MEC, 284 cases of ADCC, and 6 cases of EMEC diagnosed as primary lung carcinoma were identified. Age distribution analysis of the patients showed a unimodal distribution for both MEC and ADCC with most patients being diagnosed after the age of 40. 54% of MEC patients were male, while 48% of ADCC patients were male. The majority of patients were Caucasians (77% for MEC and 83% for ADCC patients). Both disease-specific and overall survival were worse for patients diagnosed at the age of 60 years or above. Race or sex did not significantly affect patients’ survival. High-grade MEC showed significantly worse prognosis than low or intermediate grade MEC. EMEC cases were too few (n=6) to perform reliable survival analysis, however, the age of diagnosis was higher (45 and above) with a higher incidence among black population (50%). Conclusion Comprehensive review of clinical and epidemiological features of primary salivary gland-type lung carcinoma show that the age of diagnosis and tumor grade are the most significant factor in determining the survival of these patients.

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Race Influences Stage-specific Survival in Gastric Cancer

The American Surgeon ◽

10.1177/000313481508100327 ◽

2015 ◽

Vol 81 (3) ◽

pp. 259-267 ◽

Cited By ~ 15

Author(s):

J. Harrison Howard ◽

Jason M. Hiles ◽

Anna M. Leung ◽

Stacey L. Stern ◽

Anton J. Bilchik

Keyword(s):

Gastric Cancer ◽

Overall Survival ◽

Surgical Treatment ◽

Lymph Nodes ◽

Gastric Adenocarcinoma ◽

Surgical Patients ◽

Disease Specific Survival ◽

Stage Number ◽

Survival Differences ◽

Disease Specific

Gastric adenocarcinoma studies show improved survival for Asians but have not reported stage-specific overall survival (OS) or disease-specific survival (DSS) by race. The Surveillance, Epidemiology and End Results database was queried for cases of gastric adenocarcinoma between 1998 and 2008. We evaluated OS and DSS by race and stage. Number of assessed lymph nodes was compared among surgical patients. Of 49,058 patients with complete staging data, 35,300 were white, 7709 were Asian, and 6049 were black. Asians had significantly better OS for all stages ( P < 0.001) and significantly better DSS for Stages I ( P < 0.0001) and II ( P = 0.0006). As compared with blacks, whites had significantly better DSS for Stages I ( P < 0.0001), II ( P = 0.0055), III ( P = 0.0165), and IV ( P < 0.0001). Among the 28,133 (57%) surgical patients, average number of evaluated lymph nodes was highest for Asians ( P < 0.0001). Among surgical patients with 15 or more nodes evaluated, DSS was worse in blacks with Stage I disease ( P < 0.05). Blacks with gastric adenocarcinoma have a worse DSS, which disappears when surgical treatment includes adequate lymphadenectomy. Race-associated survival differences for gastric adenocarcinoma might simply reflect variations in surgical staging techniques and socioeconomic factors.

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Re-Evaluating the Prognosis and Transformation Risk of Chronic Eosinophilic Leukemia

Blood ◽

10.1182/blood-2020-142610 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 16-16

Author(s):

Daniel O'Leary ◽

Amrita Goyal

Keyword(s):

Overall Survival ◽

T Cell ◽

Acute Leukemia ◽

Hodgkin Lymphoma ◽

Poor Prognosis ◽

Cell Lymphoma ◽

Disease Specific Survival ◽

Chronic Eosinophilic Leukemia ◽

Aggressive Disease ◽

Disease Specific

Chronic eosinophilic leukemia, not otherwise specified, (CEL-NOS) is a rare myeloproliferative neoplasm defined by an autonomous clonal expansion of eosinophil precursors. CEL-NOS has long been considered an aggressive disease with a poor prognosis and high risk of transformation to blast phase. This impression was reinforced by a case series of 10 patients with CEL-NOS in which median overall survival was 22 months and in which one half of patients transformed to acute leukemia at a median of 20 months from time of diagnosis (Helbig et al, American Journal of Hematology, 2012). However, this has not been confirmed in any population-based or large cohort studies. We sought to evaluate whether this poor prognosis is consistent across a larger patient database. We identified 487 patients with CEL-NOS in the Surveillance Epidemiology and End Result-18 database. Patients were predominantly male (60.5%) with a median age of 57 at time of diagnosis. Overall survival at 2 years from diagnosis for the cohort was 92.7% with a disease-specific survival of 97.2%. At 5 years overall survival remained relatively high (88.7%) as did disease-specific survival (95.6%). Median follow-up was 56.5 months (range 0-191 months). Median survival for patients who died of CEL was 5 months (range 0-46 months). This raises the possibility of a dichotomous patient population wherein there is one group of patients with a good prognosis, and a second group with aggressive disease and significantly poorer prognosis. Of note, the majority of the patients who died from CEL-NOS (21/23) died within two years of diagnosis. Only three of the patients studied developed acute leukemia, two with acute myeloid leukemia and one acute lymphoblastic leukemia; all three died of their second malignancy within 28-60 months. An additional 11 patients developed lymphoma, 2 Hodgkin lymphoma and 9 non-Hodgkin lymphoma (NHL), including peripheral T-cell lymphoma (3/9), diffuse large B-cell lymphoma (3/9), angioimmunoblastic T-cell lymphoma (2/9), and Burkitt's lymphoma (1/9). This data suggests that the current impression of CEL-NOS as a rare and aggressive disease with a poor prognosis may be more nuanced than previously appreciated. The limitations of SEER data are significant and include concerns regarding both accuracy of diagnosis and collection bias. However, the incongruence between current disease perception and the large-scale data available suggest that further study is both warranted and necessary. Next steps would include the creation of a prospective national registry of patients with CEL-NOS. This would assist both in giving appropriate disease-counseling for patients and in better assessing which patients might benefit from treatment with chemotherapy. Disclosures No relevant conflicts of interest to declare.

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The Impact of Diabetes on Clinical Outcomes in Chronic Lymphocytic Leukemia

Blood ◽

10.1182/blood.v126.23.2095.2095 ◽

2015 ◽

Vol 126 (23) ◽

pp. 2095-2095

Author(s):

Erlene Kuizon Seymour ◽

Kamlai Saiya-Cork ◽

Brian Parkin ◽

Kerby Shedden ◽

Jennifer Griggs ◽

...

Keyword(s):

Overall Survival ◽

High Risk ◽

Clinical Outcomes ◽

Proportional Hazards ◽

Lymphocytic Leukemia ◽

Diabetic Patients ◽

Disease Specific Survival ◽

Prognostic Variable ◽

Disease Specific

Abstract Introduction: Diabetes negatively influences cancer-related outcomes in several malignancies. Previous studies have demonstrated key drivers in the pathogenesis of chronic lymphocytic leukemia (CLL) involving the insulin and insulin-like receptor pathways. We aimed to investigate whether hyperinsulinemic states such as diabetes impact clinical outcomes in CLL patients. Methods: This is a retrospective cohort study of 291 patients enrolled into a CLL translational clinical trial from 2004-2011 at a single university. Clinical outcomes were evaluated through exhaustive medical record review. Diabetes was classified as Type 1, Type 2, or steroid-induced diabetes requiring therapy. Covariates included Rai stage and intensity of CLL treatment, determined on whether patients had low- or high-intensity regimens. The primary end points were disease-specific and overall survival. Overall survival and disease-specific survival was measured from date of diagnosis to date of death, or date of censoring, which was February 2015. Disease-specific death was defined as death due to infection, CLL progression and/or complication from CLL therapy. Causes of death were verified by 2 independent physician reviewers with expertise in CLL, and dates of death were confirmed by the national death index database. Disease-specific and overall survival was calculated using Kaplan-Meier analysis. Proportional hazards (Cox) regression models were fit to the data using overall or disease-specific survival as endpoints. We also performed a separate parallel survival analysis including only patients who were untreated prior to enrollment. Multivariate analyses were performed incorporating known high-risk CLL features including Rai stage, IgVH mutation status, del11q, del17p, and TP53 mutation status. Cox proportional hazards were used in the multivariate analysis. Results: Over ten years, 291 CLL patients were enrolled into our study. Of these, 19% had diabetes (Type 2 DM, n = 46, Type 1 DM, n = 2, steroid-induced DM, n = 8). Only 5 patients were treated with low-intensity regimens. Disease-specific survival was significantly shorter in diabetics compared to non-diabetics. The median disease-specific survival in diabetic patients was 128.4 months, compared to non-diabetics, which was not reached (p=0.0068). Overall survival was also significantly shorter in the diabetic patients. The median overall survival in diabetic patients was 124.5 months, compared to 260.0 months in non-diabetic patients (p=0.0002). The majority of deaths in the diabetic cohort (71%) and the non-diabetic cohort (77%) were due to CLL-specific deaths. There was a substantial amount of infection-related deaths in the diabetic cohort (46%) and the non-diabetic cohort (32%). The presence of diabetes retained its significance as a prognostic variable of survival when analyzed against high-risk CLL features. Table 1. Multivariate analysis of 236 CLL patients with available data points Patient variable Hazard Ratio p -value 95% CI Diabetes 2.19 0.001 1.36-3.53 Rai stage 1.29 0.309 0.79-2.08 IgVH unmutated 2.81 0.000 1.74-4.54 Deletion 17p/mTP53 2.30 0.001 1.41-3.76 Deletion 11q 1.29 0.429 0.68-2.45 When analyzing only CLL patients untreated prior to enrollment, diabetes maintained a significantly poorer disease-specific and overall survival- as well as independent prognostic variable status against high-risk CLL features. Conclusion: Diabetes is associated with significantly worse disease-specific and overall survival among patients with CLL. The 11 year difference in median overall survival is impressive, as the life expectancy of an average diabetic patient at the median age of our cohort is estimated to be 19 to 22 years based on recent national studies. CLL-related deaths comprised the majority of both diabetic and non-diabetic deaths. Diabetes is an independent poor prognostic variable when analyzed against known high-risk features of CLL. Future investigations into other clinical variables, including glycemic control and benefits of therapies with less hematologic toxicity and/or infectious risk, are of interest. Disclosures Malek: Janssen Pharmaceuticals: Research Funding; Abbvie: Equity Ownership; Gilead Sciences: Equity Ownership.

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Survival Patterns among Chronic Lymphocytic Leukemia and Other Lymphoma Patients with Family History of Lymphoma.

Blood ◽

10.1182/blood.v110.11.4683.4683 ◽

2007 ◽

Vol 110 (11) ◽

pp. 4683-4683

Author(s):

Lesley A. Anderson ◽

Ruth M. Pfeiffer ◽

Joshua S. Rapkin ◽

Gloria Gridley ◽

Lene Mellemkjaer ◽

...

Keyword(s):

Overall Survival ◽

Family History ◽

Chronic Lymphocytic Leukemia ◽

Hodgkin Lymphoma ◽

Population Based ◽

Lymphocytic Leukemia ◽

Older Age ◽

Survival Outcomes ◽

History Of ◽

Survival Patterns

Abstract Background. A role for genetic factors in chronic lymphocytic leukemia (CLL) is unequivocal based on evidence from multiply affected families, from case series, twin and case-control studies, and population-based registry studies. Similar characteristics have been observed for non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL). Population-based studies have found CLL, NHL and HL to co-occur in families. A few hospital-based series have explored survival outcomes in familial vs. sporadic CLL patients based on the hypothesis that genetic mechanisms involved in the causation of familial lymphomas might influence survival, however these have been based on small numbers with inconsistent findings. We have conducted a large study in Scandinavia including more than 41,000 lymphoma patients to quantify survival outcomes among familial vs. sporadic patients. Methods. We used the population-based central Cancer and Multigenerational Registries in Sweden and Denmark to identify all CLL (n=7749), NHL (n=25801), and HL (n=7476) patients diagnosed 1958–2001, with linkable first-degree relatives. All relatives were linked with the Cancer Registries to obtain information on CLL, NHL, and HL. Using Cox proportional hazard models, we calculated hazard ratios (HRs) and 95% confidence intervals (CIs) as measures of overall survival for CLL, NHL, and HL patients with vs. without family history of lymphoma. Results. We found 85 (1.1%) CLL, 207 (0.8%) NHL, and 96 (1.3%) HL patients with family history of lymphoma. Overall survival was similar for CLL (HR=1.24, 95% CI 0.93–1.67), NHL (HR=0.97, 95% CI 0.80–1.16), and HL (HR=0.78, 95% CI 0.51–1.19) patients with vs. without family history of lymphoma. Risk-estimates were similar when we calculated overall survival by age at lymphoma diagnosis (above vs. below median age), year at diagnosis (before vs. after 1987), and by sex. Consistent with the literature, including all CLL, NHL and HL patients, older age (p<0.001) and male gender (p<0.001) was associated with poorer survival. However, when analyses were restricted to patients with family history of lymphoma, only older age (p<0.005) remained significant. Conclusions: Survival patterns were similar for CLL, NHL, and HL patients with vs. without family history of lymphoma, suggesting that familial lymphomas do not have an altered clinical course. Overall, there is no evidence at this time to modify therapeutic strategies for patients with CLL, NHL, or HL based solely on family history.

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