Haematology

Incidence and Survival Outcomes of Primary Parotid Follicular Lymphoma (PPFL): a Seer-Based Retrospective Analysis (2025)

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Type of publication:

Conference abstract

Author(s):

Backer G.; *Arunachalam J.

Citation:

HemaSphere. Conference: 30th Congress of theEuropean Hematology Association Annual Congress, EHA2025. Milan Italy. 9(Supplement 1) (pp 1214-1215), 2025. Date of Publication: 01 Jun 2025.

Abstract:

Background Primary malignant lymphomas of the parotid glands are rare, estimated to account for just 0.6% to 5% of all tumors of the parotid gland. Primary Parotid Follicular Lymphoma (PPFL) is a rare subtype of non-Hodgkin lymphoma (NHL) originating in the parotid gland, often associated with autoimmune disorders such as Sjogren's syndrome. Due to its low incidence, comprehensive population-based studies are scarce, limiting our understanding of epidemiological factors associated with PPFL and how they impact survival outcomes. Aims Using a large, population-based cancer registry, we aimed to evaluate the incidence trends and survival patterns of PPFL for the past 20 years in the US. Methods We conducted a retrospective population-based study utilizing data between 2000-2021 from the SEER (Surveillance, Epidemiology, and End Results) database. SEER is a program of the National Cancer Institute that collects epidemiological and survival data from cancer registries across the United States. We identified and extracted data from patients diagnosed with PPFL using ICD codes specific for primary tumors of the parotid gland (C07.9) and for follicular lymphoma (ICD codes 9690, 9691, 9695, 9698). Demographic data collected included year of diagnosis, age, sex, race, and area of residence. Incidence rates (IR) were calculated per 100,000 population and were age adjusted to the US standard population using SEER*stat 8.4.4. Using GraphPad Prism software, Kaplan-Meier survival analysis was done to calculate overall survival (OS) and cancer specific survival (CSS). Log rank (Mantel-Cox) test was used to compare survival outcomes between different groups. Results 793 patients with primary parotid follicular lymphoma were identified. 52% were male. 56% were older than 65 years at the time of diagnosis. 79% of patients were white, 10% were Hispanic, and 5% were black. 18% of patients had metastatic disease at initial presentation. IR was 0.050 (SE 0.002, 95% CI 0.045-0.055) for the years 2000-2010 and 0.036 (SE 0.002, 95% CI 0.032-0.040) from 2011 to 2021. Incidence was 29.7% higher in males compared to females with p = 0.0001(IR 0.048 vs 0.037, respectively). IR was 0.049 amongst white patients (SE 0.002, 95% CI 0.046 – 0.054), 0.021 amongst black patients (SE 0.003, 95% CI 0.015 – 0.029), and 0.031 amongst hispanic patients (SE 0.004, 95% CI 0.024-0.038). Patients between the ages of 75-79 years had the highest IR of 0.241 (SE 0.024, 95% CI 0.197- 0.293). Median overall survival (mOS) was 174 months. 5-year and 10-year CSS was 90% and 84%, respectively. mOS was 180 months in patients who received chemotherapy. mOS was 106 months in adults older than 65 years, compared to those younger (p<0.0001). White patients had the poorest mOS of 161 months (p=0.0042). There was no statistically significant difference in survival outcome based on gender. mOS was 109 months in patients residing in non metropolitan counties compared to mOS of 187 months in patients residing in metropolitan counties (p=0.0005). Summary/Conclusion PPFL primarily affects older adults, with high incidences in males and white patients. Though CSS rates are generally favorable, overall survival was found to be worse in white patients, adults aged > 65, and people residing in non metropolitan counties. These differences highlight potential disparities in disease outcomes and suggest the need for future research into factors influencing survival and access to care for patients with PPFL.

DOI: 10.1002/hem3.70152

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Exploring Disparities in Survival Outcomes of Primary Cardiac Diffuse Large B-Cell Lymphoma (DLBCL): a Seer Database Analysis (2025)

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Type of publication:

Conference abstract

Author(s):

Arvind S.; Shaikh G.; *Arunachalam J.; Naagendran M.S.; Meleveedu K.

Citation:

HemaSphere. Conference: 30th Congress of theEuropean Hematology Association Annual Congress, EHA2025. Milan Italy. 9(Supplement 1) (pp 3452-3453), 2025. Date of Publication: 01 Jun 2025.

Abstract:

Background Primary Cardiac Diffuse Large B-cell lymphoma (DLBCL) representing only 1.3% of cardiac tumors and 0.5% of extranodal lymphomas is an uncommon but aggressive malignancy that exclusively involves the heart or the pericardium. Favorable clinical outcomes depend on early detection and timely treatment with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy. Little research currently exists on the impact of healthcare access, socioeconomic and demographic factors on survival rates in primary cardiac DLBCL. Aims This study aims to provide an overview of survival outcomes of primary cardiac DLBCL and their association with demographic and clinical factors. Methods We collected data from SEER (Surveillance, Epidemiology, and End Results), which is a program of the National Cancer Institute (NCI) with statistical information on cancer incidence and survival in the United States. From the Research plus data (17 registries, 2000 – 2021), we extracted patients diagnosed with Diffuse large B-cell lymphoma (DLBCL) using the ICD code 9680/3 and having the primary cancer site as heart using code C38.0. Analysis was stratified based on age, sex, race, marital status, median household income and residency. Survival analysis was done with Graphpad Prism software, and survival outcomes were compared using the Log-rank test. Kaplan-Meier curves were used to visualize the data. Results 134 patients with cardiac DLBCL were identified. 64% of the patient population was 65 and older at diagnosis. 56% males and 44% females. 64% White, 14% Hispanic, 5% Black. 92% of patients lived in counties in metropolitan areas with a population ranging from 250,000 to 1 million. The marital status distribution at the time of diagnosis was as follows: 18% were single (never married), 16% were widowed, 4% were divorced, 2% were separated, 0.75% were in a domestic partnership and 54.5% were married. 72% of patients received chemotherapy, and 10% received external beam radiation therapy. Median overall survival (mOS) for primary cardiac DLBCL was 42 months. Cancer-specific survival (CSS) was 130 months. mOS was 144 months in patients under 65 years, and mOS was 26 months in patients aged 65 and older with p value=0.0010 (HR 2.402, 95% CI 1.470 to 3.926). In stage I disease, mOS was 144 months in < 65 and 33.5 months in 65+ (p = 0.0157). In stage IV, mOS was only 9 months in the 65+ age group, compared to those younger with p = 0.0254. mOS were 109 months in those who were married at the time of diagnosis, compared to a mOS of 25 months in those who were not (p value = 0.0030, HR 0.4825 95% CI 0.2867 to 0.8118). No statistically significant difference was found in overall survival when stratified based on sex, race, residency or median household income. Summary/Conclusion While the median cancer-specific survival of cardiac DLBCL was excellent (~ 10 years), the median overall survival was noted to be significantly lower (3.5 years), highlighting non-cancer causes of mortality. However, our analysis shows that advanced age (>65 yrs) has a significant negative impact on survival. While marital status seemed to be associated with better survival, reflecting the role of social support during cancer care, a firm conclusion requires understanding of additional factors. Future studies should explore the factors contributing to the non-cancer causes of mortality in cardiac DLBCL and poorer survival outcomes in the elderly to assist develop strategies to improve outcomes for this subset.

DOI: 10.1002/hem3.70152

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Epidemiological and Survival Trends of Alk-Positive Large B-Cell Lymphoma: a Seer (Surveillance, Epidemiology, and End Results) Study (2025)

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Type of publication:

Conference abstract

Author(s):

*Arunachalam J.; Meleveedu K.

Citation:

HemaSphere. Conference: 30th Congress of the European Hematology Association Annual Congress, EHA2025. Milan Italy. 9(Supplement 1) (pp 1319-1321), 2025. Date of Publication: 01 Jun 2025.

Abstract:

Background Anaplastic lymphoma kinase-positive (ALK+) large B-cell lymphoma (LBCL) is a rare subtype of diffuse large B-cell lymphoma (DLBCL), representing < 1% of all DLBCLs. Tumor growth is driven by ALK gene rearrangements which lead to proto-oncogene activation. Unlike ALK-positive anaplastic large-cell lymphoma (ALCL), ALK+ LBCL shows plasmablastic immunophenotype and often lacks typical T cell (CD2, CD3) and B-cell (CD20, CD79a) markers. It is known to be more aggressive than typical DLBCL due to limited response to conventional systemic chemotherapies like R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone), with a dismal 5-year OS of 8% in advanced stage disease. Off-label use of ALK inhibitors has demonstrated encouraging results in multiple case studies. Aims This study aims to analyze the incidence trends, and survival outcomes of ALK+ LBCL using the Surveillance, Epidemiology, and End Results database. SEER provides population-based data on cancer incidence, survival, and treatment outcomes by compiling various cancer registries across the U.S. Methods We conducted a retrospective population-based study between 2010-2021 utilizing the SEER database. Using ICD code 9737/3, we identified patients diagnosed with ALK+ LBCL. Demographic data, including age, sex, race, and stage, were collected. Incidence rates (IR) were calculated per 100,000 and age adjusted to the US standard population. Kaplan-Meier survival analysis was performed using GraphPad Prism to estimate overall survival (OS) and cancer specific survival (CSS). Log rank test was used to detect factors associated with survival outcomes. Results A total of 58 cases of ALK+ LBCL were included. IR was 0.0049 (SE 0.0010, 95% CI 0.0031-0.0073) during the years 2010-2015 and 0.0067 (SE 0.0011, 95% CI 0.0047-0.0094) from 2016-2021. IR was 0.0025 (SE 0.0007, 95% CI 0.0013-0.0044), 0.0035 (SE 0.0007, 95% CI 0.0022-0.0053), 0.0028 (SE 0.0008, 95% CI 0.0015-0.0049), and 0.0052 (SE 0.0015, 95%CI 0.0027-0.0091) in the age groups <20 years, 20-44 years, 45-64 years, and 65+ years respectively. Incidence rate ratio (IRR) was 4.08 (95% CI 2.08 – 7.98) when comparing males and females, indicating that incidence rate in males was 4 times higher than in females. 65% of patients presented with advanced stage disease at the time of diagnosis. Median time from diagnosis to treatment was 13 days. mOS median overall survival was 101 months. The 1-year and 10- year CSS rates were 86%, and 68% respectively. mOS was 53 months for those with advanced stage disease at initial presentation. mOS was 17 months for ages >65 years (p=0.0002), compared to those aged less than 65 years. In Ann Arbor stage I disease, mOS was 144 months in patients < 65 years and 33.5 months in 65+ age group (p = 0.0157). In Ann Arbor stage IV disease, mOS was only 9 months in 65+ age group with p value of 0.0254 when comparing with the younger age group. There was no statistically significant difference in survival outcomes based on sex, and race. Summary/Conclusion This is one of the largest retrospective studies on ALK+ LBCL. We found that incidence of anaplastic lymphoma kinase-positive large B-cell lymphoma is more common in males and adults older than 65. Survival outcomes continue to be poor, especially in older adults. Further multicenter research is warranted to explore the genomic framework and discover novel combination therapies, to improve patient outcomes.

DOI: 10.1002/hem3.70152

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Testing for t(3;8) in MYC/BCL6 re-arranged large B cell lymphoma identifies a high risk subgroup with inferior survival (2024)

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Type of publication:
Journal article

Author(s):
Maybury, Bernard Douglas; James, Lisa Jane BSc (Hons); Phillips, Neil; Venkatadasari, Indrani; Qureshi, Iman; Riley, James William Elliot; Talbot, Georgina; Moosai, Shivir; Giles, Hannah Victoria Dr; Chadderton, Nicola Mrs; Dowds, James; Rakesh, Pallav; Crosland, Henry; Haslam, Aidan; *Lane, Sarah; Vega Gonzalez, Monica; Davies, David; *Cherian, George; Shenouda, Amir; Kaudlay Sathyanarayana, Praveen Kumar; Starczynski, Jane; Rudzki, Zbigniew; Chaganti, Sridhar

Citation:
Blood. 2024 Apr 22.

Abstract:
A reciprocal t(3;8) BCL6::MYC fusion is common in large B cell lymphoma (LBCL) with MYC and BCL6 disruption. These pseudo-double hit cases are not adverse, whereas t(3;8) negative MYC/BCL6 lymphoma has an inferior prognosis relative to other MYC-rearranged LBCL.

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Retrospective multicentre study comparing survival outcomes in chronic lymphocytic leukaemia (CLL) with genetic risk stratification (2023)

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Type of publication:Conference abstract

Author(s):Qureshi I.; Mandal A.; Foster N.; Rose S.; Sharma K.; McIlroy G.; *Cherian G.; *Lane S.; Wandroo F.; Talbot G.; Pemberton N.; Parry H.; Moss P.; Paneesha S.

Citation:British Journal of Haematology. Conference: 63rd Annual Scientific Meeting of the British Society for Haematology. Birmingham United Kingdom. 201(Supplement 1) (pp 67-68), 2023. Date of Publication: April 2023.

Abstract:Purpose: This retrospective West Midlands multicentre study of CLL patients was conducted to review results of genetic testing in CLL patients and impact on survival. Method(s): 349 patients across the West Midlands were included in this retrospective study collected between December 2018 and March 2022. Clinical centres were asked to obtain data relating to the type and number of lines of treatment, overall response and reported genetic abnormalities. Treatment and response were categorised as per international workshop on chronic lymphocytic leukaemia (iwCLL) criteria.1 Genetic testing comprised fluorescence in situ hybridisation (FISH) for copy number abnormalities of 17p &11q, IgHV mutation status and Oxford Gene Technology CLL Next-Generation Sequencing (NGS) Panel to detect sequence variants in key genes associated with CLL (including NOTCH1, SF3B1, BIRC3, ATM and TP53). Patients were risk stratified into good, poor or not poor risk categories as per Rodriguez-Vicente et al.2 Statistical Analysis and Results: For 349 patients analysed, 143 (41%) patients were under active surveillance, and 206 (59%) patients received 1st line treatment. Out of the 206 patients that received 1st line treatment, 92 (44%) patients proceeded to 2nd line treatment, 31 (15%) patients proceeded to 3rd line treatment and 21 (10%) of patients required treatment beyond 3rd line Within the poor risk category, 173 patients were identified with 38 had TP53 deletion or mutation and 135 patients had other poor risk mutations such as NOTCH1, SF3B1, ATM. Statistical analysis did not show a difference in survival either from the diagnosis or from the date of NGS sample in the two groups of high-risk patients. Conclusion(s): The data identifies a cohort of patients with poor outcome that are negative for TP53 mutation, highlighting the importance of NGS in CLL patients at the point of treatment.

Assembly of alternative prothrombinase by extracellular histones initiate and disseminate intravascular coagulation (2021)

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Type of publication:
Journal article

Author(s):
Abrams, Simon Timothy; Su, Dunhao; Sahraoui, Yasmina; Lin, Ziqi; Cheng, Zhenxing; Nesbitt, Kate; *Alhamdi, Yasir; Harrasser, Micaela; Du, Min; Foley, Jonathan; Lillicrap, David; Wang, Guozheng; Toh, Cheng-Hock

Citation:
Blood; 2021 Jan 7;137(1):103-114

Abstract:
Thrombin generation is pivotal to both physiological blood clot formation and pathological development of disseminated intravascular coagulation (DIC). In critical illness, extensive cell damage can release histones into the circulation, which can increase thrombin generation and cause DIC, but the molecular mechanism is not clear. Typically, thrombin is generated by the prothrombinase complex, comprising activated factor X (FXa), activated co-factor V (FVa) and phospholipids to cleave prothrombin in the presence of calcium. In this study, we found that in the presence of extracellular histones, an alternative prothrombinase could form without FVa and phospholipids. Histones directly bind to prothrombin fragments F1 and F2 specifically, to facilitate FXa cleavage of prothrombin to release active thrombin, unlike FVa which requires phospholipid surfaces to anchor the classical prothrombinase complex. In vivo, histone infusion into mice induced DIC, which was significantly abrogated when prothrombin fragments F1+F2 were infused prior to histones, to act as decoy. In a cohort of intensive care unit (ICU) patients with sepsis (n=144), circulating histone levels were significantly elevated in patients with DIC. These data suggest that histone-induced alternative prothrombinase without phospholipid anchorage may disseminate intravascular coagulation, and reveal a new molecular mechanism of thrombin generation and DIC development. In addition, histones significantly reduced the requirement for FXa in the coagulation cascade to enable clot formation in Factor VIII and IX-deficient plasma, as well as in Factor VIII-deficient mice. In conclusion, this study highlights a novel mechanism in coagulation with therapeutic potential in both targeting systemic coagulation activation as well as in correcting coagulation factor deficiency.

Preoperative anemia and outcomes in cardiovascular surgery: systematic review and meta-analysis (2019)

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Type of publication:
Systematic Review

Author(s):
*Padmanabhan, Hari; Siau, Keith; *Curtis, Jason; Ng, Alex; Menon, Shyam; Luckraz, Heyman; Brookes, Matthew J

Citation:
The Annals of Thoracic Surgery. Dec 2019; vol. 108 (no. 6); p. 1840-1848

Abstract:
BACKGROUND Pre-operative anemia is common in patients scheduled for cardiac surgery. However, its effect on postoperative outcomes remains controversial. This meta-analysis aimed to clarify the impact of anemia on outcomes following cardiac surgery.METHODS A literature search was conducted on MEDLINE, Embase, Cochrane, and Web of Science databases. The primary outcome was 30-day postoperative or in-hospital mortality. Secondary outcomes included acute kidney injury (AKI), stroke, blood transfusion, and infection. A meta-analytic model was used to determine the differences in the above postoperative outcomes between anemic and non-anemic patients. RESULTS Out of 1103 studies screened, 22 met the inclusion criteria. A total of 23624 (20.6%) out of 114277 patients were anemic. Anemia was associated with increased mortality (odds ratio [OR] 2.74, 95% confidence interval [CI] 2.32-3.24; I2=69.6%; p<0?001), AKI (OR 3.13, 95% CI 2.37-4.12; I2=71.1%; p<0?001), stroke (OR 1.46, 95% CI 1.24-1.72; I2=21.6%; p<0?001), and infection (OR 2.65, 95% CI 1.98-3.55; I2=46.7%; p<0?001). More anemic patients were transfused than non-anemic (33.3 versus 11.9%). No statistically significant association was found between mortality and blood transfusion (OR 1.35, 95% CI 0.92-1.98; I2=83.7%; p=0.12) but we were not able to compare mortality with or without transfusion in those who were or were not anemic. CONCLUSIONS Preoperative anemia is associated with adverse outcomes following cardiac surgery. These findings support the addition of preoperative anemia to future risk prediction models, and as a target for risk modification.

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Anaemia and upper GI bleeding: A local experience (2017)

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Type of publication:
Conference abstract

Author(s):
*Ding M.; *Prawiradiradja R.; *Arastu Z.; *Sabri H.; *Smith M.

Citation:
United European Gastroenterology Journal; Oct 2017; vol. 5 (no. 5)

Abstract:
Introduction: There has been significant research recently on the use of blood transfusions in upper GI bleeding (UGIB) [1] with recent evidence advocating a restrictive approach to blood transfusions as well as the use of iron therapy[2] for anaemia post UGIB. Our team conducted a local retrospective analysis on patients admitted with UGIB over a six month period and analysed the use of blood transfusions at our trust which consists of two District General Hospitals. Patient data over a period of up to 12 months post discharge was collected to monitor their anaemia. Aims & Methods: Our aim was to monitor the appropriateness of transfusions in Upper GI Bleeding as well as monitoring the response to iron therapy following discharge. All inpatients that had an Upper GI endoscopy for UGIB were analysed. Electronic patient records were obtained from our endoscopy software and hospital database. Patients were selected over a time period of six months from 1/ 6/2015 to 31/12/2015. A Student's T-Test was used to compare the average increase in haemoglobin (Hb) for patients discharged with iron therapy against those who were not. Results: There were 148 patients, 81 male and 67 female. The mean age was 69.3, minimum 20 and maximum of 98. The average Hb on admission was 103 g/L (min=32 g/L, max=178 g/L). 78 out of 148 (52.7%) patients presenting with UGIB received a blood transfusion. The mean amount of blood received for those transfused was 3.7 units. 48 out of 78 (61.5%) of blood transfusions were given when Hb was below 70 g/L. 30 of 78 (38.5%) were transfused above a Hb of 70 g/L. (36.7%, n=11) of those who were transfused with Hb above 70 had cardiac risk factors. The mortality rate in those transfused above Hb of 70 was 13.3% (n=4) vs 10.4% (n=5) 41.5% (n=44) patients who were anaemic post-UGIB were discharged with iron therapy. The average rise in Hb was 26.5% for those discharged on iron vs 12.1% for those who did not. There was a statistically significant rise in Hb for those discharged with iron therapy (p<0.005) on follow-up versus those who did not receive it (n=62). The anaemia related readmission rates were similar for patients discharged on iron or not (9.1% n=4 vs 9.7% n=6). Conclusion: The data obtained supports a restrictive transfusion policy (mortality rate of 13.3% vs 10.4%). 58.5% of patients who were anaemic on discharge did not receive any iron therapy. On follow up, there was a statistically significant rise in Hb level in the group discharged on iron. Our data affirms recent evidence favouring iron therapy post UGIB. Further education is needed to improve outcome in patients presented with GI bleed.

A rare mandibular presentation in multiple myeloma (2017)

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Type of publication:
Conference abstract

Author(s):
*Mihalache G.; *MacBean A.; *Bhatia S.

Citation:
British Journal of Oral and Maxillofacial Surgery; Dec 2017; vol. 55 (no. 10)

Abstract:
Introduction: Multiple myeloma(MM)is a relatively rare malignant haematological disease, a monoclonal malignant proliferation of plasma cells that causes osteolytic lesions in the vertebrae, ribs, pelvic bone, skull and jaw. This rare disease develops mainly in men aged 50 to 80 years (mean age, 60 years). Materials: We report on a clinical case of a 45-year-old female patient who presented with spinal and long bones pain to the hospital and she was diagnosed with multiple myeloma. In order to start her treatment (radiotherapy/ chemotherapy/ bisphosphonates) conform our hospital protocol, she came for a full oral and dental assessment. No intraorally abnormalities were seen. However the orthopantomogram showed multiple rounded lesions of various sizes which have little, if any, circumferential osteosclerotic bone reaction. Results: Patient was diagnosed with multiple myeloma with mandibular involvement. She will be followed up by our team and her dentist for monitoring the oral health. Conclusions: The clear and rare multilocular image of myeloma on the orthopantomogram makes our case unique. Knowledge about the maxillofacial manifestations of multiple myeloma is important for the diagnosis of the disease and treatment, also the follow up of these patients regarding their oral manifestations. In the clinical case presented here, we highlight the interdisciplinarity needed to obtain a diagnosis and treatment of multiple myeloma.