bluuebunny/crossref_metadata_2025_split huggingface dataset:
Bortezomib with Dexamethasone in Newly Diagnosed Patients with Primary Systemic Light Chain Amyloidosis or Multiple Myeloma-Associated AL Amyloidosis
Authors: Huang Beihui , Li Juan , Liu Junru , Zheng Dong , Chen Mei , Zhou Zhenhai , Xu Duorong , Zou Waiyi 
Published: October 2019

Abstract
Abstract 5036

Objective:
To assess the efficacy and tolerability of bortezomib with dexamethasone for patients with primary systemic light chain (AL) amyloidosis or multiple myeloma-associated AL amyloidosis.

Methods:
Twelve newly diagnosed patients with primary systemic AL amyloidosis and six patient with multiple myeloma-associated AL amyloidosis were treated with a combination of bortezomib (1. 3 mg/m2 d1, 4, 8, 11) and dexamethasone (20 mg d1–4).

Results:
Sixteen patients was evaluable. 12/16 had a hematologic response and 6/16 (37. 5%) a hematologic complete response. Median cycles to response was 1 cycle and median cycles to best response was 2 cycles. In patients with primary AL amyloidosis, 8/10 (80. 0%) had a hematologic response and 5/10 (50. 0%) a hematologic complete response. In patients with myeloma-associated AL amyloidosis, 7/10 (70. 0%) had a hematologic response and 1/6 (16. 7%) a hematologic complete response. Twelve patients (75. 0%) had a response in at least one affected organ, in which 7 in patients with primary AL amyloidosis and 5 in myeloma-associated AL amyloidosis. Person correlation between hematologic response and organ response was 0. 667 (p=0. 005). Fatigue, diarrhea and infection were the most frequent side effects. Three patients developed herpes zoster and had to stop chemotherapy.

Conclusions:
VD produces rapid and high hematological responses in the majority of patients with newly diagnosed AL regardless of primary or associated with myeloma. It is well tolerated with few side effects. This treatment may be a valid option as first-line treatment for newly diagnosed patients with primary systemic AL amyloidosis and multiple myeloma-associated AL amyloidosis.

Disclosures:
No relevant conflicts of interest to declare.
Lymphocytopenia Is Associated with an Increased Risk of Severe Infections in Patients with Multiple Myeloma Treated with Bortezomib-Based Regimens
Authors: Jung Sung-Hoon , Ahn Jae-Sook , Kang Seung-Ji , Yang Deok-Hwan , Kim Yeo-Kyeoung , Kim Hyeoung-Joon , Lee Je-Jung 
Published: October 2019

Abstract
Abstract 5042
Bortezomib is a proteasome inhibitor with potent antimyeloma activity in relapsed/refractory multiple myeloma (MM) patients. We evaluated the type and factors affecting the onset of infectious complications and mortality owing to infection in MM patients treated with bortezomib-based regimens. We reviewed 139 patients with MM treated with regimens containing bortezomib in order to assess the type and factors affecting the development of severe infections. Infections occurred in 56 (40. 3%) of 139 patients and 83 (7. 8%) cases of the 1, 069 evaluable cycles. Severe infections developed in 43 (30. 9%) patients and ten patients (7. 1%) died during bortezomib-based treatment. Multivariate analysis determined lymphocytopenia grade 3–4 (OR 3. 17, 95% CI 1. 38–7. 31, p = 0. 007) and number of cycle ° Â 8 cycles (OR 3. 91, 95% CI 1. 39–11. 02, p =0. 010) as risk factors associated with increased severe infection. This study showed that MM patients who received bortezomib-based regimens are at a higher risk of severe infections within eight cycles of treatment during especially severe lymphocytopenic period. MM patients treated with bortezomib-based regimens should be closely monitored for the development of infectious complications during lymphocytopenia.
Table I. Multivariate analysis of factors affecting severe infection development Parameter OR (95% CI) p-Value Age (> 65) 1.78 (0.77–4.13) 0.180 Immunophenotype IgA 1.51 (0.60–3.77) 0.379 Lymphocytopenia (grade 3–4) 3.17 (1.38–7.31) 0.007 Neutropenia (grade 3–4) 1.25 (0.55–2.85) 0.600 Number of cycles (°Â 8 cycles) 3.91 (1.39–11.02) 0.010

Disclosures:
No relevant conflicts of interest to declare.
Time to Treatment of Greater Than 1 Year Optimally Stratifies Patients with Improved Survival in Mantle Cell Lymphoma
Authors: Shah Bijal D. , Martin Peter , Cultrera Jennifer L. , Bello Celeste M. , Sokol Lubomir , Sotomayor Eduardo M. 
Published: October 2019

Abstract
Abstract 5082

Introduction:
Retrospective analyses suggest an improved outcome for patients with mantle cell lymphoma (MCL) in whom safe watchful waiting can be employed. We explored our own cohort of patients treated at the H Lee Moffitt Cancer Center in an effort to validate these data.

Methods:
We retrospectively identified 280 patients with MCL treated at our institution, for whom treatment data are available among 260. Patients were stratified according to whether they were treated within 3 months, within 3–12 months, and beyond 12 months. Treatment includes radiation, surgery, chemotherapy, immunotherapy, or any combination there-of. Median overall survival (OS) is calculated from date of diagnosis to death. Median progression free survival (PFS) is calculated from time of treatment start to progression or death.

Results:
Of 260 evaluable patients 191 were treated within 3 months of diagnosis, 33 were treated between 3–12mo, and 36 had received no treatment beyond one year. The median OS for each group were approximately 62mo, 74mo, and 130mo, respectively (Figure 1A). Median PFS following treatment initiation was nearly indistinguishable between the three groups, at approximately 24mo, 26mo, and 19mo, respectively (Figure 1B).

Conclusions:
Time to treatment of greater than 12 months may identify those with a more indolent course. However, once treatment is required, patients appear to experience similar treatment benefit.

Disclosures:
No relevant conflicts of interest to declare.
Adult T-Cell Leukemia/Lymphoma in Peru: A Report of 120 Cases
Authors: Beltran Brady E , Cotacallapa Erick , Castillo Jorge J 
Published: October 2019

Abstract
Abstract 5110

Background:
Adult T-cell leukemia/lymphoma (ATLL) is an aggressive subtype of peripheral T-cell lymphoma associated with the human T-cell lymphotropic virus type-I (HTLV-1). ATLL follows a specific geographic pattern and is more frequently seen in Southern Japan, the Caribbean basin, the Middle East and South America. This retrospective study attempts to describe the characteristics and define prognostic factors for patients with ATLL in Peru.

Methods:
We included consecutive cases of ATLL identified at our institution between January 1997 and January 2012. Diagnosis of ATLL was based on clinical history and histological findings consistent with ATLL, and either a positive HTLV-1 serology or evidence of HTLV-1 integration in the pathological sample. We used the Shimoyama classification to differentiate among ATLL subtypes. Patients' characteristics will be presented descriptively. For the univariate analysis, overall survival (OS) curves were estimated using the Kaplan-Meier method, and compared using the log-rank test.

Results:
We have included 120 patients who met the criteria for ATLL. The median age was 61 years (range 23–92 years), and 46% of patients was &gt;60 years. The male-to-female ratio was 1. 2:1. ATLL types were acute in 49 (40%), lymphomatous in 53 (44%), cutaneous in 12 (10%), smoldering in 3 (3%) and chronic in 3 (3%) patients. An ECOG performance status &gt;1 was seen in 51%, B symptoms were reported in 63%, stage III/IV in 85%, bone marrow involvement in 46%, and LDH was increased in 87% of the cases. An IPI score 0–2 was reported in 31% and score 3–5 in 69% of patients. Lymphocyte count &lt;1000/uL occurred in 38% and monocyte count &gt;1000/uL was in 33% of the cases. Chemotherapy was administered in 82% of patients, 18% could not receive treatment due to a bad performance status. The median OS for the entire group was 5. 5 months. Acute ATLL had a significantly shorter OS than lymphomatous (p&lt;0. 001). A high IPI score (score 3–5) was associated with a shorter OS (p=0. 01).

Conclusions:
ATLL is a heterogeneous disease with distinct clinical features and outcomes. Based on our series, which represents the largest Latin-American cohort, acute had a worse prognosis than lymphomatous ATLL, and the IPI score should be used for risk-stratification.

Disclosures:
No relevant conflicts of interest to declare.
Clinicopathologic Characteristics and Clinical Outcomes in Patients with Testicular Non-Hodgkin Lymphoma
Authors: Kim Jeongseok , Yoon Dok Hyun , Park Ji Hyun , Jang Il Young , Kim Shin , Lee Kyoungmin , Kang Eun Hee , Huh Jooryung , Park Chan-Sik , Suh Cheolwon 
Published: October 2019

Abstract
Abstract 5111

Purpose
We aimed to evaluate the clinicopathologic characteristics and clinical outcomes in patients with testicular non-Hodgkin lymphoma.

Material and Methods
We reviewed the medical records of 24 patients with testicular non-Hodgkin lymphoma diagnosed at the Asan Medical Center between November, 2000 and June, 2012.

Results
Median age of the patients was 52 years (23–79 years). Histopathologic subtypes were as follows: DLBCL (n=18, 75%), Burkitt's lymphoma (n=2, 8. 3%), extranodal natural killer/T-cell lymphoma (NKTCL) (n=2, 8. 3%), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n=1, 4. 2%) and T-cell lymphoblastic lymphoma (T-LBL) (n=1, 4. 2%). Ten patients (41. 7%) were in stage I, 1 patient (4. 2%) in stage II, and the other 13 patients (54. 2%) were in stage IV. Eastern Cooperative Oncology Group Performance Status (ECOG PS) was 1 in 22 patients (87. 5%) and >1 in 5 patients (20. 8%). Serum LDH levels were elevated in 14 patients (58. 3%). International Prognostic index (IPI) score was low (0–1) in 6 patients (25%), low-intermediate (2) in 8 patients (33. 3%), high-intermediate (3) in 7 patients (29. 2%), high (4–5) in 3 patients (12. 5%). B-symptoms were present in 4 patients (16. 7%). Bilateral testicular involvement was observed in 5 patients (20. 8%).
Fifteen patients (62. 5%) underwent orchiectomy as an initial therapeutic and diagnostic procedure. All the patients underwent chemotherapy: R-CHOP (n=16, 66. 6%), CHOP (n=2, 8. 3%), and other regimens (n=6, 25%). None received intrathecal prophylaxis just except a T-LBL patient. Prophylactic radiotherapy to contralateral testis was given in 12 patients (50%).
Twenty-one patients (87. 5%) achieved complete response. At a median follow-up duration of 22 months (1–139 months), 2 patients (8. 3%) showed disease progression and 7 patients (29. 2%) experienced disease recurrence; in the central nervous system (n=2, 8. 3%), regional lymph nodes (n=3, 12. 5%), bone marrow (n=1, 4. 2%), nasopharynx (n=1), skin (n=1), and testicular bed (n=1). Five patients (20. 8%) died of sepsis (n=3, 12. 5%) or progression of disease (n=2, 8. 3%). Median progression free survival and overall survival were 20 months (1–139 months) and 22 months (1–139 months), respectively.
ECOG PS >1 (p=0. 015) and bilateral testicular involvement (p=0. 000) were associated with a significantly short progression free survival (PFS). ECOG PS >1 (p=0. 001), high-intermediate or high risk of IPI (p=0. 010), presence of B symptoms (p=0. 035), and bilateral testicular involvement (p=0. 001) were associated with a significantly short overall survival.

Conclusions
Testicular lymphoma is a rare but aggressive extranodal lymphoma. High ECOG PS, high IPI, B symptom, and bilateral testicular involvement were associated with poor prognosis.

Disclosures:
No relevant conflicts of interest to declare.
Case Series of Primary Breast Diffuse Large B-Cll Lymphoma in Ampang Hospital, Malaysia
Authors: Kuan Jew Win , Lau Ngee-Siang , Wong Lily L , Lau Lee-gong , Chang Kian Meng 
Published: October 2019

Abstract
Abstract 5113

INTRODUCTION
Breast is a rare extranodal site of non-Hodgkin's lymphoma. Traditionally, primary breast lymphoma (PBL) is defined as disease localized to one or both breasts with or without regional lymph nodes involvement (ipsilateral axillary and/or supraclavicular lymph nodes). Recently, there is a suggestion to define breast lymphoma by the number of involved extranodal disease. The commonest histology of PBL is diffuse large B-cell lymphoma (DLBCL). Treatment with CHOP-like regime (cyclophosphamide, doxorubicin, vincristine and prednisolone) in PBL-DLBCL remains unsatisfactory with 5-year progressive free survival rates of approximately 50–65%, which is generally worse than the localized nodal or other extranodal disease. The reason to the poorer outcome might be explained by the genotype. About 80% of the PBL-DLBCL is non-germinal center B-cell or activated B-cell (ABC) subtype, a poorer prognostic genotype compared to germinal center B-cell (GCB) subtype. The data of rituximab (R) overcoming genotypic effect of PBL-DLBCL is still inconclusive. However, the recent matched pair analysis showed similar 3-year overall survival rate to nodal DLBCL if using rituximab and CHOP. Immunoblast morphology appeared to be one of the prognostic factors besides the bulkiness of the disease and the high proliferation index (PI). We present our case series of DLBCL who presented with primary breast swelling from 2009 till June 2012.

METHOD
All the DLBCL cases diagnosed from 1st January 2009 till 30th June 2012 were filtered for breast involvement from database. Only cases presented with primary breast swelling and biopsy-proved DLBCL breast lesion were studied. Six cases were identified. All are female.

CONCLUSION
Looking at the six cases of primary breast DLBCL, the outcome is poor despite rituximab based chemotherapy. Better chemotherapy regime other than CHOP and agent like bortezomib and upfront autologous HSCT should be explored.

Disclosures:
No relevant conflicts of interest to declare.
Thrombin Generation Responses to Human Factor XIa in Plasma of Animal Species
Authors: Liang Yideng , Struble Evi B , Ma Li , Woodle Samuel A , Jha Naveen , Scott Dorothy , Lee Timothy K , Ovanesov Mikhail V 
Published: October 2019

Abstract
Abstract 5142
Thromboembolic events (TEE), including deep venous thrombosis and myocardial infarction, have been reported in patients receiving immunoglobulin intravenous (IVIG). Recent research showed that many TEE-induced IVIG samples were contaminated with human coagulation factor XIa (FXIa). At present, there is no systematic investigation of animal models that can be used to assess FXIa dependent thrombogenicity risk in animals and be predictive for human response. To assist in species selection for in vivo animal studies, we compared the magnitude and kinetic parameters of thrombin generation in commercially available human and animal plasma samples in response to human FXIa. A low sample volume, highly reproducible thrombin generation test was developed for this study. In our assay, thrombin generation in plasma (20 microliters) is initiated by physiological activator tissue factor in the presence of procoagulant phospholipids, sample (FXIa), buffer and calcium (combined volume of all additions to plasma is 20 microliters). The thrombin generation response to human FXIa was evident from increased thrombin peak height and/or shortened time to thrombin peak. The minimal effective FXIa concentration was species-dependent and ranged from below 0. 1 pM in monkey plasma to over 100 nM in BalbC mouse. The FXIa sensitivity of commercial plasma samples was aligned in the following order: Rhesus and Cynomolgus Monkeys > Human Donors > New Zealand White Rabbit and Sprague Dawley Rat > Hartley Guinea Pig and CD1 Mouse > Lewis Rat > Wistar and Fisher 344 Rats > C57BL6 and Blab C Mice. However, further experiments on freshly collected guinea pig plasma demonstrated that preanalytical variables, notably, blood collection and plasma freezing and thawing techniques, are critical to achieve optimal sensitivity of the thrombin generation test to FXIa. Therefore, variable quality of commercial plasma may have affected observed interspecies differences in thrombin generation. Collectively, these findings will help to clarify differences in minimal thrombogenic doses of FXIa observed in various animal models and levels of FXIa associated with thrombotic events in humans.
This research was supported by the FDA Office of Women's Health grant (2012).

Disclosures:
No relevant conflicts of interest to declare.
Cerebral Sinovenous Thrombosis Related to Iron Deficiency Anemia As a Result of Severe Menorrhagia: A Case Report
Authors: Corrales-Medina Fernando F. , Rodriguez Nidra , Egas-Bejar Daniela E , Valdivia-Ascuna Zoila , Grant Leon , Mancias Pedro 
Published: October 2019

Abstract
Abstract 5143
Cerebral sinovenous thrombosis (CSVT) is a rare condition with a wide spectrum of non-specific symptoms, which makes it occasionally difficult to diagnose. CSVT has been associated with various etiologies including dehydration, hypercoagulable states, neoplasmic invasion of a venous sinus, intracranial and systemic infections, use of oral contraceptives, puerperium and pregnancy. Although iron deficiency anemia (IDA) has been reported as a cause of CSVT in several pediatric cases, this association is extremely rare. To our knowledge, IDA-associated CSVT in teenagers has been rarely reported.
Here, we present the case of a 13-year old female with complete thrombosis of the vein of Galen, the straight sinus, and the left internal cerebral vein related to iron deficiency anemia as a result of severe menorrhagia. She initially presented with three-week history of headaches, nausea and vomiting. She was initially diagnosed and treated for bacterial sinusitis by her primary pediatrician, without symptom relief. She then suffered a syncopal episode, reason why she was evaluated at our institution. Initial laboratories revealed a slightly increased PT (15. 6 secs), hemoglobin of 5. 1 g/dL, MCV 63. 6 and D-dimer 1. 31. Non-contrast brain CT demonstrated no evidence of stroke, hemorrhage or mass. However, an abnormal hyperdensity in the straight sinus, inferior sagittal sinus, vein of Galen, and deep cerebral veins, were suggestive of venous thrombosis. Findings were then confirmed by brain MRI/MRV/MRA and CT angiogram. A bleeding work-up performed based on her history of heavy menses was normal. Her ferritin level was significantly decreased (6 ng/mL). Iron supplementation was initiated as well as progestin-only hormonal therapy for menstrual cycle regulation. The patient's clinical status improved back to baseline during her 8-day admission. She was discharged home on anticoagulation with scheduled follow up. Brain MRI/MRV six months later showed patency of the cerebral veins and dural venous sinuses.
IDA should be considered as an underlying cause of CSVT in pediatric patients, particularly in patients with other risk factors for thrombosis including dehydration, regardless of their age. Early recognition and management of CSVT is critical to minimize the possibility of permanent neurologic damage. Physicians should be aware of the non-specific signs and symptoms of CSVT and should have a high index of suspicion in patients with anemia.

Disclosures:
No relevant conflicts of interest to declare.
Prevalence of 25-Hydroxyvitamin D Deficiency in Korean Patients with Anemia
Authors: Yoo Eun-Hyung , Cho Hyun-Jung 
Published: October 2019

Abstract
Abstract 5164

Background:
Vitamin D deficiency is a very common health problem in Korea. Vitamin D has been suggested to have an important role on nonskeletal functions including cellular proliferation and differentiation, muscle function, immunity and erythropoiesis. Recent studies have been reported that vitamin D deficiency had associated with iron deficiency anemia, anemia of chronic kidney disease, and anemia of inflammation. In this study, we investigated the prevalence of vitamin D deficiency in Korean patients with anemia and also analyzed the association between vitamin D status and specific subtypes of anemia.

Methods:
A total of 147 anemic patients (median age 66 years, range 19∼91 years) and 300 nonanemic controls (median age 60 years, range 29∼87 years) were included. Anemia was defined according to World Health Organization (WHO) criteria. Serum 25- hydroxyvitamin D [25(OH)D] was measured using electrochemiluminescence immunoassay. The deficiency of 25(OH)D was defined as <20 ng/mL and severe deficiency was defined as <10 ng/mL. We compared serum 25(OH)D levels based on the presence and subtypes of anemia.

Results:
The prevalence of 25(OH)D deficiency was 74% (109/147) and 70% (210/300) in anemic (median Hb 9. 6 g/dL) and nonanemic group (median Hb 14. 3 g/dL), respectively. The prevalence of severe 25(OH)D deficiency was significantly higher in anemic group than in nonanemic group [44. 8% (66/147) vs 11. 7% (35/300), P<0. 0001] Odds ratio for severe 25(OH)D deficiency in anemic patients was 6. 17 (95% CI 3. 820–9. 965, P<0. 0001). The prevalence of 25(OH)D deficiency was not different between iron deficiency anemia (IDA) group and anemia of chronic disease (ACD) group. However, the serum 25(OH)D levels of ACD patients were lower than those of IDA patients in male (median 25(OH)D 14. 34 ng/mL vs 23. 04 ng/mL, P=0. 04).

Conclusion:
This study demonstrates that severe vitamin D deficiency is associated with anemia in Korea. Although vitamin D deficiency is also very common in nonanemic Korean population, anemia is related to much worse vitamin D deficient status. Multiple factors including poor nutritional status and potential roles of vitamin D on inflammation and erythropoiesis might be considered.

Disclosures:
No relevant conflicts of interest to declare.