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Background@#Acquired von Willebrand syndrome (AVWS) has not been investigated in Korean patients with Philadelphia chromosome-negative myeloproliferative neoplasm. @*Methods@#This study analyzed the prevalence at diagnosis and clinical features of AVWS in patients with essential thrombocythemia (ET), polycythemia vera (PV), prefibrotic/early primary myelofibrosis (pre-PMF), or overt PMF (PMF) diagnosed between January 2019 and December 2021 at Chungam National University Hospital, Daejeon, Korea. AVWS was defined as below the lower reference limit (56%) of ristocetin cofactor activity (VWF:RCo). @*Results@#Sixty-four consecutive patients (36 with ET, 17 with PV, 6 with pre-PMF, and 5 with PMF;30 men and 34 women) with a median age of 67 years (range, 18‒87 yr) were followed for a median of 25.1 months (range, 2.6‒46.4 mo). AVWS was detected in 20 (31.3%) patients at diagnosis and was most frequent in ET patients (41.4%), followed by patients with pre-PMF (33.3%) and PV (17.6%) patients. VWF:RCo was negatively correlated with the platelet count (r=0.937; P =0.002). Only one episode of minor bleeding occurred in a patient with ET and AVWS. Younger age (＜50 yr) [odds ratio (OR), 7.08; 95% confidence interval (CI), 1.27‒39.48; P =0.026] and thrombocytosis (＞600×10 9 /L) (OR, 13.70; 95% CI, 1.35‒138.17; P =0.026) were independent risk factors for developing AVWS. @*Conclusion@#AVWS based on VWF:RCo was common in patients with ET and pre-PMF, but less common in patients with PV in the Korean population. Clinically significant bleeding is rare in these patients.
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Advances in genomics and molecular biology over the past 20 years have resulted in numerous approved molecular targeted cancer therapies. The two main approaches for targeted cancer therapy are monoclonal antibodies and small molecules. Targeted therapy is expected to exert few side effects, but a new class of toxicities has been reported. Thus, the classical chemotherapy-induced toxicities of alopecia, myelosuppression, mucositis, nausea, and vomiting have been replaced in patients receiving targeted therapies by dermatologic, cardiovascular, gastrointestinal, endocrine, ocular, and pulmonary toxicities, and infusion reactions.Current Concepts: Targeted therapy toxicities vary, but common side effects include skin rash, diarrhea, and stomatitis. Most of these side effects are mild and can be prevented and treated. Rare and dangerous side effects, including pneumonitis, cardiotoxicities, and infusion reactions, can also be induced by targeted therapies. In most cases, toxicities are low grade (grade ≤2) and can be treated effectively, but in some cases, they can be fatal without appropriate intervention. Symptoms can be nonspecific, rendering identification of early symptoms challenging. Physicians should thus be aware of these side effects and manage toxicities appropriately.Discussion and Conclusion: The side effects of targeted therapy exert a critical impact on survival and quality of life. Most patients receiving targeted therapy need help to prevent and relieve toxicities. Management of the toxicities of targeted therapy involves patient monitoring, adjusting therapeutic dose or frequency, and providing supportive care. Serious side effects require early detection and prompt intervention, including discontinuation of targeted therapy and the use of corticosteroids.
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Background@#Non-palpable splenomegaly in patients with polycythemia vera (PV) has seldom been addressed. In this retrospective study, we evaluated non-palpable, volumetric splenomegaly defined based on age- and body surface area (BSA)–matched criteria in patients with PV diagnosed according to the 2016 World Health Organization diagnostic criteria. @*Methods@#Patients with PV who underwent abdominal computed tomography (CT) and who had palpable splenomegaly at diagnosis from January 1991 to December 2020 at Chungnam National University Hospital were enrolled. The spleen volume of each patient was determined by volumetric analysis of abdominal CT and adjusted for the patient’s age and BSA. Then the degree of splenomegaly was classified as no splenomegaly, borderline volumetric splenomegaly, overt volumetric splenomegaly, or palpable splenomegaly. @*Results@#Of the 87 PV patients enrolled, 15 (17.2%) had no splenomegaly, whereas 17 (19.5%), 45 (51.7%), and 10 (11.5%) had borderline volumetric, overt volumetric, and palpable splenomegaly, respectively. The degree of splenomegaly did not affect the cumulative incidence of thrombotic vascular events (10-year incidence: 7.7%, 0%, 22.3%, and 50.7%, respectively, P = 0.414). By contrast, splenomegaly tended to adversely affect myelofibrotic transformation (10-year cumulative incidence: 0%, 0%, 7.1%, and 30.3%, respectively, P = 0.062). Moreover, the cumulative incidence of myelofibrotic transformation was significantly higher in patients with overt volumetric or palpable splenomegaly than those with no or borderline volumetric splenomegaly (10-year incidence: 0% vs. 10.3%, respectively; 15-year incidence: 0% vs. 26.3%, respectively, P = 0.020). Overall survival (OS) differed among patients with different degrees of splenomegaly (15-year OS: 100%, 78.6%, 71.7%, and 51.9%, respectively, P = 0.021). @*Conclusion@#The degree of splenomegaly, including volumetric splenomegaly, based on ageand BSA-matched reference spleen volumes at diagnosis reflects disease progression in PV patients. Therefore, volumetric splenomegaly should be evaluated at the time of diagnosis and taken into consideration when predicting the prognosis of patients with PV.
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Background@#Information on myelofibrotic and leukemic transformations in Korean Philadelphia chromosome-negative myeloproliferative neoplasms (Ph ‒ MPNs) is limited. @*Methods@#This study retrospectively analyzed transformations in patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) prefibrotic/early primary myelofibrosis (pre-PMF), or overt primary myelofibrosis (PMF) based on the 2016 World Health Organization criteria between January 1996 and December 2020 at Chungam National University Hospital, Daejeon, Korea. @*Results@#A total of 351 patients (144 with ET, 131 with PV, 45 with pre-PMF, and 31 with PMF;204 men and 147 women) with a median age of 64 years (range, 15‒91 years) were followed for a median of 4.6 years (range, 0.2‒24.8 years). The 10-year incidence of overt myelofibrosis was higher in pre-PMF than in ET (31.3% and 13.7%, respectively; P =0.031) and PV (12.2%; P =0.003). The 10-year incidence of leukemic transformation was significantly higher in PMF than in ET (40.0% and 7.9%, respectively; P =0.046), pre-PMF (4.7%; P =0.048), and PV (3.2%; P =0.031). The 5-year incidence of leukemic transformation was higher in patients with secondary myelofibrosis (SMF) than in those with PMF (19.0% and 11.4%, respectively; P =0.040). The 5-year overall survival of patients with SMF was significantly worse than that of patients with pre-PMF (74% and 93%, respectively; P =0.027) but did not differ from that of patients with PMF (57%; P =0.744). @*Conclusion@#The rates and clinical courses of myelofibrotic and leukemic transformations in Korean patients with Ph ‒ MPN did not differ from those in Western populations.
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Purpose@#The “Act on Hospice and Palliative Care and Decisions on Life-Sustaining Treatment for Patients at the End-of-Life” was enacted on February 3, 2016 and went into effect on February 4, 2018 in Korea. This study reviewed the first year of determination to life-sustaining treatment (LST) through data analysis of the National Agency for Management of Life-Sustaining Treatment. @*Materials and Methods@#The National Agency for Management of LST provided data between February 4, 2018 and January 31, 2019 anonymously from 33,549 patients. According to the forms patients were defined as either elf-determinants or family-determinants. @*Results@#The median age of the patient was 73 and the majority was male (59.9%). Cancer patients were 59% and self-determinants were 32.1%. Cancer patients had a higher rate of self-determinants than non-cancer (47.3% vs. 10.1%). Plan for hospice service was high in cancer patients among self-determinants (81.0% vs. 37.5%, p < 0.001). In comparison to family-determinants, self-determinants were younger (median age, 67 years vs. 75 years; p < 0.001) and had more cancer diagnosis (87.1% vs. 45.9%, p < 0.001). Decision of withholding or withdrawing of LSTs in cancer patients was higher than non-cancer patients in four items. @*Conclusion@#Cancer patients had a higher rate in self-determination and withholding or withdrawing of LSTs than non-cancer patients. Continued revision of the law and education of the public will be able to promote withdrawing or withholding the futile LSTs in patients at end-of-life. Further study following the revision of the law should be evaluated to change of end-of-life care.
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Purpose@#In Korea, the “Act on Hospice and Palliative Care and Decisions on Life-sustaining Treatment for Patients at the End of Life” was enacted on February 4, 2018. This study was conducted to analyze the current state of life-sustaining treatment decisions based on National Health Insurance Service (NHIS) data after the law came into force. @*Materials and Methods@#The data of 173,028 cancer deaths were extracted from NHIS qualification data between November 2015 and January 2019. @*Results@#The number of cancer deaths complied with the law process was 14,438 of 54,635 cases (26.4%). The rate of patient self-determination was 49.0%. The patients complying with the law process have used a hospice center more frequently (28% vs. 14%). However, the rate of intensive care unit (ICU) admission was similar between the patients who complied with and without the law process (ICU admission, 23% vs. 21%). There was no difference in the proportion of patients who had undergone mechanical ventilation and hemodialysis in the comparative analysis before and after the enforcement of the law and the analysis according to the compliance with the law. The patients who complied with the law process received cardiopulmonary resuscitation at a lower rate. @*Conclusion@#The law has positive effects on the rate of life-sustaining treatment decision by patient’s determination. However, there was no sufficient effect on the withholding or withdrawing of life-sustaining treatment, which could protect the patient from unnecessary or harmful interventions.
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Purpose@#The main purpose of the Life-Sustaining Treatment Decisions Act recently enacted in Korea is to respect the patient’s self-determination. We aimed to investigate the current status and features of patient self-determination after implementation of the law. @*Materials and Methods@#Between February 2018 and January 2019, 54,635 cancer deaths were identified from the National Health Insurance Service (NHIS) database. We analyzed the characteristics of decedents who complied with the law process by self-determination compared with decedents with family determination and with decedents who did not comply with the law process. @*Results@#In multivariable analysis, patients with self-determination were younger, were less likely to live in rural areas, were less likely to belong to the highest income quintile, were less likely to be treated in general hospitals, and were more likely to show a longer time from cancer diagnosis compared with patients with family determination. Compared with patients who did not comply with the law process, patients with self-determination were younger, lived in Seoul or capital area, were less likely to belong to the highest income quintile, were treated in general hospitals, were less likely to have genitourinary or hematologic malignancies, scored higher on the Charlson comorbidity index, and showed a longer time from cancer diagnosis. Patients with self-determination were more likely to use hospice and less likely to use intensive care units (ICUs) at the end-of-life (EOL). @*Conclusion@#Decedents with self-determination were more likely to be younger, reside in the Seoul or capital area, show a longer time from cancer diagnosis, and were less likely to belong to the highest income quintile. They utilized hospice more frequently, and received less ICU care at the EOL.
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Purpose@#Six forms relating to decisions on life-sustaining treatment (LST) for patients at the end-of-life (EOL) in hospital are required by the “Act on Decision of LST for Patients at the EOL.” We investigated the preparation and creation status of these documents from the database of the National Agency for Management of LST. @*Materials and Methods@#We analyzed the contents and details of each document necessary for decisions on LST, and the creation status of forms. We defined patients completing form 1 as “self-determined” of LST, and those whose family members had completed form 11/12 as “family decision” of LST. According to the determination subject, we compared the four items of LST on form 13 (the paper of implementation of LST) and the documentation time interval between forms. @*Results@#The six forms require information about the patient, doctor, specialized doctor, family members, institution, decision for LST, and intention to use hospice services. Of 44,381 who had completed at least one document, 36,693 patients had form 13. Among them, 11,531, 10,976, and 12,551 people completed forms 1, 11, and 12, respectively. The documentation time interval from forms 1, 11, or 12 to form 13 was 8.6±13.6 days, 1.0±9.5 days, and 1.5±9.7 days, respectively. @*Conclusion@#The self-determination rate of LST was 31% and the mean time interval from self-determination to implementation of LST was 8.6 days. The creation of these forms still takes place when the patients are close to death.
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Background/Aims@#Recent changes in the diagnostic criteria for myeloproliferative neoplasms (MPNs) and increasing patient numbers necessitate updating of the data on vascular events in patients with such disorders. @*Methods@#In this single-center study, thrombotic and hemorrhagic events were retrospectively analyzed in patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) prefibrotic/early primary myelofibrosis (pre-PMF), or PMF, based on the 2016 World Health Organization diagnostic criteria. @*Results@#Of a total of 335 consecutive patients (139 ET, 42 pre-PMF, 124 PV, and 30 PMF patients; 192 males and 143 females) of median age 64 years (range, 15 to 91), 112 (33.4%) experienced a total of 126 thrombotic events before diagnosis, at the time of diagnosis, or during follow-up over a median of 4.6 years (range, 0.1 to 26.5). Cerebrovascular thrombosis (18.8%) was the most common initial event, followed by coronary heart disease (10.1%) and splanchnic (1.5%) and peripheral thrombosis (1.5%). Arterial thrombosis was more common than venous thrombosis (31.3% vs. 2.1%, respectively; p = 0.001). Thrombosis was most frequent in PV patients (39.5%), followed by patients with pre-PMF (38.1%), ET (30.9%), and PMF (13.3%). Of the 112 patients who experienced thromboses, 53 (47%) and 39 (33.9%) had thrombotic events before and at the time of MPN diagnosis, respectively. Twenty-seven patients (8.1%) experienced 29 hemorrhagic events, of which gastrointestinal bleeding (n = 20) was the most common. @*Conclusions@#Most thrombotic events occurred before or at the time of diagnosis, and the prevalence of arterial thrombosis was markedly higher than that of venous thrombosis in patients with MPN.
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Background/Aims@#Recent changes in the diagnostic criteria for myeloproliferative neoplasms (MPNs) and increasing patient numbers necessitate updating of the data on vascular events in patients with such disorders. @*Methods@#In this single-center study, thrombotic and hemorrhagic events were retrospectively analyzed in patients diagnosed with essential thrombocythemia (ET), polycythemia vera (PV) prefibrotic/early primary myelofibrosis (pre-PMF), or PMF, based on the 2016 World Health Organization diagnostic criteria. @*Results@#Of a total of 335 consecutive patients (139 ET, 42 pre-PMF, 124 PV, and 30 PMF patients; 192 males and 143 females) of median age 64 years (range, 15 to 91), 112 (33.4%) experienced a total of 126 thrombotic events before diagnosis, at the time of diagnosis, or during follow-up over a median of 4.6 years (range, 0.1 to 26.5). Cerebrovascular thrombosis (18.8%) was the most common initial event, followed by coronary heart disease (10.1%) and splanchnic (1.5%) and peripheral thrombosis (1.5%). Arterial thrombosis was more common than venous thrombosis (31.3% vs. 2.1%, respectively; p = 0.001). Thrombosis was most frequent in PV patients (39.5%), followed by patients with pre-PMF (38.1%), ET (30.9%), and PMF (13.3%). Of the 112 patients who experienced thromboses, 53 (47%) and 39 (33.9%) had thrombotic events before and at the time of MPN diagnosis, respectively. Twenty-seven patients (8.1%) experienced 29 hemorrhagic events, of which gastrointestinal bleeding (n = 20) was the most common. @*Conclusions@#Most thrombotic events occurred before or at the time of diagnosis, and the prevalence of arterial thrombosis was markedly higher than that of venous thrombosis in patients with MPN.
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Background@#The prevalence of pulmonary hypertension (PH) in myeloproliferative neoplasms (MPNs) varies among studies. We analyzed the prevalence of PH in Korean patients with Philadelphia-negative (Ph-) MPNs. @*Methods@#Medical records of patients with Ph- MPNs [essential thrombocythemia (ET), polycythemia vera (PV), or primary myelofibrosis (PMF)] visiting a single hospital between 1993 and 2019 were reviewed retrospectively. Transthoracic echocardiographic examination (TTE) results were reviewed and PH was diagnosed according to established guidelines. @*Results@#Of the 320 MPN (179 ET, 107 PV, and 34 PMF) patients, 225 (121 ET, 83 PV, and 21 PMF) underwent TTE. Of these 225 MPN patients, 19 of 121 (15.7%) ET, 9 of 83 (10.8%) PV, and 6 of 21 (28.6%) PMF patients had PH. PV patients with PH were older [71 (42‒85) vs. 61.5 (26‒91) yr, respectively; P =0.049], predominantly female (male:female ratio, 0.29 vs. 1.96, respectively; P =0.010), had lower hemoglobin levels (15.9±2.6 g/dL vs. 18.4±2.6 g/dL, respectively; P =0.010), and higher platelet counts (616.6±284.2×109/L vs. 437.7±191.7×109/L, respectively; P =0.020) than PV patients without PH. PMF patients with PH had higher monocyte counts (1.3±0.5×109/L vs. 0.8±0.4×109/L, respectively; P =0.031) than those without PH. PH was a risk factor for poor survival in PV (HR, 12.4; 95% CI, 1.8‒86.6). @*Conclusion@#PH is common in patients with Ph- MPNs and hence, careful screening for PH is warranted.
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No abstract available.
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Humans , Anemia, Pernicious , Helicobacter pylori , HelicobacterABSTRACT
BACKGROUND/AIMS: Renal complications related to BCR-ABL1-negative myeloproliferative neoplasms (MPNs) have not been examined fully in Asian populations. METHODS: We analyzed estimated glomerular filtration rate (eGFR) and its changes with time retrospectively in patients with BCR-ABL1-negative MPN from 2005 to 2015. RESULTS: The prevalence of chronic kidney disease (CKD) was 11% (6.6% having stage 3 and 4.4% having stage 4). In a linear regression analysis of eGFR versus time (years), overall, patients showed increased eGFR (mL/min/1.73 m2) by 0.51 (95% confidence interval [CI], –0.30 to 1.33; p = 0.22). Patients with polycythemia vera (PV), and those treated with hydroxyurea, showed statistically significant increases in eGFR (1.59; 95% CI, 0.28 to 2.90; p = 0.22 in PV; and 1.55; 95% CI, 0.56 to 2.54; p = 0.22 in treatment with hydroxyurea). In total, 17 patients (20.5%) showed rapid loss of eGFR (7.0 × 109 /L) and high monocyte (> 0.7 × 109 /L) counts (76.5% vs. 50%, p=0.05; 52.9% vs. 28.8%, p= 0.06, respectively). More patients had high serum lactate dehydrogenase (> 500 U/L) levels (52.9% vs. 25.8%, p = 0.03) at diagnosis. CONCLUSIONS: CKD is prevalent in patients with BCR-ABL1-negative MPN. Active cytoreductive therapy has the potential to improve kidney function in BCR-ABL1-negative MPN.
Subject(s)
Humans , Asian People , Diagnosis , Glomerular Filtration Rate , Hydroxyurea , Incidence , Kidney , Kidney Diseases , L-Lactate Dehydrogenase , Linear Models , Monocytes , Neutrophils , Polycythemia Vera , Prevalence , Renal Insufficiency, Chronic , Retrospective StudiesABSTRACT
PURPOSE: With the implementation of the Act on Life Sustaining Treatment, hospice-palliative care will be extended to non-cancer diseases including the acquired immunodeficiency syndrome (AIDS). However, there are concerns about negative perceptions and prejudice toward AIDS patients. The purpose of this study was to investigate factors related with willingness to volunteer (WV) for patients with end-stage AIDS among hospice volunteers. METHODS: Participants were 326 hospice volunteers from 19 institutions. A self-administered questionnaire was employed to investigate the participants' WV for end-stage AIDS patients, and the questions were answered using an 11-point rating scale. Demographics, volunteer activity, satisfaction with hospice volunteering, knowledge of AIDS, and attitudes towards AIDS patients (i.e., fear AIDS patients, negative attitude towards AIDS patients, personal stigmatization and stigmatizing attitude) were also investigated. A multiple regression analysis was performed to examine factors associated with WV for patients with end-stage AIDS. RESULTS: WV for patients with end-stage AIDS was 2.82 points lower than that for cancer patients (P < 0.001). The multiple regression analysis showed that the higher the level of satisfaction with hospice volunteering (P=0.002) and the lower the level of “personal stigmatization” (P < 0.001), participants showed greater WV for end-stage AIDS patients. CONCLUSION: The level of satisfaction with hospice volunteering and “personal stigmatization” were factors associated with participants' WV for patients with end-stage AIDS.
Subject(s)
Humans , Acquired Immunodeficiency Syndrome , Delivery of Health Care , Demography , Hospices , Prejudice , Stereotyping , VolunteersABSTRACT
We report a 62-year-old woman with multiple myeloma associated with cryoglobulinemia accompanied by gangrene of the digits. She presented with generalized purplish net-like discoloration (livedo reticularis), which was more prominent in the lower extremities. Multiple small shallow ulcers with crusts were found in places. In addition, gangrene was observed in both ear helices, both index fingers, and several toes. The patient had monoclonal gammopathy consisting of IgG and kappa (3.95 g/dL), cryoglobulinemia, and bone marrow plasmacytosis (42%). A biopsy of a discolored skin patch on the lower leg revealed leukocytoclastic vasculitis. She was diagnosed with multiple myeloma associated with cryoglobulinemia. Immediate plasmapheresis halted the progression of the skin lesions and digital gangrene. Two cycles of thalidomide plus dexamethasone therapy led to a partial response. This case highlights the need to search for cryoglobulinemia and multiple myeloma when we see livedo reticularis or multiple skin ulcers with obscure causes.
Subject(s)
Female , Humans , Middle Aged , Biopsy , Bone Marrow , Cryoglobulinemia , Dexamethasone , Ear , Fingers , Gangrene , Immunoglobulin G , Leg , Livedo Reticularis , Lower Extremity , Multiple Myeloma , Paraproteinemias , Plasmapheresis , Skin , Skin Ulcer , Thalidomide , Toes , Ulcer , VasculitisABSTRACT
BACKGROUND: The aim of this study was to evaluate the clinical characteristics of nondiabetic nephropathy in type 2 diabetes mellitus patients and to find a clinical significance of renal biopsy and immunosuppressive treatment in such a patient. METHODS: Renal biopsy results, clinical parameters, and renal outcomes were analyzed in 75 diabetic patients who underwent kidney biopsy at Chungnam National University Hospital from January 1994 to December 2010. RESULTS: The three most common reasons for renal biopsy were nephrotic range proteinuria (44%), proteinuria without diabetic retinopathy (20%), and unexplained decline inrena lfunction (20.0%). Ten patients (13.3%) had only diabetic nephropathy (Group I); 11 patients (14.7%) had diabetic nephropathy with superimposed nondiabetic nephropathy (Group II); and 54 patients (72%) had only nondiabetic nephropathy (Group III). Membranous nephropathy (23.1%), IgA nephropathy (21.5%), and acute tubulointerstitial nephritis (15.4%) were the three most common nondiabetic nephropathies. Group III had shorter duration of diabetes and lesser diabetic retinopathy than Groups I and II (P = 0.008).Group II had the lowest baseline estimated glomerular filtration rate (P = 0.002), with the greatest proportion of renal deterioration during follow-up (median 38.0 months, P < 0.0001). The patients who were treated with intensive method showed better renal outcomes (odds ratio 4.931; P = 0.01). Absence of diabetic retinopathy was associated with favorable renal outcome in intensive treatment group (odds ratio 0.114; P = 0.032). CONCLUSION: Renal biopsy should be recommended for type 2 diabetic patients with a typical nephropathy because a considerable number of these patients may have nondiabetic nephropathies. And intensive treatment including corticosteroid or immunosuppressants could be recommended for type 2 diabetic patients with nondiabetic nephropathy, especially if the patients do not have diabetic retinopathy.