ABSTRACT
A randomized phase-II study was performed in low/int-1 risk MDS (IPSS) to study efficacy and safety of lenalidomide without (arm A) or with (arm B) ESA/G-CSF. In arm B, patients without erythroid response (HI-E) after 4 cycles received ESA; G-CSF was added if no HI-E was obtained by cycle 9. HI-E served as primary endpoint. Flow cytometry and next-generation sequencing were performed to identify predictors of response. The final evaluation comprised 184 patients; 84% non-del(5q), 16% isolated del(5q); median follow-up: 70.7 months. In arm A and B, 39 and 41% of patients achieved HI-E; median time-to-HI-E: 3.2 months for both arms, median duration of-HI-E: 9.8 months. HI-E was significantly lower in non-del(5q) vs. del(5q): 32% vs. 80%. The same accounted for transfusion independency-at-week 24 (16% vs. 67%), but similar in both arms. Apart from presence of del(5q), high percentages of bone marrow lymphocytes and progenitor B-cells, a low number of mutations, absence of ring sideroblasts, and SF3B1 mutations predicted HI-E. In conclusion, lenalidomide induced HI-E in patients with non-del(5q) and del(5q) MDS without additional effect of ESA/G-CSF. The identified predictors of response may guide application of lenalidomide in lower-risk MDS in the era of precision medicine. (EudraCT 2008-002195-10).
Subject(s)
Hematinics , Myelodysplastic Syndromes , Humans , Lenalidomide/pharmacology , Hematinics/pharmacology , Erythropoiesis , Myelodysplastic Syndromes/drug therapy , Myelodysplastic Syndromes/genetics , Granulocyte Colony-Stimulating Factor/pharmacology , Chromosome Deletion , Chromosomes, Human, Pair 5/genetics , Treatment OutcomeABSTRACT
INTRODUCTION: The web-based self-management application Oncokompas was developed to support cancer survivors to monitor health-related quality of life and symptoms (Measure) and to provide tailored information (Learn) and supportive care options (Act). In a previously reported randomised controlled trial (RCT), 68% of 655 recruited survivors were eligible, and of those 45% participated in the RCT. Among participants of the RCT that were randomised to the intervention group, 52% used Oncokompas as intended. The aim of this study was to explore reasons for not participating in the RCT, and reasons for not using Oncokompas among non-users, and the use and evaluation of Oncokompas among users. METHODS: Reasons for not participating were assessed with a study-specific questionnaire among 243 survivors who declined participation. Usage was investigated among 320 participants randomised to the intervention group of the RCT via system data and a study-specific questionnaire that was assessed during the 1 week follow-up (T1) assessment. RESULTS: Main reasons for not participating were not interested in participation in scientific research (40%) and not interested in scientific research and Oncokompas (28%). Main reasons for not being interested in Oncokompas were wanting to leave the period of being ill behind (29%), no symptom burden (23%), or lacking internet skills (18%). Out of the 320 participants in the intervention group 167 (52%) used Oncokompas as intended. Among 72 non-users, main reasons for not using Oncokompas were no symptom burden (32%) or lack of time (26%). Among 248 survivors that activated their account, satisfaction and user-friendliness were rated with a 7 (scale 0-10). Within 3 (IQR 1-4) sessions, users selected 32 (IQR 6-37) topics. Main reasons for not using healthcare options in Act were that the information in Learn was already sufficient (44%) or no supportive care needs (32%). DISCUSSION: Main reasons for not reaching or using Oncokompas were no symptom burden, no supportive care needs, or lack of time. Users selected many cancer-generic and tumour-specific topics to address, indicating added value of the wide range of available topics.
ABSTRACT
BACKGROUND: Oncokompas is a web-based self-management application that supports cancer survivors to monitor their health-related quality of life (HRQOL) and symptoms, and to obtain personalised feedback and tailored options for supportive care. In a large randomised controlled trial among survivors of head and neck cancer, colorectal cancer, and breast cancer and (non-)Hodgkin lymphoma, Oncokompas proved to improve HRQOL, and to reduce several tumour-specific symptoms. Effect sizes were however small, and no effect was observed on the primary outcome patient activation. Therefore, this study aims to explore which subgroups of cancer survivors may especially benefit from Oncokompas. MATERIALS AND METHODS: Cancer survivors (n = 625) were randomly assigned to the intervention group (access to Oncokompas, n = 320) or control group (6 months waiting list, n = 305). Outcome measures were HRQOL, tumour-specific symptoms, and patient activation. Potential moderators included socio-demographic (sex, age, marital status, education, employment), clinical (tumour type, stage, time since diagnosis, treatment modality, comorbidities), and personal factors (self-efficacy, personal control, health literacy, Internet use), and patient activation, mental adjustment to cancer, HRQOL, symptoms, and need for supportive care, measured at baseline. Linear mixed models were performed to investigate potential moderators. RESULTS: The intervention effect on HRQOL was the largest among cancer survivors with low to moderate self-efficacy, and among those with high personal control and those with high health literacy scores. Cancer survivors with higher baseline symptom scores benefitted more on head and neck (pain in the mouth, social eating, swallowing, coughing, trismus), and colorectal cancer (weight) specific symptoms. DISCUSSION: Oncokompas seems most effective in reducing symptoms in head and neck cancer and colorectal cancer survivors who report a higher burden of tumour-specific symptoms. Oncokompas seems most effective in improving HRQOL in cancer survivors with lower self-efficacy, and in cancer survivors with higher personal control, and higher health literacy.
Subject(s)
Breast Neoplasms , Cancer Survivors , Self-Management , Telemedicine , Female , Humans , Quality of LifeABSTRACT
PURPOSE: The eHealth self-management application 'Oncokompas' was developed to support cancer survivors in monitoring health-related quality of life (HRQOL) and symptoms, and obtaining personalized feedback and options for supportive care. The aim of this study was to assess the cost-utility of Oncokompas compared with care as usual (CAU) among cancer survivors. METHODS: Survivors were randomly allocated to the intervention or control group. Direct (non-)medical, indirect non-medical costs, and HRQOL were measured at 3- and 6-month follow-up, using iMTA Medical Consumption and Productivity Costs and the EuroQol-5D questionnaires. Mean cumulative costs and quality-adjusted life-years (QALYs) were compared between both groups. RESULTS: In total, 625 survivors were randomized into intervention (n = 320) or control group (n = 305). Base case analysis showed that incremental costs from a societal perspective were - 163 (95% CI, - 665 to 326), and incremental QALYs were 0.0017 (95% CI, - 0.0121 to 0.0155) in the intervention group compared with those in the control group. The probability that, compared with CAU, Oncokompas is more effective was 60%, less costly 73%, and both more effective and less costly 47%. Sensitivity analyses showed that incremental costs vary between - 40 and 69, and incremental QALYs vary between - 0.0023 and - 0.0057. CONCLUSION: Oncokompas is likely to be equally effective on utilities, and not more expensive than CAU, and will therefore contribute to sustainable cancer survivorship care in a (cost-)effective manner. IMPLICATIONS FOR CANCER SURVIVORS: Oncokompas seems to improve HRQOL and reduces the burden of several tumour-specific symptoms, while costs from a societal perspective are similar to CAU.
Subject(s)
Cancer Survivors , Neoplasms , Self-Management , Telemedicine , Aged , Cost-Benefit Analysis , Female , Humans , Male , Middle Aged , Neoplasms/therapy , Quality of Life , SurvivorsABSTRACT
BACKGROUND: Elastic compression stockings (ECS) are uncomfortable to wear but may prevent post-thrombotic syndrome (PTS). The ability to predict PTS may help clinical decision making regarding the optimal duration of ECS after deep vein thrombosis (DVT). AIMS: Predefined endpoint analysis of the Octavia study that randomized patients who compliantly used ECS up to one year after DVT to continue or discontinue ECS treatment. Primary aim was to identify predictors of PTS. METHODS: Patient characteristics were collected and ultrasonography was performed to assess reflux, residual thrombosis and persistent thrombus load 12â¯months after DVT. Multivariable analyses were performed to identify factors related to PTS. RESULTS: Thrombus scoreâ¯≥â¯3, BMIâ¯≥â¯26, duration of symptoms before DVT diagnosisâ¯≥â¯8â¯days and a Villalta score of 2-4 points were statistically significant predictors of PTS. The predictive value for PTS for the assessed variables was not different between the 2 treatment groups. In the stop ECS group, 3.2% (95%CI 0.08-18) of patients without any predictors for PTS were diagnosed with mild PTS during follow-up, and none with severe PTS, for a sensitivity of 98% (95% CI 89-100), a specificity of 14% (95% CI 10-20), a positive predictive value of 20% (95% CI 19-22), and a negative predictive value of 97% (95% CI 81-100). CONCLUSION: We identified 4 predictors of PTS occurring in the 2nd year after DVT. Our findings may be used to decide on whether to continue ECS treatment for an additional year, after one year of compliant ECS use, keeping in mind that patients with none of the predictors will have the lowest PTS incidence.
Subject(s)
Postthrombotic Syndrome/prevention & control , Stockings, Compression , Venous Thrombosis/prevention & control , Aged , Female , Humans , Incidence , Male , Middle Aged , Postthrombotic Syndrome/diagnosis , Postthrombotic Syndrome/etiology , Prognosis , Venous Thrombosis/complications , Venous Thrombosis/diagnosisABSTRACT
Essentials Imaging is warranted in the majority of patients to confirm or rule out pulmonary embolism (PE). The age-adjusted D-dimer (ADJUST) reduced the number of required imaging tests in patients ≥ 50 years. The YEARS algorithm was designed to improve the efficiency in patients with suspected PE. There was no added value of implementing ADJUST in the YEARS algorithm in our cohort. SUMMARY: Background The YEARS algorithm was designed to simplify the diagnostic work-up of pulmonary embolism (PE) and to reduce the number of necessary computed tomography pulmonary angiography (CTPA) scans. An alternative strategy to reduce the number of CTPAs is the age-adjusted D-dimer cut-off (ADJUST) in patients aged 50 years or older. We aimed to investigate whether a combination of both diagnostic strategies might save additional CTPAs. Methods The YEARS algorithm consists of three items (clinical signs of deep venous thrombosis, hemoptysis, 'PE most likely diagnosis') with simultaneous D-dimer testing using a pre-test dependent threshold. We performed a post hoc analysis in 3465 patients managed according to YEARS to compare the number of patients managed without CTPA scans and associated diagnostic failures in hypothetical scenarios with different YEARS-ADJUST combinations. Results Following the YEARS algorithm, 1651 patients (48%) were managed without CTPA; PE was diagnosed in 456 (13%) patients at baseline and 18 patients with initial normal testing suffered venous thromboembolism (VTE) during 3-month follow-up (failure rate 0.61%; 95% confidence interval [CI], 0.36-0.96). If ADJUST had been fully integrated in YEARS, 1627 patients (47%) would have been managed without CTPA (absolute decrease of 0.69%; 95% CI -1.7 to 3.0), at cost of four additional missed PE diagnoses at baseline, for a projected 3-month VTE failure rate of 0.75% (95% CI, 0.49-1.13). None of the other studied scenarios showed relevant improvements in efficiency as well, but all led to more missed diagnoses. Conclusion In our cohort, there was no added value of implementing ADJUST in the YEARS algorithm.
Subject(s)
Fibrin Fibrinogen Degradation Products/metabolism , Pulmonary Embolism/blood , Pulmonary Embolism/diagnosis , Aged , Algorithms , Computed Tomography Angiography , Female , Humans , Male , Middle Aged , Prospective Studies , Pulmonary Embolism/diagnostic imaging , Software DesignABSTRACT
OBJECTIVE: To study whether stopping elastic compression stockings (ECS) after 12 months is non-inferior to continuing them for 24 months after proximal deep venous thrombosis. DESIGN: Multicentre single blind non-inferiority randomised controlled trial. SETTING: Outpatient clinics in eight teaching hospitals in the Netherlands, including one university medical centre. PARTICIPANTS: Patients compliant with compression therapy for 12 months after symptomatic, ultrasound proven proximal deep venous thrombosis of the leg. INTERVENTIONS: Continuation or cessation of ECS 12 months after deep venous thrombosis. MAIN OUTCOME MEASURES: The primary outcome was the incidence of post-thrombotic syndrome 24 months after diagnosis of deep venous thrombosis, as assessed by the standardised Villalta scale in an intention to treat analysis. The predefined non-inferiority margin was 10%. The main secondary outcome was quality of life (VEINES-QOL/Sym). RESULTS: 518 patients compliant with ECS and free of post-thrombotic syndrome were randomised one year after diagnosis of deep venous thrombosis to stop or continue ECS therapy for another year. In the stop-ECS group, 51 of 256 patients developed post-thrombotic syndrome, with an incidence of 19.9% (95% confidence interval 16% to 24%). In the continue-ECS group, 34 of 262 patients developed post-thrombotic syndrome (incidence 13.0%, 9.9% to 17%), of whom 85% used ECS six or seven days a week during the study period, for an absolute difference of 6.9% (95% confidence interval upper limit 12.3%). Because the upper limit of the 95% confidence interval exceeds the predefined margin of 10%, non-inferiority was not reached. The number needed to treat to prevent one case of post-thrombotic syndrome by continuing ECS was 14 (95% confidence interval lower limit 8). Quality of life did not differ between the two groups. CONCLUSION: Stopping ECS after one year in compliant patients with proximal deep venous thrombosis seemed not to be non-inferior to continuing ECS therapy for two years in this non-inferiority trial. TRIAL REGISTRATION: Netherlands Trial Register NTR1442.
Subject(s)
Conservative Treatment , Lower Extremity/blood supply , Postthrombotic Syndrome , Stockings, Compression , Veins , Venous Thrombosis , Adult , Aged , Conservative Treatment/instrumentation , Conservative Treatment/methods , Female , Humans , Male , Middle Aged , Outcome and Process Assessment, Health Care , Postthrombotic Syndrome/diagnosis , Postthrombotic Syndrome/etiology , Postthrombotic Syndrome/physiopathology , Postthrombotic Syndrome/prevention & control , Tertiary Prevention/instrumentation , Tertiary Prevention/methods , Time Factors , Ultrasonography/methods , Veins/diagnostic imaging , Veins/physiopathology , Venous Thrombosis/complications , Venous Thrombosis/diagnosis , Venous Thrombosis/physiopathology , Venous Thrombosis/therapyABSTRACT
In the past decade, patients with newly diagnosed diffuse large B-cell lymphoma (DLBCL) were treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) therapy. Standard treatment is now changing as a result of deeper understanding of underlying biologic differences of such lymphomas. One of the most powerful predictors of an adverse outcome on R-CHOP therapy is the presence of a MYC gene rearrangement (MYC+ lymphoma). Determination of MYC gene rearrangement by FISH (fluorescent in situ hybridisation) has recently become a standard diagnostic procedure. In this paper, an overview of current literature on MYC function and MYC+ lymphoma patient outcome is presented. Furthermore, we present 26 patients from our tertiary referral centre who were diagnosed with MYC+ lymphoma between 2009 and 2014. In our patient series, we confirm the dismal prognosis of MYC+ lymphoma patients. Intensification of classical chemotherapy does not lead to better overall survival, justifying new treatment modalities. First line therapy should be more specifically targeted against MYC and the genes and proteins that are deregulated by MYC. To this end, the first clinical trial in which MYC+ patients will be offered targeted treatment has recently been launched.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Biomarkers, Tumor/genetics , Gene Rearrangement , Lymphoma, Large B-Cell, Diffuse/drug therapy , Proto-Oncogene Proteins c-myc/genetics , Adult , Aged , Female , Genetic Predisposition to Disease , Humans , In Situ Hybridization, Fluorescence , Lymphoma, Large B-Cell, Diffuse/genetics , Lymphoma, Large B-Cell, Diffuse/mortality , Lymphoma, Large B-Cell, Diffuse/pathology , Male , Middle Aged , Molecular Targeted Therapy , Patient Selection , Phenotype , Precision Medicine , Predictive Value of Tests , Survival Analysis , Treatment OutcomeABSTRACT
Long-term complete remission in IgD multiple myeloma (MM) is rare. This case report describes a patient with a stage IIIB IgD-MM, who was treated with conventional melphalan and prednisone chemotherapy. The monoclonal protein disappeared after four cycles and therapy was discontinued after 14 cycles. Re-evaluation after a follow up of more than 8 years demonstrates a continuing complete remission suggesting a cure. This is remarkable, considering that several adverse prognostic factors were present. In addition a concise review on IgD-MM is given.
Subject(s)
Anti-Inflammatory Agents/administration & dosage , Antineoplastic Agents, Alkylating/administration & dosage , Immunoglobulin D/blood , Melphalan/administration & dosage , Multiple Myeloma/blood , Multiple Myeloma/drug therapy , Prednisone/administration & dosage , Aged , Bone Marrow Cells/metabolism , Bone Marrow Cells/pathology , Humans , Male , Multiple Myeloma/pathology , Remission Induction/methodsABSTRACT
BACKGROUND: The effect of interferon-alpha 2b (IFN-alpha-2b) on progression-free and overall survival as well as quality of life (QoL) was studied in mainly elderly patients with multiple myeloma (MM), who reached a plateau phase after melphalan/prednisone induction. PATIENTS AND METHODS: In an open phase III trial, 262 patients, median age 69 years (range 34-91), received at least 10 monthly courses of melphalan/prednisone followed by response evaluation. Plateau phase was reached by 128 patients. Next, 90 patients were randomized between IFN-alpha-2b and no maintenance therapy. Reasons for non-randomization were: refusal (18), concomitant disease (nine), protocol violation (six), WHO performance status >2 (four) and allogeneic transplantation (one) RESULTS: At a median follow-up from diagnosis of 97 months (0-140) for those patients alive, IFN-alpha-2b therapy was associated with improved progression-free survival (median 13.5 versus 8.4 months from randomization), although this did not translate in a better overall survival (41 versus 38.4 months). One-third of patients discontinued IFN-alpha due to toxicity. No differences were observed between patient groups in QoL. CONCLUSIONS: IFN maintenance therapy in MM prolongs progression-free survival and, provided that the burden of toxicity is not too high, does not adversely affect QoL.
Subject(s)
Interferon-alpha/administration & dosage , Multiple Myeloma/drug therapy , Adult , Aged , Aged, 80 and over , Female , Humans , Interferon alpha-2 , Interferon-alpha/adverse effects , Male , Middle Aged , Multiple Myeloma/physiopathology , Multiple Myeloma/psychology , Quality of Life/psychology , Recombinant Proteins , Survival AnalysisABSTRACT
A higher prevalence of solid tumours in patients with M(onoclonal) proteinaemia without a co-existing haematological malignancy has been reported. We investigated this association by linking a population-based registry of patients with newly diagnosed M-proteinaemia (n = 1464) with the Regional Cancer Registry. Patients were followed for a median of 7.4 years for those still alive. In total 167 (11%) patients with 173 solid tumours were compared with 861 patients with 'M-proteinaemia only' (without a haematological malignancy). The M-protein isotype or level or clinical parameters did not differ between the groups. M-protein isotype was not associated with a specific tumour type. Standardised Morbidity Ratios (SMR) for nearly all solid tumours were elevated in the year of the M-protein discovery, but the excess risk disappeared during follow-up suggesting selection through diagnostic investigations rather than a causal role. In this large series of patients with both newly diagnosed M-proteinaemia and a solid tumour no relationship could be established.
Subject(s)
Neoplasms/epidemiology , Paraproteinemias/epidemiology , Adolescent , Adult , Aged , Aged, 80 and over , Female , Follow-Up Studies , Humans , Incidence , Male , Middle Aged , Neoplasms/complications , Neoplasms/therapy , Paraproteinemias/complications , PrevalenceABSTRACT
Whether the response to chemotherapy is a prognosticator in multiple myeloma (MM) is still not known. Therefore, the relationship between survival and the rate of monoclonal protein (M-protein) decrement during the first cycles of therapy was prospectively assessed in 262 patients with newly diagnosed MM that were included in a phase III trial (HOVON-16). M-proteins were collected monthly during melphalan-prednisone therapy (MP: melphalan 0.25 mg/kg, prednisone 1.0 mg/kg orally for 5 d every 4 weeks). Patients with light chain disease (n = 18), immunoglobulin M (IgM)-MM (n = 1) and no immunotyping (n = 1) were excluded. Of the 242 patients studied, 75% had IgG M-protein and 25% IgA; MM stages: I: 1%, II: 35% and III: 64%. The median M-protein decrease after the first cycle of MP was 21% for IgG and 27% for IgA, and declined to < 5% after four cycles. An obvious survival advantage was seen for patients who had an M-protein decrease of at least 30% after the first MP cycle, which became significant when an M-protein decrease of 40% or more was reached. As established prognostic parameters (Salmon & Durie stage, serum creatinine, and haemoglobin) also remained prognostically significant, we concluded that early response to MP predicts for survival in MM.
Subject(s)
Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Multiple Myeloma/drug therapy , Adult , Aged , Aged, 80 and over , Female , Humans , Immunoglobulin A/metabolism , Immunoglobulin G/metabolism , Male , Melphalan/administration & dosage , Middle Aged , Multiple Myeloma/metabolism , Myeloma Proteins/metabolism , Prednisone/administration & dosage , Prospective Studies , Survival Analysis , Treatment OutcomeABSTRACT
BACKGROUND: The treatment of posttransplant lymphoproliferative disorder (PTLD) remains empirical. We review our treatment of seven cases of PTLD consisting of five interventions: 1) reduction of immunosuppression; 2) antiviral drugs; 3) interferon-alpha; 4) gamma-globulins; and 5) anti-CD19 monoclonal antibodies. METHODS AND RESULTS; Seven consecutive patients who had undergone a simultaneous pancreas-kidney, liver, heart, or kidney transplantation were treated. One patient acquired a primary EBV infection with an oligoclonal immunoblastic lymphoma early after pancreas-kidney transplantation; all others developed a monoclonal polymorphic or immunoblastic lymphoma 2 to 123 months after transplantation. In all patients extranodal sites were involved, in three the graft was also involved. Five patients received the full quintuple approach and all rapidly obtained a complete remission (CR) with a median follow-up of 31 months (7-74 months). Of the two patients who did not receive interferon-alpha for fear of graft rejection one responded slowly with a CR after 7 months, and the other obtained a rapid CR followed by a relapse at 4 months. All three patients with a liver or heart transplant could keep their graft. All patients are still alive with a median follow-up of 31 months (7-74 months). CONCLUSION: This combined approach resulted in a favorable outcome in patients with high risk monoclonal PTLD after solid organ transplantation.
Subject(s)
Lymphoproliferative Disorders/etiology , Lymphoproliferative Disorders/surgery , Organ Transplantation/adverse effects , Acyclovir/therapeutic use , Antibodies, Monoclonal/therapeutic use , Antiviral Agents/therapeutic use , Humans , Immunoglobulins, Intravenous/therapeutic use , Interferon-alpha/therapeutic use , Lymphoproliferative Disorders/drug therapy , Prognosis , Remission Induction , Treatment OutcomeABSTRACT
We describe a young woman who displayed the "malignant" variant of the antiphospholipid syndrome (APS), also known as the "catastrophic APS." Renal insufficiency, retinopathy, cerebral infarcts, bone marrow necrosis, skin ulcers, and nasal septum perforation were the result of widespread thrombotic microangiopathy. She recovered during high intensity anticoagulation.
Subject(s)
Antiphospholipid Syndrome/physiopathology , Adolescent , Anticoagulants/therapeutic use , Antiphospholipid Syndrome/drug therapy , Catastrophic Illness , Female , HumansABSTRACT
We determined interleukin-6 (IL-6) levels in the serum of 212 well-defined patients with newly diagnosed paraproteinaemia and evaluated its discriminatory value and prognostic role in multiple myeloma (MM). Results were compared with serum neural cell adhesion molecule and beta-2-microglobulin, both established prognostic MM markers. Paraproteinaemia-related diagnoses were: MM (60), other haematological diseases (46), solid tumours (35), autoimmune diseases (17) and monoclonal gammopathy of unknown significance (MGUS) (54). The range of IL-6 levels in all diagnostic groups overlapped widely and did not serve as a discriminatory marker in newly diagnosed paraproteinaemia even when patients with infection or fever (42) were excluded. In MM high IL-6 levels (>/= 50 pg/ml) were not associated with a shorter survival (P = 0.24). We compared our results with 20 published studies on serum IL-6 in paraproteinaemia and/or MM. IL-6 data have to be related to the assay used (bio- or immunoassay) and to the status of MM (newly diagnosed, during therapy, progressive disease). We conclude that serum IL-6 is not specific for paraproteinaemia-related diseases and will not serve as a reliable discriminatory or prognostic marker in paraproteinaemia and MM.
Subject(s)
Interleukin-6/blood , Paraproteinemias/diagnosis , C-Reactive Protein/metabolism , Humans , Multiple Myeloma/diagnosis , Neural Cell Adhesion Molecules/blood , Prognosis , Survival Analysis , beta 2-Microglobulin/bloodABSTRACT
A women aged 36 with a positive family anamnesis for autoimmune endocrine diseases and a history of thyroid diseases, developed major complaints of general malaise, orthostatic hypotension and loss of appetite after the start of a treatment with levothyroxin because of (sub)clinical hypothyroidism. She was found to suffer from primary adrenocortical insufficiency masked by excessive use of liquorice and a lowered metabolism, but which via the suppletion with thyroid hormone had led to an addisonian crisis.
Subject(s)
Addison Disease/chemically induced , Hormone Replacement Therapy/adverse effects , Thyroxine/adverse effects , Adult , Autoimmune Diseases/complications , Autoimmune Diseases/drug therapy , Autoimmune Diseases/genetics , Endocrine System Diseases/complications , Endocrine System Diseases/genetics , Female , Glycyrrhiza/adverse effects , Humans , Pedigree , Plants, Medicinal , Thyroid Diseases/complications , Thyroid Diseases/drug therapyABSTRACT
Diagnoses in patients with paraproteinaemia are diverse; few (mostly single centre based) studies are known that describe incidence, diagnoses and follow-up in patients with paraproteinaemia. In the region of the Comprehensive Cancer Centre West in the Netherlands (population 1.6 million, 1992) a population-based registry was set up in the period 1991-1993. Patients (n = 1464; median age: 72 years; range: 16-102) were entered by clinical chemists, internists, haematologists, and pathologists. Multiple myeloma and plasmacytoma were diagnosed in 261 patients (18%), paraprotein-related haematological diseases in 159 patients (11%) and paraprotein-related internal diseases in 210 patients (14%). After bone marrow examination monoclonal gammopathy of unknown significance (MGUS) was diagnosed in 207 (14%) patients. No further diagnosis could be made in 627 (43%) patients mostly for lack of supplementary bone marrow and (or) X-ray examinations. Consequently, more than two-thirds of all patients with a newly found paraprotein did not show any sign of a haematological malignancy. Using these data a 'myeloma risk score' was developed to predict the presence of a multiple myeloma based on paraprotein type and concentration, aiding the physician in determining which patients should undergo further bone marrow and skeletal examinations.
Subject(s)
Multiple Myeloma/etiology , Paraproteinemias/complications , Adolescent , Adult , Aged , Aged, 80 and over , Bone Marrow/pathology , Follow-Up Studies , Humans , Middle Aged , Multiple Myeloma/diagnosis , Multiple Myeloma/epidemiology , Netherlands/epidemiology , Paraproteinemias/epidemiology , ROC Curve , Risk AssessmentABSTRACT
Cerebral watershed infarctions usually occur after a period of acute and severe systemic hypotension resulting in a distinctive clinical picture. We present a two year old girl who developed watershed infarctions after a near drowning accident. The clinical features and diagnostic aspects are described.