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Thrombotic thrombocytopenic purpura (TTP) is one of the rare group disorders classified as thrombotic microangiopathies (TMAs). Approximately 90% of TTP developed immune-mediation by the formation of antibodies against the enzyme ADAMTS-13. The exact cause is unknown. To establish an association between human leukocyte antigen (HLA) and autoimmune basis, as susceptibility or protection against the disease, we contributed a study aiming to evaluate the role of HLA in immune-mediated TTP (iTTP). Considering epidemiological factors such as age, sex, ethnicity, and geographical origins, we contributed the study in our country, Turkey, which consist of a very heterogeneous population. Patients' data collection was retrospectively from electronic database on two University hospitals having big therapeutic apheresis service. Control arm was healthy people registered as stem cell donors matched in terms of age and sex. The frequency of HLA-DRB1 and HLA-DQB1 alleles between acquired TTP and the control group was compared using the chi-square method. Yates correction and logistic regression were performed on these results. A total of 75 iTTP patients and 150 healthy individuals enrolled to the study. HLA-DRB1∗11, HLA-DQB1∗03, HLA-DRB1∗11:01, HLA-DRB1∗14:01, HLA-DRB1∗13:05, HLA-DRB1∗11 + HLA-DQB1∗03 allele pair and HLA-DRB1∗15 + HLA- DQB1∗06 were proved to be susceptibility allele pairs for iTTP. HLA-DRB1∗15, HLA-DRB1∗01:01, HLA-DRB1∗07:01, HLA-DRB1∗13:01, HLA-DRB1∗14:54, HLA-DQB1∗05:01, HLA-DQB1∗02:02 and HLA-DRB1∗07 + HLA-DQB1∗02 allele pair were found to be protective against iTTP. Our findings support an association with iTTP across very heterogenous populations in Turkey.
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Alelos , Cadenas HLA-DRB1 , Púrpura Trombocitopénica Trombótica , Humanos , Turquía/epidemiología , Púrpura Trombocitopénica Trombótica/genética , Púrpura Trombocitopénica Trombótica/inmunología , Femenino , Masculino , Adulto , Persona de Mediana Edad , Cadenas HLA-DRB1/genética , Estudios Retrospectivos , Cadenas beta de HLA-DQ/genética , Estudios de Casos y Controles , Frecuencia de los Genes , Predisposición Genética a la Enfermedad , Adulto Joven , Antígenos HLA/genética , AdolescenteRESUMEN
INTRODUCTION: Therapeutic plasma exchange (TPE) may involve complications. We aimed to review the demographic data, indications, technical information, and complications. METHODS: Data for TPE procedures (TPEPs) performed between 2004 and 2018 were retrospectively. RESULTS: This study covered 2505 TPEPs performed on 338 patients; 55% of them were female (n = 186), and the median age was 36 years (range, 11-93 years). Most TPEPs were administered for hematological (40.6%) indications. The incidence of complications on the first procedure was 3.2% (n = 80); only 16 procedures (0.6%) were failed. The complication incidence was 19.8% (n = 497), with 789 total complications. Most of the complications were patient-related (90.4%), and the most of them were urticaria (29.1%), occlusion (3.2%), and faulty systems (1.01%), respectively. The use of only fresh frozen plasma as replacement fluid caused a higher complication rate (22.1%, p < 0.01). CONCLUSION: The number of TPEPs is increasing every day. Hematologic indications for TPE and the use of fresh frozen plasma may increase the risk of complications.
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Introduction: Primary immune thrombocytopenia (ITP) is an acquired disorder of platelets with complex and unclear mechanism of increased immune distruction or impaired production of platelets. While management of ITP is evolving, there is a need for guidance particularly in certain circumstances such as pregnancy, emergency and for patients requiring co-medications. We aimed to determine the tendencies of hematologists in Turkiye on such special conditions. Methods: As a modified Delphi method, Turkish National ITP Working Group founded under Turkish Society of Hematology developed a questionnaire consisting of statements regarding pregnancy, emergency and circumstances regarding co-treatment with antiaggregant or anticoagulants. 107 Hematologists working either in university or state hospitals voted for their agreement or disagreement of the statements for two consequential rounds. Results: Participant hematologists reached an agreement on the starting treatment in pregnant patients with platelets less than 30 x109/L and delivery of either normal or cesarian section to be safely performed above 50 x109/L. For emergency and rescue management of ITP, our panel have agreed against the use of high dose corticosteroids alone, preferred a combination with transfusion or IVIG. For patients who require interventions, platelet counts >50 x109/L were regarded as safe for low risk procedures as well as co-treatment with antiplatelets or anticoagulants. Conclusion: As National ITP study group, we have observed the need to increase the practice guidance in patients with primary ITP requiring additional treatments including invasive interventions, and co-treatments towards coagulation. Decisions on the management of ITP during pregnancy should be individualized. There is a certain lack of consensus on the thresholds of platelet counts as well as co-morbidities and co-medications. This lack of consensus may be due to the variations in the practices.
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BACKGROUND: Hairy cell leukemia (HCL) is a rare mature B-cell malignancy that is primarily treated with purine analogues. However, relapse remains a significant challenge, prompting the search for alternative therapies. The BRAF V600E mutation prevalent in HCL patients provides a target for treatment with vemurafenib. PATIENTS AND METHODS: This multicenter retrospective study included nine patients with relapsed/refractory (R/R) HCL from six different centers. Patient data included demographics, prior treatments, clinical outcomes, and adverse events. RESULTS: Patients received different treatment regimens between centers, including vemurafenib alone or in combination with rituximab. Despite the differences in protocols, all patients achieved at least a partial response, with seven patients achieving a complete response. Adverse events were generally mild with manageable side effects. The absence of myelotoxic effects and manageable side effects make BRAF inhibitors attractive, especially for patients ineligible for purine analogues or those with severe neutropenia. CONCLUSION: Single agent vemurafenib or in combination with rituximab appears to be a promising therapeutic option for R/R HCL. Further research is needed to establish standardized treatment protocols and to investigate long-term outcomes.
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Protocolos de Quimioterapia Combinada Antineoplásica , Leucemia de Células Pilosas , Rituximab , Vemurafenib , Humanos , Leucemia de Células Pilosas/tratamiento farmacológico , Leucemia de Células Pilosas/patología , Vemurafenib/administración & dosificación , Vemurafenib/uso terapéutico , Vemurafenib/efectos adversos , Rituximab/administración & dosificación , Rituximab/uso terapéutico , Rituximab/efectos adversos , Masculino , Persona de Mediana Edad , Femenino , Estudios Retrospectivos , Anciano , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Protocolos de Quimioterapia Combinada Antineoplásica/efectos adversos , Adulto , Proteínas Proto-Oncogénicas B-raf/genética , Proteínas Proto-Oncogénicas B-raf/antagonistas & inhibidores , Recurrencia Local de Neoplasia/tratamiento farmacológico , Recurrencia Local de Neoplasia/patología , Resultado del Tratamiento , Anciano de 80 o más Años , Resistencia a AntineoplásicosRESUMEN
Objective: Primary immune thrombocytopenia (pITP) is an acquired autoimmune disorder related to the increased destruction and/or impaired production of platelets. Its diagnosis and management are challenging and require expertise and the interpretation of international consensus reports and guidelines with national variations in availability. We aimed to assess the agreement of hematologists in Türkiye on certain aspects of both first-line and second-line management of patients with pITP. Materials and Methods: Applying a modified Delphi method, the Turkish National ITP Working Group (14 steering committee members), founded under the auspices of the Turkish Society of Hematology, developed a 21-item questionnaire consisting of statements regarding the first-line and second-line treatment of pITP. A total of 107 adult hematologists working in either university or state hospitals voted for their agreement or disagreement with the statements in two consecutive rounds. Results: The participants reached consensus on the use of corticosteroids as first-line treatment and with limited duration. Methylprednisolone was the corticosteroid of choice rather than dexamethasone. Use of intravenous immunoglobulin was not preferred for patients without bleeding. It was also agreed that thrombopoietin receptor antagonists (TPO-RAs) or rituximab should be recommended as second-line treatment and that splenectomy could be considered 12-24 months after diagnosis in patients with chronic pITP. Conclusion: The optimization of the dose and duration of TPO-RAs in addition to corticosteroids is necessary to improve the management of patients with pITP.
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Consenso , Púrpura Trombocitopénica Idiopática , Humanos , Púrpura Trombocitopénica Idiopática/diagnóstico , Púrpura Trombocitopénica Idiopática/terapia , Púrpura Trombocitopénica Idiopática/tratamiento farmacológico , Adulto , Técnica Delphi , Manejo de la Enfermedad , Encuestas y Cuestionarios , Turquía/epidemiología , Esplenectomía , Corticoesteroides/uso terapéutico , Femenino , Guías de Práctica Clínica como AsuntoRESUMEN
INTRODUCTION: The advent of tyrosine kinase inhibitors (TKIs) was revolutionary in the management of chronic myeloid leukemia (CML). Although TKIs were generally considered to be safe, they can be associated with renal injury. We evaluated the effect of TKIs on renal functions in a cohort of patients with long-term follow-up. MATERIAL AND METHODS: We retrospectively examined patients with chronic phase CML treated with TKIs. We analyzed the estimated glomerular filtration rate (eGFR) of patients from the initiation of TKI to the last follow-up. eGFR values of CML patients were compared to those of patients with stage 1 or 2 chronic kidney disease (CKD). RESULTS: A total of 195 patients with CML and 138 patients with CKD were examined. eGFR decline was 1.556 ml/min/1.73m2/year for patients with CML (P = .221). Patients receiving second-generation TKIs (2GTKI) were estimated to have 0.583 ml/min/1.73m2 higher eGFR value than that of the imatinib group, but it was not significant (P = .871). eGFR of patients who had used bosutinib had a downward trend. Duration of TKI therapy, age, and hypertension were found to be significant factors in eGFR decline for CML patients. Lower baseline GFR was associated with an increased risk of CKD development. CONCLUSION: Imatinib could result in a decline in eGFR which was clinically similar to early-stage CKD patients. We did not observe significant kidney function deterioration in patients receiving 2GTKIs including dasatinib and nilotinib. We recommend close renal function monitoring in patients receiving imatinib, especially for elderly patients with lower baseline eGFR and hypertension.
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Hipertensión , Leucemia Mielógena Crónica BCR-ABL Positiva , Insuficiencia Renal Crónica , Humanos , Anciano , Mesilato de Imatinib , Inhibidores de Proteínas Quinasas/efectos adversos , Tasa de Filtración Glomerular , Estudios Retrospectivos , Leucemia Mielógena Crónica BCR-ABL Positiva/tratamiento farmacológico , Dasatinib/efectos adversos , Insuficiencia Renal Crónica/etiologíaRESUMEN
Thrombocytopenia has been reported more frequently with obinutuzumab in clinical trials where it was directly compared with rituximab. However, more significant than the frequency, a unique form of severe thrombocytopenia manifesting very early after the first obinutuzumab infusion has been published in case reports. To further explore this subject, we went through the records of our clinic to identify similar cases of obinutuzumab-induced acute thrombocytopenia (OIAT). Among 24 patients who received obinutuzumab outside of clinical trials, we recovered three cases with OIAT. This paper describes these three cases in detail, placing emphasis on the timing, severity, and the clinical course. Notably, all three patients developed severe OIAT within 5 days of their first obinutuzumab exposure, responded well to transfusion, and recovered within a few days without severe bleeding. None of the patients experienced a similar event in the second course of the obinutuzumab-based therapy. Our observations suggest that OIAT may be a frequent, possibly non-relapsing, and unique event that deserves more attention than it currently receives.
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BACKGROUND: Medical education requires the implementation of different teaching methods and strategies for future doctors to achieve broad learning objectives. This wide range of methods and strategies includes the use of Information Technologies. For a long time, there was a call for a change in medical education for blending new teaching approaches to lessen medical students' class time. The COVID-19 pandemic then sped up the transition to the new way of medical education and classroom lectures were quickly moved to a virtual environment. We expect that these changes will continue, and online learning will be one of the main teaching strategies in medical education. Therefore, educational experiences during the COVID-19 pandemic will improve our understanding of online learning and will help to develop blended medical school curricula in the future. For this reason, we aimed to determine students' overall satisfaction with their online learning experience and to define the main factors affecting students' satisfaction with their online learning program at Cerrahpasa Medical Faculty. METHODS: A cross-sectional survey study was conducted to determine medical students' overall satisfaction with online learning methods and to identify factors associated with positive and negative satisfaction levels. A questionnaire, consisting of 24 questions to collect demographic characteristics, factors associated with online education experience and overall satisfaction levels was developed and distributed to 1600 medical students. Multivariable linear regression analysis was used to determine the factors associated with positive and negative satisfaction levels. RESULTS: Regression analysis showed that being familiar with online teaching techniques (ß = 0.19, 95% CI [0.07, 0.30], faculty members' higher online teaching skill levels (ß = 0.42, 95% CI [0.32, 0.51], interactive online teaching approaches (ß = 0.54, 95% CI [0.41, 0.67], having a personal workspace (ß = 0.43, 95% CI [0.19, 0.67], and a self-reported longer attention span (ß = 0.75, 95% CI [0.57, 0.92] were associated with higher overall satisfaction with online learning. The occurrence of technical problems (ß = -0.19, 95% CI [-0.26, -0.12] was associated with lower overall satisfaction. CONCLUSIONS: Higher online teaching skills of faculty members, use of interactive approaches, students' familiarity with online teaching techniques, provision of a personal workspace, and self-reported longer attention spans positively contributed to higher levels of student satisfaction with online learning. Considering the increasing significance of online educational methods, our study identified key components that affect students' level of satisfaction. This information might contribute to the development of online educational programs in the future.
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COVID-19 , Educación a Distancia , Estudiantes de Medicina , Humanos , Educación a Distancia/métodos , Pandemias , Estudios Transversales , Satisfacción Personal , Encuestas y Cuestionarios , COVID-19/epidemiología , Análisis de RegresiónRESUMEN
Hodgkin Lymphoma (HL) is often curable with ABVD therapy and improving outcomes is a main goal of ongoing research. Bleomycin-associated pneumonitis (BAPT) is a potentially life-threatening complication that necessitates bleomycin discontinuation. We conducted this study on a homogenous cohort of advanced stage HL treated only with ABVD for frontline therapy to assess if bleomycin discontinuation increases the risk of lymphoma progression. After the exclusion of patients who received radiotherapy or other drugs, 106 and 28 patients in the six-cycle ABVD and BAPT groups respectively had similar survival curves for progression and death with a 49-month median follow-up. PFS rates were also very similar at two and four years from diagnosis with 2-year PFS rates of 83.9% and 82.1% (RR = 1.1 95%CI = 0.45-2.2). Outcome comparisons were also similar between the two groups when stratified according to early response assessment with PET/CT. Patients who discontinued bleomycin due to toxicity did not experience an increased risk of progression compared to patients who completed six ABVD cycles.
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Pulmonary hypertension (PH) is a complex disorder that should be managed with a multidisciplinary approach. Although most of the underlying causes of left heart disease can be easily diagnosed with cardiac imaging, some pathologies might necessitate careful investigation to go beyond the obvious. High-output heart failure (HF) due to arteriovenous malformation (AVMs) is an unnoticeable cause for HF and PH. Patients with hepatic AVMs should always be carefully evaluated with regard to hereditary hemorrhagic telangiectasia (HHT) since they can have multiple signs related to the other systems without any symptoms. In this case report, we discussed a patient who was initially diagnosed as PH associated with HF with preserved ejection fraction but eventually was found to have PH associated with high-output HF due to hereditary hemorrhagic telangiectasia (HHT, or Osler Weber Rendu syndrome) after detailed evaluation.
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Malformaciones Arteriovenosas , Cardiopatías , Insuficiencia Cardíaca , Hipertensión Pulmonar , Telangiectasia Hemorrágica Hereditaria , Humanos , Telangiectasia Hemorrágica Hereditaria/complicaciones , Telangiectasia Hemorrágica Hereditaria/diagnóstico por imagen , Hipertensión Pulmonar/etiología , Hipertensión Pulmonar/complicaciones , Malformaciones Arteriovenosas/complicaciones , Malformaciones Arteriovenosas/diagnóstico por imagen , Insuficiencia Cardíaca/complicaciones , Cardiopatías/complicacionesRESUMEN
INTRODUCTION: In polycythemia vera (PV) patients undergoing phlebotomy, iron deficiency (ID) may develop. ID has been linked to restless legs syndrome (RLS), and in one study, 29.6% of PV patients had RLS. We aimed to evaluate the frequency of RLS in PV and to evaluate factors that might play a role in RLS development among PV and essential thrombocythemia (ET) patients. METHODS: We consecutively included PV cases as the patient group, and ET and ID patients and healthy subjects (HSs) were included as controls. Those with conditions that could lead to RLS were excluded. All subjects were questioned according to the diagnostic criteria of the International Restless Legs Syndrome Study Group. RESULTS: Twenty-seven PV, 23 ET, and 22 ID patients and 23 HSs were included. RLS was detected in 25.9%, 34.8%, and 45.5% of PV, ET, and ID patients, respectively. None of the HSs had RLS. In univariate analysis, interferon-α and anagrelide use, magnesium levels, and the Leeds assessment of neuropathic symptoms and signs (LANSS) scores had a significant impact on RLS in PV and ET patients (p = 0.014, p = 0.032, p = 0.036, and p = 0.003, respectively). CONCLUSION: RLS was more common among PV and ET patients than HSs, which was irrespective to the iron status. RLS was more frequent in ET patients than that observed in PV cases, indicating that ID may not be the only causative factor for RLS development in PV. Further prospective studies are needed to determine the prevalence and risk factors of RLS developing in PV and ET.
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Deficiencias de Hierro , Policitemia Vera , Síndrome de las Piernas Inquietas , Humanos , Policitemia Vera/complicaciones , Policitemia Vera/epidemiología , Policitemia Vera/diagnóstico , Estudios Transversales , Síndrome de las Piernas Inquietas/epidemiología , Síndrome de las Piernas Inquietas/etiología , PrevalenciaRESUMEN
This survey study aimed to evaluate the level of awareness and knowledge of acquired hemophilia A (AHA) among physicians from various specialties. Data were collected by administering a questionnaire containing questions about two patient profiles to evaluate the approaches of physicians from different specialties. The study included a total of 945 physicians. The most common suspected diagnosis for the clinical patient profile was immune thrombocytopenia, followed by hemophilia. For the laboratory patient profile, the most common suspected diagnosis was hemophilia. While only 9.4% of the physicians stated that they had good knowledge of this disease, only 21.6% wanted to learn more about AHA. Most of the participating physicians do not rank AHA first in the differential diagnosis of a patient with clinical and laboratory findings of AHA. Appropriate educational activities to raise awareness of AHA will help reduce disease-related morbidity and mortality.
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Hemofilia A , Médicos , Púrpura Trombocitopénica Idiopática , Trombocitopenia , Humanos , Hemofilia A/diagnósticoRESUMEN
Secondary immunodeficiency (SID) can occur as a result of multiple factors, including hematological malignancies, hematopoietic stem cell transplantation (HSCT), immunosuppressive treatment, biologics, and anti-inflammatory drugs. SID includes disorders resulting from impairment of both cellular and humoral immunity. This review focuses on the current risk factors, implications, and challenges in managing SID patients with impaired humoral immunity, which includes quantitative (hypogammaglobulinemia) and/or functional antibody and B-cell deficiencies specifically related to hematological malignancies and post-HSCT. Increased physician awareness is needed surrounding the disease presentation and early risk factors, as SID may be caused by several etiologies. Careful clinical assessment is then required to optimize management, which encompasses close monitoring of clinical parameters, vaccination, antibiotic prophylaxis, and immunoglobulin replacement therapy (IGRT). Novel methods of IGRT administration are associated with enhanced pharmacokinetics, IgG trough level stability, no need for venous access, as well as fewer systemic adverse events and better administration flexibility compared with traditional methods. Published international guidelines supported by observations from clinical data are broadly followed; however, best practices within each country have nuances that underline the need to tailor treatment plans to the individual patient.
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Objectives: Acute myeloid leukemia (AML) is a highly heterogeneous disease. Although patients can be classified into risk groups based on their genetic changes, the prognosis of disease within these categories varies widely. This situation raises the need to search for new molecular markers related to AML. Serine peptidase inhibitor Kazal type 2 (SPINK2) has recently been reported to be upregulated in AML and associated with poor outcomes by meta-analysis and in a limited number of AML patients. Methods: We analyzed SPINK2 mRNA expression in 62 patients (45 adult and 17 pediatric) with AML and 11 cell lines using quantitative Real-Time PCR (qRT-PCR). SPINK2 protein level was determined using ELISA in cell lines. Results: We found that the expression of SPINK2 mRNA and protein levels in AML cell lines (HL60 and NB4) have increased compared to other cell lines (K562, Jurkat and NALM6, MCF7, HeLa, HUVEC, hFOB, 293T, U87). SPINK2 mRNA expression was upregulated in patients with AML compared to controls (p=0.004) and significantly lower in t(8;21)-positive patients compared to negative patients (p=0.0006). Conclusions: Our results suggest that SPINK2 serves an important role in AML development. Further studies are needed to evaluate SPINK2 expression in AML patients with t(8.21) and investigate to clarify its prognostic value in various subgroups of AML.
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BACKGROUND: Cytomegalovirus (CMV) disease impacts morbidity and mortality in hematopoietic cell transplant (HCT) recipients. This systematic review summarized data on the epidemiology, management, and burden of CMV post-HCT outside of Europe and North America. METHODS: The MEDLINE, Embase, and Cochrane databases were searched for observational studies and treatment guidelines in HCT recipients across 15 selected countries from Asia-Pacific, Latin America, and Middle East (search period: 1 January 2011-17 September 2021). Outcomes included incidence of CMV infection/disease, recurrence, risk factors, CMV-related mortality, treatments, refractory, resistant CMV, and burden. RESULTS: Of 2708 references identified, 68 were eligible (67 studies and one guideline; 45/67 studies specific to adult allogeneic HCT recipients). The rates of CMV infection and disease within 1 year of allogeneic HCT were 24.9%-61.2% (23 studies) and 2.9%-15.7% (10 studies), respectively. Recurrence occurred in 19.8%-37.9% of cases (11 studies). Up to 10% of HCT recipients died of CMV-related causes. In all countries, first-line treatment for CMV infection/disease involved intravenous ganciclovir or valganciclovir. Conventional treatments were associated with serious adverse events such as myelosuppression (10.0%) or neutropenia only (30.0%, 39.8%) and nephrotoxicity (11.0%) (three studies), frequently leading to treatment discontinuation (up to 13.6%). Refractory CMV was reported in 2.9%, 13.0%, and 28.9% of treated patients (three studies) with resistant CMV diagnosed in 0%-10% of recipients (five studies). Patient-reported outcomes and economic data were scarce. CONCLUSION: The incidence of CMV infection and disease post-HCT is high outside of North America and Europe. CMV resistance and toxicity highlight a major unmet need with current conventional treatments.
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Infecciones por Citomegalovirus , Trasplante de Células Madre Hematopoyéticas , Adulto , Humanos , Citomegalovirus , Trasplante de Células Madre Hematopoyéticas/efectos adversos , Receptores de Trasplantes , Infecciones por Citomegalovirus/tratamiento farmacológico , Infecciones por Citomegalovirus/epidemiología , Costo de Enfermedad , Europa (Continente)/epidemiología , América del Norte/epidemiologíaRESUMEN
Acquired hemophilia A (AHA) is a rare disease caused by autoantibodies inhibiting factor VIII (FVIII) activity. Although the conditionis usually idiopathic, there may be other underlying diseases. Treatment consists of two steps: treatment of acute bleeding and immunosuppression. In this multicenter study, we aimed to demonstrate the clinical characteristics, management details, and survival of AHA patients in Turkey. Data was collected from eleven centers in Turkey. aPTT, FVIII, FVIII inhibitor, and hemoglobin (HB) levels, mixing test results, and demographics at diagnosis, treatment information, adverse events, bleeding episodes during follow-up, relapses, and outcome were analyzed. Twenty-nine patients were analyzed (58.6% female). No underlying disorder could be detected in 14 patients. The most prevalent etiologies were pregnancy, malignancy and infections. The median FVIII activity and FVIII inhibitor titer at diagnosis were 0.7% (0.0-29.4%) and 32.6 BU (0.6-135.6 BU) respectively. Bleeding was severe in 44.8% of patients. The HB value was significantly lower in patients with severe bleeding. Most of the patients (n = 25, 86.2%) had only one bleeding episode without relapse, three patients (10.3%) had two bleeding episodes, and one patient had more than three bleedings. 21 (75%) patients received hemostatic therapy. The use of recombinant FVIIa was slightly higher than activated prothrombin complex concentrate (15 versus 10 patients). Immunosuppressive treatment was initiated in 26 (93%) patients. Regimens containing steroid, cyclophosphamide, and rituximab in different combinations were the most preferred. The median follow-up period was 13 months (2-156 months). Median overall survival was 154.97 months. Four and six-year survival were 90.9 ± 0.8% and 77.9 ± 14.1% respectively. This is a unique study that investigated the demographic characteristics, treatment approaches, and patient survival of AHA in Turkey.
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Acute hemolytic transfusion reactions (AHTRs) are feared complications of packed red blood cell (PRBC) transfusions. This study aimed to investigate the clinical consequences of isolated enzyme-phase crossmatch-incompatible PRBC transfusions by clinically observing all events during the study period at a single institution with the primary goal of detecting AHTRs. Ninety-four transfusions of interest were administered during the study period. Laboratory investigations were adequate in 73 episodes, where no AHTR developed and a mean hemoglobin concentration rise of 1.1 g/dL was documented. Three transfusions were terminated prematurely; however, further investigations ruled out AHTR. The remaining 21 transfusions were also completed uneventfully without noteworthy clinical deterioration. This study's results provide clinical validation to omit pretransfusion screening with enzyme-phase crossmatch and document the safety and short-term efficacy of isolated enzyme-phase incompatible transfusions. The findings may encourage future clinical research to better understand the long-term efficacy of such transfusions, which may be valuable for transfusion-dependent patients.
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Transfusión de Eritrocitos , Reacción a la Transfusión , Humanos , Transfusión de Eritrocitos/efectos adversos , Estudios Transversales , Transfusión Sanguínea , Tipificación y Pruebas Cruzadas Sanguíneas , Incompatibilidad de Grupos SanguíneosRESUMEN
BACKGROUND: Severe acute kidney injury (AKI) requiring urgent hemodialysis (uHD) is associated with considerable morbidity and mortality in patients with multiple myeloma (MM). PURPOSE: To evaluate the renal function, outcome, and survival status of patients with MM who received uHD and to compare their overall survival with MM patients who did not receive uHD. MATERIALS AND METHODS: A total of 70 eligible MM patients who received uHD were included together with 70 control patients with MM. RESULTS: In the study group, 11 patients (15.7%) were known to have pre-existing chronic kidney disease. Thirty-four percent of the study group had AKI requiring uHD at MM diagnosis. Seventy-eight percent of the study group had severe AKI due to myeloma kidney. Renal function recovered in 36 patients (51.4%). Patients with MM who became hemodialysis dependent had significantly higher serum creatinine (sCr) levels at the time of AKI compared to patients with renal recovery (p < 0.05). Logistic regression analysis showed that high sCr on admission was significantly associated with hemodialysis dependence (odds ratio 0.78; 95% CI: 0.63-0.96; p = 0.018). The median overall survival was 30 months [IQR: 26] in the study group and 84 months [IQR: 96.25] in the control group (p < 0.05). Cox regression analysis showed that the need for uHD at initial MM diagnosis was associated with reduced survival (hazard ratio (HR) 1.9; 95% CI: 1.1-3.2; p = 0.017). Renal recovery did not provide a survival benefit. CONCLUSION: The need for uHD was associated with poor survival. Recovery of renal function was not associated with improved survival.