A doença das aglutininas frias é um tipo de anemia hemolítica autoimune definida pela presença de autoanticorpos frios (autoanticorpos ativos em temperaturas abaixo de 30°C).
Introdução
O que você precisa saber de cara
A doença das aglutininas frias é um tipo de anemia hemolítica autoimune definida pela presença de autoanticorpos frios (autoanticorpos ativos em temperaturas abaixo de 30°C).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 5 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 14 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Doença de aglutininas frias
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Outros ensaios clínicos
23 ensaios clínicos encontrados, 3 ativos.
Publicações mais relevantes
Autoimmune hemolytic anemia: New frontiers in diagnosis and therapy.
Autoimmune hemolytic anemias comprise warm AIHA (wAIHA), due to IgG autoantibodies reacting at body temperatures, and cold agglutinin disease (CAD), due to IgM reacting in cold and strongly activating complement. The diagnosis, based on the direct antiglobulin test (DAT), is sometimes difficult since the DAT is performed with various methods with different sensitivity and specificity. Novel investigation includes DAT by flow-cytometry that helps the diagnosis of DAT-negative forms and in-deep bone marrow examination that aims to understand AIHA pathogenesis. Therapy is different in wAIHA and CAD: in the former, steroids represent the first-line, followed by rituximab, splenectomy and immunosuppressors, while in the latter rituximab is recommended frontline, followed by rituximab plus chemotherapy or the complement inhibitor sutimlimab. Plasmacell-directed therapies (bortezomib and daratumumab) may be administered in refractory patients. Several new therapies are under investigation, including inhibitors of Bruton's and spleen tyrosine kinases, neonatal Fc receptor, and cytokines, and bi-specific monoclonal antibodies.
Mutations in histone lysine methyltransferase genes are associated with autoimmune cytopenias: a single-center study.
Epigenetic dysregulation is increasingly recognized as a contributor to autoimmunity and autoimmune cytopenias (AIC). Histone lysine methyltransferases (MTs) are key regulators of gene expression through epigenetic modification. Germline MT mutations are associated with immunodeficiency syndromes such as Kabuki syndrome, which frequently co-occurs with AIC, while somatic KMT2D mutations have been reported in cold agglutinin disease. This study aimed to (1) determine the frequency of mutations in histone methyltransferase genes (KMT2D, KMT2A, KMT2C, and KDM6A) among patients with AIC, and (2) compare clinical, laboratory, and immunologic characteristics, as well as treatment responses, between patients with and without MT mutations. We retrospectively analyzed 534 patients who underwent comprehensive next-generation sequencing of bone marrow or peripheral blood; 80 had a diagnosis of AIC. Patients were categorized as MT-positive (MT⁺) or MT-negative (MT⁻) based on the presence of mutations in the specified genes. MT⁺ patients were significantly more likely to develop AIC compared with MT⁻ patients (25/90 vs 55/444). MT⁺ patients with AIC exhibited significantly lower serum immunoglobulin G levels, and those with autoimmune hemolytic anemia were more likely to demonstrate complement involvement on the direct antiglobulin test. MT variants identified in the AIC cohort were absent from the 1000 Genomes database and were predicted to be among the top 1% of the most deleterious variants based on Phred scores. These findings suggest that mutations in histone methyltransferase genes may play a role in the development of AIC. Prospective studies are warranted to validate these associations and to elucidate the epigenetic mechanisms underlying autoimmunity, which may ultimately support biomarker discovery and personalized approaches to disease management.
Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
Monoclonal gammopathies of clinical significance represent a heterogeneous spectrum of non-malignant disorders that may involve multiple organ systems or remain organ-restricted. These conditions are driven by circulating monoclonal proteins (MIg) secreted by small, often indolent, B-cell or plasma cell clones. Accumulating evidence has demonstrated that MIg may possess both thrombogenic and hemorrhagic potential, mediated through complex and multifactorial interactions with coagulation pathways, platelets, and the vascular endothelium. In this review, we critically appraise the spectrum of hemostatic complications associated with monoclonal gammopathies, encompassing entities such as amyloidosis, cold agglutinin disease, M-protein-associated antiphospholipid antibody syndrome, anti-PF4 antibody-mediated disorders, and thrombotic microangiopathies, among others. Given their substantial clinical burden and frequent under recognition, we propose that these MIg-driven hemostatic disorders be classified separately as monoclonal gammopathies of thrombotic/hemorrhagic significance (MGTHS) encompassing both thrombotic and hemorrhagic subtypes. We additionally outline a proposed diagnostic approach emphasizing systematic evaluation for alternative etiologies, characterization of the monoclonal protein, and assessment for clinical, laboratory, and treatment-response features supporting pathogenic attribution. Establishing such a nosological framework highlights the clinical importance of these conditions and underscores the need for systematic recognition, diagnostic evaluation, and multidisciplinary management of these often overlooked but clinically consequential entities.
Cryoglobulinemia Type II: Sustained Remission After B-Cell-Directed Therapy.
BACKGROUND Cryoglobulinemia is a rare disease with a prevalence of <5 cases per 10 000. In many cases there is significant diagnostic delay, leading to years of morbidity. There are no randomized trials on non-infectious type II cryoglobulinemia, and choice of therapy is based on clinical expertise and observational data. Therapy based on the monoclonal anti-CD20 antibody rituximab in combination with glucocorticosteroids is the preferred choice in many centers. This strategy induces clinical remission in two-thirds of patients. However, one-third of the initially-responding patients experience relapse within the first year. As such, many patients will not achieve sustained remission. CASE REPORT We describe the clinical course of 2 patients with non-infectious type II cryoglobulinemia. They were initially treated according to standard clinical practice, without lasting symptom relief. A novel treatment strategy was attempted, targeting the underlying B-cell clone responsible for the production of disease-inducing monoclonal immunoglobulins. These protocols, initially developed for treatment of chronic cold agglutinin disease, contain rituximab in combination with fludarabine or bendamustine. Sustained clinical, immunological, and hematological remissions were achieved in both patients, and the treatment was well tolerated with no need for hospitalization or other supportive measures. CONCLUSIONS Treating patients with type II cryoglobulinemia using chemo-immune therapy in the same regimens as used in cold agglutinin disease could be considered as a therapeutic option. Based on our observations in the 2 patients described in this case series, this approach seems feasible and well tolerated. Deep sustained remissions may be possible.
Framework-mediated binding of foreign and self-glycans by IGHV4-34 antibodies.
Cold agglutinin disease is an autoimmune condition characterised by expression of self-reactive antibodies to I/i carbohydrate blood group antigens (polymers of N-acetyllactosamine or 'LacNAc' units) resulting in anaemia through the agglutination and complement-mediated destruction of red blood cells. This antibody response is dominated by antibodies of the human IGHV4-34 germline, which display a unique hydrophobic patch formed by germline-encoded tryptophan and tyrosine residues at positions 7 and 25 within framework 1 (FR1). Although the requirement for conservation of this FR1 patch for binding to the I/i carbohydrate antigen has been well established, structural insights regarding the mechanistic role of the FR1 patch have remained elusive. Intriguingly, recent papers describing IGHV4-34 antibodies bound to the glycan-adorned surface of the HIV envelope glycoprotein shed light on the utility of this FR1 surface. Rather than indirectly shaping the conformation of the conventional antigen binding site as previously proposed, the structures reveal direct interfaces between the hydrophobic FR1 patch and high mannose glycans projected by HIV. Given the stereochemical similarities between these glycans and LacNAc, these structures suggest how I/i self-antigen glycans might be similarly engaged by IGHV4-34 antibodies in a framework-centric non-CDR mode and provide a rationale for the preservation of this otherwise self-reactive antibody germline.
Publicações recentes
Safety and Effectiveness of Sutimlimab in Cold Agglutinin Disease: A Real-World International Experience.
Case Report: Refractory cold agglutinin disease with hypersplenism: efficacy of splenectomy in a patient treated with sutimlimab.
Clinicopathological features of primary cold agglutinin disease in the Japanese population: Heterogeneous characteristics and diagnostic challenges.
Sutimlimab vs B-Cell-Targeted Therapy in Cold Agglutinin Disease: Which Is the Optimal Approach?
Severe Intravascular Hemolysis and Acute Kidney Injury Triggered by Mycoplasma-Associated Cold Agglutinin Disease: A Case Report.
📚 EuropePMC434 artigos no totalmostrando 198
Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
Journal of thrombosis and haemostasis : JTHAutoimmune hemolytic anemia: New frontiers in diagnosis and therapy.
Blood reviewsCryoglobulinemia Type II: Sustained Remission After B-Cell-Directed Therapy.
The American journal of case reportsFramework-mediated binding of foreign and self-glycans by IGHV4-34 antibodies.
Frontiers in immunology[Pure red cell aplasia in a patient with cold agglutinin disease-associated lymphoproliferative disorder].
[Rinsho ketsueki] The Japanese journal of clinical hematologyA Cold Case: Cold Agglutinin Disease Complicating Stem Cell Transplant.
Case reports in transplantationMonoclonal gammopathies of cutaneous significance: A nomenclature and pathophysiology-based classification.
Journal of the American Academy of DermatologySutimlimab for Relapsed Cold Agglutinin Disease: Real-Life Evidence of Sustained Response With Extended-Interval Dosing.
European journal of haematologyDouble Agglutination Observed in Mycoplasma-Associated Kawasaki Disease: Pathophysiological Insights and Considerations for Treatment Strategy.
CureusA case of concurrent cold agglutinin disease and C3 glomerulonephritis requiring differentiation from other iatrogenic immunodeficiency-associated lymphoproliferative disorder in a patient with rheumatoid arthritis and Sjögren's disease.
Immunological medicineTheir Blood Ran Cold: A Case Series on Cold Agglutinin Disease.
QJM : monthly journal of the Association of Physicians[Advances in the treatment of cold agglutinin disease and their impact on quality of life].
[Rinsho ketsueki] The Japanese journal of clinical hematologyPhagocytosis of Lymphocytes and Erythrocytes by B-Cell Lymphoma in a Patient With a Clinical Suspicion of Pure Red Cell Aplasia.
Clinical case reportsParoxysmal Cold Hemoglobinuria Associated With Monoclonal B-Cell Lymphocytosis in an Elderly Patient: A Report of a Rare Case.
CureusAutoimmune Hemolytic Anemia in Non-Hodgkin's Lymphoma: Pathogenesis, Diagnosis, and Management.
Nigerian journal of clinical practiceMutations in histone lysine methyltransferase genes are associated with autoimmune cytopenias: a single-center study.
Blood vessels, thrombosis & hemostasisSutimlimab in Patients With Cold Agglutinin Disease (CAD): Results From a Managed Access Program.
European journal of haematologyConvergent mutation trajectories convert functional self-tolerance in IGHV4-34 B cells to genetic tolerance encoded in the antibody.
Proceedings of the National Academy of Sciences of the United States of AmericaIptacopan for Immune Thrombocytopenia and Cold Agglutinin Disease: A Global Phase 2 Basket Clinical Trial.
American journal of hematologyDiagnosis and management of cold agglutinin disease.
Hematology. American Society of Hematology. Education ProgramManagement of autoimmune hemolytic anemia.
Hematology. American Society of Hematology. Education ProgramPrecision targeting of autoreactive B cells in systemic lupus erythematosus using anti-9G4 idiotope synthetic immune receptor T cells.
bioRxiv : the preprint server for biologyAdvances in Complement Inhibition Therapies for Paroxysmal Nocturnal Hemoglobinuria and Autoimmune Hemolytic Disorders.
Journal of blood medicineDurable responses and favorable cost-effectiveness of rituximab-based chemoimmunotherapy for primary cold agglutinin disease.
Blood researchCase Report: Coexisting cold agglutinin disease and acquired hemophilia A: a rituximab-responsive dual autoimmune disorder.
Frontiers in medicineComplement-targeting therapies in hemolytic diseases.
Current opinion in immunologyA Rare Combination: Cold Agglutinin Disease Followed by Waldenström Macroglobulinemia-A Case of Early Treatment Response.
Diagnostics (Basel, Switzerland)Cerebral venous sinus thrombosis with cold agglutinin disease in summer: illustrative case.
Journal of neurosurgery. Case lessonsPartial arch replacement for type A aortic dissection with cold agglutinin disease after sutimlimab.
Journal of cardiology casesReal-world efficacy and safety of immunochemotherapy in cold agglutinin-mediated autoimmune haemolytic anaemia.
British journal of haematologyIptacopan for cold agglutinin disease: a case report with literature review.
Frontiers in immunologyRefractory cold autoimmune hemolytic anemia as initial presentation of gastric B cell lymphoma: a case report.
Journal of medical case reportsCold Agglutinin Syndrome as the Initial Presentation of Acute Myeloid Leukemia: A Case Report.
Clinical case reportsRosai-Dorfman-Destombes disease in an elderly man. A case of necrosis in the lymph node.
American journal of blood researchUnifying serological testing for cold agglutinins.
Vox sanguinisCold Agglutinin Disease in the Setting of Pancreatic Adenocarcinoma.
European journal of case reports in internal medicineIn response: Approach to autologous stem cell harvesting and transplantation in patients with cold-agglutinin disease or cryoglobulins.
TransfusionA unique case of red blood cell agglutination reaction on glass surface in otherwise normal sample.
Asian journal of transfusion scienceBeneath the surface in autoimmune hemolytic anemia: pathogenetic networks, therapeutic advancements and open questions.
Frontiers in immunologyA Case of Primary Cold Agglutinin-Mediated Hemolytic Anemia Successfully Treated with Steroids.
Cureus[Treatment strategies for autoimmune hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyThe Role of Cold Agglutinins Screening in Cardiac Surgery: Necessity or Redundancy?
Sisli Etfal Hastanesi tip bulteniA Case of Cold Agglutinin Disease With Transformation to High-Grade Lymphoma During Sutimlimab Treatment.
CureusSuccessful treatment for distal-arch aortic aneurysm in a cold agglutinin-positive patient via physician-modified thoracic endovascular aortic repair: a case report.
General thoracic and cardiovascular surgery casesA Rare Case of Cold Antibody-Mediated Autoimmune Haemolytic Anaemia in Early Systemic Lupus Erythematosus.
Cureus[Cold agglutinin disease (CAD)].
Innere Medizin (Heidelberg, Germany)[Anterolateral Partial Sternotomy as an Effective Approach for Open Repair After Debranching Thoracic Endovascular Aortic Repair in Aortic Arch Aneurysm].
Kyobu geka. The Japanese journal of thoracic surgeryReal-World Use of Rituximab in the Treatment of Cold Agglutinin Disease in the United States: A Retrospective Study.
EJHaemA case of autoimmune hemolytic anemia with cold agglutinin disease post-operation for recurrent ovarian cancer.
International cancer conference journalPrevalence and incidence of primary autoimmune hemolytic anemia and cold agglutinin disease in the United States, 2016-2023.
PloS oneConcurrence of mixed cryoglobulinaemia and cold agglutinin disease, and the putative role for a stereotyped immunoglobulin light chain.
British journal of haematologyDRESS Syndrome with Cold Agglutinins: An Unusual Immune Response to Anticonvulsants.
Current drug safetyAnesthetic Management During Total Abdominal Hysterectomy in a Patient With Cold Agglutinin Disease: A Case Report.
CureusAnesthesia for Urgent Gastroscopy in Cold Agglutinin Disease: A Case Report and Literature Review.
CureusA middle-aged woman with the initial symptom of acrocyanosis diagnosed with cold agglutinin disease: A case report.
SAGE open medical case reportsIntervascular Large B-cell Lymphoma with Severe Anemia and a High-titer of Cold Agglutinin.
Internal medicine (Tokyo, Japan)Ambulatory total knee arthroplasty in a patient with cold agglutinin disease.
Canadian journal of anaesthesia = Journal canadien d'anesthesie[Clinical and Laboratory Characteristics of Cold Agglutinin Disease Patients with Positive Results of Acidified-Serum Lysis Test].
Zhongguo shi yan xue ye xue za zhiConfirmation of Fixed Quarterly Riliprubart Regimen in Patients with Cold Agglutinin Disease Using Population PK/PD and Exposure-Response Analyses.
Clinical pharmacology and therapeuticsSpinal Anesthesia in a Patient With Cold Agglutinin Disease Presenting for Total Knee Arthroplasty in a Community Hospital Setting.
CureusCold agglutinin disease coexisting with left main coronary artery involvement and ventricular dysfunction - a challenging triad.
Indian journal of thoracic and cardiovascular surgeryTreatment of autoimmune hemolytic anemia: novel and investigational approaches.
Minerva medicaA rare case of gastrointestinal diffuse large B cell lymphoma with cold agglutinin disease presentation.
The Kaohsiung journal of medical sciencesIntracapillary Monoclonal IgM Deposition Concomitant With Cold Agglutinin Disease.
Kidney medicineAbdominal Aortic Aneurysm with Primary Cold Agglutinin Disease Treated with Endovascular Aortic Repair.
Annals of vascular diseasesApproach to autologous stem cell transplantation in a patient with severe cold agglutinin disease, a case report.
TransfusionIntraoperative vascular anastomosis occlusion due to cold agglutinin disease during brain surgery: a case report.
JA clinical reportsVery low doses of rituximab in autoimmune hemolytic anemia-an open-label, phase II pilot trial.
Frontiers in medicineSevere mycoplasma pneumoniae pneumonia combined with cold agglutinin disease and pulmonary embolism in childhood: A case report and review of the literature.
African journal of reproductive healthBreast Adenocarcinoma and Cold Agglutinin Disease: A Paraneoplastic Syndrome.
CureusSuccessful rituximab treatment in a seronegative rheumatoid arthritis patient with concurrent cold agglutinin syndrome and immune thrombocytopenia.
Rheumatology internationalStay Away From the Cold: An Unusual Case of Cold Agglutinin Disease Presenting as Recurrent Transient Ischemic Attacks.
CureusJalink M, Jacobs CF, Khwaja J, et al. Daratumumab monotherapy in refractory warm autoimmune hemolytic anemia and cold agglutinin disease. Blood Adv. 2024;8(11):2622-2634.
Blood advancesAnesthesia Management of Cold Agglutinin Disease in a Pregnant Patient: A Case Report.
Clinical case reportsNavigating cold agglutinin-induced hemolytic anemia in developing countries: A case report and literature review.
SAGE open medical case reportsAutoimmune haemolytic anaemias.
Nature reviews. Disease primersSafety and efficacy of pegcetacoplan treatment for cold agglutinin disease and warm antibody autoimmune hemolytic anemia.
BloodParsaclisib for the treatment of primary autoimmune hemolytic anemia: Results from a phase 2, open-label study.
American journal of hematologyLong-term safety profile of sutimlimab in adult Japanese patients with cold agglutinin disease.
International journal of hematologyThe Vital Role of Immunohematology in Diagnosing Paroxysmal Cold Hemoglobinuria: A Clinical Case Report.
CureusAutoimmune Hemolytic Anemias: Challenges in Diagnosis and Therapy.
Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie[Current recommendations for the diagnosis and treatment of autoimmune hemolytic anemia based on etiology and pathology].
[Rinsho ketsueki] The Japanese journal of clinical hematologySutimlimab for Cold Agglutinin Disease.
Journal of the advanced practitioner in oncologyCold Agglutinin Disease: A Rare Paraneoplastic Manifestation of a Thyroid Malignancy.
Cureus[The treatment strategies of autoimmune hemolytic anemia].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi[Cold agglutinin syndrome associated with infectious mononucleosis: A case report].
Revista medica del Instituto Mexicano del Seguro SocialSustained improvements in patient-reported outcomes after long-term sutimlimab in patients with cold agglutinin disease: results from the CADENZA study open-label extension.
EClinicalMedicineLong-term efficacy and safety of continued complement C1s inhibition with sutimlimab in cold agglutinin disease: CADENZA study Part B.
EClinicalMedicineConcurrent existence of cold agglutinin disease and ANCA-associated vasculitis with fulminant pulmonary-renal syndrome poses significant treatment challenges.
BMJ case reportsEfficacy of sutimlimab for cold agglutinin disease in a patient on chronic hemodialysis.
CEN case reports[Cold agglutinin disease: pathology, diagnosis, and treatment].
[Rinsho ketsueki] The Japanese journal of clinical hematologyThe impact of individual clinical features in cold agglutinin disease: hemolytic versus non-hemolytic symptoms.
Expert review of hematologyCold-antibody Autoimmune Hemolytic Anemia: its Association with Neoplastic Disease and Impact on Therapy.
Current oncology reportsObinutuzumab for the Treatment of Cold Agglutinin Disease: A Case Report.
Turkish journal of haematology : official journal of Turkish Society of HaematologyThe evolving management algorithm for the patient with newly diagnosed cold agglutinin disease.
Expert review of hematologyA Case of Renal Iron Overload Associated with Cold Agglutinin Disease Successfully Managed by Rituximab.
Clinical hematology internationalRöth A, Broome CM, Barcellini W, et al. Long-term sutimlimab improves quality of life for patients with cold agglutinin disease: CARDINAL 2-year follow-up. Blood Adv. 2023;7(19):5890-5897.
Blood advancesNew classifications of B-cell neoplasms: a comparison of 5th WHO and International Consensus classifications.
International journal of hematologyCost-effectiveness of sutimlimab in cold agglutinin disease.
American journal of hematology[Sutimlimab for cold agglutinin disease, a newcomer among complement inhibitors in immunohematology].
La Revue de medecine interneSuccessful Management of Total Plasma Exchange for Hemolytic Cold Agglutinin Disease.
Internal medicine (Tokyo, Japan)Successful treatment of concurrent cold agglutinin disease and myelodysplastic syndrome.
Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for HaemapheresisWhen you think you should transfuse…don't!
Laboratory medicineDaratumumab monotherapy in refractory warm autoimmune hemolytic anemia and cold agglutinin disease.
Blood advancesCOVID-19 vaccine safety and immunogenicity in patients with cold agglutinin disease receiving concomitant sutimlimab.
American journal of hematologyA Rare Case Presentation: EBV-related Cold Agglutinin Disease and Venous Thrombosis.
The Pediatric infectious disease journalCold Agglutinin Disease and COVID-19: A Scoping Review of Treatments and Outcomes.
Journal of clinical medicine researchClinical and laboratory characteristics of patients with cold agglutinin disease: A retrospective analysis at a tertiary medical center.
Asian journal of transfusion scienceSutimlimab suppresses SARS-CoV-2 mRNA vaccine-induced hemolytic crisis in a patient with cold agglutinin disease.
Journal of clinical and experimental hematopathology : JCEHGene expression analysis revealed downregulation of complement receptor 1 in clonal B cells in cold agglutinin disease.
Clinical and experimental immunologyMonoclonal Gammopathy of Undetermined Cardiovascular Significance; Current Evidence and Novel Insights.
Journal of cardiovascular development and diseaseThe Importance of Early Suspicion for Cold Autoimmune Hemolytic Anemia.
Cureus[Sjögren's syndrome combined with cold agglutinin disease: A case report].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesCold Agglutinin Disease in a Rhesus Macaque (Macaca mulatta).
Comparative medicineSafety, tolerability, and activity of the active C1s antibody riliprubart in cold agglutinin disease: a phase 1b study.
BloodNovel pharmacotherapy for drug-induced immune hemolytic anemia.
Expert opinion on pharmacotherapyRecombinant erythropoietin in autoimmune hemolytic anemia with inadequate bone marrow response: a prospective analysis.
Blood advancesCytokine polymorphisms in patients with autoimmune hemolytic anemia.
Frontiers in immunologyDaratumumab-based regimen for cold agglutinin disease refractory to therapy directing B-cell clone.
Leukemia & lymphomaOrganizing Pneumonia in a Case of Cold Agglutinin Disease with Pulmonary Thrombosis.
Internal medicine (Tokyo, Japan)Cold agglutinin disease and cryoglobulinaemia: A frequent coexistence with clinical impact.
British journal of haematologyCold agglutinin disease in childhood: a case series with review of literature.
Recenti progressi in medicinaAntibody based therapeutics for autoimmune hemolytic anemia.
Expert opinion on biological therapyTotal arch replacement for an aortic arch aneurysm with cold agglutinin disease after rituximab and plasmapheresis.
Journal of cardiothoracic surgerySevere autoimmune hemolytic anemia; epidemiology, clinical management, outcomes and knowledge gaps.
Frontiers in immunology[Novel therapeutic agents for hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyA case of cold agglutinin disease in a bottlenose dolphin (Tursiops truncatus).
The Journal of veterinary medical scienceSystemic Lupus Erythematosus and Antiphospholipid Syndrome Accompanied by Mixed-Type Autoimmune Hemolytic Anemia.
Case reports in rheumatologyClinical and clonal characteristics of monoclonal immunoglobulin M-associated type I cryoglobulinaemia.
British journal of haematologyRefractory cold agglutinin disease successfully treated with daratumumab. A case report and review of literature.
Hematology (Amsterdam, Netherlands)Cold agglutinin disease: A case report with atypical clinical findings.
SAGE open medical case reportsCold Agglutinins Causing Intracoronary Hemagglutination During Premature Ventricular Contraction Ablation Requiring Thrombectomy and Venoarterial Extracorporeal Membrane Oxygenation.
Journal of cardiothoracic and vascular anesthesiaA Rare Case of Cold Agglutinin Syndrome Associated With Legionella Pneumonia.
CureusLong-term sutimlimab improves quality of life for patients with cold agglutinin disease: CARDINAL 2-year follow-up.
Blood advancesLow-grade B cell lymphoma in the perirenal space of the left kidney associated with high titer cold agglutinin disease.
American journal of blood research[Novel anti-complement therapeutics for hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyHemolytic Anemia in the Setting of Atypical Pneumonia: A Case of Cold Agglutinin Disease.
CureusMolecular pharmacology in complement-mediated hemolytic disorders.
European journal of haematologyComplement-directed therapy for cold agglutinin disease: sutimlimab.
Expert review of hematologyThe Histopathology of Cold Agglutinin Disease-Associated B-Cell Lymphoproliferative Disease.
American journal of clinical pathologyA Case Report of Cold Agglutinin Disease, Severe B12 Deficiency, and Pernicious Anemia: A Deadly Coincidence.
CureusSustained inhibition of complement C1s with sutimlimab over 2 years in patients with cold agglutinin disease.
American journal of hematologyStrategies to overcome the diagnostic challenges of autoimmune hemolytic anemias.
Expert review of hematologyPrimary cold agglutinin disease: a recently recognised diagnostic entity in WHO and ICC.
HistopathologyThe choice of new treatments in autoimmune hemolytic anemia: how to pick from the basket?
Frontiers in immunologySutimlimab Pharmacokinetics and Pharmacodynamics in Patients with Cold Agglutinin Disease.
The Journal of pharmacology and experimental therapeuticsSutimlimab for the Treatment of Cold Agglutinin Disease.
HemaSphereAnti-C1s humanized monoclonal antibody SAR445088: A classical pathway complement inhibitor specific for the active form of C1s.
Clinical immunology (Orlando, Fla.)A case of cold agglutinin syndrome associated with chronic lymphocytic leukaemia harbouring mutations in CARD11 and KMT2D.
International journal of hematologyMonoclonal antibodies for treatment of cold agglutinin disease.
Expert opinion on biological therapyDevelopment of the Cold Agglutinin Disease Symptoms and Impact Questionnaire (CAD-SIQ).
European journal of haematologyUncovering the Unusual: A Case of Mixed Connective Tissue Disease With Rare Presentation, Atypical Complications, and Therapeutic Dilemmas.
CureusIgM monoclonal gammopathy of undetermined significance: clinicopathologic features with and without IgM-related disorders.
HaematologicaThree different pathways of IgM-antibody-dependent hemolysis are mainly regulated by complement.
Frontiers in immunologySpurious macrocytic anaemia in a patient with systemic lupus erythematosus: cold agglutinin disease.
Clinical rheumatologyRise of the planet of rare anemias: An update on emerging treatment strategies.
Frontiers in medicineDaratumumab as a novel treatment option in refractory ITP.
Blood cells, molecules & diseasesNeutrophilic erythrophagocytosis in myelodysplastic syndrome and cold agglutinin disease co-occurrence.
Clinical case reportsSuccessful Management of Refractory Autoimmune Hemolytic Anemia with Cold Agglutinin Disease with Splenectomy: A Case Report with Review of Literature.
The Kobe journal of medical sciencesPerioperative Management of a Patient With Severe Cold Agglutinin Disease Undergoing Total Hip Arthroplasty With a Cemented Stem: A Case Report.
A&A practiceMedically-attended anxiety and depression is increased among newly diagnosed patients with cold agglutinin disease: Analysis of an integrated claim-clinical cohort in the United States.
PloS oneAn atypical case of refractory passenger lymphocyte syndrome after renal transplantation.
NefrologiaCold AIHA and the best treatment strategies.
Hematology. American Society of Hematology. Education ProgramSutimlimab: A Complement C1s Inhibitor for the Management of Cold Agglutinin Disease-Associated Hemolysis.
The Annals of pharmacotherapyIncidentally discovered cold hemagglutinins within autologous blood bag and cardioplegia line in a patient with a recent history of COVID-19 undergoing coronary artery surgery.
PerfusionAn observational analysis of disease burden in patients with cold agglutinin disease: Results from a large US electronic health record database.
Journal of managed care & specialty pharmacyBilateral retinal vasculitis associated with cold agglutinin disease treated with obinutuzumab and infliximab.
American journal of ophthalmology case reportsPlasma cell neoplasms and related entities-evolution in diagnosis and classification.
Virchows Archiv : an international journal of pathologySutimlimab provides clinically meaningful improvements in patient-reported outcomes in patients with cold agglutinin disease: Results from the randomised, placebo-controlled, Phase 3 CADENZA study.
European journal of haematologySustained hematologic remission after discontinuation of sutimlimab treatment in patients with cold agglutinin disease.
Blood advancesChronic cold agglutinin disease after a third COVID-19 mRNA vaccination.
International journal of hematologyComplement C1s as a diagnostic marker and therapeutic target: Progress and propective.
Frontiers in immunologyDual arterial thrombosis: A diagnostic enigma.
Annals of African medicine[Advances in understanding the pathogenesis and treatment of autoimmune hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyCryoglobulins and cold agglutinins for hand arm vibration syndrome.
Occupational medicine (Oxford, England)Current Treatment Options in Cold Agglutinin Disease: B-Cell Directed or Complement Directed Therapy?
Transfusion medicine reviewsDifferential Diagnosis of Waldenström's Macroglobulinemia and Early Management: Perspectives from Clinical Practice.
Blood and lymphatic cancer : targets and therapySutimlimab improves quality of life in patients with cold agglutinin disease: results of patient-reported outcomes from the CARDINAL study.
Annals of hematologyPractical therapy for primary autoimmune hemolytic anemia in adults.
Clinical and experimental medicine[Recent progress in the diagnosis and treatment of cold agglutinin disease].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiHow do I warm HPC(A) products to maximize cell viability in the setting of cold agglutinin disease?
TransfusionSutimlimab for treatment of cold agglutinin disease: why, how and for whom?
ImmunotherapyThe Burden of Cold Agglutinin Disease on Patients' Daily Life: Web-Based Cross-sectional Survey of 50 American Patients.
JMIR formative researchThe significance of antiglobulin (Coombs) test reactivity in patients with COVID-19.
Immunobiology[Thrombotic risk in autoimmune hemolytic anemia].
[Rinsho ketsueki] The Japanese journal of clinical hematologyNovel Insights into Factor D Inhibition.
International journal of molecular sciencesIgM monoclonal gammopathies of clinical significance: diagnosis and management.
HaematologicaNovel Monoclonal Antibody Therapy in a Patient With Treatment-Refractory Warm Autoimmune Hemolytic Anemia.
CureusHeavy chain/light chain assay is a useful biomarker for diagnosis and management of patients with cold agglutinin disease.
British journal of haematologyAutoimmune hemolytic anemia: causes and consequences.
Expert review of clinical immunology[Splenic lymphoma, diagnosis and treatment].
La Revue de medecine interneSutimlimab in patients with cold agglutinin disease: results of the randomized placebo-controlled phase 3 CADENZA trial.
BloodSustained sutimlimab response for 3 years in patients with cold agglutinin disease: A phase I, open-label, extension trial.
British journal of haematologyDevelopment of New Drugs for Autoimmune Hemolytic Anemia.
PharmaceuticsMicrovascular free tissue transfer in the setting of cold agglutinin disease.
MicrosurgeryBone marrow and peripheral blood involvement of relapsed diffuse large B-cell lymphoma after prior cold agglutinin disease.
International journal of laboratory hematologyMortality in cold agglutinin disease shows seasonal pattern.
TransfusionCold agglutinin disease secondary to severe SARS-CoV-2 treated with eculizumab.
BMJ case reportsAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Autoimmune hemolytic anemia: New frontiers in diagnosis and therapy.
- Mutations in histone lysine methyltransferase genes are associated with autoimmune cytopenias: a single-center study.
- Monoclonal Gammopathies of Thrombotic and Hemorrhagic Significance: Mapping into a classification schema.
- Cryoglobulinemia Type II: Sustained Remission After B-Cell-Directed Therapy.
- Framework-mediated binding of foreign and self-glycans by IGHV4-34 antibodies.
- Safety and Effectiveness of Sutimlimab in Cold Agglutinin Disease: A Real-World International Experience.
- Case Report: Refractory cold agglutinin disease with hypersplenism: efficacy of splenectomy in a patient treated with sutimlimab.
- Clinicopathological features of primary cold agglutinin disease in the Japanese population: Heterogeneous characteristics and diagnostic challenges.
- Sutimlimab vs B-Cell-Targeted Therapy in Cold Agglutinin Disease: Which Is the Optimal Approach?
- Severe Intravascular Hemolysis and Acute Kidney Injury Triggered by Mycoplasma-Associated Cold Agglutinin Disease: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:56425(Orphanet)
- MONDO:0018922(MONDO)
- GARD:6130(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Q3961632(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
