Agglutination of labeled RBC

https://ijshr.com/IJSHR_Vol.5_Issue.3_July2020/IJSHR_Abstract.0016.html, 2020

Cold agglutinin disease is a type of auto immune hemolytic anemia characterized by cold agglutinins which were first described by Landsteiner in 1903. The implicated autoantibodies are IgM type which bind to red blood cells (I, i, H antigen) at lower or colder temperatures leading to hemolysis. The hemolytic potential of such antibodies is usually determined by their titer, specificity and thermal amplitude. In this case, 31 year old female presented with fever, severe pallor and jaundice in a cold winter night. The samples were sent to Department of Transfusion Medicine to provide compatible blood units for transfusion in view of severe anemia. Visible agglutinates in anticoagulated sample and discrepant results in immunohematology laboratory were the first observation to suspect cold agglutinins. The resolution of same was done with simple pre-warming technique using conventional tube test. High titers correlated with severity of symptoms due to hemolysis. The compatible blood units obtained were transfused with special precautions and transfusion was uneventful. This case highlights the importance of simple step of maintaining blood sample of patient of cold antibodies at 37 0 C and using conventional tube technique for immunohematological tests.

IntroductIon Cold agglutinin disease (CAD) is a complement-mediated immune hemolytic disease characterized by red cell destruction in the reticuloendothelial system. [1] About half of CAD patients were idiopathic without any underlying cause. The secondary cases were with the underlying disorder such as a viral infection, autoimmune disease, and lymphoid malignancy. The incidence of CAD is around 1/million, accounting for 15%-25% of autoimmune hemolytic anemia. [1] CAD mainly affected middle-aged or elderly people but was also reported in young people. [2] In CAD, the autoantibody, mainly of Immunoglobulin (Ig) M class and agglutinate red cells at 3°C-4°C. [3] We report serological and clinical findings of two CAD cases with similar serological findings but different clinical outcomes. The purpose of this study was to increase our horizon on the clinical and serological characteristics of CAD, and the information learned from this study would help in early diagnosis and better management of these patients. case reports Case 1 A 61-year-old female patient presented with the complaints of weakness, body pain, and difficulty in breathing to our medical outpatient department. On physical examination, pallor was evident with hemoglobin (Hb): 6.2 g/dL and hematocrit (HCT): 18.9%, blood urea 141.00 mg/dl, uric acid 14.80 mg/dl, and serum creatinine 2.7 mg/dl. Peripheral blood smear showed red cell agglutination and rouleaux formation [Figure 1a]. White blood cells show a few atypical cells (lymphoplasmacytic), neutrophils show a toxic granulation with cytoplasmic vacuolization and reduced platelets. A blood requisition form and blood samples were received in the blood bank. Immunohematology workup: on naked eye examination, EDTA and plain sample vials showed visible agglutinates. Blood grouping by conventional tube technique revealed a discrepancy. Cell grouping revealed B positive, while serum Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia associated with autoantibodies that react mainly in the cold. CAD can interfere with immunohematology tests. We report two cases of CAD presented initially as blood grouping discrepancy leading us to further workup. Case I was a 61-year-old female with symptoms of weakness, bone pain, and difficulty breathing with laboratory findings of high cold agglutinin titers of 1:1024. Bone marrow aspirate revealed a mixture of >30% plasma cell and plasma blast. Bone marrow biopsy revealed an interstitial, focal, and nodular pattern of involvement by plasma cells, leading to the diagnosis of plasma cell dyscrasias. Case II analyzed was benign CAD with cold agglutinin titers of 1:64, with no other abnormality. Both patients were managed successfully. We report serological and clinical findings of two CAD cases with similar serological findings but different clinical outcomes.

Scandinavian Journal of Immunology, 2001

International Journal Of Community Medicine And Public Health, 2020

Autoimmune hemolytic anemia is a disease characterized by destruction of red blood cells (RBC) and anemia, caused by production of antibodies released against the body's own RBCs. While this condition is more commonly idiopathic, it may accompany autoimmune diseases as well. Cold agglutinin disease (CAD) is a rare subtype of acquired autoimmune hemoliytic anemia, however, is an idiosyncratic clinical and pathological terminology, usually seen in older ages. This condition is caused by IgM antibodies called "cold agglutinins" formed against I antigens on RBC membranes which cause agglutination of RBCs at lower temperatures. In this case, a 56 year old male patient who was diagnosed with CAD secondary to Epstein-Barr virus (EBV) infection is being presented.

International Journal of Research in Medical Sciences, 2016

Autoimmune hemolytic anemia (AIHA) is characterized by the presence of agglutinins directed against autologous erythrocytes causing their reduced survival. Approximately 20% of AIHA are associated with cold-reactive antibodies. About half of patients are termed idiopathic without any underlying causes. Secondary cases are associated with underlying diseases or with certain drugs. We report herein a case of idiopathic cold autoimmune hemolytic anemia in a 65 year old lady without any underlying cause who presented with grouping discrepancy and responded well to treatment. Blood transfusion was completely avoided by keeping her warm and other conservative management including hematinic. At the time of discharge from the hospital her haematocrit reached 24%. Patient now is being followed as an outpatient and she continues to work daily albeit acro-cyanosis occurs sometimes on exposure to cold water. To our knowledge a careful communication between the transfusion services and clinicians can avoid unnecessary blood transfusions in such cases especially in clinically responding patients.

Journal of the College of Physicians and Surgeons Pakistan

Autoimmune hemolytic anemia (AIHA) is a form of hemolytic anemia in which red cells lysis occurs due to presence of an autoantibody. Association of AIHA is well known with lymphoproliferative disorders, especially with non-Hodgkin's lymphoma. However, AIHA in association with Hodgkin's lymphoma is seen occasionally. Of the AIHA associated with Hodgkin's lymphoma, most are of warm type or mixed type. Cold AIHA, as seen in our case, is very rare in Hodgkin's lymphoma.

2023

Cold agglutinin disease (CAD) is a rare form of autoimmune hemolytic anemia (AIHA), in which IgM-specific antibodies cause the agglutination of red blood cells (RBCs) at temperatures < 37°C and activate the classical pathway of complement leading to extravascular hemolysis, C3b-coated RBCs are phagocytosed by the macrophages of the reticuloendothelial system (predominantly in the liver). Up to date there are two clinical pathologic entities recognized as distinct with different therapeutic implications: cold agglutinin disease and cold agglutinin syndrome (CAS). Primary CAD is recognized as clonal B-cell lymphoproliferative disorder of the bone marrow, clinical and imagistic evidence of associated malignancy. CAS arises in the setting of an underlying disorder such as infection, autoimmune disease, or malignancy (non-Hodgkin lymphoma or other malignant process). The diagnosis of CAD is often delayed due to the unpredictable clinical course. In spite of the current therapeutic options which are directed at the pathogenic B cells or the complement system, the low response rates and frequent relapses lead to challenges regarding the management of this disease.

Asian Journal of Transfusion Science, 2015

Background and Aim: Cold agglutinin syndrome (CAS) primary or secondary represents approximately 16-32% of autoimmune hemolytic anemia cases. Most patients present with mild, chronic hemolytic anemia with exacerbation of the condition in the cold environment. Red cell transfusions are only indicated when there is a life-threatening anemia causing crisis. We studied the clinical and serological characterization of CAS with the aim that the information gained from this study would help in proper diagnosis and management of these patients. Materials and Methods: The prospective study included nine patients who were admitted with severe anemia. Detailed work-up were conducted to establish the diagnosis, severity of in vivo hemolysis and transfusion management. Results: All patients presented with pallor, weakness, fatigue and painful fingers and toes with exacerbation of symptoms in winter months. Secondary CAS was observed in three patients suffering from malignant lymphoma. Red cells of all patients were coated with complements (C3) more specifically C3d. In one patient suffering from malignant lymphoma, the cold autoagglutinin titer was as high as 4096. Autoantibody in seven patients was specific to "I" antigen and one to "i" antigen. Conclusions: We conclude that detailed clinical and serological characterization is needed to diagnose and manage CAS. Whereas avoidance of cold exposure is the primary therapy, but no critical patient should be denied blood transfusion due to serological complications. All transfusion services should follow the correct protocol to maximize blood safety in CAS.

Journal of medical case reports, 2018

Cold agglutinin disease is a rare disorder characterized by an autoimmune hemolytic anemia occurring at low temperatures. Physical examination findings, often limited to acrocyanosis, are combined with a thermal amplitude test to help establish the diagnosis. Thermal amplitude testing determines the highest temperature at which the cold agglutination will occur and is an important parameter in diagnosing cold agglutinin disease. Here we describe a 57-year-old white man of German and Nicaraguan descent with known chronic cold agglutinin disease who presented to our ophthalmology clinic for evaluation of a cataract. During routine cataract surgery, the lowered temperature of the conjunctiva from intermittent flow of balanced salt solution at room temperature induced a cold agglutination reaction in conjunctival vessels easily visible under a surgical microscope. To the best of our knowledge, this method of demonstrating cold agglutinin disease has not been described in the literature ...

Hematology/Oncology Clinics of North America, 2015