First Case Report of the Association of HbE Trait and Cold Agglutinin Disease

10.5580/1053 ◽  
2011 ◽  
Vol 7 (2) ◽  
Keyword(s):  
Case Report ◽  
Cold Agglutinin ◽  
Cold Agglutinin Disease ◽  
First Case

Perioperative considerations in the management of cold agglutinin disease in laparoscopic surgery

2021 ◽  
Vol 14 (5) ◽  
pp. e241294
Author(s):  
Yisi D Ji ◽  
Paul M Cavallaro ◽  
Britlyn D Orgill
Keyword(s):  
Case Report ◽  
Laparoscopic Surgery ◽  
Acute Cholecystitis ◽  
Operating Room ◽  
Patient Outcomes ◽  
Cold Agglutinin ◽  
Surgical Team ◽  
Cold Agglutinin Disease ◽  
Disease Exacerbation ◽  
Multidisciplinary Collaboration

An 80-year-old man with idiopathic cold agglutinin disease presented with acute cholecystitis. We describe operating room and anaesthetic considerations for patients with cold agglutinin disease and measures that can be taken to prevent disease exacerbation in this case report. Multidisciplinary collaboration and planning between the operative room staff, anaesthesia team and surgical team are needed to ensure safe surgery and optimal patient outcomes.

Successful treatment of relapsed and refractory cold agglutinin disease with bortezomib: case report

2019 ◽  
Vol 85 ◽  
pp. S68-S69
Author(s):  
S. Çelik ◽  
Z. Güven ◽  
B. Eser ◽  
L. Kaynar ◽  
M. Çetin ◽  
...  
Keyword(s):  
Case Report ◽  
Successful Treatment ◽  
Cold Agglutinin ◽  
Cold Agglutinin Disease

Late Occuring Severe Autoimmune Hemolytic Anemia in a Liver Transplanted Child.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 4025-4025
Author(s):  
Hulya Ozsahin ◽  
Michela Schaeppi ◽  
Thierry Peyrard ◽  
Fabienne Gumy-Pause ◽  
Klara Posfay-Barbe ◽  
...  
Keyword(s):  
Case Report ◽  
Liver Transplant ◽  
Lymphoproliferative Disease ◽  
Direct Antiglobulin Test ◽  
Cold Agglutinin ◽  
Reference Laboratory ◽  
Solid Organ ◽  
First Case ◽  
Thermal Amplitude ◽  
Antiglobulin Test

Abstract INTRODUCTION Autoimmune haemolytic anaemia (AIHA) can complicate solid organ as well as bone marrow transplantation both in children and adults. We describe the first case report of a child with AIHA due to mixed type warm-acting IgM and warm IgG auto-antibodies, 8 months after liver transplantation. CASE REPORT A sixteen-month old boy (A Rh D positive blood group,), 8 months after an orthotopic liver transplant (full cadaveric liver, male A Rh D negative) presented with fever and moderate hepatitis. Two weeks after this episode he developed severe anemia with a Hb level of 39 g/L. His red cells were A1 Rh(D) positive. Plasma testing could not be interpreted due to positive reactions with all test cells. Screening for irregular antibodies was positive. The direct antiglobulin test was repeatedly negative. Further analysis revealed a cold agglutinin with low titre (8 at 15°C) but high thermal amplitude (22–37°C) although of IgM nature, and the absence of any underlying alloantibodies. Only Rh null red blood cells were not agglutinated by the autoantibody. Therapy included keeping body temperature over 37° C, transfusions of A Rh(D) positive warmed crossmatched blood units, intravenous immunoglobulins (1g/kg/d x 5 days), and methylprednisolone (20 mg/kg/d). Tacrolimus was replaced by cyclosporin A. Further investigations revealed panagglutinating IgG autoantibodies in the plasma and on the erythrocytes and the monospecific direct antiglobulin test became positive for IgM, IgG, C3c and C3d. French National Reference Laboratory for Immuno-Hematology and Rare Blood Groups confirmed these findings and showed both the IgM and IgG autoantibodies to be directed against the Rh proteins (anti-RH29 antibody) and to be strongly active at 37°C. Crossmatched group O Rh(D) positive red blood cell (RBC) units were transfused, however with limited effect and progressive haemolysis. Hb level dropped to 33 g/L. One cryopreserved O Rh null RBC unit was obtained from the French National Rare Blood Bank. Rituximab® (375 mg/m2 once weekly) (anti-CD 20 monoclonal antibody) was also introduced. Immediately following this transfusion and 24 hours after the first dose of rituximab, Hb levels increased and were stable at 80 g/L. No further transfusions were needed and the haemolytic parameters normalized slowly. Total 4 doses of rituximab were administered.Ten weeks after admission, the child could be discharged. At 18 months’ follow-up, there is no recurrence of AIHA. DISCUSSION Only few cases of AIHA due to warm acting “cold” agglutinins have been described, generally resulting in death. AIHA in patients with liver transplant has been previously reported, mainly due to warm autoantibodies or to classical cold agglutinin disease associated with viral infections, lymphoproliferative disease or autoimmune disorders. Our case is interesting in that this unusual AIHA occurred not only in a liver transplanted child, but also late after transplantation. Although extensive investigations revealed no aetiology, the hypothesis of a viral infection-triggered AIHA with first an IgM, then a mixture of IgM and IgG autoantibodies directed against the same epitope is plausible. This case illustrates the efficacy of rituximab in AIHA not responding to first-line therapy and the importance of international collaboration to provide extremely rare compatible blood units.

Rituximab as Successful Therapy in a Patient with Refractory Paroxysmal Cold Hemaglobinuria.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3730-3730
Author(s):  
Ahrin B. Koppel ◽  
Stephen W. Lim ◽  
Melanie Osby ◽  
George Garratty ◽  
Dennis Goldfinger
Keyword(s):  
Case Report ◽  
Medical Center ◽  
Oral Prednisone ◽  
Single Agent ◽  
Hemoglobin Concentration ◽  
Cold Agglutinin ◽  
Igg Antibody ◽  
Syphilis Infection ◽  
Cold Temperatures ◽  
First Case

Abstract Background: Paroxysmal cold hemaglobinuria (PCH) is caused by an IgG autoantibody which behaves as a biphasic hemolysin, attaching to RBCs at cold temperatures and activating complement at warmer temperatures, leading to hemolysis. This antibody, known as the Donath-Landsteiner antibody (DL-A), frequently shows specificity for the P-antigen. PCH was historically associated with syphilis infection. More recently, the DL-A has been found primarily in children with acquired autoimmune hemolytic anemia (AIHA) following a viral illness. In adults, PCH is rare and may occur as an idiopathic disease or in association with a lymphoproliferative disorder. Cases in children usually resolve spontaneously, whereas the adult form can be chronic and pose a therapeutic challenge, since treatment with steroids and splenectomy may be ineffective. Recently rituximab has been demonstrated to be a useful agent in treating AIHA that is resistant to conventional therapies. Case Report: A 64-year-old woman presented to another hospital with three months of progressive weakness. She was found to be severely anemic. Gastrointestinal blood loss was ruled out. Extensive work up was obtained with CT imaging and bone marrow biopsy, which showed no evidence of malignancy. A hemolytic process was identified and she was placed on oral prednisone 60mg daily. The patient then presented to Cedars-Sinai Medical Center three months later with recurrent fatigue and a hemoglobin concentration (Hb) of 6.6 g/dL. Lab values revealed an elevated reticulocyte count (7.9%), WBC 27.6, total bilirubin 3.5 mg/dL, indirect fraction 3.4 mg/dL, elevated LDH 445 U/L, absent haptoglobin, and microspherocytes on peripheral blood smear. The Direct Antiglobulin (Coombs) Test (DAT) was positive with an anti-complement reagent and negative with an anti-IgG reagent, leading to the suspicion of a DL-A or cold agglutinin. Cold agglutinin titer was normal. A Donath-Landsteiner test was positive, confirming the diagnosis of PCH. Steroids were rapidly tapered and she was given rituximab 375 mg/m2. Her Hb increased and evidence of hemolysis ceased. The patient received 3 additional doses of rituximab weekly. Her Hb recovered to normal. The patient did well for 9 months until she presented again with acute hemolysis (Hb 8.8 g/dL.) The DAT was again positive with an anti-complement reagent and negative with an anti-IgG reagent. She was given a single dose of rituximab with cessation of hemolysis. She received another 3 doses, which resulted in stabilization of her Hb. She remains well at 6 months follow-up. Discussion: The most frequent form of AIHA is due to a warm, IgG antibody and is commonly responsive to steroids or splenectomy, whereas in cold agglutinin disease, caused by an IgM antibody these therapies are usually ineffective. The use of rituximab has been reported as a useful treatment for both warm and cold AIHA refractory to conventional therapy. This is the first case report to our knowledge of a patient with adult PCH refractory to steroids successfully treated with rituximab. This patient responded dramatically to rituximab on two separate occasions, and has remained in remission since the second cycle after treatment with this single agent. Rituximab may represent an effective therapy for adult patients with chronic PCH.

Steroid-Responsive Idiopathic Cold Agglutinin Disease: A Case Report

1989 ◽  
Vol 81 (3) ◽  
pp. 166-168 ◽  
Author(s):  
M. Lahav ◽  
I. Rosenberg ◽  
A.J. Wysenbeek
Keyword(s):  
Case Report ◽  
Cold Agglutinin ◽  
Cold Agglutinin Disease

scholarly journals Intraoperative pulmonary thromboembolism in a patient with cold agglutinin disease: a case report

2020 ◽  
Vol 27 (5) ◽  
pp. 411-412
Author(s):  
Haruna Yamamoto ◽  
Satoru Kayama ◽  
Taichi Onimaru ◽  
Midori Imaizumi ◽  
Kinuko Kubota ◽  
...  
Keyword(s):  
Case Report ◽  
Pulmonary Thromboembolism ◽  
Cold Agglutinin ◽  
Cold Agglutinin Disease

Anesthetic Management for the Open Heart Surgery in a Patient with Cold Agglutinin Disease: Case report

2003 ◽  
Vol 45 (1) ◽  
pp. 157
Author(s):  
Francis Sahngun Nahm ◽  
Jong Sung Kim ◽  
Byung Moon Ham ◽  
Hyun Soo Moon ◽  
Sung Hee Han
Keyword(s):  
Case Report ◽  
Heart Surgery ◽  
Anesthetic Management ◽  
Open Heart Surgery ◽  
Cold Agglutinin ◽  
Open Heart ◽  
Cold Agglutinin Disease ◽  
Disease Case

Unusually Severe Course of Autoimmune Hemolytic Anemia / Cold Agglutinin Disease: A Case Report

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S165-S165
Author(s):  
M M Yilmaz ◽  
Y Tanhehco
Keyword(s):  
Case Report ◽  
Plasma Exchange ◽  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Middle Lobe ◽  
Cold Agglutinin ◽  
Upper Respiratory Tract ◽  
Cold Agglutinin Disease ◽  
Cell Surface Antigens ◽  
Short Period

Abstract Introduction/Objective Autoimmune hemolytic anemia (AIHA) is a group of disorders with limited epidemiological and clinical data, characterized by hemolysis due to autoantibodies against red blood cell surface antigens. Cold agglutinins account for about 25% of all AIHA, which can agglutinate erythrocytes at 0-4 °C. Cold agglutinin disease (CAD) is a self limited disease and usually does not cause significant hemolysis. Here, we report a case with an unusually severe course for secondary CAD and role of plasma exchange in the management. Methods A 53-year-old male patient with no known past medical history presented to the emergency department with shortness of breath and syncope after a week of upper respiratory tract infection symptoms. The patient became profoundly anemic (presented with 8.1 g/dL hemoglobin level and progressively decline to 4.2 g/dL) in a short period of time. Upon initial evaluation, his peripheral blood smear demonstrated clumps of RBCs. Direct anti-globulin testing was negative for IgG but positive for C3 which is consistent with CAD. A respiratory PCR panel detected Rhinovirus. Mycoplasma and EBV IgM were negative. Additionally, chest X-ray showed right middle lobe pneumonia that was treated with antibiotics. Patient received multiple transfusions of pre-warmed pRBCS and showed initial improvement but eventually went into respiratory failure and cardiac arrest with return of spontaneous circulation after 8 minutes of CPR. Subsequently, plasma exchange was started. Only after intiation of plasma exchange, the patient’s ongoing hemolysis was stabilized. Conclusion This case report presents a patient with unexpectedly rapid and severe hemolysis from secondary cold agglutinin disease. Interestingly, the case appeared not to be caused by EBV or Mycoplasma pneumonia infection but Rhinovirus. Further studies confirmed patient had no autoimmune disorder or lymphoid malignancy that may had initiated secondary CAD. On the management aspect, pre-warmed RBC transfusions were not sufficient to stabilize the patient’s condition. Plasma exchange was able to control ongoing hemolysis within 2 sessions successfully.

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