Pneumococcal-vaccine-conjugate/prednisolone/tak-816

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Cold agglutinin disease and rebound effect: case report A 2-month-old girl developed cold agglutinin disease following administration of pneumococcal-vaccine-conjugate and tak-816. Further she exhibited rebound of cold agglutinin disease following gradual taper of prednisolone [routes not stated; not all dosages stated]. The girl was admitted for jaundice and poor feeding for 3 days following administration of first doses of tak-816 [haemophilus influenzae type b conjugate vaccine] and pneumococcal-vaccine-conjugate [pneumococcal conjugate vaccine]. Physical examination showed generalised pallor and scleral icterus, without acrocyanosis or hepatosplenomegaly. Laboratory findings were as follows; haemoglobin (Hb) 3.8 g/dL, mean corpuscular volume 87.3fl, reticulocyte count 3.4%, total bilirubin (TB) 10.4 mg/dL, direct bilirubin 0.8 mg/dL, lactate dehydrogenase (LDH ) 606 U/l, haptoglobin 3 mg/dL, and no hemoglobinuria. She was positive for direct antiglobulin test (DAT) with polyspecific anti-IgG and anti-C3 at 4 °C and room temperature, but was negative at 37 °C. No spherocytes were noted in peripheral blood smears. Bone marrow smears revealed erythroid hyperplasia. Based on findings she was suspected with cold autoimmune haemolytic anaemia. Further examination revealed that monospecific DAT was negative for C3 and positive for IgG. Her cold agglutinin titer was 1:16. The autoantibody was specific for the I-antigen. She was negative for Donath–Landsteiner antibody test. Serology tests were negative for infectious aetiology. Thus a diagnosis of vaccination attributed low-titer cold agglutinin disease was confirmed. The girl initially received transfusion of one unit of packed red blood cells due to severe anemia and then she started receiving prednisolone 2 mg/kg/day. At 37 °C, a crossmatch test was performed and ABO-compatible red blood cells were transfused through an in-line warmer to mitigate the risk of transfusion-related haemolysis. Her Hb increment following the transfusion was lower than expected, but LDH and TB levels normalised 2 weeks following the admission. Thus blood transfusion frequency was gradually decreased. Later prednisolone dose was tapered to 1 mg/kg/day. Her haemolysis relapsed following the reduction prednisolone dose. Therefore prednisolone dose was again increased to 2 mg/kg/day. But, she had continual reduction in Hb and she required transfusion despite high-dose steroid. She also stared receiving second line treatment with high-dose immune-globulin [IVIG]. Thus her Hb levels increased temporarily with immune-globulin, but she required frequent transfusions. She received second dose of immune-globulin. Following two cycles of immune-globulin, her Hb levels stabilised at about 10 g/dL and her transfusion requirement decreased. She received a total of 10 units of red blood cells transfusions over a period of 85 days. Over the following 2 months prednisolone was tapered off. She was discharged on oral hydrocortisone. Hydrocortisone was discontinued 2 months following the discharge. At

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