Acute hemolytic transfusion reaction (AHTR) is a potentially serious complication associated with the transfusion of ABO incompatible blood. It results in disseminated intravascular coagulation (DIC), shock, renal failure and death. Patient misidentification, sample mislabeling are some of the causes of an AHTR. To-date there is no data available on the use of RBCET in the management of an AHTR. We report the clinical course and successful management of an AHTR following massive ABO incompatible blood transfusion. The patient is a 68-year-old female with known coronary artery disease and type O Rh negative blood, who presented to the emergency room with unstable angina. She was taken emergently to the operating room for a quadruple coronary artery bypass graft. Due to sample mislabeling, she received 7 units of incompatible type A Rh negative packed red blood cells (PRBC) intra-operatively. During the immediate post-operative period, she was noted to have increased drainage from the surgical site, decreased urine output, hematuria and a precipitous drop in hemoglobin to 6.5 g/dL. She underwent re-exploration and received an additional 2 units of incompatible type A Rh negative PRBC and other blood products including fresh frozen plasma (FFP), platelets and cryoprecipitate that were ABO incompatible. In the recovery room, family members confirmed her blood type as type O Rh negative. Immediate re-typing utilizing pre-transfusion blood samples collected for CBC and chemistry, and obtaining blood bank records from her previous admission from another hospital a diagnosis of ABO incompatible AHTR was established. Further work-up revealed a strong positive DAT (3+ IgG) with a positive eluate (patient’s anti-A antibodies coating the transfused type A RBC’s). Hemolysis parameters showed elevated LDH 753 IU, bilirubin 2.1 mg/dL, and decreased haptoglobin 29.4 mg/dL. Her urine output continued to decrease with increase in serum creatinine to 2.4mg/dL. A continuous veno-venous hemodialysis (CVVHD) was started along with fluid resuscitation and urine alkalinization. A RBCET was performed within 11 hours after the transfusion of the last incompatible unit, which was followed by a second RBCET 24 hours later. Each RBCET consisted of 8 units of type O Rh negative blood. Following the two RBCET her blood type became type O Rh negative. Hemolysis parameters improved over the next several days and became normal. Serum creatinine progressively improved with increasing urine output. Dialysis was discontinued on day 13. Patient continued to do well and was discharged home in good condition with a normal renal function three weeks after her initial admission. The severity and course of an AHTR is dictated by the load of incompatible blood cells in the circulation. The high mortality rate associated with an AHTR warrants an aggressive approach in addition to supportive care. RBCET is used in the management of severe hemolytic disease of the new born, complications of sickle cell disease, and to lower the parasite load in malaria and babesiosis. The role of RBCET in AHTR is not established. There are two case reports in the Japanese literature where a RBCET was performed in the management of an AHTR with successful outcome. The course of this patient suggests that aggressive management including supportive care, timely RBCET, and renal replacement treatment may prevent DIC, organ damage and alter the grave prognosis associated with AHTR following incompatible blood transfusion.
Intranasal desmopressin (IN-DDAVP) is used for home treatment of menorrhagia in women with inherited bleeding disorders. The effect of IN-DDAVP on laboratory haemostatic parameters in women with menorrhagia related to platelet dysfunction is unknown. We evaluated the effects of IN-DDAVP on haemostatic parameters in women with menorrhagia and platelet dysfunction and correlated them with menstrual flow. Eleven women (aged 18-45) with menorrhagia and haemostatic abnormalities had determination of von Willebrand factor antigen (VWF:Ag), von Willebrand factor ristocetin cofactor (VWF:RCo) activity, factor VIII coagulant activity (FVIII:C), platelet aggregation and platelet adenosine tri-phosphate (ATP) release pre-IN-DDAVP and 60-min post-IN-DDAVP. Eight of eleven women underwent platelet function analyzer (PFA-100) closure time determination with collagen/adrenaline and collagen/adenosine diphosphate cartridges pretreatment and post-treatment. IN-DDAVP was administered during two consecutive menstrual cycles. Menstrual flow was assessed during each cycle using a pictorial blood assessment chart. Treatment with IN-DDAVP resulted in elevated VWF levels and shortened PFA-100 closure time with significant inverse correlation between shortening of PFA-100 closure times and increases in VWF levels. There were also significant inverse correlations between changes in menstrual flow and changes in VWF:Ag (P = 0.02), VWF:RCo (P = 0.04) and FVIII:C (P = 0.006), following treatment. In vitro platelet aggregation and platelet ATP release response did not correct and did not correlate with changes in menstrual flow. Our results demonstrate a correlation between haemostatic parameters and menstrual flow following IN-DDAVP in women with menorrhagia and platelet dysfunction.
Development of inhibitors is a serious complication in hemophilia A patients (factor VIII deficiency) who receive plasma-derived or recombinant products. It occurs in about 20–30% of patients with severe or moderate hemophilia A. The presence of a high titer inhibitor (titer > 5BU), is associated with increased morbidity and mortality, complicates disease management, and increases cost of treatment. Treatments used for the management of acute bleeds include high doses of human FVIII, porcine FVIII, prothrombin complex concentrates, FVIII bypassing activity (FEIBA), and recombinant VIIa (rVIIa)). The induction of immune tolerance (IT)with daily high doses of FVIII has been reported to eradicate the inhibitor in 60–80% of patients; although it is a very costly procedure, the response appears to be dependent on the level of the inhibitor with patients with low inhibitor titer (<10 BU) responding better to IT treatment. To rapidly reduce/eradicate the alloimmune factor VIII antibodies immunomodulatory agents, including immunoglobulin, corticosteroids, cyclophosphamide, azathioprine, and plasmapheresis have been used. Rituximab, an anti-CD20 monoclonal antibody, eliminates circulating B cells and has been shown to reduce/eradicate alloimmune factor VIII antibodies in children with severe hemophilia A. We report the clinical course and response to rituximab treatment in a 24 year old male with moderate hemophilia A and high titer inhibitor. The patient presented with a massive retroperitoneal bleed with Hb 6.4g/dL and an inhibitor titer of 41 BU reaching a maximum of 111 BU over the next four weeks. For the acute bleeding episode, he received FEIBA and rVIIa. For the treatment of inhibitor, he was given rituximab 375 mg/m2 × 4 together with prednisone 1 mg/Kg daily. Prednisone was slowly tapered off over the next several weeks. Three months later, his inhibitor titer had decreased to 35 BU and remained below 15 BU for the next 30 weeks. The course of this patient’s response suggests that rituximab in combination with prednisone may rapidly reduce inhibitor titer in adult patients with hemophilia and high titer inhibitor. It is possible the rituximab therapy may replace IT therapy or be used as an adjunct to IT therapy. Also, the combination of rituximab and prednisone treatment may reduce/prevent the exposure of young patients to cytotoxic chemotherapy. Randomized clinical trials will be required to determine whether the combination of rituximab and prednisone is a better option compared to cytoxan and prednisone to reduce/eradicate inhibitors in young adult hemophilia patients with high titer inhibitor. Figure Figure
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