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examined by Gastroenterology since May 2013&#44; with no history of nephrology problems&#46; Following progressive anaemia &#40;haemoglobin&#58; 6&#46;5g&#47;dl&#41;&#44; the patient was referred to the hospital centre for a red blood cell transfusion&#46; We observed a post-transfusion reaction and progressive deterioration of renal function&#44; reaching a maximum creatinine level of 7&#46;67mg&#47;dl&#44; urea 171mg&#47;dl and oligoanuria&#46; The patient was admitted to the Nephrology Department&#44; where a complete study was carried out&#46; We observed values of haemoglobin up to 4&#46;2mg&#47;dl&#44; LDH 997UI&#47;l&#44; indirect bilirubin 1&#46;1mg&#47;dl&#44; reticulocytes 2&#46;9&#37;&#44; haptoglobin 36&#46;40mg&#47;dl and anisopoikilocytosis with polychromasia in the blood smear&#46; Haematuria and pyuria were detected in the urine test strip&#46; The data suggested autoimmune intravascular haemolytic anaemia due to blood incompatibility&#46;</p><p class="elsevierStylePara">The Haematology Department was consulted&#44; which reported the presence of irregular antibodies of anti-Tja specificity at a titre of 1&#58;16&#46; These results are compatible with the absence of a high-incidence antigen in the general population&#44; the P antigen&#46; During the hospital admission&#44; renal function improved&#46; The patient required various sessions of haemodialysis&#44; support treatment with intense fluid therapy and alkalinisation&#44; and treatment of anaemia with erythropoietin and intravenous iron&#44; and four units of packed erythrocyte transfusion&#46; Currently the patient has a creatinine level of 0&#46;3mg&#47;dl and haemoglobin level of 10&#46;6g&#47;dl&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">This case covers two conditions rare in normal clinical practice&#58; the absence of the P antigen and acute renal failure &#40;ARF&#41; due to haemoglobinuria&#46; Despite the diagnostic suspicion being intravascular haemolytic anaemia secondary to blood incompatibility&#44; doubts arose due to the urine testing negative for haemosiderin and haemoglobin&#44; as well as in the Coombs test&#46;</p><p class="elsevierStylePara">The absence of the P&#44; Pk and P1 antigens&#44; structurally related&#44; occurred as a result of the loss of activity of 4-alpha-galactosyltransferase&#44; a type II membrane protein with 353 amino acids&#46; In addition to being associated with post-transfusion reactions&#44; it can also cause abortions through haemolytic disease of the fetus and newborn and&#44; according to some theories&#44; different infectious diseases &#40;infectious erythema or haemolytic-uraemic syndrome&#41;&#46;<span class="elsevierStyleSup">2&#44;3</span></p><p class="elsevierStylePara">ARF can be triggered by rhabdomyolysis and haemolysis&#44; due to myoglobin and haemoglobin respectively&#44; proteins that contain the &#8220;haemo&#8221; pigment which damages the kidney&#44; causing tubular obstruction&#44; acute tubular necrosis and vasoconstriction&#46; Haemoglobin has a greater molecular weight &#40;65&#160;000Da&#41; than myoglobin &#40;17&#160;000Da&#41; and&#44; in contrast&#44; binds to proteins such as haptoglobin&#44; making its filtration and excretion more difficult&#46; Both cases presented dark-coloured urine&#44; testing positive for blood in the urine test strip&#44; and increase in plasma LDH levels&#46; In myoglobinuria&#44; the level of creatine-kinase enzyme increased and in the case of haemolysis&#44; we detected anaemia&#44; a decrease in haptoglobin and an increase of indirect bilirubin with the appearance of dysmorphic red blood cells in peripheral blood&#46;<span class="elsevierStyleSup">4&#44;5</span></p><p class="elsevierStylePara">Treating a rare blood-type is a complicated task because of the difficulty in finding blood of the same phenotype&#46; Consequently&#44; the options were to carry out cryopreservation of autologous red blood cells or to find compatible individuals within the patient&#8217;s family&#46; In our case&#44; blood from another hospital was reserved and blood from a family member was used&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">Lecture fees&#58; Yes&#44; but not related with the content of the published study&#46;</p><p class="elsevierStylePara">Council Membership&#58; The author &#40;F&#46;J&#46; Gainza&#41; is a member of the editorial committee of the magazine <span class="elsevierStyleItalic">Nefrolog&#237;a</span> and a reviewer of other international magazines&#46;</p><p class="elsevierStylePara">Travel expenses&#58; Not related to the content of the study&#46; Routine funding for meetings and conferences organized by scientific societies&#46;</p>"
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Acute renal failure due to haemoglobinuria secondary to P antigen
Fracaso renal agudo por hemoglobinuria secundaria a antígeno P
Clara Gómez-Péreza, Ane Mujika-Lópezb, Teresa Visus-Fernández-de Manzanosb, Eva Álvarez-Quintanab, Francisco J. Gainza-de los Ríosb, Nerea Gómez-Larrambeb
a Servicio de Hematología, Hospital Universitario de Cruces, Barakaldo, Vizcaya,
b Servicio de Nefrología, Hospital Universitario de Cruces, Barakaldo, Vizcaya,
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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">To the Editor&#44;</span></p><p class="elsevierStylePara">The P antigen is a high-incidence antigen present in erythrocytes&#44; platelets&#44; lymphocytes&#44; fibroblasts&#44; the placenta and uroepithelial cells&#46; More than 99&#46;9&#37; of the population has this antigen&#46; Individuals with P phenotype are characterised by a lack of Pk&#44; P and P1 erythrocyte antigens and by forming anti-Tja antibodies &#40;anti-PP1PK&#41;&#44; without previous immunization&#46; Knowledge of this type of antibodies is important because they are involved in severe post-transfusion reactions&#46;<span class="elsevierStyleSup">1</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE REPORT</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">The patient was a 68-year-old female&#44; whose only history of significance was iron deficiency anaemia&#44; examined by Gastroenterology since May 2013&#44; with no history of nephrology problems&#46; Following progressive anaemia &#40;haemoglobin&#58; 6&#46;5g&#47;dl&#41;&#44; the patient was referred to the hospital centre for a red blood cell transfusion&#46; We observed a post-transfusion reaction and progressive deterioration of renal function&#44; reaching a maximum creatinine level of 7&#46;67mg&#47;dl&#44; urea 171mg&#47;dl and oligoanuria&#46; The patient was admitted to the Nephrology Department&#44; where a complete study was carried out&#46; We observed values of haemoglobin up to 4&#46;2mg&#47;dl&#44; LDH 997UI&#47;l&#44; indirect bilirubin 1&#46;1mg&#47;dl&#44; reticulocytes 2&#46;9&#37;&#44; haptoglobin 36&#46;40mg&#47;dl and anisopoikilocytosis with polychromasia in the blood smear&#46; Haematuria and pyuria were detected in the urine test strip&#46; The data suggested autoimmune intravascular haemolytic anaemia due to blood incompatibility&#46;</p><p class="elsevierStylePara">The Haematology Department was consulted&#44; which reported the presence of irregular antibodies of anti-Tja specificity at a titre of 1&#58;16&#46; These results are compatible with the absence of a high-incidence antigen in the general population&#44; the P antigen&#46; During the hospital admission&#44; renal function improved&#46; The patient required various sessions of haemodialysis&#44; support treatment with intense fluid therapy and alkalinisation&#44; and treatment of anaemia with erythropoietin and intravenous iron&#44; and four units of packed erythrocyte transfusion&#46; Currently the patient has a creatinine level of 0&#46;3mg&#47;dl and haemoglobin level of 10&#46;6g&#47;dl&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">This case covers two conditions rare in normal clinical practice&#58; the absence of the P antigen and acute renal failure &#40;ARF&#41; due to haemoglobinuria&#46; Despite the diagnostic suspicion being intravascular haemolytic anaemia secondary to blood incompatibility&#44; doubts arose due to the urine testing negative for haemosiderin and haemoglobin&#44; as well as in the Coombs test&#46;</p><p class="elsevierStylePara">The absence of the P&#44; Pk and P1 antigens&#44; structurally related&#44; occurred as a result of the loss of activity of 4-alpha-galactosyltransferase&#44; a type II membrane protein with 353 amino acids&#46; In addition to being associated with post-transfusion reactions&#44; it can also cause abortions through haemolytic disease of the fetus and newborn and&#44; according to some theories&#44; different infectious diseases &#40;infectious erythema or haemolytic-uraemic syndrome&#41;&#46;<span class="elsevierStyleSup">2&#44;3</span></p><p class="elsevierStylePara">ARF can be triggered by rhabdomyolysis and haemolysis&#44; due to myoglobin and haemoglobin respectively&#44; proteins that contain the &#8220;haemo&#8221; pigment which damages the kidney&#44; causing tubular obstruction&#44; acute tubular necrosis and vasoconstriction&#46; Haemoglobin has a greater molecular weight &#40;65&#160;000Da&#41; than myoglobin &#40;17&#160;000Da&#41; and&#44; in contrast&#44; binds to proteins such as haptoglobin&#44; making its filtration and excretion more difficult&#46; Both cases presented dark-coloured urine&#44; testing positive for blood in the urine test strip&#44; and increase in plasma LDH levels&#46; In myoglobinuria&#44; the level of creatine-kinase enzyme increased and in the case of haemolysis&#44; we detected anaemia&#44; a decrease in haptoglobin and an increase of indirect bilirubin with the appearance of dysmorphic red blood cells in peripheral blood&#46;<span class="elsevierStyleSup">4&#44;5</span></p><p class="elsevierStylePara">Treating a rare blood-type is a complicated task because of the difficulty in finding blood of the same phenotype&#46; Consequently&#44; the options were to carry out cryopreservation of autologous red blood cells or to find compatible individuals within the patient&#8217;s family&#46; In our case&#44; blood from another hospital was reserved and blood from a family member was used&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">&#160;</span></p><p class="elsevierStylePara"><span class="elsevierStyleBold">Conflicts of interest</span></p><p class="elsevierStylePara">&#160;</p><p class="elsevierStylePara">Lecture fees&#58; Yes&#44; but not related with the content of the published study&#46;</p><p class="elsevierStylePara">Council Membership&#58; The author &#40;F&#46;J&#46; Gainza&#41; is a member of the editorial committee of the magazine <span class="elsevierStyleItalic">Nefrolog&#237;a</span> and a reviewer of other international magazines&#46;</p><p class="elsevierStylePara">Travel expenses&#58; Not related to the content of the study&#46; Routine funding for meetings and conferences organized by scientific societies&#46;</p>"
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