Transfuzijsko liječenje u KBC Sestre milosrdnice tijekom dvanaest godina

Transfusion treatment is administered according to clinical and laboratory results, with ongoing patient assessments. Decisions on necessary measures to prevent any adverse and unexpected events and reactions are made on the basis of hemovigilance and ongoing gathering and analysis of relevant data. Information about transfusion treatment at the Sestre milosrdnice University Hospital Center, Vinogradska site, was retrospectively collected for a period of twelve years (2001-2012). In that period, 14137.25±1693.07 units of all blood products were used, where red blood cells (RBC) accounted for 67.34%, fresh frozen plasma (FFP) for 17.55%, and platelet concentrates (PC) for 14.32%. During the study period, the consumption of RBC was even, of FFP decreased by 45% and of PC increased by 58%. RBC transfusions were received by 10.43% of hospitalized patients, 1.46% of them during surgical procedures. Transfusions of all blood products were received by 14.63% of patients. We found 247 adverse reactions to all blood products. Febrile nonhemolytic and allergic reactions were quite equally represented, 49.5% each. As for other reactions (1%), one transfusion associated circulatory overload and one transfusion related acute lung injury were recorded. There were no fatal post-transfusion reactions.Transfuzijsko liječenje se provodi prema kliničkim i laboratorijskim nalazima uz stalnu procjenu bolesnikova stanja. Nadzorom transfuzijskog liječenja (hemovigilancija) uz kontinuirano prikupljanje i analizu podataka o neželjenim i neočekivanim događajima i reakcijama prosuđuje se o potrebnim mjerama kojima bi se oni spriječili. Retrogradno su prikupljeni podaci o transfuzijskom liječenju bolesnika u KBC Sestre milosrdnice, lokacija Vinogradska, tijekom dvanaest godina (2001.-2012.). Potrošeno je godišnje 14137,25±1693,07 doza svih krvnih pripravaka. Udio eritrokoncentrata (KE) bio je 67,34%, svježe smrznute plazme (SSP) 17,55% i trombokoncentrata (KT) 14,32%. Tijekom promatranog razdoblja potrošnja KE bila je ujednačena, SSP smanjena za 45% i KT povećana za 58%. Transfuzije KE je primilo 10,43% hospitaliziranih bolesnika. Tijekom kirurških zahvata transfuzije KE je primilo 10,11% bolesnika, odnosno 1,46% hospitaliziranih bolesnika. Transfuzije svih krvnih pripravaka primilo je 14,63% bolesnika. U promatranom razdoblju prijavljeno je 247 poslijetransfuzijskih reakcija na sve krvne pripravke. U zbroju svih reakcija podjednako su zastupljene febrilne nehemolitičke i alergijske reakcije (49,5%). Od ostalih (1%) jedna je bila preopterećenje kardiovaskularnog sustava i jedna akutna plućna insuficijencija uzrokovana transfuzijom. Nije bilo poslijetransfuzijskih reakcija sa smrtnim ishodom

SEVERE HEMOLYTIC DISEASE IN A NEWBORN CAUSED BY ANTI-K ANTIBODIES

Hemolitička bolest novorođenčeta (HBN) posljedica je majčine aloimunizacije na eritrocitne antigene fetusa. Majčina protutijela IgG razreda kroz posteljicu prelaze u fetalnu cirkulaciju te vezanjem za površinske eritrocitne antigene uzrokuju njihovo razaranje. U najtežim slučajevima razvija se hidrops fetusa, a najčešće hiperbilirubinemija i anemija novorođenčeta. Učestalost anti-K protutijela kao uzroka HBN-a slijedi odmah iza anti-D protutijela. Tijekom 80-ih godina prošlog stoljeća uočen je različiti mehanizam djelovanja anti-D i anti-K protutijela. HBN uzrokovana anti-K protutijelima je pretežno posljedica supresije eritropoeze, a hemoliza fetalnih eritrocita je manje izražena. HBN se može javiti i u prvoj trudnoći. U slučaju koji prikazujemo Kell negativna majka se imunizirala trudnoćom s Kell pozitivnim djetetom, što je dovelo do teške anemije novorođenčeta. Nalaz anti-K protutijela u majčinom serumu i na djetetovim eritrocitima, uz odgovarajući Kell fenotip oca, potvrdili su dijagnozu. Zahvaljujući pravodobnom i adekvatnom tretmanu transfuzijama, djevojčica je imala potpuni i brzi oporavak, bez posljedica za rast i razvoj.Hemolytic disease of the newborn (HDN) is a consequence of the mother\u27s alloimmunization towards fetal erythrocyte antigens. Maternal IgG class antibodies cross the placenta into the fetal circulation and attach to the antigenic sites on the surface of the erythrocytes, hence destroying them. Hydrops fetalis is the most severe form of the disease, but hyperbilirubinemia and anemia are more common. The incidence of HDN caused by anti-K antibodies comes immediately after the incidence of HDN caused by anti-D antibodies. During the 1980\u27s a different mechanism was noted for anti-D and anti-K antibodies. Suppression of erythropoesis, rather than hemolysis, is the predominant mechanism in HDN related to Kell aloimmunization, and may occur during the first pregnancy. In our case, the aloimmunization of the K-negative mother occured during pregnancy with a K-positive fetus, leading to severe anemia in the newborn. Serological findings of anti-K antibodies in the mother\u27s serum, and the presence of Kell antigens on the surface of the child\u27s erythrocytes, compiled with the father\u27s Kell antigen status established the diagnosis. Due to timely and adequate treatment with transfusions , the girl recovered completely without consequences to growth and development

THE IMPORTANCE OF ANTENATAL IMMUNOPROPHYLAXIS FOR PREVENTION OF HEMOLYTIC DISEASE OF THE FETUS AND NEWBORN

Hemolitička bolest fetusa i novorođenčeta (HBFN) je posljedica majčine aloimunizacije na eritrocitne antigene fetusa. Aloimunizacija na D antigen iz Rhesus (Rh) sustava krvnih grupa ima posebno značenje budući da se radi o najjačem eritrocitnom imunogenu. Otkako se unatrag četiri desetljeća rutinski provodi postnatalna profilaksa imunizacije davanjem anti-RhD imunoglobulina RhD negativnim ženama, drastično je smanjen mortalitet zbog HBFN. Uvođenjem antenatalne profilakse klinički značajna HBFN je postala izuzetno rijetka. Sporadični teški oblici bolesti su uglavnom posljedica nedosljednosti u provođenju profilakse. U slučaju koji opisujemo nije prepoznat rizik imunizacije tijekom prve majčine trudnoće, te je izostala antenatalna prevencija. Nakon primarne imunizacije, u drugoj je trudnoći s RhD pozitivnim djetetom došlo do žestokog sekundarnog imunološkog odgovora majke i ranog razvoja teške fetalne anemije. Intrauterine transfuzije su spasile vitalno ugroženi fetus, ali su istodobno uzrokovale snažnu eritroidnu supresiju. Anemija koja je trajala mjesecima nakon rođenja liječena je ponavljanim transfuzijama, te humanim rekombinantnim eritropoetinom. Unatoč teškoj kliničkoj slici, kratkoročni ishod bolesti je povoljan i dječak zasada ima uredan rast i razvoj. Ipak, rizici kasnih posljedica, a posebice neurorazvojnih odstupanja nalažu daljnje pomno praćenje djeteta. Opisani slučaj ukazuje na trajnu aktualnost problematike Rh imunizacije u nas. Provođenje antenatalne imunoprofilakse je prvi i ključni korak u kvalitetnoj prevenciji HBFN.Hemolytic disease of the fetus and newborn (HDFN) is a consequence of maternal alloimmunization against fetal red blood cell antigens. Alloimmunization against D antigen from Rhesus (Rh) blood group system is particularly important because of its strong immunogenicity. During the last few decades, the introduction of RhD prophylaxis by postpartum administration of anti-D immunoglobulin to RhD negative women, now improved with antenatal prophylaxis, has led to a dramatic decrease in perinatal mortality and morbidity from HDFN. However, severe cases have not disappeared, mostly due to prophylaxis failure. In our case, inappropriate prenatal care during the first pregnancy in an RhD negative mother resulted in primary immunization. In the next pregnancy with an RhD positive child, the mother’s secondary immune response was extremely strong and led to early development of severe fetal anemia. The fetus survived thanks to the treatment with intrauterine transfusions (IUT), but they caused suppression of erythropoiesis, which lasted for months after birth. The long lasting, late anemia was treated with repeated postnatal red cell transfusions and recombinant human erythropoietin (rHuEPO). Despite the severity of HDFN in our case, the short-term outcome is good. The boy has normal growth until now, but due to the possibility of an adverse long-term neurodevelopmental outcome, this case requires continuous follow up. It also reminds of the fact that RhD alloimmunization remains an actual problem in daily routine. Antenatal prophylaxis is a crucial step in quality care of those who are at a risk of HDFN

Kombinirana megaloblastična i imunohemolitička anemija - Prikaz slučaja

A 55-year-old female with a history of psychosis and rheumatoid arthritis was admitted to the hospital for fatigue and dizziness. At admission, macrocytic anemia, high serum lactic acid dehydrogenase (LDH) and gastrin concentrations, decreased serum vitamin B concentration, with macroovalocytes and poikilocytes in peripheral blood smear suggested the diagnosis of pernicious anemia. Indirect antiglobulin test (IAT) was negative. Surprisingly, treatment by vitamin B and folic acid administered for two weeks was ineffective and followed by transitory worsening of hemoglobin concentration on day 8. Repeat direct antiglobulin test (DAT) and IAT were positive. This immunotransfusion conversion, suggesting the presence of autoimmune hemolytic anemia, could be explained by change in the macroblastic erythrocyte population, i.e. emerging red cells with completely exposed membrane antigens due to vitamin B treatment and/or higher degree of dysregulation of the lymphocyte clone secreting erythrocyte autoantibodies. We proposed the coexistence of pernicious and autoimmune hemolytic anemia; therefore, methylprednisolone was added to vitamin B treatment. This therapy successfully improved hemoglobin and erythrocyte concentration. Although megaloblastic-pernicious anemia is a common disease, association of pernicious and autoimmune hemolytic anemia with two mechanisms of hemolysis (ineffective erythropoiesis and immune mechanism) is a rare condition, with only several dozens of cases described so far.Opisuje se 55-godišnja bolesnica koja je primljena u bolnicu zbog slabosti i vrtoglavica. Ranije je bila liječena od reumatoidnog artritisa i psihoze. Pri dolasku su nalazi makrocitne anemije s makroovalocitima i hipersegmentiranim neutrofilima u perifernom razmazu, u serumu visoka koncentracija laktat dehidrogenaze (LDH) i gastrina, te snižena koncentracija vitamina B upućivali na dijagnozu perniciozne anemije. Indirektni antiglobulinski test (IAT) bio je negativan. Dvotjedno liječenje vitaminom B i folnom kiselinom ne samo da nije imalo učinka, nego se osmog dana liječenja pogoršao stupanj anemije. Razmatralo se liječenje transfuzijom eritrocita, ali su kontrolni IAT i direktni antiglobulinski test (DAT) sada bili pozitivni, što je ukazivalo na autoimunu hemolitičku anemiju (AIHA). Ova imunotransfuziološka konverzija, uz ostale razloge možda vezana i uz dvotjedno liječenje vitaminom B , mogla je biti uzrokovana pojačanom reaktivnošću i "gubitkom nadzora" klona limfocita koji luče anteritrocitna antitijela i/ili promjenama u sazrijevanju i konformaciji eritrocitne membrane makroblasta s jačim izražajem antigena na koje su reagirali "nekontrolirani" limfociti. Anemija je tada shvaćena kao združena pojava perniciozne anemije i AIHA. Dodatkom metilprednisolona vitaminu B postignut je porast hemoglobina i eritrocita, a stanje bolesnice se popravilo. Iako je perniciozna anemija česta bolest, udružena perniciozna i autoimuna hemolitička anemija su opisane samo u nekoliko desetaka slučajeva, uglavnom u sastavu drugih autoimunih bolesti

Combined megaloblastic and immunohemolytic anemia associated - case report

A 55-year-old female with a history of psychosis and rheumatoid arthritis was admitted to the hospital for fatigue and dizziness. At admission, macrocytic anemia, high serum lactic acid dehydrogenase (LDH) and gastrin concentrations, decreased serum vitamin B concentration, with macroovalocytes and poikilocytes in peripheral blood smear suggested the diagnosis of pernicious anemia. Indirect antiglobulin test (IAT) was negative. Surprisingly, treatment by vitamin B and folic acid administered for two weeks was ineffective and followed by transitory worsening of hemoglobin concentration on day 8. Repeat direct antiglobulin test (DAT) and IAT were positive. This immunotransfusion conversion, suggesting the presence of autoimmune hemolytic anemia, could be explained by change in the macroblastic erythrocyte population, i.e. emerging red cells with completely exposed membrane antigens due to vitamin B treatment and/or higher degree of dysregulation of the lymphocyte clone secreting erythrocyte autoantibodies. We proposed the coexistence of pernicious and autoimmune hemolytic anemia; therefore, methylprednisolone was added to vitamin B treatment. This therapy successfully improved hemoglobin and erythrocyte concentration. Although megaloblastic-pernicious anemia is a common disease, association of pernicious and autoimmune hemolytic anemia with two mechanisms of hemolysis (ineffective erythropoiesis and immune mechanism) is a rare condition, with only several dozens of cases described so far