Transfusion-related acute lung injury in a young woman with ectopic pregnancy - case report and literature review.

BACKGROUND: Transfusion-related acute lung injury (TRALI) is a major cause of death associated with transfusion. Nevertheless it is still misdiagnosed and underreported. We present a case of this serious complication in order to make it more familiar to clinicians and indicate proper medical management, which is significant not only for the patients presenting TRALI symptoms but also for future recipients of blood components. CASE PRESENTATION: A 32-year-old white woman with in-vitro fertilization in anamnesis was admitted to Department of Obstetrics and Gynecology because of abdominal pain. Ultrasonography examination revealed pathological mass in left adnexa. The patient underwent laparoscopic left salpingectomy. The following day she reported progressive pain in lower abdomen with signs of peritoneal irritation. Emergency laparotomy was performed and active bleeding from fallopian tube was stopped. Four units of red blood cells concentrate and six units of fresh frozen plasma were transfused. Within two hours of surgery acute respiratory distress symptoms occurred, bilateral infiltrates were found on chest X-ray. The patient responded to supportive treatment (oxygen therapy, dexamethasone, diuretics) and her state improved within 12 hours. Serological diagnostics revealed anti-HLA antibodies in one donor which reacted with patient's granulocytes. Clinical picture and anti-leukocyte antibodies detected in blood component allowed to identify the immune-mediated TRALI. CONCLUSIONS: Transfusion-related acute lung injury is a life-threatening complication of transfusion which manifests as non-cardiogenic pulmonary edema. Each suspected case of this syndrome should be reported to blood center in order to confirm the diagnosis and implement preventive measures (exclusion of implicated donor from further blood donation).

[PREVFNAIT prevention of foetal/neonatal alloimmune thrombocytopenia (FNAIT) in Polish foetuses and newborns--the PREVFNAIT program]. / Zapobieganie alloimmunologicznej maloplytkowosci plodów i noworodków (AIMPN) w Polsce--program PREVFNAIT.

The scientific goals related to the grant include 1) estimation of FNAIT prevalence in Poland and 2) search for biomarkers to predict the risk of the antibody production and severity of fetal thrombocytopenia. Fetal/Neonatal Alloimmune Thrombocytopenia (FNAIT) is caused by destruction of fetal blood platelets due to maternal antibodies. This condition, which most commonly results from incompatibility between the mother and the fetus for the Human Platelet Antigen-1a (HPA-1a), may lead to intracranial hemorrhage, damage of the central nervous system (CNS) and even death of the fetus or the newborn. It can be the cause of strokes in term newborns. FNAIT is usually attributed to the presence of anti-HPA-1a antibodies. Its incidence rate is estimated at approximately 1/1000-2000 live births. In the absence of a screening program, it is usually diagnosed after birth of a child with symptoms of thrombocytopenia or CNS hemorrhage. Monitoring of antibody production and thrombocytopenia treatment to effectively minimize the risk of stroke are therefore launched only at the next pregnancy. Testing indications are broader to include fetal ultrasound for symptoms of stroke to the CNS, ventricular enlargement or hydrocephalus, and obstetric failure. Diagnostic process is also recommended prior to the planned cordocentesis, in vitro fertilization and in sisters of mothers with children with FNAIT history. HPA-1a testing remains the best method for diagnosing pregnancies at risk. The detection frequency for FNAIT in Poland remains low. Therefore, the Institute of Hematology and Transfusion Medicine (IHTM) will have performed such HPA-1a antigen testing in 30 000 Polish women within the framework of the PREVFNAIT program by March 2016. HPA-1a negative women (2% of the population) are a risk group for production of anti- HPA-1a antibodies responsible for FNAIT therefore all of them will be monitored for the presence and activity of anti-HPA-1a antibodies. Such testing will be performed free of charge for the women.

[Reticulated platelet count used for differentiation of the type of thrombocytopenia in pregnant women]. / Przydatnosc badania retykuloplytek w róznicowaniu maloplytkowosci wystepujacej u kobiet ciezarnych.

BACKGROUND: Analysis of reticulated platelets (RP), the youngest form of platelets in peripheral blood, is useful for assessment of thrombopoietic response in thrombocytopenic conditions due to intense immunological platelet destruction. AIM: The aim of the study was to assess the value of RP measurement for differentiation between pregnancy-related thrombocytopenia (PT), immunological thrombocytopenia (IM) and hereditary thrombocytopenia (HT) in pregnant women with platelet count below 100 G/L. MATERIAL: The study included 49 pregnant thrombocytopenia women (21 with PT, 22 with IM, 6 with HR) as well as 22 healthy pregnant women (Control). METHODS: The percentage of RP in peripheral blood was measured using double staining with: PE-labeled anti CD41 (Dako) and thiazole orange (Beckton Dickinson). The measurements were performed several times during pregnancy (II and III trimester) and delivery. Anti-platelet antibodies were tested by immunofluorescence and immunoensimatic assays. HPA1a antigen was determined by PCR/SSP. RESULTS: The average platelet count in all groups of thrombocytopenia women was significantly lower than in control group. The mean RP percentage in the control group (5.31%) was within the range of the haematological normal value (0.5-6%), and for thrombocytopenia women it was: 9.19% for PT women, 14.75% for IM women and 14.94% for HT women and was significantly higher than that in the control group. In the group of IM pregnant women the RP percentage was significantly higher in the II trimester than in the PT women. Anti-platelet antibody and HPA1a antigen testing excluded alloimmunological/fetus/neonatal thrombocytopenia in the study material. CONCLUSION: RP analysis has been proved useful for preliminary differentiation of PT and IT in the II trimester of pregnancy. Higher RP percentage informs the physician of the likelihood of immunological thrombocytopenia in the pregnant woman as well as of the delivery of a thrombocytopenia child.

[Post transfusion purpura--a serious underdiagnosed delayed post-transfusion event]. / Skaza krwotoczna maloplytkowa--rzadko rozpoznawane pózne powiklanie poprzetoczeniowe.

UNLABELLED: Post-transfusion purpura (PTP), a delayed post-transfusion event, is underdiagnosed, not only in Poland. Thrombocytopenia results from the destruction of patients' and transfused platelets by platelet specific antibodies (anti-HPA). The incidence of PTP is not estabilished. THE AIM: Analysis of 10 cases with PTP diagnosed in Poland during last 25 years. MATERIAL AND METHODS: 9 women and 1 man with normal platelet count who developed thrombocytopenia with diathesis haemorrhagica after transfusion of red cells or/and platelets. Platelet specific antibodies (anti-HPA) were examined by the platelet immunofluorescence test and by the monoclonal immobilization of platelet antibodies (MAIPA) assay; leucocyte antibodies by standard lymophocytotoxicity test (LCT). RESULTS: In all 10 patients the PTP was diagnosed because: 1) thrombocytopenia with diathesis haemorrhagia occured 3-10 days after the transfusion (in 5 cases blood was transfused during the operation), 2) in all the patients anti-HPA were detected (in 9- anti-HPA-1a, in 1 case anti-HPA-3a), 3) a primary HPA alloimmunization was very probable. A recovery of platelets count occured in 6 patients within 8-34 days after corticosteroids and/or IVIG, however, therapeutic effect of them was difficult to assess due to a great probability of spontaneous remission up to 1 month. Four patients died due to their basic disease although the impact of the PTP cannot be excluded. CONCLUSION: The diagnosis of PTP was possible because of the detection of anti-HPA antibodies in patients who developed thrombocytopoenia after blood transfusion.

[Transfusion-Related Acute Lung Injury a serious, underdiagnosed transfusion-related event]. / Potransfuzyjna ostra niewydolnosc oddechowa--grozne, zbyt rzadko rozpoznawane powiklanie poprzetoczeniowe.

UNLABELLED: Transfusion-Related Acute Lung Injury (TRALI) has been diagnosed very rare until recent years. However, the growing interest in TRALI allows to asses, that it is the second commonest cause of transfusion association death. In Poland only single cases of TRALI have been published. AIM: We analyzed 34 cases with dyspnea reported as a post-transfusion event and examined leukocyte antibodies, which are supposed to be an important pathogenic factor in TRALI. RESULTS: 34 patients were classified into: the group A--patients with a pulmonary oedema after exclusion of other reasons (TRALI, n= 11); the group B-- patients with pulmonary oedema, but with difficulties to exclude other reasons (possible TRALI, n=15); the group C--post transfusion dyspnea without pulmonary oedema (patients where not classified as the TRALI, n=8). In all the groups other clinical symptoms were also analyzed. The leukocyte antibodies were most often detected in the group A (91%), less often in the group B (53%) and C (37.5%). CONCLUSIONS: Transfusion-related dyspnea should be individually analyzed before the final diagnosis of TRALI. If in the donor of transfused blood the leukocyte antibodies are detected, the "trace back" procedure should be started to see whether in other patients a transfusion-related dyspnea was diagnosed. This procedure is important for potential exclusion of a given donor from blood donation.

Fetal/Neonatal Alloimmune Thrombocytopenia: Pathogenesis, Diagnostics and Prevention.

Fetal/neonatal alloimmune thrombocytopenia (FNAIT) is a relatively rare condition (1/1000-1/2000) that was granted orphan status by the European Medicines Agency in 2011. Clinical consequences of FNAIT, however, may be severe. A thrombocytopenic fetus or new-born is at risk of intracranial hemorrhage that may result in lifelong disability or death. Preventing such bleeding is thus vital and requires a solution. Anti-HPA1a antibodies are the most frequent cause of FNAIT in Caucasians. Its pathogenesis is similar to hemolytic disease of the newborn (HDN) due to anti-RhD antibodies, but is characterized by platelet destruction and is more often observed in the first pregnancy. In 75 % of these women, alloimmunization by HPA-1a antigens, however, occurs at delivery, which enables development of antibody-mediated immune suppression to prevent maternal immunization. As for HDN, the recurrence rate of FNAIT is high. For advancing diagnostic efforts and treatment, it is thereby crucial to understand the pathogenesis of FNAIT, including cellular immunity involvement. This review presents the current knowledge on FNAIT. Also described is a program for HPA-1a screening in identifying HPA-1a negative pregnant women at risk of immunization. This program is now performed at the Institute of Hematology and Transfusion Medicine in cooperation with the Department of Obstetrics and Gynecology of the Medical Centre of Postgraduate Education in Warsaw as well as the UiT The Arctic University of Norway.

Transfusion-related acute lung injury: a dangerous and underdiagnosed noncardiogenic pulmonary edema.

Transfusion-related acute lung injury (TRALI) is one of the leading causes of death associated with transfusion of blood and blood components. The understanding of the etiology and pathophysiology of this syndrome has much improved during the last decades, nevertheless numerous issues are still unresolved and symptomatic treatment remains the cornerstone of medical management. Consequently more attention is directed at primary as well as secondary prevention. The awareness of the problem within the medical society is still unsatisfactory which results in a high number of unrecognized cases or of inaccurate diagnoses one of which is cardiogenic pulmonary edema. The aim of this review is to make the TRALI syndrome more familiar to clinicians and to emphasize how significant proper medical management is both for the patients presenting TRALI symptoms as well as for future recipients of blood components.

Lysophosphatidylcholines: bioactive lipids generated during storage of blood components.

Transfusion-related acute lung injury (TRALI) is suggested to be a "two hit" event, resulting from priming and activation of pulmonary neutrophils. It is known that neutrophil activation may result from infusion of lysophosphatidylcholines (LysoPCs) accumulated during storage of blood components. The aim of our study was to verify whether the LysoPCs are released into the storage medium of blood components. We measured the LysoPCs concentration in the supernatants from stored apheresis platelet concentrates (PLTs), packed non-leukoreduced red blood cell concentrates (RBCs), leukoreduced red blood cell concentrates (L-RBCs), fresh frozen plasma (FFP) and donor plasma (control). Lipids were separated on high-performance thin-layer chromatography, detected by primulin spray and quantified by photodensitometric scanning. The LysoPCs concentration in donor plasma was similar to that in FFP. During storage the LysoPCs content in PLTs increased almost two-fold as compared to the fresh isolated platelets. In RBCs and L-RBCs the LysoPC level was very low or below detection limit and did not increase throughout the storage period. According to our observations bioactive LysoPCs may be considered a neutrophil-activating factor only following PLT transfusions but not RBCs transfusions.

[Chronic neutropenia - experience from the Department of Immunology, Children's Memorial Health Institute]. / Przewlekla neutropenia - Doswiadczena Wlasne Oddzialu Immunologii Instytutu ,, Pomnik -Centrum Zdrowia Dziecka" w Warszawie z Lat 1985-2006.

UNLABELLED: Chronic neutropenia (CN) is defined by an absolute neutrophil count (ANC) below 1500/ul, lasting at least 6 months. AIM: clinical course and treatment of children afflicted with CN was analysed. MATERIAL AND METHODS: we present 60 children treated in our department due to CN. The diagnosis was based on: bone marrow smears, ANC, immunologic investigation. RESULTS: we established the diagnosis of: Kostmann disease (KD), cyclic neutropenia (CyN), hyperIgM syndrome (HIGM), Shwachman-Diamond syndrome (SDS), severe chronic neutropenia (SCN) and chronic benign neutropenia (CBN) in: 4, 2, 2, 1, 21 and 20 children respectively. Due to positive results of tests: MAIGA, GIFT or GAT autoimmune neutropenia of infancy (AIN) was confirmed in 7 children. In 3 infants neutropenia was connected with HCMV- infection and Gancyclovir therapy. RHuG-CSF treatment was implemented in 14 and effective in 13 patients. A girl, suffering from KD, during rHuG-CSF therapy, developed chronic myeloblasts leucaemia and died. A boy, with the same diagnosis, underwent bone marrow transplantation from related donor but died from invasive pulmonary aspergillosis. Antibacterial prophylaxis was necessary in 29 children. We used Amoxicillin or Trimethoprim/Sulfametoxazole, obtaining decrease of frequency and severity of infections. During observation period all children suffered from upper respiratory tract infections, 19 had chronic gingivitis. Severe infections- bacterial pneumonia, sepsis, severe varicella and measles were observed in 30, 5, 2 and 1 patient respectively. A teenager, affected with SCN, died due to fulminant Clostridium perfringens infection. CONCLUSIONS: 1. RHuG-CSF therapy is essential in children with KD and SCN (when accompanied by severe infections). 2. AIN proved to be a mild condition, although ANC decreased below 500. In this entity rHuG-CSF is recommended during severe infections and before surgery. 3. Antibiotic prophylaxis is recommended for children with: KD, CyN, GSD1b, CN in 1st year of life, HIGM; in other cases it is considered individually.