A high prevalence of neutrophil-specific antibodies in ELANE-mutated severe congenital neutropenia.

An assay for neutrophil-specific antibodies is frequently used in the workup of chronic severe neutropenia and is suggestive of autoimmune, or sporadically alloimmune neutropenia, rather than severe congenital neutropenia (SCN). We analyzed a neutropenia consortium database for the outcomes of antibody testing initiated before receiving genetic diagnosis in Polish SCN cohort. Test results, performed in a single reference laboratory, were available for 14 patients with ELANE-mutated SCN or cyclic neutropenia, and were frequently positive (36%). We note that the trigger for genetic studies in severe neutropenia should not be affected by antibody-positivity and should be clinically driven.

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).

[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.