Immunosupressive therapy in a patient with postpartum acquired hemophilia a: Case presentation

Edinsel faktör inhibitörleri çoğunlukla kan pıh- tılaşma proteinlerini direk olarak engelleyen antikorlardır. Bu antikorlar; hemofili A’daki an- tikor gelişimi, post partum dönem, çeşitli im- münolojik hastalıklarla ilişkili olan durumlar, çeşitli kanserlerle ilişkili olan durumlar ve yaşlı hastalar gibi birçok klinik durumda görülür. Önemli oranda mortalite ve morbiditeye neden olan edinsel hemofili A (AHA); öz geçmiş ve soy geçmişinde kanama hastalığı olmayan, kanama ve uzamış aPTT’si olan hastalarda ayırıcı tanıda düşünülmelidir. Edinsel hemofilinin tedavi stra- tejisi hakkında henüz bir görüş birliği yoktur. Biz burada; immünsupressif tedavi ile başarılı bir şekilde tedavi edilen post partum edinsel (AHA)’lı bir vaka bildirdik.Acquired factor inhibitors are antibodies that inhibit directly blood clotting proteins. These are seen in many clinical situations such as inhi- bitors in hemophilia A, postpartum, in older pa- tients, in association with various immunologic disorders and various malignancies. Acquired hemophilia A which can cause a considerable proportion of mortality and morbidity, should be taken in consideration as a differentional di- agnosis in patients who have prolonged aPTT and bleeding without a personal or family his- tory of bleeding disorder. There is no consensus about the treatment strategy of acquired he- mophilia A yet. We reported a case of postpar- tum acquired hemophilia A who was treated with immunosupressive therapy

The Association Between Gene Polymorphisms and Leukocytosis with Thrombotic Complications in Patients with Essential Thrombocythemia and Polycythemia Vera

OBJECTIVE: Vascular events are a common complication in patients with polycythemia vera (PV) and essential thrombocythemia (ET). This study aimed to analyze the association between PAI-1 4G/5G and ACE I/D gene polymorphisms, and leukocytosis with thrombosis in patients with PV and ET. METHODS: In total, 64 patients with ET and PV were evaluated. Arterial or venous thrombosis, such as cerebral transient ischemic attack, ischemic stroke, myocardial infarction, peripheral arterial thrombosis, deep venous thrombosis, and pulmonary embolism, were defined as a vascular event. DNA samples were screened for mutations via reverse hybridization strip assay. RESULTS: In terms of PAI-1 gene polymorphism, the frequency of the 4G and 5G allele was 48.5% and 51.5%, respectively. The ACE allele frequency was 51.2% and 48.8% for D and I, respectively. There wasn't an association between occurrence of vascular events and the frequency of any allele. In terms of occurrence of vascular events, there weren't any significance differences between the patients that were carrying the ACE D/D homozygous allele to ACE I/D and those that carried the I/I allele (P = 0.93). There wasn't a significant difference in occurrence of vascular events between the PAI-1 5G/5G homozygote allele carriers, and the 4G/5G and 4G/4G allele carriers (P = 0.97). Vascular events were significantly more common in the patients with leukocytosis (leukocyte count >10 × 109 L–1) than in those without leukocytosis (leukocyte count ≤10 × 109 L–1) (P = 0.00). Age >60 years was also a significant risk factor for occurrence of vascular events(P = 0.008). CONCLUSION: PAI-1 and ACE gene polymorphisms were not considered new risk factors for thrombosis in PV and ET patients. On the other hand, leukocytosis at diagnosis was associated with the occurrence of vascular events in the patients with ET and PV

The Role of Azacitidine in the Treatment of Elderly Patients with Acute Myeloid Leukemia: Results of a Retrospective Multicenter Study

Objective: In this study, we aimed to investigate the efficacy and safety of azacitidine (AZA) in elderly patients with acute myeloid leukemia (AML), including patients with >30% bone marrow (BM) blasts. Materials and Methods: In this retrospective multicenter study, 130 patients of &#8805;60 years old who were ineligible for intensive chemotherapy or had progressed despite conventional treatment were included. Results: The median age was 73 years and 61.5% of patients had >30% BM blasts. Patients received AZA for a median of four cycles (range: 1-21). Initial overall response [including complete remission (CR)/CR with incomplete recovery/partial remission] was 36.2%. Hematologic improvement (HI) of any kind was documented in 37.7% of all patients. HI was also documented in 27.1% of patients who were unresponsive to treatment.nMedian overall survival (OS) was 18 months for responders and 12 months for nonresponders (p=0.005). In the unresponsive patient group, any HI improved OS compared to patients without any HI (median OS was 14 months versus 10 months, p=0.068). Eastern Cooperative Oncology Group performance status of <2, increasing number of AZA cycles (&#8805;5 courses), and any HI predicted better OS. Age, AML type, and BM blast percentage had no impact. Conclusion: We conclude that AZA is effective and well tolerated in elderly comorbid AML patients, irrespective of BM blast count, and HI should be considered a sufficient response to continue treatment with AZA