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Seattle, WA, United States

Gernsheimer T.B.,Puget Sound Blood Center
Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program

Thrombocytopenia is a common finding in pregnancy. Establishing the diagnosis of immune thrombocytopenia (ITP) in a pregnant patient is similar to doing so in a nonpregnant patient, except that the evaluation must specifically rule out other disorders of pregnancy associated with low platelet counts that present different risks to the mother and fetus and may require alternate distinct therapy. Many of the same treatment modalities are used to manage the pregnant patient with ITP, but others have not been determined to be safe for the fetus, are limited to a particular gestational period, or side effects may be more problematic during pregnancy. The therapeutic objective differs from that in chronic ITP in the adult because many pregnant patients recover or improve spontaneously after delivery and therefore maintenance of a safe platelet count, rather than prolonged remission, is the goal. Thrombocytopenia may the limit choices of anesthesia, but does not guide mode of delivery, and the fetus is rarely severely affected at birth. Patients should be advised that a history of ITP or ITP in a previous pregnancy is not a contraindication to future pregnancies and that, with proper management and monitoring, positive outcomes can be expected in the majority of patients. Source

Konkle B.A.,Puget Sound Blood Center | Konkle B.A.,University of Washington

In this issue of Blood, Leizorovicz et al show that progression of superficial venous thrombosis of the lower extremity is associated with a significant risk of doi:10.1182/blood-2013-07-514067 deep vein thrombosis and pulmonary embolism regardless of proximity to the saphenofemoral venous junction. © 2013 by The American Society of Hematology. Source

Reems J.-A.,Puget Sound Blood Center

Allogeneic platelet transfusions protect patients from bleeding episodes and also make aggressive medical procedures such as those involving marrow transplants requiring chemotherapy and/or radiotherapy possible. These patients are dependent upon an unfailing supply of platelets that can sometimes be in short supply due to high demands coupled with an extremely short expiration date for platelet products of only 5 days. One approach that is under investigation to overcome platelet shortages is to harness the extraordinary capabilities of stem cells to proliferate and differentiate into various cell types and to use this ability to specifically produce clinical scale quantities of functional platelets in bioreactors. To accomplish such an enormous and complex task requires an appreciation of the regulatory mechanisms that occur during the development of megakaryocytes (MKs) and the subsequent biogenesis of functional platelets from mature MKs. This means understanding the complex network of intracellular and extracellular regulatory mechanisms that act at each phase of a developmental process that ushers stem cells along the MK lineage to produce billions of platelets per day in a healthy individual. © 2011 American Association of Blood Banks. Source

To evaluate the utility of a centralized transfusion service model in preventing the transfusion of incompatible units in patients with sickle cell disease (SCD). The serologic records of transfused patients with SCD were reviewed. The index hospital was where an alloantibody was initially detected. In total, 150 patients with SCD were evaluated; 66 (44.0%) of 150 were alloimmunized. In 42 (63.6%) of these patients, 1 or more antibodies evanesced. The median number of hospitals visited by patients with SCD for RBC transfusion with 1 or more evanesced antibodies was three (range, one to eight); the median number of nonindex hospitals was two (range, one to seven). Of the patients with evanesced antibodies, 28.6% received transfusions at various nonindex hospitals 20 or more times after the antibody evanesced. A centralized database can help identify patients with SCD who have evanesced alloantibodies and prevent issuing incompatible RBC units. Source

Wong T.,Puget Sound Blood Center | Recht M.,Oregon Health And Science University

Haemophilia A and B are X-linked bleeding disorders due to the inherited deficiency of factor VIII or factor IX, respectively. Of the approximately 1 per 500010000 male births affected by haemophilia, 80 are deficient in factor VIII and 20 are deficient in factor IX. Haemophilia is characterized by spontaneous and provoked joint, muscle, gastrointestinal and CNS bleeding leading to major morbidity and even mortality if left untreated or under-treated. The evolution of haemophilia management has been marked by tragedy and triumph over recent decades. Clotting factors and replacement strategies continue to evolve for patients without inhibitors. For patients with an inhibitor, factor replacement for acute bleeding episodes and immune tolerance, immune modulation and extracorporeal methods for inhibitor reduction are the cornerstone of care. In addition, adjuvant therapies such as desmopressin, antifibrinolytics and topical agents also contribute to improved outcomes for patients with and without inhibitors. The future direction of haemophilia care is promising with new longer-acting clotting factors and genetic therapies, including gene transfer and premature termination codon suppressors. With these current and future treatment modalities, the morbidity and mortality rates in patients with haemophilia certainly will continue to improve. © 2011 Adis Data Information BV. All rights reserved. Source

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