American journal of hematology
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Systemic bleeding at the time of postpartum hemorrhage (PPH) is usually the result of coagulopathy that has developed acutely as a result of massive hemorrhage after uterotonics and sutures have failed. Occasionally, the patient has a preexisting coagulopathy, but more often, coagulopathy arises acutely as the result of massive hemorrhage, which is usually related to obstetrical and less often surgical bleeding. Despite being able to identify risk factors for PPH in the antenatal and intrapartum period, the majority of women who ultimately develop PPH do not have any such factors and every pregnancy is at risk. ⋯ Recombinant activated factor VIIa (rFVIIa) has been used in the management of severe PPH unresponsive to blood component therapy. Coagulation laboratory evaluation may be useful in guiding hemostatic management during massive PPH, but for the results to be useful, they must be rapidly available and provide information that would not be available from clinical assessment alone. The hematologist or hemostasis expert has the opportunity to make the difference between life and death for the patient experiencing massive PPH.
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Review
Blood component support in acquired coagulopathic conditions: is there a method to the madness?
Acquired coagulopathies are often detected by laboratory investigation in clinical practice. There is a poor correlation between mild to moderate abnormalities of laboratory test and bleeding tendency. ⋯ However, prophylactic transfusion of these products in a nonbleeding patient to correct mild to moderate abnormality of a coagulation test especially preprocedure is not evidence-based. This article reviews the management of bleeding due to oral anticoagulants and antiplatelet agents, disseminated intravascular coagulation, chronic liver disease, and trauma.
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Review
Corticosteroids and rituximab as adjunctive treatments for thrombotic thrombocytopenic purpura.
Although treatment with plasma exchange increased the survival of patients with thrombotic thrombocytopenia purpura to 80% in the 1980s, no further increase of survival occurred over the next 20 years. However, more consistent use of adjuvant treatment with corticosteroids and rituximab in recent years has begun to further increase survival as well as decrease the frequency of relapse. ⋯ Fewer days of plasma exchange have resulted in fewer complications, such as central venous catheter-related systemic infections. Future potential options for adjuvant treatment, recombinant ADAMTS13 to correct severe ADAMTS13 deficiency, and agents to block von Willebrand factor-mediated platelet thrombosis are being investigated.
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T-cell lymphomas make up approximately 10-15% of lymphoid malignancies. The frequency of these lymphomas varies geographically, with the highest incidence in parts of Asia. ⋯ Anaplastic large-cell lymphoma is the only subgroup to have a good response to a cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP)-like regimen. Angioimmunoblastic T-cell lymphoma has a prolonged disease-free survival in only ∼20% of patients, but younger patients who have an autotransplant in remission seem to do better. PTCL-not otherwise specified (NOS) is not one disease. Anthracycline containing regimens have disappointing results and a new approach is needed. NK/T-cell lymphoma localized to the nose and nasal sinuses seems to be best treated with radiotherapy containing regimens. Enteropathy associated PTCL and hepatosplenic PTCL are rare disorders with a generally poor response to therapy, although selected patients with enteropathy associated PTCL seem to benefit from intensive therapy.
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Evaluating a child with symptoms of easy bruising and/or bleeding remains a challenge in pediatric hematology, and there is no "one size fits all" approach. This review focuses on recent research in three elements of the evaluation of a child with a suspected bleeding disorder. We will first discuss the development of the standardized Pediatric Bleeding Questionnaire, and its applications in research and clinical settings. ⋯ Platelet function disorders are among the most common inherited bleeding disorders. However, testing for such disorders is time-consuming and requires a step-wise approach. We will review the indications for and limitations of the most commonly utilized platelet function laboratory studies.