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8 results

  • Chapter 53 - Thrombotic Thrombocytopenic Purpura in Pregnancy

  • from Section 9 - Hematologic Conditions in Pregnancy
  • Edited by Amira El-Messidi, McGill University, Montréal, Alan D. Cameron, University of Glasgow
  • Book:
    OSCEs in Obstetrics and Maternal-Fetal Medicine
    Published online:
    23 February 2023
    Print publication:
    16 February 2023, pp 680-689

  • Summary

    During your call duty, a healthy 32-year-old primigravida at 22+3 weeks’ gestation, confirmed by first-trimester sonography, presents to the obstetrics emergency assessment unit of your hospital center with new-onset, asymptomatic port-wine-colored urine with chills and an oral temperature of 39.1°C at home; she also notes a two-day history of headache, now accompanied by visual changes. Your obstetric trainee informs you that clinical history is not suggestive of an infectious etiology, although comprehensive investigations are pending. She has no obstetric complaints, and fetal viability was ascertained upon presentation. Routine prenatal laboratory investigations, aneuploidy screening, and fetal morphology survey were unremarkable. The laboratory urgently notifies you that the platelet concentration is 12 × 109/L, confirmed on manual count; other requested laboratory tests are in progress.

  • 3.10.4 - General Haematology for Intensive Care

  • from Section 3.10 - Haematological and Oncological Disorders
  • Edited by Ned Gilbert-Kawai, The Royal Liverpool Hospital, Debashish Dutta, Princess Alexandra Hospital NHS Trust, Harlow, Carl Waldmann, Royal Berkshire Hospital, Reading
  • Book:
    Intensive Care Medicine
    Published online:
    27 July 2023
    Print publication:
    01 December 2021, pp 336-342

  • Summary

    Key Learning Points

    1. 1. Anaemia in critical illness is often multifactorial. Careful assessment of history and trend of haemoglobin and full blood count parameters can help direct further investigations.

    2. 2. Restrictive red cell transfusion strategies are associated with lower mortality in the intensive care unit. The default transfusion threshold is 70 g/l unless there are specific conditions, e.g. cardiac disease.

    3. 3. Thrombocytopenia in critical illness is common and often reflects abnormal physiology; therefore, the mainstay of management is to treat the underlying cause.

    4. 4. Patients with sickle cell disease (SCD) have highly variable clinical presentations due to different sickle genotypes and clinical heterogeneity within each genotype. Therefore, the severity of previous sickle cell crises is the best indicator of severity of their condition. Management should be led by haematologists with experience in SCD.

    5. 5. Therapeutic plasma exchange removes harmful large molecules from the circulation. Protocols vary according to indication; therefore, management should be directed by the clinical team with expertise in the underlying condition.

  • Gamunex® in Guillain-Barré Syndrome: A Postmarketing, Retrospective, Observational Study

  • Zaeem A. Siddiqi, Kecia Courtney, Kim Hanna, Elsa Mondou, Vera Bril
  • Journal:
    Canadian Journal of Neurological Sciences / Volume 44 / Issue 6 / November 2017
    Published online by Cambridge University Press:
    24 July 2017, pp. 711-717
    • Article
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  • Background: The objective of this retrospective study was to evaluate the effectiveness and safety of Gamunex® (immune globulin [human] 10%; hereinafter “Gamunex”) when administered intravenously in the initial treatment of Guillain-Barré syndrome (GBS). The study was conducted as a postapproval commitment for Health Canada. Methods: A medical chart review for hospitalized patients diagnosed with GBS and treated with Gamunex (Gamunex 10% and IGIVnex® 10%; N=109; n=69 evaluable) was conducted at seven Canadian study centers in reverse chronological order. The primary endpoint for assessing effectiveness was the proportion of patients with treatment success compared with a prospectively defined historical effectiveness threshold for plasma exchange (PE) treatment (55.05%). Treatment success was assessed as ≥1 point improvement from baseline on the GBS Disability Scale or abbreviated GBS Disability Scale. Cases were not evaluable if treatment success, relapse, or treatment failure could not be determined by the available chart data. Results: Applying a conservative estimate with all nonevaluable patients (n= 40) classified as treatment failures, Gamunex treatment success was estimated at 57.8% (63 of 109 patients), which exceeded the predefined historical PE effectiveness threshold. In the evaluable population of this study, Gamunex treatment was successful in 91.3% of patients (63/69). Some 23 (21.1%) of 109 Gamunex-treated patients experienced ≥1 adverse event; the profile and frequency were consistent with the adverse events reported for Gamunex in the product’s labeling and with the natural clinical course of GBS. Conclusions: The effectiveness of Gamunex for treatment of GBS was comparable to PE therapy. Gamunex was observed to have an acceptable safety profile in this study population.

  • Chapter 20 - Nutrition

  • from Section 3 - Special critical care tools and techniques
  • Edited by Marc van de Velde, Helen Scholefield, Lauren A. Plante
  • Book:
    Maternal Critical Care
    Published online:
    05 July 2013
    Print publication:
    04 July 2013, pp 203-216

  • Summary

    This chapter focuses on three entities namely disseminated intravascular coagulation (DIC), HELLP syndrome, and thrombotic thrombocytopenic purpura (TTP), which represents unique and critical threats to the well-being of mother and fetus during peripartum period. It is concerned with the etiology, clinical features, diagnostic methods and management of these entities. In non-bleeding patients with DIC, platelets and factor replacement should not be administered prophylactically or based on laboratory tests alone. The treatment of HELLP involves monitoring and responding to maternal signs and symptoms, particularly when pre-eclampsia is present, and includes fluid management and the use of antihypertensive agents and magnesium sulfate for seizure prophylaxis. Plasma exchange is the treatment of choice for TTP. The optimal treatment regimen for obstetric coagulation disorders continues to evolve, given the frequently dynamic clinical situation, the presence and health of the fetus, and a growing interest in conducting investigations during the peripartum period.

  • 17 - Clinical and biological features of acute disseminated encephalomyelitis

  • from Section 4 - ADEM
  • Edited by Dorothée Chabas, University of California, San Francisco, Emmanuelle L. Waubant, University of California, San Francisco
  • Book:
    Demyelinating Disorders of the Central Nervous System in Childhood
    Published online:
    11 April 2011
    Print publication:
    17 March 2011, pp 183-201

  • Summary

    Plasma exchange involves non-selectively removing plasma components that are potentially pathogenic in immune-mediated diseases from a patient's blood. Intravenous immunoglobulin used for therapeutic purposes are pooled human immunoglobulins derived from the plasma of 10000-100000 healthy donors. The major component of intravenous immunoglobulins (IVIg) preparations is IgG; very small amounts of other immunoglobulins, immunoglobulin fragments, and other smaller plasma proteins are also present. IVIg affect several components of the immune system and have been used successfully in the treatment of several neuroimmunological disorders. IVIg therapy has been evaluated both as a treatment of acute demyelinating relapses as well as a maintenance therapy to alter the disease course in adult patients with multiple sclerosis (MS). This chapter focuses on studies pertaining to the treatment of acute relapses. Plasma exchange might be an effective and relatively well-tolerated treatment option for severe demyelinating relapses in MS patients.

  • 51 - KOHLMEIER-DEGOS’ DISEASE (MALIGNANT ATROPHIC PAPULOSIS)

  • from PART V: - SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM
  • Edited by Louis R. Caplan, Julien Bogousslavsky
  • Book:
    Uncommon Causes of Stroke
    Published online:
    06 January 2010
    Print publication:
    09 October 2008, pp 377-380

  • Summary

    The underlying pathological mechanism of thrombotic thrombocytopenic purpura (TTP) is the presence of microvascular thrombi that partially occlude the vascular lumins with overlying proliferative endothelial cells. Coombs-negative hemolytic anemia and severe thrombocytopenia owing to platelet clumping in the microcirculation are the most outstanding laboratory abnormalities. Classically, TTP has been recognized by the pentad of microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, fever, and renal involvement, though only 20 to 40 percentage of patients will manifest the classic pentad. TTP remains a life-threatening disease the mortality rate of which may be as high as 90 percentage when untreated. Diagnosis is mainly based on hematological findings and a broad variety of neurological abnormalities, including ischemic or, less often, hemorrhagic stroke. Plasma exchange (PE) is currently the mainstay of treatment; however, rapid advances in the understanding of TTP pathophysiology may offer more specific and effective therapies in the near future.

  • 62 - MOYA-MOYA SYNDROME

  • from PART VI: - NONINFLAMMATORY DISORDERS OF THE ARTERIAL WALL
  • Edited by Louis R. Caplan, Julien Bogousslavsky
  • Book:
    Uncommon Causes of Stroke
    Published online:
    06 January 2010
    Print publication:
    09 October 2008, pp 465-478

  • Summary

    Kohlmeier-Degos' disease (KDD) is more common in white boys and men with age of onset ranging between 3 weeks of age to 67 years old, most commonly occurring in the second and third decades. The etiology and pathogenesis, although still unknown, is speculated to be immunologic dysfunction, coagulation or fibrinolysis abnormalities, or possible viral infections. Any organ system may be involved; the clinical manifestations are a result of multifocal infarctions. Laparoscopic examination, however, shows lesions similar in appearance to the skin lesions that may involve multiple areas of the small intestine. These lesions are secondary to infarction and have also been found in the esophagus, duodenum, stomach, colon, and rectum. Ischemia and infarction are key pathophysiological mechanisms of the lesions seen. Therapies that have been used include antiplatelets, anticoagulants, immunosuppressive agents, plasma exchange, and nicotine patches. Surgery is indicated in cases of intestinal perforation.