Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome, 1996 to 2011 (CME)

Som, Sumit; Deford, Cassandra C.; Kaiser, Mandi L.; Terrell, Deirdra R.; Hovinga, Johanna A. Kremer; Lämmle, Bernhard; George, James N.; Vesely, Sara K.

doi:10.1111/j.1537-2995.2012.03646.x

Cited by 71 publications

(72 citation statements)

References 20 publications

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“…PEX has become the standard of treatment for TTP and it has reduced the mortality rate from an estimated 90% to less than 10% [19–22]. The addition of glucocorticoids and rituximab in select cases has further improved the survival and reduced duration of PEX [23]. Although randomized controlled trials are lacking, the usual standard approach is continuation of the PEX and glucocorticoids until the platelet count has been normal for at least 2 days.…”

Section: Discussionmentioning

confidence: 99%

Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature

et al. 2016

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Patient: Female, 34Final Diagnosis: Refractory thrombotic thrombocytopenic purpuraSymptoms: FatigueMedication: —Clinical Procedure: Plasma exchangeSpecialty: Rheumatology • Hematology and Critical CareObjective:Rare co-existance of disease or pathologyBackground:Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE. In rare cases, TTP may precede the diagnosis of SLE or occur concurrently.Case Report:We present a case of a previously healthy 34-year-old female who presented with dizziness and flu-like symptoms and was found to have thrombocytopenia, hemolytic anemia, and schistocytes in the peripheral smear. She was subsequently diagnosed with TTP and started on plasmapheresis and high-dose steroids, but without a sustained response. A diagnosis of refractory TTP was made, and she was transferred to our facility for further management. Initially, the patient was started on rituximab, but her condition continued to deteriorate, with worsening thrombocytopenia. Later, she also fulfilled the Systemic Lupus International Collaborating Clinics (SLICC) criteria for diagnosis of SLE. Treatment of TTP in SLE patients is generally similar to that in the general population, but in refractory cases there are few reports in the literature that show the efficacy of cyclophosphamide. We started our patient on cyclophosphamide and noticed a sustained improvement in the platelet count in the following weeks.Conclusions:Thrombotic thrombocytopenic purpura is a life-threatening hematological emergency which must be diagnosed and treated in a timely manner. Refractory cases of TTP have been described in the literature, but without clear evidence-based guidelines for its management, and is solely based on expert opinion and previous case reports. Further studies are needed to establish guidelines for its management. We present this case to highlight the role that cyclophosphamide might carry in those cases and to be a foundation for these future studies.

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Section: Discussionmentioning

confidence: 99%

Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature

et al. 2016

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“…Analyses of the E. coli O104:H4 outbreak provided no evidence of the value of eculizumab and plasma exchange (127)(128)(129). Risks of plasma exchange include complications of catheter insertion, hypocalcemia, and exposure to blood products (130). It is important to remember that patients with HUS often deteriorate after they are initially diagnosed, that the median number of days of anuria (among those whose urine output ceases) is 8 (20), and that gradual spontaneous resolution of the microangiopathy and recovery of renal and neurologic function are the rule and not the exceptions.…”

Section: Use Of Plasmapheresis and Eculizumabmentioning

confidence: 99%

Shiga Toxin/Verocytotoxin-Producing Escherichia coli Infections: Practical Clinical Perspectives

2014

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Escherichia coli strains that produce Shiga toxins/verotoxins are rare, but important, causes of human disease. They are responsible for a spectrum of illnesses that range from the asymptomatic to the life-threatening hemolyticuremic syndrome; diseases caused by E. coli belonging to serotype O157:H7 are exceptionally severe. Each illness has a fairly predictable trajectory, and good clinical practice at one phase can be inappropriate at other phases. Early recognition, rapid and definitive microbiology, and strategic selection of tests increase the likelihood of good outcomes. The best management of these infections consists of avoiding antibiotics, antimotility agents, and narcotics and implementing aggressive intravenous volume expansion, especially in the early phases of illness.

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“…19 Therapeutic plasma exchange can reduce the mortality from 90% to approximately 13%, 19 although complications from plasma exchange are not uncommon. 20 TTP is caused by a severe deficiency (eg, ,5% activity) of the metalloproteinase ADAMTS13, which is responsible for cleaving large multimers of von Willebrand factor. Currently, the assay of this enzyme in specialized laboratories often has a turnaround time of 10 to 14 days.…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

The role of hematologists in a changing United States health care system

Wallace

Connell

Abkowitz

2015

Blood

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Major and ongoing changes in health care financing and delivery in the United States have altered opportunities and incentives for new physicians to specialize in nonmalignant hematology. At the same time, effective clinical tools and strategies continue to rapidly emerge. Consequently, there is an imperative to foster workforce innovation to ensure sustainable professional roles for hematologists, reliable patient access to optimal hematology expertise, and optimal patient outcomes. The American Society of Hematology is building a collection of case studies to guide the creation of institutionally supported systems-based clinical hematologist positions that predominantly focus on nonmalignant hematology. These roles offer a mix of guidance regarding patient management and the appropriate use and stewardship of clinical resources, as well as development of new testing procedures and protocols.

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Decreasing frequency of plasma exchange complications in patients treated for thrombotic thrombocytopenic purpura‐hemolytic uremic syndrome, 1996 to 2011 (CME)

Cited by 71 publications

References 20 publications

Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature

Systemic Lupus Erythematosus Presenting as Refractory Thrombotic Thrombocytopenic Purpura: A Diagnostic and Management Challenge. A Case Report and Concise Review of the Literature

Shiga Toxin/Verocytotoxin-Producing Escherichia coli Infections: Practical Clinical Perspectives

The role of hematologists in a changing United States health care system

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