Life-threatening postpartum hemolysis, elevated liver functions tests, low platelets syndrome versus thrombocytopenic purpura - Therapeutic plasma exchange is the answer

Background: Thrombotic thrombocytopenic purpura (TTP) is an uncommon haematological disease which can occur at any age and may present with COVID-19. This case describes a COVID-19 complication associated with a presentation resembling TTP. Case description: A 51-year-old man who had received a kidney transplant and was on immunosuppressant medication, was admitted to a critical care unit with severe COVID-19 pneumonia/acute respiratory distress syndrome (ARDS) which required intubation, mechanical ventilation and inotropic support. The course was complicated by the classic pentad of thrombocytopenia, intravascular haemolysis, acute kidney injury, neurological symptoms and fever, which prompted the diagnosis of probable TTP. After five sessions of therapeutic plasma exchange, the patient’s general status improved, he was weaned off mechanical ventilation and his renal panel and haemolytic markers normalized. Conclusion: TTP is a life-threatening condition which requires urgent management with therapeutic plasma exchange. This case highlights some possible complications of COVID-19 generally and in immunocompromised patients specifically. The potential role of plasma exchange in COVID-19 patients without a positive diagnosis of TTP (the so-called ‘TTP resembling presentation’) is an area of further research.

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Treatment of thrombotic thrombocytopenic purpura beyond therapeutic plasma exchange

Hematology ◽

10.1182/asheducation-2015.1.637 ◽

2015 ◽

Vol 2015 (1) ◽

pp. 637-643 ◽

Cited By ~ 25

Author(s):

Paul Coppo ◽

Antoine Froissart

Keyword(s):

Plasma Exchange ◽

Thrombotic Thrombocytopenic Purpura ◽

Survival Rates ◽

Therapeutic Plasma Exchange ◽

Response To Treatment ◽

Thrombocytopenic Purpura ◽

Therapeutic Landscape ◽

Life Threatening ◽

Adamts13 Deficiency ◽

Von Willebrand

Abstract Daily therapeutic plasma exchange (TPE) transformed the historically fatal prognosis of acquired, anti-ADAMTS13 antibody-mediated thrombotic thrombocytopenic purpura (TTP), leading to the current overall survival rates of 80%-85%. However, relapses occur in ∼40% of patients and refractory disease with fatal outcomes still occurs. In this context, the introduction of rituximab has probably been the second major breakthrough in TTP management. Rituximab is now routinely recommended during the acute phase, typically in patients with a suboptimal response to treatment, or even as frontline therapy, with high response rates. In more severe patients, salvage strategies may include twice-daily TPE, pulses of cyclophosphamide, vincristine, as well as splenectomy in more desperate cases. In this life-threatening disease, relapse prevention represents a major goal. Persistent severe acquired ADAMTS13 deficiency in patients who are otherwise in remission is associated with a high risk of relapse and preemptive treatment with rituximab may be considered in this context. In the coming years, the TTP therapeutic landscape should be enriched by original strategies stemming from clinical experience and new agents that are currently being evaluated in large, ideally international, clinical trials. Promising agents under evaluation include N-acetylcysteine, bortezomib, recombinant ADAMTS13, and inhibitors of the glycoprotein-Ib/IX-von Willebrand factor axis.

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A Case of Thrombotic Thrombocytopenic Purpura with Exacerbation after Incomplete Remission of Therapeutic Plasma Exchange

The Korean Journal of Blood Transfusion ◽

10.17945/kjbt.2017.28.3.298 ◽

2017 ◽

Vol 28 (3) ◽

pp. 298-303

Author(s):

Yong-Hak Sohn ◽

Chunhwa Ihm ◽

Eun-Hye Choi ◽

Sang Kwang Lee

Keyword(s):

Plasma Exchange ◽

Thrombotic Thrombocytopenic Purpura ◽

Therapeutic Plasma Exchange ◽

Thrombocytopenic Purpura

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Therapeutic plasma exchange in adult critically ill patients with life-threatening SARS-CoV-2 disease: A pilot study

Journal of Critical Care ◽

10.1016/j.jcrc.2020.07.001 ◽

2020 ◽

Vol 60 ◽

pp. 328-333 ◽

Cited By ~ 7

Author(s):

Fahad Faqihi ◽

Abdulrahman Alharthy ◽

Mohammed Alodat ◽

Demetrios J. Kutsogiannis ◽

Peter G. Brindley ◽

...

Keyword(s):

Pilot Study ◽

Plasma Exchange ◽

Critically Ill ◽

Critically Ill Patients ◽

Therapeutic Plasma Exchange ◽

Life Threatening

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Life-threatening COVID-19 presenting as stroke with antiphospholipid antibodies and low ADAMTS-13 activity, and the role of therapeutic plasma exchange: A case series

SAGE Open Medical Case Reports ◽

10.1177/2050313x20964089 ◽

2020 ◽

Vol 8 ◽

pp. 2050313X2096408

Author(s):

Abdulrahman Alharthy ◽

Fahad Faqihi ◽

Abdullah Balhamar ◽

Ziad A Memish ◽

Dimitrios Karakitsos

Keyword(s):

Intensive Care Unit ◽

Intensive Care ◽

Plasma Exchange ◽

Plasma Volume ◽

Antiphospholipid Antibodies ◽

Rescue Therapy ◽

Antiviral Treatment ◽

Case Series ◽

Therapeutic Plasma Exchange ◽

Life Threatening

We present a case series of three patients with COVID-19 who were admitted to our intensive care unit due to acute respiratory distress syndrome, brain infarction, pulmonary embolism, and antiphospholipid antibodies. We applied therapeutic plasma exchange on all cases. On intensive care unit admission, all patients had low (<10) Glasgow Coma Scale, and central nervous imaging showed multiple brain infarctions. COVID-19 was confirmed by reverse transcriptase polymerase chain reaction assays. Patients underwent rescue therapeutic plasma exchange using the Spectra OptiaTM Apheresis System (Terumo BCT Inc., USA), which operates with acid-citrate dextrose anticoagulant as per Kidney Disease Improving Global Outcomes 2019 guidelines. A dose of 1.5 plasma volume was used for the first dose and then 1 plasma volume daily for a total of five doses. Plasma was replaced with Octaplas LG® (Octapharma AG, USA), which is an artificial fresh frozen plasma product that has undergone viral inactivation by prion reduction technology. We administered ARDS-net/prone positioning ventilation, empiric antiviral treatment, therapeutic anticoagulation, and intensive care unit supportive care. Laboratory tests showed lymphocytopenia; elevated levels of D-dimer, fibrinogen, total bilirubin, C-reactive protein, lactate dehydrogenase, and ferritin; as well as low levels of ADAMTS-13 activity and antibody. Serology tests depicted positive IgM and IgG antiphospholipid antibodies (anti-cardiolipin and anti-β2-glycoprotein I antibodies). No side effects of therapeutic plasma exchange were recorded. After the completion of therapeutic plasma exchange, patients improved clinically and gradually recovered neurologically (after 27–32 days). To conclude, in life-threatening COVID-19, especially when immune dysregulation features such as antiphospholipid antibodies exist, therapeutic plasma exchange could be an effective rescue therapy.

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Pembrolizumab-induced thrombotic thrombocytopenic purpura

Journal of Oncology Pharmacy Practice ◽

10.1177/1078155219887212 ◽

2019 ◽

Vol 26 (5) ◽

pp. 1237-1240 ◽

Cited By ~ 4

Author(s):

Marcus SR Dickey ◽

Anant J Raina ◽

Peter J Gilbar ◽

Brendan L Wisniowski ◽

Joel T Collins ◽

...

Keyword(s):

Adverse Events ◽

Plasma Exchange ◽

Thrombotic Thrombocytopenic Purpura ◽

Immune Checkpoint ◽

Checkpoint Inhibitors ◽

Oral Prednisolone ◽

Autoimmune Disorder ◽

Community Acquired Pneumonia ◽

Thrombocytopenic Purpura ◽

Life Threatening

Introduction Pembrolizumab is a humanised monoclonal antibody targeting the receptor programmed cell death protein-1 (PD-1), with anti-tumour activity demonstrated for many malignancies. Such immune checkpoint inhibitors are associated with many immune-related adverse events including rash, colitis, hepatitis, pneumonitis, endocrinopathy and, rarely, haematological adverse events, including immune-related thrombocytopenia. Case report We report a 60-year-old female with metastatic non-small cell lung cancer treated with pembrolizumab every three weeks. Following her fifth cycle, she presented to our hospital with community-acquired pneumonia. Thrombocytopenia developed the next day and, after detailed investigations, thrombotic thrombocytopenic purpura was diagnosed. Management and outcome Pembrolizumab was immediately ceased and plasma exchange commenced along with IV methylprednisolone 250 mg daily for three days followed by oral prednisolone. After five days of plasma exchange, platelet counts normalised and haemolytic anaemia resolved. Discussion Acquired thrombotic thrombocytopenic purpura is an autoimmune disorder caused by an inhibitory autoantibody against ADAMTS-13. While most cases of acquired thrombotic thrombocytopenic purpura are idiopathic, certain conditions (e.g. bacterial infection, autoimmune disorders, malignancies) and medications are associated with thrombotic thrombocytopenic purpura. Other potential causes were eliminated in our patient. As acquired thrombotic thrombocytopenic purpura is an autoimmune disorder, pembrolizumab, given its unique mechanism of action and association with immune-related adverse events, is believed to be implicated in the development of thrombotic thrombocytopenic purpura. This case is one of only two linking anti-PD-1 therapy to thrombotic thrombocytopenic purpura development (the other occurring in a patient on nivolumab plus ipilimumab). Thrombotic thrombocytopenic purpura is life-threatening and clinicians are advised to be aware of its possible occurrence in immune checkpoint inhibitor-treated patients.

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