Life-threatening thrombotic thrombocytopenic purpura (TTP) in a patient with sickle cell-hemoglobin C disease

AbstractImmune thrombotic thrombocytopenic purpura (iTTP) is a rare autoimmune disorder characterized by severely reduced activity of the von Willebrand factor (VWF)-cleaving protease ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) due to autoantibodies. This leads to the development of pathogenic multimers of VWF, causing a thrombotic microangiopathy with decreased number of platelets, hemolysis, and life-threatening tissue ischemia of mostly brain, heart, and kidneys. Standard treatment of iTTP involves daily plasma exchange to remove ultra large multimers of VWF, inhibitors, substituting ADAMTS13, and the accompaniment of an immunosuppressive treatment with steroids. Recently, caplacizumab was approved for iTTP. Caplacizumab is a nanobody binding the A1 domain of VWF, blocking its interaction with glycoprotein Ib–IX–V platelet receptor and therefore preventing platelet aggregation. VWF activities may serve as therapeutic drug monitoring of caplacizumab, whereas ADAMTS13 activities may be used for biomarkers to guide caplacizumab treatment modalities and overall treatment duration. Additional immunosuppressive treatment by inhibiting autoantibody formation (e.g., the use of Rituximab, a chimeric monoclonal antibody directed against the B-cell antigen CD20) is a further treatment option. Infections are well-known causes for an acute episode for patients with iTTP. The novel SARS-CoV-2 virus is mainly associated with acute respiratory distress as well as diffuse endothelial inflammation and increased coagulopathy. However, little is known about an infection with SARS-CoV-2 virus triggering iTTP relapses. We herein report the case of an acute iTTP episode accompanying a SARS-CoV-2 infection.

Download Full-text

Thrombotic thrombocytopenic purpura: pathophysiology, treatment, and related nursing care

Critical Care Nurse ◽

10.4037/ccn1995.15.6.44 ◽

1995 ◽

Vol 15 (6) ◽

pp. 44-52

Author(s):

M Kajs-Wyllie

Keyword(s):

Critical Care ◽

Thrombotic Thrombocytopenic Purpura ◽

Nursing Care ◽

Critical Care Unit ◽

Family Members ◽

Vital Role ◽

Critical Care Nurses ◽

Thrombocytopenic Purpura ◽

Rare Syndrome ◽

Life Threatening

The patient diagnosed with TTP presents to the critical care unit with myriad life-threatening problems. Knowledge of the pathophysiology and treatment of this rare syndrome is essential to plan care appropriately. However, despite immediate diagnosis and intervention, the outcome may not be successful. Critical care nurses play a vital role in caring for these patients, as well as helping family members deal with this devastating disease.

Download Full-text

How I treat thrombotic thrombocytopenic purpura in pregnancy

Blood ◽

10.1182/blood.2019000962 ◽

2020 ◽

Vol 136 (19) ◽

pp. 2125-2132

Author(s):

Barbara Ferrari ◽

Flora Peyvandi

Keyword(s):

Differential Diagnosis ◽

Thrombotic Thrombocytopenic Purpura ◽

Current Knowledge ◽

Fetal Outcome ◽

Subsequent Pregnancy ◽

Thrombocytopenic Purpura ◽

Severe Deficiency ◽

Life Threatening ◽

High Index Of Suspicion ◽

In Pregnancy

Abstract Thrombotic thrombocytopenic purpura (TTP) is an acute, life-threatening thrombotic microangiopathy (TMA) caused by acquired or congenital severe deficiency of ADAMTS13. Pregnancy is a recognized risk factor for precipitating acute (first or recurrent) episodes of TTP. Differential diagnosis with other TMAs is particularly difficult when the first TTP event occurs during pregnancy; a high index of suspicion and prompt recognition of TTP are essential for achieving a good maternal and fetal outcome. An accurate distinction between congenital and acquired cases of pregnancy-related TTP is mandatory for safe subsequent pregnancy planning. In this article, we summarize the current knowledge on pregnancy-associated TTP and describe how we manage TTP during pregnancy in our clinical practice.

Download Full-text

Rituximab Twice Weekly for Refractory Thrombotic Thrombocytopenic Purpura in a Critically Ill Patient with Acute Respiratory Distress Syndrome

Case Reports in Oncology ◽

10.1159/000505236 ◽

2020 ◽

Vol 13 (1) ◽

pp. 153-157

Author(s):

Bahjat Azrieh ◽

Arwa Alsaud ◽

Khaldun Obeidat ◽

Amr Ashour ◽

Seham Elebbi ◽

...

Keyword(s):

Acute Respiratory Distress Syndrome ◽

Respiratory Distress Syndrome ◽

Respiratory Distress ◽

Thrombotic Thrombocytopenic Purpura ◽

Distress Syndrome ◽

Standard Of Care ◽

Critically Ill Patient ◽

Microangiopathic Hemolytic Anemia ◽

Thrombocytopenic Purpura ◽

Life Threatening

Thrombotic thrombocytopenic purpura (TTP) is a rare, serious, life-threatening disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, and hypercoagulability. The etiology is a deficiency of ADAMTS13 which is usually caused by acquired antibodies. Plasma exchange and steroids is the standard of care in the treatment of TTP. However, there are refractory cases of TTP which require further management. Rituximab appears to be a safe and effective therapy for refractory and relapsing TTP. Here we report a challenging case of TTP that responded to treatment with rituximab twice weekly. According to our knowledge, rituximab twice weekly has never been used for TTP before.

Download Full-text

Thrombotic Thrombocytopenic Purpura Following Honeybee Envenomation: A Case Report

International Journal of Medical Toxicology and Forensic Medicine ◽

10.32598/ijmtfm.v10i2.28794 ◽

2020 ◽

Vol 10 (2) ◽

Author(s):

Zahra Khalighi ◽

Golnaz Azami ◽

Elham Shafiei ◽

Ali Sahebi ◽

Aliashraf Mozafari

Keyword(s):

Thrombotic Thrombocytopenic Purpura ◽

Case Reports ◽

Major Complication ◽

The Body ◽

Severe Thrombocytopenia ◽

Thrombocytopenic Purpura ◽

Toxic Reaction ◽

Bee Sting ◽

Life Threatening ◽

Underlying Diseases

Background: Thrombotic Thrombocytopenic Purpura (TTP) is a rare and life-threatening disorder characterized by severe thrombocytopenia, microangiopathic hemolytic anemia, fever, renal dysfunction, and neurological deficient. TTP leads to the formation of blood clots in small blood vessels throughout the body. TTP is associated with many risk factors such as pregnancy, HIV, cancer, lupus, and infections. Recently there have been few published case reports of bee sting associated TTP.Methods: A 67-year-old man from a rural area of the Southwest Province of Iran, Ilam, was referred to the academic general hospital because of fever, chills, sweating, vomiting and dizziness following the honeybee sting on his body. Results: this study showed that,multiple co-morbidities including CVD and diabetes, along with coagulation abnormalities after honeybee stings, seriously exacerbated patient hemodynamic status.Conclusion: TTP, as a major complication due to the toxic reaction of a large number of bee stings with underlying diseases in patients, should be given more attention.

Download Full-text

Thrombotic Thrombocytopenic Purpura and Sickle Cell Crisis

Clinical and Applied Thrombosis/Hemostasis ◽

10.1177/1076029608323804 ◽

2008 ◽

Vol 16 (2) ◽

pp. 224-227 ◽

Cited By ~ 15

Author(s):

Suresh G. Shelat

Keyword(s):

Red Blood Cells ◽

Thrombotic Thrombocytopenic Purpura ◽

Sickle Cell ◽

Blood Cells ◽

Signs And Symptoms ◽

Activity Level ◽

Acute Chest Syndrome ◽

Thrombocytopenic Purpura ◽

Nucleated Red Blood Cells ◽

Elevated Lactate

Described is a case of acute chest syndrome in a sickle-cell patient (hemoglobin SS) who also developed signs and symptoms of thrombotic thrombocytopenic purpura, including thrombocytopenia and hemolysis (anemia, elevated lactate dehydrogenase, presence of schistocytes, dark-colored plasma, and elevations in nucleated red blood cells). The ADAMTS13 activity level was normal. Discussed are the diagnosis and therapeutic management issues and the challenges of differentiating the vasoocclusive and hemolytic complications of sickling red blood cells from the thrombotic microangiopathy of thrombotic thrombocytopenic purpura.

Download Full-text

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.

Download Full-text