Abstract:Extreme thrombocytosis induces an acquired thrombotic-hemorrhagic diathesis, and left uncontrolled is a harbinger of potentially fatal vascular complications. Currently, cytoreduction with medical therapy remains the mainstay of hyperthrombocytosis management. However, it offers a less-than-ideal option in situations where a rapid reduction in platelets is urgently needed, as in the presence of vital end-organ ischemia or to ameliorate of life-threatening hemorrhage. The role of thrombocytapheresis, or platele… Show more
“…There is lack of evidence to predict the kinetics of platelet rise in these cases. For this reason, even though he did not present any symptoms or thrombotic event associated with thrombocytosis, and was receiving hydroxyurea and anagrelide, it was decided to perform therapeutic thrombocytapheresis, because the effect of this double pharmacologic cytoreduction is not immediate . In addition, this indication is in line with the recommendation of the Guidelines of the American Society for Apheresis where prophylactic thrombocytapheresis in recommended in thrombocytosis cases although a category III, that is, decision making should be individualized.…”
Section: Discussionmentioning
confidence: 97%
“…The efficiency platelet reduction with Spectra Optia observed in our case was significantly higher than previously reported. There are a variety of studies comparing this equipment related to platelet donation in healthy individuals, however for therapeutic use is not frequent and the only equipment approved in the United States is COBE Spectra . Spectra Optia has not been yet FDA cleared for therapeutic thrombocytapheresis.…”
Section: Discussionmentioning
confidence: 99%
“…There are a variety of studies comparing this equipment related to platelet donation in healthy individuals, however for therapeutic use is not frequent and the only equipment approved in the United States is COBE Spectra. 7 Spectra Optia has not been yet FDA cleared for therapeutic thrombocytapheresis. Boddu et al presented a case-based review of four patients treated with therapeutic thrombocytapheresis in which platelet count was reduced 30%-60% after processing 1.5-2 total blood volumes; unfortunately, no information is provided regarding the apheresis separators used.…”
Essential thrombocytosis (ET) is a chronic myeloproliferative neoplasm characterized by the presence of thrombocytosis and it can be complicated by thrombotic and/or hemorrhagic events. Treatment options include low‐dose aspirin and cytoreductive agents such as hydroxyurea. In cases of extreme thrombocytosis, therapeutic thrombocytapheresis can be a useful procedure. We present a case of a 61‐year‐old‐man previously diagnosed with CALR‐mutated ET, who develop acute myeloid leukemia. When recovering after induction chemotherapy, he developed an extreme thrombocytosis up to 2337 × 109/L regardless hydroxyurea was started. Two therapeutic trombocytapheresis were performed and anagrelide was added to cytoreductive regimen. Platelet count stabilized around 570 × 109/L. Both procedures were performed with the Spectra Optia Apheresis System version 11.3 (Terumo BCT) and we decided to use a higher collection preference and lower collection speed than manufacturer's recommendations. Platelet count decreased from 2380 × 109/L to 1035 × 109/L in the first procedure and from 1813 × 109/L to 768 × 109 in the second procedure. Platelet collection efficiency was calculated to be 110.3% and 86.1% in the first and second thrombocytapheresis, respectively. Therapeutic thrombocytapheresis with Spectra Optia is a safe and efficient therapy to treat patients with primary thrombocytosis while effect of cytoreductive agents is attained. Platelet collection efficiency was calculated to be higher than previously reported. We suggest that changes in technical parameters such as a deeper aspiration point and/or lower collection speed may increase procedure's efficiency.
“…There is lack of evidence to predict the kinetics of platelet rise in these cases. For this reason, even though he did not present any symptoms or thrombotic event associated with thrombocytosis, and was receiving hydroxyurea and anagrelide, it was decided to perform therapeutic thrombocytapheresis, because the effect of this double pharmacologic cytoreduction is not immediate . In addition, this indication is in line with the recommendation of the Guidelines of the American Society for Apheresis where prophylactic thrombocytapheresis in recommended in thrombocytosis cases although a category III, that is, decision making should be individualized.…”
Section: Discussionmentioning
confidence: 97%
“…The efficiency platelet reduction with Spectra Optia observed in our case was significantly higher than previously reported. There are a variety of studies comparing this equipment related to platelet donation in healthy individuals, however for therapeutic use is not frequent and the only equipment approved in the United States is COBE Spectra . Spectra Optia has not been yet FDA cleared for therapeutic thrombocytapheresis.…”
Section: Discussionmentioning
confidence: 99%
“…There are a variety of studies comparing this equipment related to platelet donation in healthy individuals, however for therapeutic use is not frequent and the only equipment approved in the United States is COBE Spectra. 7 Spectra Optia has not been yet FDA cleared for therapeutic thrombocytapheresis. Boddu et al presented a case-based review of four patients treated with therapeutic thrombocytapheresis in which platelet count was reduced 30%-60% after processing 1.5-2 total blood volumes; unfortunately, no information is provided regarding the apheresis separators used.…”
Essential thrombocytosis (ET) is a chronic myeloproliferative neoplasm characterized by the presence of thrombocytosis and it can be complicated by thrombotic and/or hemorrhagic events. Treatment options include low‐dose aspirin and cytoreductive agents such as hydroxyurea. In cases of extreme thrombocytosis, therapeutic thrombocytapheresis can be a useful procedure. We present a case of a 61‐year‐old‐man previously diagnosed with CALR‐mutated ET, who develop acute myeloid leukemia. When recovering after induction chemotherapy, he developed an extreme thrombocytosis up to 2337 × 109/L regardless hydroxyurea was started. Two therapeutic trombocytapheresis were performed and anagrelide was added to cytoreductive regimen. Platelet count stabilized around 570 × 109/L. Both procedures were performed with the Spectra Optia Apheresis System version 11.3 (Terumo BCT) and we decided to use a higher collection preference and lower collection speed than manufacturer's recommendations. Platelet count decreased from 2380 × 109/L to 1035 × 109/L in the first procedure and from 1813 × 109/L to 768 × 109 in the second procedure. Platelet collection efficiency was calculated to be 110.3% and 86.1% in the first and second thrombocytapheresis, respectively. Therapeutic thrombocytapheresis with Spectra Optia is a safe and efficient therapy to treat patients with primary thrombocytosis while effect of cytoreductive agents is attained. Platelet collection efficiency was calculated to be higher than previously reported. We suggest that changes in technical parameters such as a deeper aspiration point and/or lower collection speed may increase procedure's efficiency.
“…The role of thrombocytapheresis in management of bleeding secondary to extremely high platelet counts is not well defined in MF patients. However, like any other thrombocytosis in general, it may be considered on case by case basis, if urgent platelet reduction is desired in bleeding secondary to acquired von Willebrand’s syndrome [54]. Per current ASFA guidelines, thrombocytapheresis is a category 2 (second line therapy either as a standalone treatment or in conjunction with other modes of treatment), grade 2C recommendation (weak recommendation, low quality or very low quality evidence) for symptomatic thrombocytosis [55].…”
Bleeding and thrombosis are long recognized complications of myelofibrosis (MF) and contribute significantly to its morbidity and mortality. However, so far, few studies have evaluated the frequency of these events, their characteristics, and their prognostic impact. Based on these studies, thrombotic events in MF are about as common as in essential thrombocytemia (ET) but less common than in polycythemia vera (PV), while bleeding events are relatively more common in MF than in ET or PV. The emergence of the concept of prefibrotic primary MF (PMF), which is associated with a higher frequency of thrombohemorrhagic complications than ET, and the growing evidence that prefibrotic PMF may also have a different thrombotic and bleeding risk profiles than fibrotic (overt) PMF, have emphasized the need for a reappraisal of the risk of thrombosis and hemorrhage in patients with MF. In this review, we discuss the frequency of thrombosis and bleeding in patients with MF, including prefibrotic PMF and their established and potential risk factors.
“…With extreme thrombocytosis in an emergency situation thrombocytopheresis could be considered. In most situations urgent cytoreductive therapies are what is needed but there may be individual cases where thrombocytophersis should be considered as a possible therapeutic option [74].…”
Myeloproliferative neoplasms (MPNs) are associated with a variety of symptoms and signs which cause major morbidity for the patients. The disorders are associated with increased incidence of thromboembolic and hemorrhagic events which can lead to complications and shortened life expectancy. Areas covered: Using systematic literature review and expert clinical and research experience the authors discuss strategies for the management of symptoms and signs including pruritus, fatigue, splenomegaly, and cytopenia. Cytoreduction including treatments to inhibit the JAK/STAT pathway are considered. Pathogenesis and prevention and treatment of thrombotic and hemorrhagic events and their management is addressed and the suggested management of the special situations such as surgery and pregnancy are discussed. Expert commentary: Management of disease has traditionally focused on symptom treatment and complication prevention but the discovery of driver mutations has led to treatments aiming to eliminate the clone, which should be the ultimate goal of therapy. A future challenge is to develop safe and effective MPN therapy and to personalize therapy.
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