Table of Contents
Definition / general | Essential features | Terminology | Pathophysiology | Clinical features | Symptoms | Vascular access | Indications | Volume exchanged & technical details | Adverse events | Laboratory | Case reports | Treatment | Peripheral smear images | Sample assessment & plan | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Adkins BD, Booth GS. Plateletpheresis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/transfusionmedplateletpheresis.html. Accessed April 19th, 2024.
Definition / general
- Thrombocytapheresis (or plateletpheresis) is the removal of platelet cells via apheresis
Essential features
- Therapeutic thrombocytapheresis is performed to acutely lower a patient's platelet (PLT) count due to symptoms associated with thrombocytosis, such as hemorrhagic or thromboembolic complications
Terminology
- Thrombocytosis (J Clin Apher 2023;38:77, Blood 2014;124:1997)
- Usually defined as a platelet count > 450,000 - 500,000/µL
- Can be caused by an underlying hematologic malignancy (primary thrombocytosis) or by a reactive process (secondary thrombocytosis) (Leuk Res 2017;58:14)
- Primary thrombocytosis
- Due to a myeloproliferative disorder (MPD), including polycythemia vera, essential thrombocythemia and chronic myelogenous leukemia (CML) (see Peripheral smear images)
- Shorter platelet survival; normal circulating lifespan of a platelet is 8 - 10 days
- Increase in markers of platelet activation, such as platelet factor 4, thrombomodulin and beta thromboglobulin
- May experience hemorrhage or thrombosis
- Hemorrhage risk due to acquired von Willebrand syndrome
- Thrombosis usually occurs when platelet count is > 600,000/µL but risk does not correlate well with platelet count
- Secondary thrombocytosis
- More common, accounting for 80 - 90% of cases (Intern Med 2022;61:3323)
- Platelet function is relatively normal
- Carries significantly smaller risk of hemorrhage or thrombosis
Pathophysiology
- Thrombosis can occur as a result of congestion and increased viscosity secondary to high platelet counts
- This tends to be more common in clonal thrombocytosis unless patients have underlying predisposition to clot formation (N Engl J Med 2004;350:1211)
- Likewise, bleeding tends to occur as a result of qualitative platelet defects seen in myeloproliferative disorders
(N Engl J Med 2004;350:1211)
- Extremely high platelet counts tend to associate with an acquired von Willebrand syndrome with clearance of high molecular weight von Willebrand factor (vWF) and bleeding (Haematologica 2002;87:264)
Clinical features
- Hemorrhage or thromboembolism associated with platelet counts > 450,000/µL (Semin Thromb Hemost 2013;39:101)
Symptoms
- Patients may present with platelet type bleeding at mucous membranes or gastrointestinal tract, digital ischemia or massive thromboembolic events such as pulmonary embolism or hepatic vein thrombosis (N Engl J Med 2004;350:1211)
- Platelet count does not predict symptoms (J Clin Apher 2023;38:77)
Vascular access
- Usually performed emergently using femoral apheresis or a dialysis catheter, which do not require radiographic confirmation
- Background on femoral vein vascular access (StatPearls: Femoral Vein Central Venous Access [Accessed 31 January 2024])
- Central venous catheter, which requires radiographic confirmation, can be used in nonemergent situations
Indications
- The only indications for thrombocytapheresis according to the American Society for Apheresis (ASFA) are symptomatic and prophylactic or secondary thrombocytosis (J Clin Apher 2023;38:77)
- Symptomatic thrombocytosis - ASFA category II
- Bring platelet count to < 350 - 450 x 109/L in patients with an acute thrombohemorrhagic event
- Prophylaxis - ASFA category III
- Pregnant patients, surgical patients, etc.
- < 450 - 600 x 109/L may be an appropriate count for prophylaxis per ASFA
- Symptomatic thrombocytosis - ASFA category II
Volume exchanged & technical details
- 1 procedure or until resolution of symptoms
- 1.5 - 2 blood volumes
- This usually reduces the platelet count by 30 - 60%
- Patients with spleens will recover counts more quickly than asplenic patients due to splenic platelet content (Leuk Res 2017;58:14)
Adverse events
- This is an apheresis procedure, with its typical complications
- Also complications from the underlying myeloproliferative neoplasm, hypotension, citrate related toxicity, bleeding and infection
Laboratory
- Elevated platelet count
- Reduced vWF and factor VIII levels
Case reports
- 24 year old woman with essential thrombocythemia was treated prophylactically with thrombocytapheresis (Case Rep Oncol 2020;13:675)
- 25 year old man with triple negative essential thrombocythemia presented with thrombosis and acquired von Willebrand syndrome and was treated with thrombocytapheresis (Am J Case Rep 2020;21:e924560)
- 51 year old woman with essential thrombocythemia was treated for postoperative bleeding with 2 thrombocytapheresis procedures (Transfusion 2021;61:3277)
Treatment
- Treatment of underlying condition should be initiated for primary thrombocytosis with cytoreductive agents (hydroxyurea) (J Clin Apher 2023;38:77)
- Interferon alpha should be used in pregnancy
- In symptomatic or prophylactic thrombocytapheresis, the platelet goal should be < 450 x 109/L
Sample assessment & plan
- Assessment: A 63 year old man with JAK2+ myeloproliferative neoplasm underwent thrombocytapheresis due to acute thromboembolism.
- Plan: 1.5 blood volumes were processed. If counts remain high or patient experiences symptoms related to thrombocytosis, further intervention may be considered.
Board review style question #1
A patient presents with essential thrombocythemia and has ongoing mucocutaneous and gastrointestinal bleeding. A platelet count is found to be > 2,000 x 109. Unfortunately, 2 days later the patient remains symptomatic. What is the goal platelet count?
- < 10 - 20 x 109/L
- < 350 - 450 x 109/L
- < 750 - 1,000 x 109/L
- < 1,500 - 1,750 x 109/L
Board review style answer #1
B. < 350 - 450 x 109/L. ASFA guidelines recommend a count of 350 - 450 x 109/L for symptomatic thrombocytosis. The remaining answers fall outside of ASFA recommendations. Answer A is incorrect because it is too low and could lead to bleeding complications, requiring platelet transfusion. Answers C and D are incorrect because < 750 - 1,000 x 109/L and < 1,500 - 1,750 x 109/L are both still too high and could potentially contribute to depletion of von Willebrand factor, furthering the symptomatic thrombocytosis associated bleeds.
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Reference: Plateletpheresis
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Board review style question #2
Why do asplenic patients with thrombocytosis recover their platelet counts more slowly than patients with spleens after thrombocytapheresis?
- Absence of extramedullary hematopoiesis
- Asplenia impacts fluid balance leading to thrombocytopenia
- Lack of splenic macrophages
- Spleen helps maintain platelet count with sequestered platelets
Board review style answer #2
D. Spleen helps maintain platelet count with sequestered platelets. The spleen contains ~25 - 30% of the body's platelets and some of these will peripheralize after thrombocytapheresis or in times of stress in patients with a spleen (Leuk Res 2017;58:14). Asplenic patients do not have this residual pool of platelets (StatPearls: Physiology, Spleen [Accessed 31 January 2024]). Answer A is incorrect because extramedullary hematopoiesis occurs mainly in very young patients or those with a bone marrow filling lesion, such as a hematologic malignancy, metastasis or marrow fibrosis; nevertheless, it does not act as a platelet reserve but rather a producer of platelets. Answer B is incorrect because fluid balance is maintained predominantly by the kidneys as opposed to the spleen. Answer C is incorrect because splenic macrophages help clear aging platelets and red cells and do not release or store platelets.
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Reference: Plateletpheresis
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