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Rezaei A, Vosoughi T, Ahmadzadeh Deylami A, Salehi Kahyesh R. Plasma Exchange on Platelet Count in Thrombotic Thrombocytopenic Purpura: A Case Report. bloodj 2026; 23 (1) :53-60
URL: http://bloodjournal.ir/article-1-1608-en.html
Plasma Exchange on Platelet Count in Thrombotic Thrombocytopenic Purpura: A Case Report
Atefeh Rezaei , Tina Vosoughi , Ahmad Ahmadzadeh Deylami , Roya Salehi Kahyesh *
Keywords: Purpura, Thrombotic Thrombocytopenic, Platelet Count, Thrombocytopenia, Plasmapheresis
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 References:
    1. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: clinical experience in 108 patients. N Engl J Med. 1991; 325(6): 398-403. [DOI:10.1056/NEJM199108083250605] [PMID]
    2. Zheng XL, Vesely SK, Cataland SR, Coppo P, Geldziler B, Iorio A, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020; 18(10): 2486-95. https://doi.org/10.1111/jth.15010 [DOI:10.1111/jth.15006] [PMID]
    3. Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The 8th Special Issue. J Clin Apher. 2019; 34(3): 171-354. [DOI:10.1002/jca.21705] [PMID]
    4. Cuker A, Cataland SR, Coppo P, de la Rubia J, Friedman K D, George JN, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021; 137(14): 1855-61. [DOI:10.1182/blood.2020009150] [PMID]
    5. Ortel TL, Neumann I, Ageno W, Beyth R, Clark NP, Cuker A, et al. American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism. Blood Adv. 2020; 4(19): 4693-738. [DOI:10.1182/bloodadvances.2020001830] [PMID] []
    1. Picod A, Veyradier A, Coppo P. Should all patients with immune-mediated thrombotic thrombocytopenic purpura receive caplacizumab? J Thromb Haemost. 2021; 19(1): 58-67. [DOI:10.1111/jth.15194] [PMID]
    2. Matsumoto M, Miyakawa Y, Kokame K, Ueda Y, Wada H, Higasa S, et al. Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) in Japan 2023. Int J Hematol. 2023;118(5):529-46. [DOI:10.1007/s12185-023-03657-0] [PMID] []
    3. Westwood JP, Scully M. Management of acquired, immune thrombocytopenic purpura (iTTP): beyond the acute phase. Ther Adv Hematol. 2022; 13: 20406207221112217. [DOI:10.1177/20406207221112217] [PMID] []
    4. Jestin M, Benhamou Y, Schelpe AS, Roose E, Provôt F, Galicier L, et al. Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura. Blood. 2018; 132(20):2143-53. [DOI:10.1182/blood-2018-04-840090] [PMID]
    5. Doyle  AJ,  Stubbs  MJ,   Dutt T, Lester W, Thomas W, van Veen J,et al. Long-term risk of relapse in immune-mediated thrombotic thrombocytopenic purpura and the role of anti-CD20 therapy. Blood. 2023; 141(3): 285-94. [DOI:10.1182/blood.2022017023] [PMID]
    1. Scully M, Cataland SR, Peyvandi F, Coppo P, Knöbl P, Kremer Hovinga JA, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019; 380(4): 335-46. [DOI:10.1056/NEJMoa1806311] [PMID]
    2. Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL,
      Miyata T, Vanhoorelbeke K. Thrombotic thrombocytopenic purpura. Nat Rev Dis Primers. 2017; 3: 17020. [      DOI:10.1038/nrdp.2017.20] [PMID]
    3. Bendapudi PK, Hurwitz S, Fry A, Marques MB, Waldo SW, Li A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol. 2017; 4(4): e157-e164. [DOI:10.1016/S2352-3026(17)30026-1] [PMID]




Plasma Exchange on Platelet Count in Thrombotic Thrombocytopenic Purpura: A Case Report

Atefeh Rezaei1      , Tina Vosoughi1      , Ahmad Ahmadzadeh Deylami1      , Roya Salehi Kahyesh1

1 Thalassemia &Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapour University of Medical Sciences, Ahvaz, Iran
Received: 2025/12/29
Accepted: 2026/04/13
 





     http://dx.doi.org/10.61186/bloodj.22.1.19
    



Citation:
Rezaei A, Vosoughi T, Ahmadzadeh Deylami A, Salehi Kahyesh R. Plasma Exchange on Platelet Count in Thrombotic Thrombocytopenic Purpura: A Case Report. J Iran Blood Transfus. 2026: 23 (1): 53-60
    



Correspondence: Salehi Kahyesh, Assistant Professor of Thalassemia & Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapour University of Medical Sciences.
P.O.Box: 6138933333, Ahvaz, Iran.
Tel: (+98613) 33750416
E-mail:
1- Acridine Orange
1- Biological safety cabinet
1- Platelet Concentrate
2- Food and Drug Administration
3- Normal Skin Flora
4- Platelet Rich Plasma-Platelet Concentrate
5- Eosin-Methylene blue
6- Thioglycolate
1- Acridine Orange
1- Biological safety cabinet
1- Platelet Concentrate
2- Food and Drug Administration
3- Normal Skin Flora
4- Platelet Rich Plasma-Platelet Concentrate
5- Eosin-Methylene blue
6- Thioglycolate
royaarta@yahoo.com  		 A B S T R A C T
Background and Objectives
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, neurological symptoms, renal dysfunction, and fever. Although therapeutic plasma exchange (TPE) is the cornerstone of treatment management, responses can vary significantly among patients. This report describes an unusual recurrence of TTP in which platelet recovery was inconsistent with biochemical recovery, emphasizing the need for individualized treatment strategies.
Case Report
A 38-year-old female patient with a 2-year history of TTP presented to Baqaei 2 Hospital in Ahvaz with presentations including altered level of consciousness, seizures, anemia, thrombocytopenia, and marked elevation of lactate dehydrogenase (LDH). At initial evaluation, the patient's platelet count was 25 ×109 /L, hemoglobin level was 7.5 g/dL, and LDH was 2300 units/L. Treatment with plasma exchange with corticosteroids was initiated, which resulted in initial improvement in platelet count and hemolysis indices. After six sessions of plasma exchange, the platelet count increased to more than 150×109/L on one occasion; however, a decrease to  83×109/L was observed in the next session. Despite continuing treatment for 12 sessions, the platelet count only reached 107×109 /L. After completing 18 sessions of daily plasma exchange, although the LDH level returned to the normal range, the platelet count still did not exceed 150×109 /L, indicating limited efficacy of additional plasma exchange sessions. Subsequently, plasma exchange was discontinued and corticosteroids were continued with the addition of rituximab (375 mg weekly for four weeks), resulting in a gradual and sustained increase in platelet count to the normal range.
Conclusions 
This study demonstrates that hematologic improvement in TTP is not always accompanied by biochemical normalization. Persistent thrombocytopenia with normal LDH may indicate immune or endothelial factors that are not resolved by plasma exchange alone. Close monitoring, individual adjustments, and early initiation of immunotherapy such as rituximab are essential to optimize outcomes.
Key words: Purpura،  Thrombotic Thrombocytopenic, Platelet Count, Thrombocytopenia, Plasmapheresis
 


Copyright © 2025 Journal of Iranian Blood Transfusion, Published by Blood Transfusion Research Center.
This work is licensed under a Creative Common Attribution-Non Commercial 4.0 International license.





 
Type of Study: Research | Subject: Hematology
References
1. Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: clinical experience in 108 patients. N Engl J Med. 1991; 325(6): 398-403. [DOI:10.1056/NEJM199108083250605] [PMID]
2. Zheng XL, Vesely SK, Cataland SR, Coppo P, Geldziler B, Iorio A, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020; 18(10): 2486-95. https://doi.org/10.1111/jth.15010 [DOI:10.1111/jth.15006] [PMID]
3. Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice - Evidence-Based Approach from the Writing Committee of the American Society for Apheresis: The 8th Special Issue. J Clin Apher. 2019; 34(3): 171-354. [DOI:10.1002/jca.21705] [PMID]
4. Cuker A, Cataland SR, Coppo P, de la Rubia J, Friedman K D, George JN, et al. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021; 137(14): 1855-61. [DOI:10.1182/blood.2020009150] [PMID]
5. Ortel TL, Neumann I, Ageno W, Beyth R, Clark NP, Cuker A, et al. American Society of Hematology 2020 guidelines for management of venous thromboembolism: treatment of deep vein thrombosis and pulmonary embolism. Blood Adv. 2020; 4(19): 4693-738. [DOI:10.1182/bloodadvances.2020001830] [PMID] []
6. Picod A, Veyradier A, Coppo P. Should all patients with immune-mediated thrombotic thrombocytopenic purpura receive caplacizumab? J Thromb Haemost. 2021; 19(1): 58-67. [DOI:10.1111/jth.15194] [PMID]
7. Matsumoto M, Miyakawa Y, Kokame K, Ueda Y, Wada H, Higasa S, et al. Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) in Japan 2023. Int J Hematol. 2023;118(5):529-46. [DOI:10.1007/s12185-023-03657-0] [PMID] []
8. Westwood JP, Scully M. Management of acquired, immune thrombocytopenic purpura (iTTP): beyond the acute phase. Ther Adv Hematol. 2022; 13: 20406207221112217. [DOI:10.1177/20406207221112217] [PMID] []
9. Jestin M, Benhamou Y, Schelpe AS, Roose E, Provôt F, Galicier L, et al. Preemptive rituximab prevents long-term relapses in immune-mediated thrombotic thrombocytopenic purpura. Blood. 2018; 132(20):2143-53. [DOI:10.1182/blood-2018-04-840090] [PMID]
10. Doyle AJ, Stubbs MJ, Dutt T, Lester W, Thomas W, van Veen J,et al. Long-term risk of relapse in immune-mediated thrombotic thrombocytopenic purpura and the role of anti-CD20 therapy. Blood. 2023; 141(3): 285-94. [DOI:10.1182/blood.2022017023] [PMID]
11. Scully M, Cataland SR, Peyvandi F, Coppo P, Knöbl P, Kremer Hovinga JA, et al. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019; 380(4): 335-46. [DOI:10.1056/NEJMoa1806311] [PMID]
12. Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL, Miyata T, Vanhoorelbeke K. Thrombotic thrombocytopenic purpura. Nat Rev Dis Primers. 2017; 3: 17020. [DOI:10.1038/nrdp.2017.20] [PMID]
13. Bendapudi PK, Hurwitz S, Fry A, Marques MB, Waldo SW, Li A, et al. Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol. 2017; 4(4): e157-e164. [DOI:10.1016/S2352-3026(17)30026-1] [PMID]
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