Article menu

Rapidly identifying thrombotic thrombocytopenic purpura (TTP) can save lives¹

TTP is a life-threatening thrombotic microangiopathy (TMA) that requires quick diagnosis and urgent treatment. ^1,2

The prognosis of thrombotic thrombocytopenic purpura (TTP) can be unpredictable^1,2

Patients can experience refractory disease or recurrence of their TTP (exacerbation or relapse), highlighting the need for vigilant monitoring^3,4

The risks and effects of TTP extend beyond the first acute events that patients experience.^5-7

Here are some of the various ways TTP can affect your patients after diagnosis and treatment.

TTP episode

Even with plasma exchange therapy (PEX), mortality risk persists^8-10

acute mortality associated with episodes of aTTP^9,10

Refractory TTP

Occurs when patients do not have a clinical response after 5 sessions of PEX. Clinical response is defined as sustained platelet count ≥150 × 109/L and LDH <1.5 times ULN and no clinical evidence of new or progressive organ injury.⁴

Refractory TTP can indicate poor prognosis for survival³

of aTTP patients experience unpredictable refractoriness

TTP exacerbation

A platelet count decrease after a clinical response and before a clinical remission. A clinical exacerbation is defined as platelet count decrease to <150 × 10⁹/L (with other causes of thrombocytopenia excluded), with or without clinical evidence of new or progressive ischemic organ injury, within 30 days of stopping plasma exchange or anti-vWF therapy.⁴

TTP exacerbation is a threat to patient recovery

of patients have ≥1 TTP episode within 30 days of stopping plasma exchange^*
^*Retrospective review of French Reference Centre for TMA registry (N=388).

TTP relapse

A new episode of TTP after achieving clinical remission.^† A clinical relapse is defined as platelet count decrease to <150 × 109/L (with other causes of thrombocytopenia ruled out), with or without clinical evidence of new ischemic organ injury and a confirmed documentation of severe ADAMTS13 deficiency.⁴

ADAMTS13 = a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13.

^†Clinical remission is defined as sustained clinical response with no plasma exchange and no anti-vWF therapy for ≥30 days or attainment of ADAMTS13 remission.

TTP exacerbation is a threat to patient recovery¹¹

reported relapse rate in studies of TTP⁷

Monitor patients closely. Make them aware of the potential for recurrence (exacerbation or relapse) and educate on symptoms and triggers so they know to reach out immediately.

ULN=upper limit of normal.

References

Scully M, Cataland SR, Peyvandi F, et al; HERCULES Investigators. Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura. N Engl J Med. 2019;380(4):335-346. doi:10.1056/NEJMoa1806311
Joly BS, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura. Blood. 2017;129(21):2836-2846. doi:10.1182/blood-2016-10-709857
Sayani FA, Abrams CS. How I treat refractory thrombotic thrombocytopenic purpura. Blood. 2015;125(25):3860-3867. doi:10.1182/blood-2014-11-551580
Cuker A, Cataland SR, Coppo P, et al; International Working Group for Thrombotic Thrombocytopenic Purpura. Redefining outcomes in immune TTP: an international working group consensus report. Blood. 2021;137(14):1855-1861. doi:10.1182/blood.2020009150
Han B, Page EE, Stewart LM, et al. Depression and cognitive impairment following recovery from thrombotic thrombocytopenic purpura. Am J Hematol. 2015;90(8):709-714. doi:10.1002/ajh.24060
Deford CC, Reese JA, Schwartz LH, et al. Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura. Blood. 2013;122(12):2023-2029. doi:10.1182/blood-2013-04-496752
Thejeel B, Garg AX, Clark WF, et al. Long-term outcomes of thrombotic microangiopathy treated with plasma exchange: a systematic review. Am J Hematol. 2016;91(6):626-630. doi:10.1002/ajh.24339
Goel R, King KE, Takemoto CM, Ness PM, Tobian AAR. Prognostic risk-stratified score for predicting mortality in hospitalized patients with thrombotic thrombocytopenic purpura: nationally representative data from 2007 to 2012. Transfusion. 2016;56(6):1451-1458. doi:10.1111/trf.13586
Kremer Hovinga JA, Vesely SK, Terrell DR, Lämmle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2010;115(8):1500-1511. doi:10.1182/blood-2009-09-243790
Peyvandi F, Scully M, Kremer Hovinga JA, et al. Caplacizumab reduces the frequency of major thromboembolic events, exacerbations and death in patients with acquired thrombotic thrombocytopenic purpura. J Thromb Haemost. 2017;15(7):1448-1452. doi:10.1111/jth.13716
Grall M, Azoulay E, Galicier L, et al. Thrombotic thrombocytopenic purpura misdiagnosed as autoimmune cytopenia: causes of diagnostic errors and consequence on outcome. Experience of the French Thrombotic Microangiopathies Reference Centre. Am J Hematol. 2017;92(4):381-387. doi:10.1002/ajh.24665

The long-term impact of thrombotic thrombocytopenic purpura (TTP)

Patients can experience the effects of TTP long after their first episodes

The consequences of TTP can lead to many long-term complications and health risks. Approaching TTP with a long-term mindset can help ensure comprehensive care for your patients.^1-3

Microthrombi may result in long-term neurocognitive decline in patients with TTP¹

63% of patients with TTP have neurological signs and symptoms during their first event, including^2-4

Coma
Focal neurological signs
Personality changes
Transient ischemic attack
Seizures
Stroke

In these patients, studies have shown⁴

Persistent neurological impairment during remission (49%)

Disorientation
Loss of concentration
Dizziness
Lack of balance
Headache
Diplopia

Significant impairment in memory domains

(direct, backward, and deferred memory) vs the general population

Higher prevalence of relapse in TTP

vs those without neurological symptoms (41% vs 8%)

Patients with TTP may have increased risk of stroke after recovery⁵

Stroke during the median observation follow-up period (3.08 years) after recovery from TTP occurred in 13.1% of patients, which is five-fold higher than the expected prevalence from an age- and sex-matched reference population.

Stroke risk is common after recovery from TTP and is associated with ADAMTS13 levels

ADAMTS13=a disintegrin and metalloproteinase with thrombospondin type 1 motif, 13.

Low ADAMTS13 activity after recovery from an acute episode is associated with an increased risk of ischemic stroke during the follow-up period (P=0.007).

of patients with >70% ADAMTS13 levels experienced stroke (0/22)

Patients with TTP reported decreased quality of life^1,4

TTP affects patients’ quality of life over the long term, owing to exacerbations, relapses, and sustained neurocognitive defects. Patients’ responses across health-related quality of life measures suggest that living with TTP is a considerable emotional burden.

Clinical anxiety and depression are serious risks for patients with TTP⁴

of patients with >70% ADAMTS13 levels experienced stroke (0/22)

It’s important to monitor the physical and mental health of your patients with TTP.^1,4,5

Refences

Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2486-2495. doi:10.1111/jth.15006
Han B, Page EE, Stewart LM, et al. Depression and cognitive impairment following recovery from thrombotic thrombocytopenic purpura. Am J Hematol. 2015;90(8):709-714. doi:10.1002/ajh.24060
Deford CC, Reese JA, Schwartz LH, et al. Multiple major morbidities and increased mortality during long-term follow-up after recovery from thrombotic thrombocytopenic purpura. Blood. 2013;122(12):2023-2029. doi:10.1182/blood-2013-04-496752
Riva S, Mancini I, Maino A, et al. Long-term neuropsychological sequelae, emotional wellbeing and quality of life in patients with acquired thrombotic thrombocytopenic purpura. Haematologica. 2020;105(7):1957-1962. doi:10.3324/haematol.2019.226423
Upreti H, Kasmani J, Dane K, et al. Reduced ADAMTS13 activity during TTP remission is associated with stroke in TTP survivors. Blood. 2019;134(13):1037-1045. doi:10.1182/blood.2019001056