The clinical picture of thrombotic microangiopathy in patients older than 60 years of age

Schmidt, Julien; Zafrani, Lara; Lemiale, Virginie; Stépanian, Alain; Joly, Bérangère S.; Azoulay, Élie; Mariotte, Éric

doi:10.1111/bjh.17176

Cited by 5 publications

(7 citation statements)

References 12 publications

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“…Therefore, the diagnosis of MAHA is based on a triad of thrombocytopenia, LDH elevation and the presence of schistocytes, along with a negative direct antiglobulin test. The search for schistocytes is a major diagnostic element and should be repeated in case of initial negativity [ 57 ].…”

Section: Hematological Complications Requiring Emergency Chemotherapy...mentioning

confidence: 99%

Management of hematological patients requiring emergency chemotherapy in the intensive care unit

Lafarge,

Chean,

Whiting

et al. 2024

Intensive Care Med

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Hematological malignancies may require rapid-onset treatment because of their short doubling time, notably observed in acute leukemias and specific high-grade lymphomas. Furthermore, in targeted onco-hematological scenarios, chemotherapy is deemed necessary as an emergency measure when facing short-term, life-threatening complications associated with highly chemosensitive hematological malignancies. The risks inherent in the disease itself, or in the initiation of treatment, may then require admission to the intensive care unit (ICU) to optimize monitoring and initial management protocols. Hyperleukocytosis and leukostasis in acute leukemias, tumor lysis syndrome, and disseminated intravascular coagulation are the most frequent onco-hematological complications requiring the implementation of emergency chemotherapy in the ICU. Chemotherapy must also be started urgently in secondary hemophagocytic lymphohistiocytosis. Tumor-induced microangiopathic hemolytic anemia and plasma hyperviscosity due to malignant monoclonal gammopathy represent infrequent yet substantial indications for emergency chemotherapy. In all cases, the administration of emergency chemotherapy in the ICU requires close collaboration between intensivists and hematology specialists. In this review, we provide valuable insights that aid in the identification and treatment of patients requiring emergency chemotherapy in the ICU, offering diagnostic tools and guidance for their overall initial management. Supplementary Information The online version contains supplementary material available at 10.1007/s00134-024-07454-z.

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Section: Hematological Complications Requiring Emergency Chemotherapy...mentioning

confidence: 99%

Management of hematological patients requiring emergency chemotherapy in the intensive care unit

Lafarge,

Chean,

Whiting

et al. 2024

Intensive Care Med

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“…Elderly patients present more comorbidities (mental alteration, stroke, renal dysfunction, cancer) than the rest of the population, which contributes to delay the diagnosis of TMA. They also seem to develop more TTP and secondary TMA associated with cancer or medication [ 39 ]. A better awareness of TMA-related complications could contribute to a faster diagnosis and a better management of these elderly patients whose greater frailty contributes to an increased mortality rate.…”

Section: Discussionmentioning

confidence: 99%

Incidence of thrombotic microangiopathies in Quebec: insight from a laboratory centralizing ADAMTS-13 testing

Merlen

Pépin

Barry

et al. 2022

Orphanet J Rare Dis

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Background Thrombotic microangiopathies (TMA) are serious medical conditions requiring a prompt diagnosis to adapt treatment. The determination of ADAMTS-13 activity enables discriminating thrombotic thrombocytopenic purpura (TTP) from other forms of TMA. The purpose of this study was to provide an estimate of the incidence of TTP and TMA in the Canadian Quebec province using data collected from a laboratory centralizing ADAMTS-13 testing for the whole province. Results From 2012 to 2019, 846 patients were evaluated for plasma ADAMTS-13 activity due to a suspicion of TMA. TTP was identified in 147 patients. Of these, 118 patients with a median age of 51.5 years and a male–female ratio of 1:1.4 had their first episode of TTP during the study period. The number of ADAMTS-13 tests performed and the number of patients with suspected TMA increased annually by 19% and 21% respectively. While the incidence of non-TTP TMA increased annually, that for TTP remained unchanged. This averaged 10.2 (95% CI 5.9–14.4) per million persons per year for suspected non-TTP TMA and 1.8 (95% CI 1.3–2.4) for confirmed TTP. The incidence rate of TMA other than TTP was higher in the age group 70–79 years (21.8; 95% CI 5.4–38.1) for females and in the age group 80–89 years (24.4; 95% CI 7.2–41.7) for males compared to other age groups. The incidence rate of TTP was higher in the age group 40–49 years (4.0; 95% CI 2.0–5.9) for women and in the age group 60–69 years (3.4; 95% CI 1.1–5.6) for men compared to other age groups. Conclusion The analysis of centralized data measuring ADAMTS-13 activity allowed us to adequately establish the incidence rate and demographic characteristics of TMA, particularly TTP, in Quebec. TTP incidence remained stable while suspected non-TTP TMA steadily increased from 2012 to 2019.

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“…The prevalence of older patients with iTTP is increasing due to the aging of the general population and the possibly of improvements in disease recognition. Recent, dedicated studies [33][34][35] have underlined that older iTTP patients have an atypical clinical presentation and a poorer response to treatment and prognosis, which are detailed hereafter.…”

Section: Immune Mediated Thrombotic Thrombocytopenic Purpura In Older...mentioning

confidence: 99%

“…Delirium and behavioral abnormalities are often at the forefront of iTTP's clinical presentation in older patients, as opposed to headache and abdominal pain, which are more frequent in younger patients. Renal and cardiac involvement are more frequent and severe in older patients, whereas hematologic features such as thrombocytopenia and anemia are less pronounced [33], despite a seemingly increased gastro-enteral bleeding rate [35].…”

Section: Clinical Presentation and Diagnosismentioning

confidence: 99%

“…These differences translate into poorer performances of both the French and PLASMIC scores [33,36] and a longer time from admission to diagnosis for older patients (3 versus 1 day) [33,35], even though the proportion of iTTP amongst TMA does not seem to decrease with advanced age [35]. Recently, proteinuria and blood pressure were reported as potential leads for improving the performances of these scores [37][38][39][40], but these results warrant confirmation and their added value for older patients more frequently affected by hypertension, diabetes mellitus, and/or chronic kidney disease remains to be evaluated.…”

Section: Clinical Presentation and Diagnosismentioning

confidence: 99%

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The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

Joseph

Joly

Picod

et al. 2023

JCM

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Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic microangiopathy (TMA) related to a severe ADAMTS13 deficiency, the specific von Willebrand factor (VWF)-cleaving protease. This deficiency is often immune-mediated (iTTP) and related to the presence of anti-ADAMTS13 autoantibodies that enhance its clearance or inhibit its VWF processing activity. iTTP management may be challenging at extreme ages of life. International cohorts of people with TTP report delayed diagnoses and misdiagnoses in children and elderly people. Child-onset iTTP shares many features with adult-onset iTTP: a female predominance, an idiopathic presentation, and the presence of neurological disorders and therapeutic strategies. Long-term follow-ups and a transition from childhood to adulthood are crucial to preventing iTTP relapses, in order to identify the occurrence of other autoimmune disorders and psychosocial sequelae. In contrast, older iTTP patients have an atypical clinical presentation, with delirium, an atypical neurological presentation, and severe renal and cardiac damages. They also have a poorer response to treatment and prognosis. Long-term sequelae are highly prevalent in older patients. Prediction scores for iTTP diagnoses are not used for children and have a lower sensitivity and specificity in patients over 60 years old. ADAMTS13 remains the unique biological marker that is able to definitely confirm or rule out the diagnosis of iTTP and predict relapses during follow-ups.

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The clinical picture of thrombotic microangiopathy in patients older than 60 years of age

Cited by 5 publications

References 12 publications

Management of hematological patients requiring emergency chemotherapy in the intensive care unit

Management of hematological patients requiring emergency chemotherapy in the intensive care unit

Incidence of thrombotic microangiopathies in Quebec: insight from a laboratory centralizing ADAMTS-13 testing

The Specificities of Thrombotic Thrombocytopenic Purpura at Extreme Ages: A Narrative Review

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