DRESS syndrome and thrombotic thrombocytopaenic purpura: are they related?

Sandouk, Zahrae; Alirhayim, Zaid; Khoulani, Dania; Hassan, Syed Adeel

doi:10.1136/bcr-2012-007558

Cited by 12 publications

(7 citation statements)

References 10 publications

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“…[ 8 ] Up to now, only 2 cases reporting a potential association between DRESS and thrombotic thrombocytopenic purpura, a type of TMA, have been published. [ 2 , 3 ] ADAMTS13 testing can be useful in differentiating TTP from others TMA, as in our case. [ 9 ]…”

Section: Discussionmentioning

confidence: 59%

DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome

Bobot

Coen

Simon³

et al. 2018

Medicine

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Rationale:The life-threatening drug rash with eosinophilia and systemic symptoms (DRESS) syndrome occurs most commonly after exposure to drugs, clinical features mimic those found with other serious systemic disorders. It is rarely associated with thrombotic microangiopathy.Patient concerns:We describe the unique case of a 44-year-old man who simultaneously experienced DRESS syndrome with thrombotic microangiopathy (TMA) after a 5 days treatment with fluindione.Diagnoses:Clinical evaluation leads to the discovery of an underlying lymphangiomatosis, due to a Noonan syndrome.Intervetions:The anticoagulant was withdrawn, and corticosteroids (1 mg/kg/day) and acenocoumarol were started.Outcomes:Clinical improvement ensued. At follow-up the patient is well.Lessons:The association of DRESS with TMA is a rare condition; we believe that the presence of the underlying Noonan syndrome could have been the trigger. Moreover, we speculate about the potential interrelations between these entities.

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Section: Discussionmentioning

confidence: 59%

DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome

Bobot

Coen

Simon³

et al. 2018

Medicine

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“…These complications can evolve to posterior reversible encephalopathy syndrome (PRES), as reported by at least two studies [ 22 , 24 , 25 ], and sometimes to coma [ 26 ]. Most CNS lesions are characterized as hemorrhagic or ischemic cerebral infarctions [ 20 , 26 , 27 , 29 ] and, therefore, TMA may be misdiagnosed as a case of stroke [ 28 , 29 ]. Given that TMA is a medical emergency associated with neurological sequelae and a fatality rate of 20%, a quick and precise diagnosis is essential to prevent adverse outcomes [ 10 ].…”

Section: Discussionmentioning

confidence: 99%

“…The lack of ADAMTS13 tests is explained by the fact that, despite being necessary to confirm TTP diagnosis, the test is not easily available in the real world, particularly in low-and middle-income countries. For that reason, the PLASMIC score has been used to predict low ADAMTS13 activity [19][20][21][22][23][24][25][26][27][28][29][30][31][32]. The use of PLASMIC score in this study allowed us to suspect that most of our patients had TTP even though ADAMTS13 results were not available.…”

Section: Plos Onementioning

confidence: 98%

Neurological manifestations in thrombotic microangiopathy: Imaging features, risk factors and clinical course

et al. 2022

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Background and purpose Thrombotic microangiopathy (TMA) is a group of microvascular occlusive disorders that presents with neurological involvement in up to 87% of the cases. Although the central nervous system (CNS) is an important target organ in TMA, the role of neurological manifestations in the disease clinical course is not well established. In this study, we described the neurological manifestations and CNS radiological aspects in patients with a first, acute TMA event. We also examined the association between severe neurological involvement and adverse clinical outcomes in TMA. Methods A cohort of patients diagnosed with a first TMA event between 1995 and 2016 was included, their medical charts and imaging tests were retrospectively evaluated. Results A total of 49 patients were included, 85.7% were women and the mean age was 36.5 years-old (SD 13.0). Neurological manifestations were described in 85.7% of the patients, most of them (88%) were considered severe and consisted of confusion, compromised sensorimotor function, stupor, seizures, and personality change. Imaging tests were performed in 62% of the patients with neurological manifestations and detected acute CNS lesions, such as posterior reversible encephalopathy syndrome, hemorrhagic and ischemic stroke were observed, in 7 (27%) of them. While the need for intensive care unit admission was greater and longer among patients with severe neurological manifestations, the number of plasma exchange sessions, the total duration of hospitalization and in-hospital death were similar between groups. Conclusions Severe neurological manifestations are common in first TMA events and are responsible for a worse disease presentation at admission. While the effect of neurological manifestations on acute TMA clinical course seems to be modest, these manifestations may have an important impact on the development of chronic cognitive impairment, which highlights the need for proper diagnosis and treatment.

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“…In Asia, the DIHS/DRESS represents almost one tenth of all adverse drug reaction cases, with a mortality rate of 3–10% [17–20]. Various newly developed autoimmune diseases are considered sequelae of the DIHS/DRESS, including thrombotic thrombocytopenic purpura (TTP) [21], autoimmune hemolytic anemia (AHA) [22], sclerodermoid graft-versus-host disease-like lesions [23], and systemic lupus erythematosus (SLE) [24]. FT1DM is one of the most important sequelae of DIHS/DRESS.…”

Section: Discussionmentioning

confidence: 99%

Co-Occurrence of Multiple Endocrine Abnormalities Induced by the DIHS/DRESS

Deng

et al. 2019

International Journal of Endocrinology

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Background Drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS) is a severe adverse reaction caused by specific drugs. However, little information is available about sequelae following DIHS/DRESS resolution from an endocrinologist's perspective. This study aimed to investigate the endocrine sequelae following DIHS/DRESS, from clinical feature to etiology. Methods We retrospectively analyzed the patients diagnosed with DIHS/DRESS in Peking Union Medical College Hospital (PUMCH) during the period of 1 January 2012 to 31 December 2017, and those who developed endocrine disorders after DIHS/DRESS were further examined. We also reviewed the literature, from 1 January 2000 to 31 December 2017, on involvement of endocrine glands in DIHS/DRESS patients. Results Three patients developed both autoimmune thyroid disease (AITD) and type 1 diabetes (T1DM)/fulminant type 1 diabetes (FT1DM) of the 45 patients. Seven cases involving more than two endocrine glands were reported in the literature. Our results indicated that DIHS/DRESS is a potential etiological factor of autoimmune polyendocrine syndrome (APS), especially APS III. Conclusions Patients require careful long-term follow-up after DIHS/DRESS. Involvement of endocrine glands, especially FT1DM, should always be monitored in patients with a history of DIHS/DRESS. This study indicated that DIHS/DRESS could lead to APS, especially APS III, providing novel insights into the etiological factors of APS.

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DRESS syndrome and thrombotic thrombocytopaenic purpura: are they related?

Cited by 12 publications

References 10 publications

DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome

DRESS syndrome with thrombotic microangiopathy revealing a Noonan syndrome

Neurological manifestations in thrombotic microangiopathy: Imaging features, risk factors and clinical course

Co-Occurrence of Multiple Endocrine Abnormalities Induced by the DIHS/DRESS

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