POEMS syndrome, arterial thrombosis and thrombocythaemia

Zénone, T; Bastion, Y.; Salles, Gilles; Rieux, Claire; Morel, Dominique; Felman, Pascale; Espinouse, Daniel; Bryon, Paul‐André

doi:10.1046/j.1365-2796.1996.498839000.x

Cited by 22 publications

(10 citation statements)

References 15 publications

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“…The myeloproliferative and plasma cell disorder, such as thrombocytosis or polycythaemia, may contributed to the development of thrombotic complications in POEMS patients [14-16]. Moreover, Zenone et al[17] reported a patient with POEMS syndrome who developed arterial thrombosis attributed to essential thrombocythaemia. However, the development of myeloproliferative and plasma cell disorder in the same patient is rare because of the rarity of each of these conditions.…”

Section: Discussionmentioning

confidence: 99%

Young patient with arterial thrombosis and skin changes as the onset manifestations: POEMS syndrome

et al. 2016

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POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Arterial thrombosis is a distinctively unusual feature in patients with POEMS syndrome. We report a 33-year-old man with intermittent amaurosis of left eye and skin changes as the onset manifestations, who was finally confirmed as having POEMS syndrome. Most notably, this was a young man without high risk factors of arterial thrombosis and no monoclonal protein was detected until the repeated measurement later. This case evokes the need to consider the diagnosis of POEMS syndrome for young patients with symptoms of arterial thrombosis but no high risk factors of thrombosis.

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Section: Discussionmentioning

confidence: 99%

Young patient with arterial thrombosis and skin changes as the onset manifestations: POEMS syndrome

et al. 2016

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“…The vertebral signal abnormalities observed in the spinal MRI do not contradict this diagnosis [13]. In addition to several single case reports [1,2,7,8,10,17,19,20,22,23,29], two larger series have demonstrated the high frequency of moderate thrombocytosis in POEMS syndrome [4,24]. Nevertheless, the unique and paradoxical combination of vitamin B 12 deficiency, which is usually associated with thrombocytopenia [9], and thrombocytosis has been reported only once in POEMS syndrome before [28].…”

Section: Discussionmentioning

confidence: 99%

Multiple vascular abnormalities and a paradoxical combination of vitamin B 12 deficiency and thrombocytosis in a case with POEMS syndrome

Lewerenz

Gocht

Hoeger

et al. 2003

Journal of Neurology

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POEMS/Crow-Fukase syndrome is a rare multisystem disorder associated with elevated vascular endothelial growth factor (VEGF), which clinically presents with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. We report a case of POEMS syndrome due to a gammopathy of undetermined significance with thrombocytosis, vitamin B(12) deficiency, highly elevated VEGF and in addition to glomeruloid angiomas two previously undescribed proliferative vascular lesions: a spinal arteriovenous fistula and a plexogenic pulmonary arteriopathy, which ultimately resulted in lethal pulmonary hypertension. We assume that the high VEGF levels caused the vascular abnormalities observed in our patient.

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“…7,42,46 Both arterial and venous thromboses have been described in patients with POEMS syndrome. In our series, there were 18 [47][48][49][50][51][52][53][54][55][56][57][58][59][60] Vasospastic angina, 61,62 pulmonary embolism, and Budd-Chiari syndrome also have been described. 63,64 In 4 of our patients, renal failure developed as a preterminal event.…”

Section: Less-recognized Featuresmentioning

confidence: 95%

POEMS syndrome: definitions and long-term outcome

Dispenzieri

Kyle

Lacy

et al. 2003

Blood

705

806

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The POEMS syndrome (coined to refer to polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) remains poorly understood. Ambiguity exists over the features necessary to establish the diagnosis, treatment efficacy, and prognosis. We identified 99 patients with POEMS syndrome. Minimal criteria were a sensorimotor peripheral neuropathy and evidence of a monoclonal plasmaproliferative disorder. To distinguish POEMS from neuropathy associated with monoclonal gammopathy of undetermined significance, additional criteria were included: a bone lesion, Castleman disease, organomegaly (or lymphadenopathy), endocrinopathy, edema (peripheral edema, ascites, or effusions), and skin changes. The median age at presentation was 51 years; 63% were men. Median survival was 165 months. With the exception of fingernail clubbing (P ‫؍‬ .03) and extravascular volume overload (P ‫؍‬ .04), no presenting feature, including the number of presenting features, was predictive of survival. Response to therapy (P < .001) was predictive of survival. Pulmonary hypertension, renal failure, thrombotic events, and congestive heart failure were observed and appear to be part of the syndrome. In 18 patients (18%), new disease manifestations developed over time. More than 50% of patients had a response to radiation, and 22% to 50% had responses to prednisone and a combination of melphalan and prednisone, respectively. We conclude that the median survival of patients with POEMS syndrome is 165 months, independent of the number of syndrome features, bone lesions, or plasma cells at diagnosis. Additional features of the syndrome often develop, but the complications of classic multiple myeloma rarely

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POEMS syndrome, arterial thrombosis and thrombocythaemia

Cited by 22 publications

References 15 publications

Young patient with arterial thrombosis and skin changes as the onset manifestations: POEMS syndrome

Young patient with arterial thrombosis and skin changes as the onset manifestations: POEMS syndrome

Multiple vascular abnormalities and a paradoxical combination of vitamin B 12 deficiency and thrombocytosis in a case with POEMS syndrome

POEMS syndrome: definitions and long-term outcome

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