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

Abstract

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.

Recurrent Crow-Fukase Syndrome Associated with Increased Serum Levels of Vascular Endothelial Growth Factor: A Case Report and Review of the Literature

“…In the previous 17 out of 27 case reports, the VEGF level decreased and the symptoms improved after treatment (Watanabe et al 1996(Watanabe et al , 1998Arimura et al 1997;Soubrier et al 1998;Henzan et al 1999;Niimi et al 2000;Ichikawa et al 2001;Shinde et al 2001;Araki et al 2002;Endo et al 2002;Jaccard et al 2002;Koga et al 2002;Minamitani et al 2002;Lewerenz et al 2003;Takahashi et al 2003;Takakura et al 2003;Tokashiki et al 2003;Baba et al 2004;Matsui et al 2004;Ozaki et al 2004;Takai et al 2004;Yoritaka et al 2004;Badros et al 2005;Imai et A major criterion and 3 minor criteria are needed for the diagnosis of CFS (Arimura et al ). al.…”

Severe Pulmonary Hypertension Caused by Smoldering Plasma Cell Myeloma: An Autopsy Case of POEMS Syndrome

“…Lesprit et al [8] investigated further 5 patients with high serum concentrations of IL-1 β , IL-6, TNF- α , and VEGF. Isolated increase of serum VEGF has been reported in a number of cases of POEMS syndrome presenting with PH [9, 11, 18] and the serum level of VEGF became normalized in association with amelioration of PH [11]. In our case, involvement of such cytokines in the development of PH was unclear because any such cytokines were not checked antemortem.…”

“…Many anecdotal case reports dealing with this condition have been published [9–12, 15–24] and some studies indicate that the incidence of PH in the POEMS syndrome is 5 to 40% [2, 3, 8]. Although detailed mechanisms are unknown, some cytokines are reported to be involved in the pathogenesis [8–12, 18, 22, 25]. Rose et al [22] found elevated serum levels of IL-6 in 2 patients with POEMS syndrome presenting with PH.…”

“…Regarding histopathological examinations on PH in POEMS syndrome patients, only a few limited studies have so far been performed [8, 18]. Lesprit et al [8] reported histological findings in a single case, where eccentric intimal fibrosis, medial hypertrophy, and marked dilatation of the arteries and arterioles of the lungs were observed.…”

“…Lesprit et al [8] reported histological findings in a single case, where eccentric intimal fibrosis, medial hypertrophy, and marked dilatation of the arteries and arterioles of the lungs were observed. Lewerenz et al [18] also documented intimal fibrosis and medial hypertrophy of the pulmonary artery. These histological findings featuring plexogenic pulmonary arteriopathy mimicked the findings of idiopathic PH.…”