Crow-Fukase Syndrome Associated with High-Output Heart Failure.

Abstract

A 64-year-old womanwas admitted with systemic edema and exertional dyspnea. High-output heart failure was diagnosed by right heart catheterization and she was treated with diuretics. After 3 weeks, her symptoms disappeared but a high cardiac output state persisted. A diagnosis of Crow-Fukasesyndromewas madebased on the presence of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes. Her serum vascular endothelial growth factor (VEGF) level was markedly elevated after recovery from heart failure. Wesuspect that an elevated VEGFlevel and a high cardiac output state mayplay a role in the pathogenesis of heart failure in Crow-Fukase syndrome. (Internal Medicine 41: 638-641, 2002)

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

“…Judging from the present and previous histopathological findings, it could be concluded that the pathogenesis of PH in plasma cell disorders (PCDs) is heterogeneous. On the other hand, in most cases of the POEMS syndrome presenting with PH, plasma cells were found to almost exclusively have lambda-type light chains [2, 8, 9, 17, 18, 22, 24]. Although our case is unusual in that the light chain was of kappa type, it is congruous with the results of other studies dealing with plasma cell myeloma and PH [28, 29].…”

“…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].…”

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.…”

Pulmonary Hypertension and Refractory Heart Failure in a Patient with Crow-Fukase (POEMS) Syndrome

“…The transpulmonary pres- sure gradient (mean pulmonary arterial pressure-mean pulmonary capillary wedge pressure) was 19 mmHg, and the patient was thought to be out of proportion due to pulmonary hypertension caused by left heart disease with diastolic dysfunction (11). Only a few patients with pulmonary hypertension with Crow-Fukase syndrome have undergone cardiac catheterization, and most of them had normal pulmonary capillary wedge pressures (12)(13)(14)(15)(16). As far as we know, there are only two case reports of out of proportion pulmonary hypertension in patients with Crow-Fukase syndrome (17,18), and one of these patients had left ventricular hypertrophy (18).…”