A case of POEMS syndrome associated with essential thrombocythaemia and dermal mastocytosis.

We describe a case of POEMS syndrome presenting with the recognized features of polyneuropathy, organomegaly, endocrine abnormalities, monoclonal protein, skin changes and anasarca. The patient was found to have both a solitary sclerotic plasmacytoma of the pelvis and evidence of Castleman's disease of lymph nodes. A number of unusual and unique features are also documented. Histological examination of affected skin demonstrated changes similar to urticaria pigmentosa including local oedema and mast cell infiltration. There was marked thrombocythaemia which has been seen in only one previous case and in addition the patient developed diffuse vascular calcification in the absence of recognized aetiological factors. Radiotherapy of the pelvic lesion and chemotherapy to control the myeloproliferative disorder gave rise to significant improvement in neuropathy. Control of anasarca required steroid therapy in addition to diuretics. The significance of these observations is discussed in relation to previous reports

Interaction of Metal Cations with Alkylnitriles in the Gas Phase: Solvation of Metal Ions by the Hydrocarbon Chain

Relative affinity measurements of monovalent metal ions (M=Li+, Na+, Cu+ and Ag+) toward aliphatic nitriles have been performed using the kinetic method by dissociation of metal-bound dimer ions of the type R1C equivalent to N-M+-N equivalent to CR2. It was found, particularly for Cu+ and Ag+, that the affinity toward nitriles having long chains (>C-6) is markedly enhanced. This is attributed to a bidentate interaction of the metal ion with the nitrile moiety and the aliphatic chain. Theoretical calculations on the copper complexes show that these bidentate structures enjoy about 30% greater copper-ion affinities compared to their linear counterparts. Such aliphatic interactions also play a major role in the dissociation chemistry of copper-bound tetramers of the kind (RC equivalent to N)(4)Cu2+center dot where the long aliphatic chain R curls around the copper ion to facilitate electron transfer or a redox reaction to produce (RC equivalent to N)(2)Cu+ + RC equivalent to N+center dot + RC equivalent to N