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Plasma protein electrophoresis before and after treatment with prednisone and melphalan. The narrowbased peak, formerly evident in the y-globulin region has disappeared.
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A new syndrome has been described comprising polyneuropathy, oedema, hyperpigmentation and thickening of the skin, gynaecomastia in males and amenorrhoea in females, monoclonal gammopathy, papilloedema and diabetes. There is frequent osteosclerosis with or without plasmacytoma, hepatosplenomegaly and polycythaemia. There is a good response to corti...
Citations
... Suele asociarse a mieloma osteoscleroso, cursa con paraproteinemia (pp) inferior a la del mieloma clásico y afecta a adultos jóvenes 10 . Es de curso más indolente, con mayor presencia de síntomas paraneoplásicos, menor frecuencia de dolor óseo, habiéndose descrito también en Europa y Norteamérica [13][14][15][16][17][18][19][20] . Las adenopatías generalizadas pueden encontrarse hasta en un 65% de los casos, especialmente en los japoneses 12 , asociados o no a lesiones óseas, y con ap de enf de Castleman 12,21,22 . ...
Plasma cell myeloma or multiple myeloma is a disease resulting from the proliferation in the bone marrow of a clone of neoplastic cells that are closely related, both morphologically and functionally, to plasma cells. The term plasma cell leukaemia may be used to designate a de novo leukaemia or the terminal phase of myeloma when neoplastic cells are present in the peripheral blood in large numbers. Patients with de novo or primary plasma cell leukaemia show clinical features that are common in myeloma such as bone pain, lytic lesions, hypercalcaemia and renal failure but they have a higher incidence of extramedullary lesions and, in addition, often have hepatomegaly and splenomegaly. Bone marrow biopsy can be of use both in the diagnosis of myeloma and in assessing the prognosis. A trephine biopsy at diagnosis is recommended since it may be needed as a baseline to assess a post‐treatment biopsy when no adequate aspirate is obtained.
Multiple myelomaOther syndromes associated with secretion of a paraprotein
Other syndromes associated with secretion of a paraproteinReferences
objective: To determine whether there are peculiarities of the POEMS syndrome (a multisystemic disorder associated with polyneuropathy, organomegaly, endocrinopathy of various forms, production of a monoclonal [M] component, and skin changes) in Caucasian patients, especially signs and symptoms absent in other series; and to attempt a reappraisal of the neuropathy and endocrinopathy to find a unifying mechanism.design: A retrospective, cooperative study compared 25 cases, observed over a 15-year period, with two published series of patients, one of Japanese patients and one of American patients, and with a review of the literature on non-Asian cases. Details were obtained of patients' medical history, physical examination, immunochemical and hormonal testing, roentgenographic examination, computed tomography imaging, and electromyography.results: The main features of the syndrome found in these patients were those first described in Japan: polyneuropathy, enlargement of the lymph nodes, liver, and spleen, endocrine disturbances, low concentration of the monoclonal component, hyperpigmentation, and hypertrichosis. Three other symptoms were found more frequently than previously reported: skin angiomas, scleroderma changes of the hands, and thrombocytosis. Electromyography and nerve biopsy showed a variety of abnormalities ranging from demyelination to axonal degeneration. Nerve deposits of immunoglobulin were absent. Organomegaly seemed to be heterogeneous. Pathologic findings in the enlarged lymph nodes and spleen were compatible with Castleman's disease. Liver biopsies were usually normal. The endocrine changes were surprisingly diverse, with some observations combining unrelated primary and secondary insufficiencies. No single hypothesis emerged regarding their mechanism. All M components had a λ-light chain. An IgG M component was found more frequently in solitary lesions. An IgA M component was found more frequently in patients without bone lesions. Sedimentation rate was usually normal. Radiotherapy of solitary plasmacytomas was followed by dramatic improvement of extramedullary signs and symptoms in all cases.conclusions: The symptoms, clinical course, and management of the patients reported here were similar to those observed in the literature. This study confirms the existence of a close link between symptoms and λ-light-chain production. There are indications that a plasma cell growth factor that does not cross-react with interleukin-6 (IL-6) may be involved.
A 40 year old Chinese woman presented with anasarca and later developed features of POEMS syndrome. These are peripheral neuropathy (P), organomegaly (O), endocrine dysfunction (E), monoclonal gammopathy (M), and skin changes (S) which are usually associated with plasma cell dyscrasia. In our patient, monoclonal gammopathy was not detected on immunofixation electrophoresis and was revealed only after analysis of kappa/lambda light chain ratio of the raised serum IgA immunoglobulin. Needle liver biopsy of her grossly enlarged liver showed marked accumulation of glycogen and presence of giant mitochondria in the hepatocytes, a feature not previously reported.
A patient with classical POEMS syndrome associated with a myeloproliferative disorder is presented, in whom extensive arterial calcification was seen at an early age in the absence of other predisposing factors. Radiographs also demonstrated several small discrete sclerotic foci elsewhere in the skeleton and areas of new bone formation around the spine which were described in previous cases. Histological studies in this patient demonstrated an osteosclerotic solitary plasmacytoma of bone and Castleman's disease of lymph nodes, both of which have previously been causally associated with POEMS syndrome. In addition, dermal infiltration by mast cells was demonstrated.
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.
