Osteosclerotic Myeloma with Polyneuropathy

Abstract

Five cases of histologically verified plasma cell myeloma in sclerotic skeletal foci and polyneuropathy are reported. Thirty similar cases were collected from the literature. They illustrate a special form of plasma cell neoplasia. The characteristic features are osteosclerosis, polyneuropathy resembling polyradiculitis, approximately normal hemoglobin concentration, bone marrow smears and ESR, low concentration of M-protein, and absence of Bence-Jones' proteinuria. Slow progression of the disease is a possible additional feature. It is hypothesized that autoimmune mechanisms are involved in the pathogenesis. This hypothesis is based on the observation of circulating immune complexes, positive Waaler's reaction and relative increase in the number of circulating B-lymphocytes.

Successful Treatment of POEMS Syndrome Complicated by Severe Congestive Heart Failure with Thalidomide

“…Although the relationship has not been fully elucidated, marked improvement of CHF has been reported along with the treatment of POEMS syndrome in most of these patients (13,(15)(16)(17)(18). As the pathogenesis of CHF in these patients, ischemic or hypertrophic cardiomyopathy has been suggested in previous reports (14,15,18 (19,20 …”

“…[17] [16] [15] [14] veloped CHF with the progression of POEMS syndrome (2). Although the relationship has not been fully elucidated, marked improvement of CHF has been reported along with the treatment of POEMS syndrome in most of these patients (13,(15)(16)(17)(18).…”

How I treat POEMS syndrome

“…42 For this first group, radiation doses of 40 Gy are most standard because the goal is potential cure rather than mere palliation. 9,[43][44][45][46] If a patient is rapidly deteriorating, simultaneous use of corticosteroids (eg, dexamethasone 40 mg days 1-4 every 2 weeks or daily prednisone at ϳ 1 mg/kg) is reasonable as adjuvant therapy. The corticosteroids can be tapered over the ensuing months, but be vigilant looking for signs of adrenal insufficiency, especially if they had decreased adrenal reserve at diagnosis.…”

POEMS syndrome: 2011 update on diagnosis, risk‐stratification, and management

“…A starting dose of prednisone ranging between 20 and 1,500 mg/day has been used. No evidence based recommendation can be given as to the appropriate dose, but personal experience would place the daily starting anywhere between 1 and 2 [39,[148][149][150][151] 50% of patients have significant clinical improvement Melphalan-Dexamethasone [146] 81% hematologic response rate; 100% with some neurologic improvement Corticosteroids [3,39,55,73,152] 15% of patients have significant clinical improvement High-dose chemotherapy with PBSCT [52,124,[130][131][132][133][134][135][136][137]139,[153][154][155][156][157][158] 100% of surviving patients have significant clinical improvement Thal after MP [159] No hematological response but improved ascites; stabilized PN, splenomegaly, pulmonary hypertension Thal 1 Dex after CAD [160] CD/POEMS: improved ascites, effusions, pulmonary hypertension, PN, renal function, IL-6 level Thal 1 Dex [161] N 5 9; VEGF improved in all; PN improved in 66%; stable in 33%; improved edema; no HCR Thal after VAD, CTX, bevacizumab [23] Improved cardiopulmonary status, but no improved PN and rising VEGF Lenalidomide 1 Dex [140] Improved ascites, PS, PN, VEGF, testosterone, pulmonary function tests Lenalidomide1 Dex [141] N59; all had hematologic response; clinical responses in all evaluable patients including PS, neurological syndrome, edema, and VEGF. Bortez1AD after VAD, CMP, and AD [142] Improved M-protein, VEGF, paresthesias, splenomegaly, effusions, muscle strength, gynecomastia, and skin changes...…”