POEMS Syndrome Symptoms and Treatment

POEMS Syndrome Symptoms and TreatmentPOEMS Syndrome Paraproteinemic neuropathies, Organomegaly, Endocrinopathy, M-protein and Skin changesAbstractThe POEMS syndrome, also known as Crow-Fukase syndrome, is a rare multi organ discommode characterized by polyneuropathy, organomegaly, endocrinopathy, M-protein spike and clamber changes. new(prenominal) associated features, such as sclerotic bone lesions, edema, ascites, hematological disorders and Castleman disease clear also be feed. We report a case of POEMS syndrome who presented with insidious onset, continuous tense sensorimotor polyneuropathy, pedal edema, ascites, hepatomegaly along with skin changes. X-ray hip showed osteosclerotic lesions. Thyroid function analyses showed hypothyroidism. M-protein (IgG) monoclonal exercise set was seen on immunoelectrophoresis. The patient role was started on melphalan and corticosteroid combination therapy. We emphasis on the importance of recognizing a challenging diagnosis of a rare disease, which is shown to be treatment responsive. doorPOEMS syndrome is a rare paraneoplastic disorder of plasma cellular phone dyscrasias, which was first exposit in 1956 by Crow and then in 1968 by Fukase 1. The parent POEMS was given to it by Bardwick and co-workers in 1980 based on five salient features polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes 1. It is more than prevalent in men, with manly to feminine ratio of 2.51. It normally manifests in 5th and 6th ten dollar bills of life. Its inheritance is chatoyant and its pathophysiology is still non well understood.Case PresentationA 40-year-old male presented with progressive weakness, tingling and numbness sensation in both write down limbs for two years. He had swelling of lower limbs, abdominal dilation and dermatologic changes in form of discoloration and thickening of skin everyplace the cheeks, nose, pass on and feet for one year. He had also gave history of erectile dysfunction and waiver of libido for six months. There was no history of syncope, bony pain or drug abuse. He had no previous history of hypertension, diabetes or tuberculosis.On physical examination, the patient had bilateral pedal edema direct 1.c and abdominal distention designing 2.a. Skin was thickened and hyperpigmented over the face, fingers of the hands and shin (Figure.1a,b,c). Bilateral gynecomastia and testicular atrophy were present. Abdominal examination showed hepatomegaly and ascites. Higher mental functions and terminology were normal. Fundus examination showed papilledema on both side and rest of the cranial restiveness examination were normal. Motor power in upper limbs was normal and in lower limbs showed predominant distal weakness Medical Research Council (MRC) 4/5 at hip joint and 4-/5 at ankle joint). latterly tendon reflexes in upper limb were diminished (+1) including biceps, triceps and supinator and absent in lower limbs. There was 30% loss in pain, touch and temperature virtuoso in both lower limbs below knees. Posterior column sensations (joint rate and vibration sense) were also impaired in lower limbs below the precedent superior iliac spine. Romberg sign was positive.Hemogram, liver and renal function tests, vigor enzymes (creatine phosphokinase), serum ferritin and vitamin B12 level were normal. Serum total protein was 7.1 gm/dL, albumin 3 gm/dL, globulin 4.1 gm/dl, and AG ratio 11.3. fasting and postprandial blood sugar level were normal. Thyroid function test showed raised TSH level (16.62 /ml). Luteinizing hormone (LH) and testosterone levels were 15 IU/L (1.8-8.6 IU/L) and 111 ng/L (300-1,000 ng/dL), respectively. Anti atomic antibody (ANA), Rheumatoid factor out (RF), serum human immunodeficiency computer virus (HIV) ELISA test, hepatitis B and C markers were negative. Ultrasonography of abdomen showed hepatomegaly (16 cm), conquer ascites and enlargement of multiple lymph nodes along the iliac vessels. Fine n eedle determination cytology of mesenteric lymph node was inconclusive. Ascitic fluid examination revealed exudative nature (SAAG passwordPOEMS syndrome is a rare, multiple system disorder, characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal or M-protein band and skin changes. Any three of the five features may be present to establish diagnosis 2. However, some authors fuck off proposed clinical criteria for diagnosis in which includes two major criteria, which can be either presence of polyneuropathy or plasma cell proliferative disorder. Minor criteria include sclerotic bone lesions, organomegaly, edema, endocrinopathy, papilledema or skin changes 2.Polyneuropathy is a predominant feature of POEMS syndrome and is found in 90% of the cases. It is usually a sensorimotor, axonal and demyelinating type polyneuropathy 3. As in our patient, both axonal and demyelinating polyneuropathy are seen on electrodiagnostic studies. The appliance of neuropathy is not kn own but the young evidence of the presence of anti-neural antibodies points to an immunological mechanism 4. Endocrinopathies occur with a relative oftenness of 60%-80% and the most common are gonadal failure (70%) and glucose fanaticism/diabetes mellitus (50%).Hypo or hyperthyroidism, hyperprolactinemia and adrenal insufficiency have also been reported. The mechanism of endocrinopathy is also not obvious however, involvement of direct acting antibodies against hypothalamo-hypophyseal-axis and hormone end organs has been hypothesized 5. In our patient, impotence, loss of libido and testicular atrophy and hypothyroidism on ancillary laboratory investigation were present.Increased levels of vascular endothelial step-up factor (VEGF) are found in POEMS syndrome. VEGF subjoins microvascular permeability, thereby inducing edema, ascites and pleural effusions as were present in our patient 6,7. However, measurement of VEGF level in ascites was not available in our patient. Papillede ma may be seen in well-nigh 37% of patients and is not associated with the increase in intracranial pressure. The real understanding of papilledema is not still known. Hepatomegaly may be seen in up to 50% of patients with splenomegaly and lymphadenopathy occurring less often. The hyperpigmentation over the face, legs and hands was also observed in our case. The skin changes usually observed in POEMS syndrome are hyperpigmentation, lichenification, hypertrichosis, sclerodermoid changes and glomeruloid hemangiomas. Skin biopsy may show inflammation, fibrosis, or nonspecific changes. Monoclonal protein is detected in more than 90% of patients and may become positive in the follow-up of patients who have no monoclonal gammopathy initially 8.Nearly all cases reported in the literature show lambda positivity as in our patient. It may be rarely found in urine and cerebrospinal fluid (CSF). The instinctive course of POEMS syndrome is chronic, with a reported median survival for a decade (8-13.8ys). The morbidity depends on the extent and number of systems involved. The cause of POEMS syndrome is still unknown. It is alluring to incriminate the presence of lambda light chains in the pathogenesis because of their unexpected frequency (more than 95% of patients), but histopathologic review of affected organs and nerves does not support that it is a form of deposition disorder. Increased levels of cytokines IL-1, TNF- and IL-6, more specifically VEGF, appear to play a pathogenic role in the disorder 9,10.In view of the constellation of a mixed polyneuropathy, monoclonal gammopathy, osteosclerotic myeloma, extravascular meretriciousness overload, bilateral papilledema, skin changes and endocrinopathies, our patient was diagnosed as POEMS syndrome. However, some other close first derivative diagnosis like tuberculosis and hemochromatosis were ruled out with fascinate investigations. Patient was treated with combination of alkylating agent melphalan and corticosteroi d. To conclude, when a patient present with unexplained sensorimotor polyneuropathy, signs of extravascular volume overload and evidence of other system involvement, a high index of suspicion should be unploughed for a diagnosis of POEMS syndrome, to avoid missing this rare syndrome, which is amenable to treatment.Figure LegendsFigure 1. Photographs of patient showing skin hyperpigmentation over the face, hand and limbs (thin arrows). impenetrable arrow showing pitting edema over the left leg.Figure 2. Photograph of patient (a) showing abdominal distention (free fluid was confirm by ultrasonography). X-ray pelvic bone (b) showing multiple sclerotic lesion over right iliac crest (thin arrow) and one large osteosclerotic lesion (thick arrow) over the neck of left femur (b).Figure 3. Nerve conduction show showing axonal and demyelinating neuropathy in right median nerve.Figure 4. swot marrow smear in centre reveals one large irregular plasma cell, which has prominent nucleoli and abundant cytoplasm. There is loss of normal nuclear configuration with fraying border.ReferencesBardwick PA, Zvaifler NJ, Gill GN, Newman D, Greenway GD, Resnick DL. Plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, M proteins and skin changes the POEMS syndrome Report on two cases and a review of the literature. 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