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Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome.

Kumar S, Sharma S - Oxf Med Case Reports (2015)

Bottom Line: Immunoelectrophoresis using the immunofixation method revealed lambda chain monoclonal gammopathy.The patient was given radiotherapy, followed by a combination therapy of melphalan and dexamethasone.We emphasize the importance of recognizing a challenging diagnosis of a rare disease, which is shown to be treatment responsive.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurology , Sawai Man Singh Medical College , Jaipur, Rajasthan , India.

ABSTRACT
POEMS syndrome (Crow-Fukase syndrome) is a rare paraneoplastic disorder. It is characterized by peripheral neuropathy, elevated vascular endothelial growth factors (VEGFs), monoclonal gammopathy, sclerotic bone lesions and Castleman disease. Other important clinical features are organomegaly, edema, ascites, papilledema, endocrinopathy, skin changes and thrombocytosis. A high index of suspicion, a detailed clinical history and examination followed by appropriate laboratory investigations like VEGF level, radiological skeletal survey and bone marrow biopsy are required to diagnose POEMS syndrome. We report a case of POEMS syndrome who presented with insidious onset, progressive sensorimotor polyneuropathy, pedal edema, ascites, hepatomegaly, skin changes and hypothyroidism. X-ray of the pelvis showed osteosclerotic lesions. Immunoelectrophoresis using the immunofixation method revealed lambda chain monoclonal gammopathy. The patient was given radiotherapy, followed by a combination therapy of melphalan and dexamethasone. We emphasize the importance of recognizing a challenging diagnosis of a rare disease, which is shown to be treatment responsive.

No MeSH data available.


Related in: MedlinePlus

Photographs of patient showing pitting edema over the left legs [thick arrow (a)]. Hyperpigmented skin is seen over the lower limbs, hands and face [thin arrows (a), (c) and (d)]. The photograph also showing abdominal distention (b). Free fluid was confirmed by abdominal ultrasonography.
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OMV023F1: Photographs of patient showing pitting edema over the left legs [thick arrow (a)]. Hyperpigmented skin is seen over the lower limbs, hands and face [thin arrows (a), (c) and (d)]. The photograph also showing abdominal distention (b). Free fluid was confirmed by abdominal ultrasonography.

Mentions: On physical examination, bilateral pedal edema was present (Fig. 1a and b). Skin was thickened and hyperpigmented over the face, fingers of the hands and shin (Fig. 1a–d). Bilateral gynecomastia and testicular atrophy were also present. Abdominal examination showed hepatomegaly and ascites. Higher mental functions were normal. Examination of the fundi revealed bilateral papilledema, but otherwise cranial nerve examination was unremarkable. Muscle power in upper limbs was normal and predominant distal weakness [Medical Research Council (MRC) grade 4/5 at hip joints and 4-/5 at ankle joints] was present in lower limbs. Deep tendon reflexes in the upper limb were diminished (+1) including biceps, triceps and supinator and absent in lower limbs (knee and ankle). All modalities of sensations including pain, touch, temperature, vibration and joint position senses were impaired below knees. Romberg's sign was positive.Figure 1:


Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome.

Kumar S, Sharma S - Oxf Med Case Reports (2015)

Photographs of patient showing pitting edema over the left legs [thick arrow (a)]. Hyperpigmented skin is seen over the lower limbs, hands and face [thin arrows (a), (c) and (d)]. The photograph also showing abdominal distention (b). Free fluid was confirmed by abdominal ultrasonography.
© Copyright Policy - creative-commons
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4664845&req=5

OMV023F1: Photographs of patient showing pitting edema over the left legs [thick arrow (a)]. Hyperpigmented skin is seen over the lower limbs, hands and face [thin arrows (a), (c) and (d)]. The photograph also showing abdominal distention (b). Free fluid was confirmed by abdominal ultrasonography.
Mentions: On physical examination, bilateral pedal edema was present (Fig. 1a and b). Skin was thickened and hyperpigmented over the face, fingers of the hands and shin (Fig. 1a–d). Bilateral gynecomastia and testicular atrophy were also present. Abdominal examination showed hepatomegaly and ascites. Higher mental functions were normal. Examination of the fundi revealed bilateral papilledema, but otherwise cranial nerve examination was unremarkable. Muscle power in upper limbs was normal and predominant distal weakness [Medical Research Council (MRC) grade 4/5 at hip joints and 4-/5 at ankle joints] was present in lower limbs. Deep tendon reflexes in the upper limb were diminished (+1) including biceps, triceps and supinator and absent in lower limbs (knee and ankle). All modalities of sensations including pain, touch, temperature, vibration and joint position senses were impaired below knees. Romberg's sign was positive.Figure 1:

Bottom Line: Immunoelectrophoresis using the immunofixation method revealed lambda chain monoclonal gammopathy.The patient was given radiotherapy, followed by a combination therapy of melphalan and dexamethasone.We emphasize the importance of recognizing a challenging diagnosis of a rare disease, which is shown to be treatment responsive.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Neurology , Sawai Man Singh Medical College , Jaipur, Rajasthan , India.

ABSTRACT
POEMS syndrome (Crow-Fukase syndrome) is a rare paraneoplastic disorder. It is characterized by peripheral neuropathy, elevated vascular endothelial growth factors (VEGFs), monoclonal gammopathy, sclerotic bone lesions and Castleman disease. Other important clinical features are organomegaly, edema, ascites, papilledema, endocrinopathy, skin changes and thrombocytosis. A high index of suspicion, a detailed clinical history and examination followed by appropriate laboratory investigations like VEGF level, radiological skeletal survey and bone marrow biopsy are required to diagnose POEMS syndrome. We report a case of POEMS syndrome who presented with insidious onset, progressive sensorimotor polyneuropathy, pedal edema, ascites, hepatomegaly, skin changes and hypothyroidism. X-ray of the pelvis showed osteosclerotic lesions. Immunoelectrophoresis using the immunofixation method revealed lambda chain monoclonal gammopathy. The patient was given radiotherapy, followed by a combination therapy of melphalan and dexamethasone. We emphasize the importance of recognizing a challenging diagnosis of a rare disease, which is shown to be treatment responsive.

No MeSH data available.


Related in: MedlinePlus