RESUMEN
@#<p style="text-align: justify;" data-mce-style="text-align: justify;"><span style="color: #525a63; font-family: Lato, sans-serif; font-size: 16px; font-style: normal; font-variant-ligatures: normal; font-variant-caps: normal; font-weight: 400; letter-spacing: normal; orphans: 2; text-align: start; text-indent: 0px; text-transform: none; white-space: normal; widows: 2; word-spacing: 0px; -webkit-text-stroke-width: 0px; background-color: #ffffff; text-decoration-thickness: initial; text-decoration-style: initial; text-decoration-color: initial; display: inline !important; float: none;" data-mce-style="color: #525a63; font-family: Lato, sans-serif; font-size: 16px; font-style: normal; font-variant-ligatures: normal; font-variant-caps: normal; font-weight: 400; letter-spacing: normal; orphans: 2; text-align: start; text-indent: 0px; text-transform: none; white-space: normal; widows: 2; word-spacing: 0px; -webkit-text-stroke-width: 0px; background-color: #ffffff; text-decoration-thickness: initial; text-decoration-style: initial; text-decoration-color: initial; display: inline !important; float: none;">POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) also known as Crow-Fukase syndrome or Takatsuki syndrome is a rare and disabling paraneoplastic syndrome that frequently occurs in the fifth or sixth decade of life without a known standard first-line therapy. A 34-year-old Filipino male nurse, who presented with gradually progressive distal lower extremity weakness and sharp shooting pain in bilateral legs accompanied by mild joint effusion on the left knee, hypertrichosis, bilateral conjunctival injection, and gynecomastia underwent extensive workup and was diagnosed with POEMS syndrome. Complete blood count revealed erythrocytosis and thrombocytosis with elevated serum VEGF (vascular endothelial growth factor) and elevated monoclonal serum free lambda light chains. The electrophysiologic studies revealed chronic demyelinating sensorimotor polyneuropathy while bone marrow core biopsy and bone marrow aspirate smear immunohistochemical staining showed it to be positive for lambda and CD138. He had an initial unsuccessful treatment course with melphalan and prednisone. Hence, bortezomib and dexamethasone were given which gave significant improvement in symptoms from the overall neuropathy limitation score of 5 to 1.</span></p>
RESUMEN
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is an autoimmune condition that primarily targets smallto medium sized vessels in multiple organ systems, with sinonasal, pulmonary and renal involvement being common.The present case study discusses a known case of ANCA associated vasculitis, type 2 diabetes and hypertension, whopresented with altered sensorium and weakness of the left side of the body. Few months before the onset of symptoms,the patient received rituximab injection for treating cutaneous panniculitis and high pANCA titers. MRI of brain and CECTof chest were suggestive of vasculitis and blood culture showed the growth of Burkholderia cepacia. She was treated withbroad spectrum antibiotics and other supportive treatment. Despite all the efforts including ionotropic and ventilatorsupports, the patient succumbed to the infection.
RESUMEN
POEMs syndrome is a rare paraneoplastic disorder related to plasma cell dyscrasia. The present study deals with anunusual case of POEMs syndrome in a young male who came with ascending demyelinating polyneuropathy, resultingin complete quadriparesis. The diagnosis was based on clinical findings of gynecomastia, hyperpigmentation andhypertrichosis of skin and lymphadenitis. Detailed laboratory investigations and imaging confirmed POEMS syndrome.In view of the rapidly progressing polyneuropathy, the patient was treated with chemotherapy followed by stem celltransplantation. The patient recovered completely after the treatment.
RESUMEN
Crow-Fukase syndrome is diagnosed by the presence of polyneuropathy in conjunction with several other characteristic generalized symptoms, including organomegaly, endocrinopathy, M protein, and skin changes (POEMS syndrome). Rehabilitation is very important for patients with Crow-Fukase syndrome because progressive polyneuropathy reduces a patient's muscle strength. We report a case of long-term rehabilitation after high-dose chemotherapy with autologous peripheral blood stem cell transplantation. After transplantation, neurologic improvement began, and following rehabilitation therapy, the patient's proximal lower extremity muscle strength recovered to an almost normal level. At 2 months after transplantation, the patient could walk again using a cane and an ankle-foot orthosis. At six and a half years after transplantation, the neuropathy was still improving and there was no recurrence of other symptoms. We suggest that rehabilitation combined with autologous peripheral blood stem cell transplantation for Crow-Fukase syndrome is very useful for improving the disuse condition and for recovering muscle strength.
RESUMEN
Os autores relatam o caso de um paciente do sexo masculino com astenia, edema, polineuropatia, hepatoesplenomegalia, linfadenopatia, múltiplas disfunções endócrinas, alterações de pele, lesões osteoescleróticas e discreto pico monoclonal de IgA e cadeias leves do tipo lambda. Obteve-se o diagnóstico de síndrome de POEMS. A síndrome de POEMS, apesar de rara, deve entrar no diagnóstico diferencial de diversas doenças multissistêmicas (AU)
A case of a man with asthenia, edema, polyneuropathy, hepatosplenomegaly, lymphadenopathy, multiple endocrine disfunctions, skin changes, sclerotic bone lesions and mild IgA and lambda light chains monoclonal spike. The diagnostic of POEMS syndrome was made. POEMS Syndrome, although rare, must be included in the differential diagnosis of many multisistemic disorders (AU)