Desde la pérdida de la fuerza hasta el compromiso sistémico: serie de casos del síndrome POEMS / From limb weakness to a systemic involvement: Poems syndrome case two report and review of the literature

El síndrome POEMS es un trastorno paraneoplásico raro y poco frecuente, que se presenta principalmente en la sexta década de la vida, caracterizado por el compromiso multisistémico con predominio de neuropatía desmielinizante. Abarca diversas y heterogéneas manifestaciones clínicas y su diagnóstico requiere un alto índice de sospecha. Se presentan dos casos de pacientes que consultaron por cuadros poco frecuentes en los que la pérdida de la fuerza orientó al acercamiento de una afectación multisistémica que concluyó con el diagnóstico de esta enfermedad(AU)

POEMS syndrome is a rare and infrequent paraneoplastic syndrome, which occurs mainly in the sixth decade of life, characterized by multisystem involvement with a predominance of demyelinating neuropathy, which encompasses diverse and heterogeneous clinical manifestations and whose diagnosis requires a high index of suspicion. We present two cases of patients who consulted due to unusual symptoms and whose loss of strength led to an approach due to multisystem involvement that concluded with the diagnosis of this disease(AU)

Presentación de un caso de síndrome de Cooke-Apert-Gallais / Presentation of a case of Cooke-Apert-Gallais syndrome

Se estudió una paciente de 47 años de edad con antecedentes de buena salud, sin antecedentes atópicos personales ni familiares, que acude a la consulta de endocrinología por presentar aumento del vello corporal con dos años de evolución. Al examen físico se le constató rubicundez facial, escudo pubiano masculino, predominio de la cintura escapular sobre la cintura abdominal e hipertrofia de 3,5 cm del clítoris. Referente a los exámenes complementarios presentó un marcado aumento de la testosterona en sangre así que se le practicó una histerectomía con doble anisectomía y los resultados de la biopsia fueron: un tumor benigno de células de Leydig. Se concluyó que se trataba de un Síndrome de Cooke-Apert-Gallais por un tumor productor de testosterona en el ovario(AU)

A study was conducted of a female 47-year-old patient with a history of good health and no personal or family atopic antecedents, who attends endocrinology consultation due to increased growth of body hair of two years' evolution. Physical examination revealed facial reddishness, a male pubic shield, predominance of the scapular waist over the abdominal waist, and 3.5 cm hypertrophy of the clitoris. Complementary tests found a marked increase in blood testosterone, which led to the performance of hysterectomy with double adnexectomy. The result of the biopsy was benign Leydig cell tumor. The final diagnosis was Cooke-Apert-Gallais syndrome due to a testosterone-producing tumor in the ovary(AU)

Prevalence of dual endocrinopathy: hypothyroidism and gestational diabetes mellitus in patients of preeclampsia

Background: Hypertension associated with proteinuria greater than 0.3g/L in a 24-hour urine collection or 1+ by qualitative urine examination, after 20 weeks of gestation is preeclampsia. The present study was conducted to evaluate prevalence of dual endocrinopathy (hypothyroidism and gestational diabetes mellitus) in patients of preeclampsia.Methods: The observational study was conducted within a period of one year from November 2015 to October 2016 in the Department of Obstetrics and Gynaecology SMGS Hospital, GMC, Jammu. A total of 400 patients of preeclampsia were included in the study and underwent serum TSH and oral glucose tolerance test.Results: In this study 32.5% of the preeclampsia patients (130 out of 400) had hypothyroidism, 15.25% patients (61 out of 400 preeclampsia) had gestational diabetes mellitus, 9.75 % patients of preeclampsia had dual endocrinopathy  (both hypothyroidism and gestational diabetes mellitus).Conclusions: The current study concluded that dual endocrinopathy (both hypothyroidism and gestational diabetes mellitus) has substantially higher prevalence (9.75%) in the patients of preeclampsia.

Thyroid profile in type 2 diabetes mellitus

Background: Diabetes mellitus is an endocrine disorder which involves multiple organ systems and leads to significant morbidity and mortality. Diabetes mellitus has been defined as “A metabolic syndrome characterized by chronic hyperglycemia and disturbance of carbohydrate, fat and protein metabolism associated with absolute or relative deficiency in insulin secretion and or insulin action”. Thyroid diseases are also a common endocrinopathy seen in the adult population. Thyroid hormones are intimately involved in cellular metabolism. The present work is a modest attempt to study the prevalence of thyroid disorders in patients with type 2 diabetes mellitus.Methods: The study was carried out in total 108 diabetic patients without known thyroid disorder admitted in various Medical wards of R.N.T. Medical college and attached group of hospitals, Udaipur. It was a cross Sectional study done over a period of 10 months. Results: In the present study, 13% of patients with type 2 diabetes mellitus had abnormal thyroid profile. Out of which the most common presentation was sub clinical hypothyroidism found in 9.25% followed by1.9% had overt hypothyroidism and 1.9% had sub clinical hyperthyroidism. In persons with abnormal thyroid profile 85.7% were females and 14.3% were males which was statistically significant.Conclusions: Prevalence of thyroid dysfunction is common among T2DM patients and is higher in females than in males. There is no significant correlation between thyroid dysfunction and age, diabetes control, family history, type of treatment and HbA1c level in diabetic patients.

Clinical and electrophysiological profiles in early recognition of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes syndrome / 中华医学杂志(英文版)

Background@#The detection of polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome at early stage is challenging for neurologists. Since polyneuropathy could be the first manifestation, it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). The present study aimed to determine the clinical and electrophysiological features of POEMS syndrome to distinguish from CIDP.@*Methods@#The data of a group of patients with POEMS (n = 17) and patients with CIDP (n = 17) in Zhongshan Hospital Fudan University from January 2015 to September 2017 were analyzed in this retrospective study. The clinical features, neurological symptoms, and electrophysiological findings were compared between the two groups.@*Results@#Clinically, patients with POEMS demonstrated significantly more neuropathic pain in the lower extremities than patients with CIDP (58.8% vs. 11.8%, P = 0.01). Multisystem features like edema, skin change, organomegaly, and thrombocytosis were also pointed towards the diagnosis of POEMS syndrome. Electrophysiologically, terminal latency index (TLI) was significantly higher in patients with POEMS than that in patients with CIDP (median nerve: 0.39 [0.17–0.52] vs. 0.30 (0.07–0.69), Z = –2.413, P = 0.016; ulnar nerve: 0.55 [0.23–0.78] vs. 0.42 [0.12–0.70], Z = –2.034, P = 0.042). Patients with POEMS demonstrated a higher frequency of absent compound muscle action potential of the tibial nerve (52.9% vs. 17.6%, P = 0.031), less conduction block (ulnar nerve: 0 vs. 35.3%, P = 0.018), and less temporal dispersion (median nerve: 17.6% vs. 58.8%, P = 0.032) than CIDP group. The combination of positive serum monoclonal protein and high TLI (if either one or both were present) discriminated POEMS from CIDP with a sensitivity of 94.1% and 47.1% and specificity of 76.5% and 100.0%, respectively.@*Conclusions@#POEMS syndrome could be distinguished from CIDP through typical clinical and electrophysiological characteristics in practice. The combination of serum monoclonal protein and high TLI might raise the sensitivity of detecting POEMS syndrome.

Síndrome de POEMS, el desafío diagnóstico de un caso infrecuente / POEMS syndrome, the diagnostic challenge of an infrequent case / Síndrome de POEMS, o desafio diagnóstico de um caso infrequente

Se reporta el caso de un hombre de 45 años con síntomas y signos consistentes con el Sindrome de POEMS (del inglés: polineuropatía, organomegalia, endocrinopatía, gammapatía monoclonal y cambios dérmicos), un raro desorden paraneoplásico. El mismo contaba con antecedentes de tabaquismo, hipotiroidismo y últimamente había perdido 20 kg de peso. Se destaca que una historia clínica y revisión detallada seguida de estudios de laboratorio, radiología y biopsia de médula ósea, entre otros, son herramientas necesarias para reconocer los componentes de este síndrome y no demorar el diagnóstico. El paciente presentó 2 criterios obligatorios (gammapatía monoclonal y neuropatia periférica sensitivo-motora), un criterio mayor (lesión ósea) y varios criterios menores (desórdenes endocrinos, manifestaciones cutáneas, organomegalia). Actualmente se encuentra bajo supervisión hematológica y continúa su seguimiento neurológico, lo que muestra una buena respuesta a la terapia específica. Las enfermedades raras como este síndrome resultan un desafío diagnóstico para los profesionales de la salud.

The case of a 45 - year- old- man whose symptoms and signs were consistent with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes), a rare paraneoplastic disorder, has been reported. He had a previous history of smoking, hypothyroidism and a 20 kg weight loss. It was emphasized that a detailed clinical history and examination followed by laboratory and radiological studies and bone marrow biopsy, among others tests, were necessary in order to recognise the components of this syndrome and not to delay the time of diagnosis. This patient had two mandatory criteria (monoclonal gammopathy and sensorimotor polyneuropathy), one major criterion (bone lesion) and several minor criteria (endocrine disorders, cutaneous manifestations, organomegaly). He is currently under the supervision of the doctors of the hematology department and continues neurological follow-up, having a good response to the specific therapy. Rare diseases like this syndrome are a diagnostic challenge for health professionals.

Informa-se o caso de um homem de 45 anos com sintomas e sinais compatíveis com a Síndrome de POEMS (do inglês: polineuropatia, organomegalia, endocrinopatia, gamopatia monoclonal e alterações cutâneas), um distúrbio paraneoplásico raro. O homem tinha antecedentes de tabagismo, hipotiroidismo e ultimamente tinha perdido 20 kg de peso. Enfatizamos que um prontuario médico e exame detalhado, seguido de estudos de laboratório e radiológicos, e uma biópsia de medula óssea, dentre outros, são ferramentas necessárias para reconhecer os componentes desta síndrome e não demorar o tempo de diagnóstico. Nosso paciente apresentou dois critérios obrigatórios (gamopatia monoclonal e neuropatia periférica sensório-motora), um critério maior (lesão óssea) e vários critérios menores (anormalidades endócrinas, alterações cutâneas, organomegalia). Encontra-se atualmente sob supervisão hematológica e continua seu seguimento neurológico, mostrando uma resposta boa à tera,pia concreta. Doenças raras como essa síndrome são um desafio diagnóstico para os profissionais da saúde.

Síndrome de Albrigth-Mccune Sternberg: reporte de un caso y revisión de la literatura / Albright-Mccune Sternberg syndrome: case report and literature review

El síndrome de Albright-McCune Sternberg (SAMS) es un desorden raro que se origina de una mutación del gen GNAS1. Se caracteriza por presentar un fenotipo típico, el cual incluye fibrodisplasia (FD) poliostótica, pubertad precoz (PP), pigmentaciones café au lait (café con leche) junto con otras endocrinopatías. La presente investigación trata de una paciente femenina de 22 años de edad con SAMS la cual presenta algunos signos y síntomas del síndrome tales como: FD poliostótica, pigmentaciones color café con leche en la piel, PP e hipotiroidismo. Acudió por dolor a nivel de las encías inferiores producto de un aumento óseo bimaxilar, inicia su enfermedad actual en mayo de 2013 presentando dolor a nivel de mucosa gingival inferior, localizado, punzante, de intensidad moderada, el cual se agrava ante la masticación y dura hasta el cese del estimulo. La FD fue diagnosticada posterior a la realización de una biopsia de tejido óseo y estudios radiográficos, la paciente presentó metrorragia a los nueve meses de edad el cual se repitió a los cinco años y persistió de manera intermitente hasta los veinte años de edad donde fue diagnosticada con ovarios poliquísticos por lo cual se le prescribió etinilestradiol y acetato de ciproterona. Aunque el SAMS generalmente cursa con una hiperfunción endocrina la paciente tiene un diagnóstico de hipotiroidismo por lo cual está bajo tratamiento con levotiroxina.

The Albright-McCune Sternberg syndrome (AMSS) is a rare disorder that arises from a mutation of the GNAS1 gene. It is characterized by a typical phenotype, which includes polyostotic fibrous dysplasia (FD), precocious puberty (PP), cafe-au-lait pigmentations and other endocrinopathies. The following research is about a 22 year old female patient with SAMS which presents some signs and symptoms of the syndrome such as polyostotic FD, pigmentation of the skin, PP and hypothyroidism. She attended by pain in the lower gum product of increased bimaxillary volume it began in may 2013 having pain in lower gingiva localized, throbbing, of moderate intensity, which is exacerbated by chewing and lasts until the cessation of the stimulus. The FD was diagnosed after performing a bone biopsy and radiographic studies, the patient had metrorrhagia at nine months of age which was repeated at five years and persisted intermittently until twenty years where he was diagnosed with polycystic ovaries so was prescribed ethinylestradiol and cyproterone acetate. Although the AMSS usually occurs with endocrine hyperfunction the patient has a diagnosis of hypothyroidism which is treated with levothyroxine.

Síndrome de Poems: reporte de un caso clínico / Poems syndrome: a case report

El Síndrome de POEMS (polineuropatía, organomegalia, endocrinopatía, pico gamma monoclonal ycambios en la piel) es una discrasia de células plasmáticas que se manifiesta con un característicoconjunto de trastornos para neoplásicos. Los cambios en la piel, como hiperpigmentación cutáneageneralizada, son debidos al aumento en la secreción del factor de crecimiento del endotelio vascular.Se reporta el caso de una mujer de 67 años de edad que se presentó con astenia, adinamia y uncuadro importante de anemia, asociados a diabetes mellitus, hipotiroidismo y polineuropatía motora ysensitiva de moderada intensidad. Sus cambios en la piel consistieron en hiperpigmentación, manchasvioláceas en los labios y acentuación de los pliegues de las manos. Los estudios subsecuentesevidenciaron el pico gamma monoclonal y la polineuropatía axonal sensitivo-motora en elelectromiograma. Se brindó el tratamiento quimioterapéutico correspondiente pero sufriócomplicaciones mielosupresoras por esta razón, desarrollo una neumonía intrahospitalaria y falleció aconsecuencia.

The POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes)is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. The skinchanges, as generalized skin hyperpigmentation, are related with elevated levels of vascularendothelial growth factor. We report a clinical case of 67 years old woman who presented withasthenia, adynamia and important anemia, associated to diabetes mellitus, hypothyroidism and amoderated intensity motor-sensitive polyneuropathy. The skin changes presented by this patientwere hyperpigmentation, violet spots in the lips and accentuation of the lines in the palm of the hand.Subsequent analysis showed the M–protein and the electromyography showed a motor-sensitivepolyneuropathy in both legs. Adequate chemotherapy was given but mielosuppressive effects wereshown, she acquired nosocomial pneumonia and died as consequence.

Cetoacidose diabética canina / Canine ketoacidosis

A cetoacidose diabética (CAD), uma das complicações metabólicas em pacientes com diabetes mellitus (DM), caracteriza-se por hipercetonemia e alterações do equilíbrio ácido-base, juntamente com as alterações clínicas e laboratoriais compatíveis. Este trabalho objetiva apresentar uma revisão crítica dos principais pontos da etiopatogenia, dos sinais clínicos e das alterações laboratoriais da CAD, bem como discorrer sobre prognóstico e modalidades terapêuticas mais recentes, visando a fornecer subsídios ao clínico de pequenos animais.

The diabetic ketoacidosis (DKA), one of the metabolic complications in patients with diabetes mellitus (DM), is characterized by hiperketonemia and acid-basic disturbances, in association with suitable clinic and laboratorial changes. This research aims to present a critical review on the main aspects of ethiopathogenesis, clinical sings and laboratorial changes of DKA, as well as the prognosis and the most recent therapeutic manners, in order to provide assistance to the small animal clinic.

Hipertireoidismo felino / Feline hyperthyroidism

O hipertireoidismo é considerado a endocrinopatia mais comum em felinos de meia idade a idosos, nos Estados Unidos e na Europa. No entanto, ainda existem poucos casos relatados no Brasil. O diagnóstico precoce e o tratamento adequado tornam o prognóstico do animal mais favorável, podendo até se obter a cura do paciente. Devido à importância desta afecção em felinos, são abordados, nesta revisão, os aspectos clínicos, diagnósticos e terapêuticos do hipertireoidismo felino.

Hyperthyroidism is the most common endocrinopathy of middle aged and older cats in the United States and Europe, however there are few related cases in Brazil yet. The early diagnosis and the correct treatment become the prognosis more favorable, leading to patient cure. This paper reviews the clinical, diagnosis and therapeutic aspects of feline hyperthyroidism.

Hipertireoidismo relacionado à síndrome de McCune Albright: relato de dois casos e revisão da literatura / Hyperthyroidism related to McCune-Albright syndrome: report of two cases and review of the literature

INTRODUÇÃO: A síndrome de McCune Albright é uma doença rara, clinicamente definida pela combinação de displasia fibrosa poliostótica, manchas café-com-leite e endocrinopatias com hiperfunção, como puberdade precoce, hipertireoidismo, acromegalia e outras. A fisiopatologia biomolecular baseia-se em uma mutação ativadora do gene para a subunidade alfa da proteína de membrana Gs, que estimula a produção intracelular de AMPc, conferindo secreção autônoma à glândula em particular. A glândula tireóide é freqüentemente envolvida nesta doença, sendo o hipertireoidismo a segunda endocrinopatia mais comum após a puberdade precoce. OBJETIVO: Realizar uma revisão da literatura no período de 1937 a 1997, incluindo nossa casuística, e discutir o tratamento utilizado para o hipertireoidismo nesta síndrome. RESULTADOS: Do total de 85 casos, em 26 com hipertireoidismo se descreve o tipo de tratamento utilizado. Entre esses, 15 foram submetidos à cirurgia (tireoidectomia total), seis foram submetidos à iodoterapia e cinco foram submetidos a tratamento com drogas antitireoidianas (DAT - propiltiuracil, carbimazol ou metimazol), pois em alguns houve associação dos tratamentos citados anteriormente. CONCLUSÃO: A síndrome de McCune-Albright é uma doença rara, que inclui displasia fibrosa poliostótica, manchas café-com-leite e endocrinopatias com hiperfunção. Os casos acompanhados com hipertireoidismo são inicialmente tratados clinicamente com DAT, porém a ablação cirúrgica ou com radioiodoterapia é a opção definitiva mais indicada.

INTRODUCTION: McCune-Albright syndrome is a sporadic disease clinicaly characterized by polyostotic fibrous dysplasia, "café-au-lait" cutaneous spots and hyperfunctional endocrinopathies, such as precocious puberty, hyperthyroidism, acromegaly and others. The biologic physiopathology of the disease is based on an activating mutation of the gene for the Gs protein which mediates the activation of adenyl cyclase and subsequent gland autonomous secretion. The thyroid gland is usually involved in this disease, being hyperthyroidism the second most common endocrinopathy seen after precocious puberty. OBJECTIVE: Revision of the literature since 1937 to 1997, adding our casuistic and discussing the adequate treatment for the hyperthyroidism in the syndrome. RESULTS: Among 85 cases identified with the syndrome, hyperthyroidism treatment was described in 26 of them. Fifteen were submitted to surgery (total thyroidectomy), 6 were submitted to iodotherapy and 5 were treated with antithyroid drugs (ATD - propylthiouracil, carbimazole or methimazole). In some cases, treatment association was done. CONCLUSION: McCune-Albright syndrome is a rare disease, combining polyostotic fibrous dysplasia, "café-au-lait" cutaneous spots and hyperfunctional endocrinopathies. Cases with hyperthyroidism are treated iniatially clinically, but definitive ablation with surgery or radioiodine treatment is the most indicated option.

Síndrome de POEMS (mieloma osteoesclerótico) / POEMS syndrome (osteosclerotic myeloma)

A síndrome de POEMS é uma entidade clínica única definida pela presença concomitante de doença monoclonal de plasmócitos, polineuropatia periférica e outras manifestações paraneoplásicas tais como organomegalias, endocrinopatias, alterações cutâneas, papiledema, lesões osteoescleróticas e sobrecarga de volume extravascular. Para o diagnóstico da doença não é necessária a presença de todas as manifestações relacionadas e o reconhecimento precoce desta patologia é importante para reduzir a morbidade. A síndrome de POEMS também é conhecida como Mieloma Osteoesclerótico, síndrome de Crow-Fukase e síndrome de Takatsuki. A sobrevida média dos pacientes com síndrome de POEMS é significativamente superior àquela esperada para os portadores de mieloma múltiplo (165 e 38 meses, respectivamente), independente do número de manifestações presentes ao diagnóstico e da intensidade de infiltração da medula óssea por plasmócitos. O tratamento padrão da síndrome de POEMS ainda não está estabelecido e as principais opções são radioterapia, corticosteróides e agentes alquilantes, inclusive na forma de quimioterapia de altas doses com transplante autólogo de células-tronco hematopoiéticas.

POEMS syndrome is a unique clinical entity defined by the presence of a monoclonal plasma cell disorder, peripheral neuropathy, and other paraneoplastic features such as organomegaly, endocrinopathy, skin changes, papilledema, sclerotic bone lesions, extravascular volume overload. Not all features of the disease are required to make the diagnosis, and early recognition is important to reduce morbidity. Other names for the syndrome include ostesclerotic myeloma, Crow-Fukase syndrome, or Takatsuki syndrome. The median survival of patients with POEMS syndrome is superior to those with multiple myeloma (165 and 38 months, respectively), independent of the number of presenting features, bone lesions, or plasma cells at diagnosis. There is no standard treatment for this disorder, the mainstays of therapy for patients with POEMS include irradiation, corticosteroids, and alkylator-based therapy, including high-dose chemotherapy with peripheral blood stem cell transplantation.

Endocrine Function and Growth in Children with Craniopharyngioma

PURPOSE: Craniopharyngiomas are often accompanied by severe endocrine disorders. Although there is universal growth hormone deficiency(GHD), the resulting growth pattern is very heterogeneous. We report the growth and endocrine outcome of 44 children with craniopharyngioma, with emphasis on initial symptoms, growth before and during growth hormone(GH) treatment and spontaneous growth in spite of GHD. METHODS: We performed a retrospective study of 44 children treated at our centre between 1984 and 2002. RESULTS: About 30% of patients had symptoms suggesting endocrine disorder at diagnosis. After surgery, multiple endocrinopathies were almost universal. Before GH therapy, height velocity was 8.00+/-2.71 cm/yr in the normal growth group(n=11) and 1.79+/-1.10 cm/yr in the subnormal growth group(n=7) during the first year and during the second year, 6.76+/-2.49 cm/yr and 2.29+/-1.33 cm/yr, respectively. There was no difference of body mass index(BMI) change between before and after surgery in the two groups. Height standard deviation score(SDS) was -1.46+/-0.74 in the normal growth group and -0.43+/-0.97 in the subnormal growth group. Before GH treatment height SDS was -1.31+/-1.25 and BMI was 20.46 & gt; or =3.60. During GH treatment, height SDS increased to -0.60+/-1.37 in the first, and to -0.41+/-1.54 in the second year(P & lt;0.05), but BMI did not change significantly. CONCLUSION: The endocrine morbidity could develop in most children with craniopharyngioma before and after the operation and should be managed properly. Although all treated patients benefit from GH therapy, further studies are necessary to find out the possible mechanism of growth regulation in normally growing children, despite GH deficient.

Endocrinopathy in Hemochromatosis Patients Multi-Transfused for Aplastic Anemia / 대한소아혈액종양학회지

PURPOSE: Chronic blood transfusions result in excessive iron deposition leading to eventual tissue damage and impaired function of organs, such as the liver, spleen, pancreas, skin, thyroid, and heart. We evaluated the body iron status and endocrinopathy in repeatedly transfused patients with aplastic anemia (AA). METHODS: Fourteen patients with AA who were transfused with more than 10 Units of packed RBC since 1996 were evaluated. We evaluated the correlation of amount of blood transfused with status of iron stores (determined by serum iron, TIBC, ferritin and transferrin saturation) and organ damage. RESULTS: Patients received a median of 61 units (range 11~168 units) of PRC. Twelve patients (85.7%) had elevated serum ferritin levels, and 11 (78.6%) had elevated transferrin saturation. Serum ferritin (P<0.01; r=0.868), and transferrin saturation (P<0.05; r=0.569) were significantly correlated with the amount of PRC transfused, respectively. Five patients had clinically significant iron overload despite the use of deferoxamine. Organ damage caused by transfusion iron overload were skin pigmentation (N=3), hepatic (N=1) and endocrinologic abnormalities. Diabetes (N=3), hypothyroidism (N=3), and hyogonadotropic hypogonadism (N=1) were observed. No patient developed clinically significant arthropathy or cardiac disease. CONCLUSION: AA patients who received chronic blood transfusions develop iron overload which may lead to endocrinopathy. Iron status and organ dysfunction should be monitored and effective measures to prevent iron overload should be applied in patients who need chronic transfusions.

A Case of POEMS Syndrome / 대한피부과학회지

POEMS syndrome is an unusual plasma cell dyscrasia with multisystemie manifestations featuring polyneuropathy, organomegaly, endocrinopsthy, M protein and skin changes. We have seen a 49-year-old woman presenting with hyperpigmentation, hypertrichosis, hyperhidrosis, and taut thickened skin of the extremities as a skin manifestation of this syndrome. We review the literature and report a case.