scholarly journals POEMS-syndrome: diagnostic difficulties

2020 ◽  
Vol 92 (7) ◽  
pp. 104-108
Author(s):  
P. A. Lebedev ◽  
E. V. Paranina ◽  
V. A. Rossiyev ◽  
E. Y. Fedorova ◽  
A. S. Nikolaeva
Keyword(s):  
Paraneoplastic Syndrome ◽  
Early Onset ◽  
Clinical Case ◽  
Specific Treatment ◽  
Poems Syndrome ◽  
Skin Changes ◽  
Monoclonal Protein ◽  
Diagnostic Difficulties

POEMS syndrome is a rare paraneoplastic syndrome whose name is an acronym formed from the initial letters of the names of the symptoms originally used to determine it: polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Due to the rarity of the disease and the small number of cases described in the literature, its diagnosis is difficult. The average time from onset of symptoms to diagnosis is 18 months. Moreover, the prognosis of the disease depends on the early onset of specific treatment. The article describes a clinical case of POEMS-syndrome in a 53-year-old man, which illustrates the difficulties associated with the timely recognition of this unusual disease.

Proposal of new clinical diagnostic criteria for POEMS syndrome

2018 ◽  
Vol 90 (2) ◽  
pp. 133-137 ◽  
Author(s):  
Tomoki Suichi ◽  
Sonoko Misawa ◽  
Yasunori Sato ◽  
Minako Beppu ◽  
Emiko Sakaida ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Plasma Cell ◽  
Characteristic Curve ◽  
Specific Treatment ◽  
Poems Syndrome ◽  
Bone Lesions ◽  
Standard Group ◽  
Skin Changes ◽  
Immunoglobulin Light Chain Amyloidosis ◽  
Serious Disease

ObjectiveTo propose the optimal diagnostic criteria for polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome using appropriate statistical methods and disease controls.MethodsThis retrospective cohort study included 104 consecutive patients with suspected POEMS syndrome, among whom a gold standard group of 60 patients with definitive POEMS syndrome diagnosis were followed for at least 12 months to strictly exclude other disorders mimicking POEMS syndrome and to confirm response to POEMS syndrome-specific treatment. Thirty patients with chronic inflammatory demyelinating polyradiculoneuropathy (demyelinating polyradiculoneuropathy controls) and 30 with multiple myeloma or immunoglobulin light chain amyloidosis (monoclonal plasma cell proliferation controls) were also included. Logistic regression analyses were performed to determine optimal combination of clinical and laboratory abnormalities, characteristic of POEMS syndrome.ResultsThe diagnostic criteria were statistically defined as the presence of the three major criteria (polyneuropathy (typically demyelinating), monoclonal plasma cell proliferative disorder and elevated vascular endothelial growth factor) and at least two of the four minor criteria (oedema/effusion, skin changes, organomegaly and sclerotic bone lesions), based on best performance by area under the receiver operating characteristic curve analyses. The sensitivity and specificity were 100% and 100%, respectively; the diagnostic accuracy of the proposed criteria was equivalent to somewhat complicated previous criteria.ConclusionsThe statistically defined, simple diagnostic criteria for POEMS syndrome could accelerate early diagnosis and treatment, thereby contribute to better outcome in patients with this serious disease. Prospective larger studies are required to confirm the validity.

scholarly journals Paraneoplastic Sjogren’s syndrome in gastric cancer

2018 ◽  
Vol 99 (2) ◽  
pp. 301-304
Author(s):  
R F Khamitov ◽  
A F Molostvova ◽  
L M Salimova
Keyword(s):  
Gastric Cancer ◽  
Sjögren’S Syndrome ◽  
Paraneoplastic Syndrome ◽  
Clinical Case ◽  
Sjögren's Syndrome ◽  
Specific Treatment ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Oncologic Diseases ◽  
Sjögren‘S Syndrome

In clinical practice, various masks of oncological diseases are often found. Some of them can manifest as a variety of syndromes or symptom complexes that resemble many non-oncologic diseases, including diffuse connective tissue diseases. In some cases, paraneoplastic syndrome facilitates diagnosing a malignant neoplasm in the early stages, but, unfortunately, can also mimic the tumor process by its more prominent manifestations, which lead to late establishment of the true cause of the disease, and therefore postpone specific treatment, creating significant clinical problems. Aim of the study was to reveal pathogenetic relationship between cancer and paraneoplastic syndrome on the example of our clinical observation. A clinical case of diagnosed paraneoplastic syndrome in the form of secondary Sjogren's syndrome, which developed long before the diagnosis of stomach cancer was made, as well as the results of clinical, instrumental, and laboratory examination of the patient, are presented. Questions of epidemiology, etiopathogenesis, and clinical picture of paraneoplastic syndrome are covered. The presented clinical case made it possible to outline the features of the course of paraneoplastic Sjogren's syndrome in gastric cancer and to identify a number of criteria for the diagnostic algorithm of this nosology. In particular, such criteria include general pathogenetic mechanisms, development only in malignant tumors, nonspecific clinical and laboratory manifestations, lack of parallelism with local symptoms of the tumor, the possibility of occurrence of paraneoplastic Sjogren's syndrome before development of local tumor symptoms and reappearance after its relapse. Oncologic diseases are characterized not only by specific symptoms characteristic for a certain organ damage (pain, bleeding, dysfunction, etc.), but also by a variety of nonspecific manifestations (fatigue, subfebrile temperature, weight loss, etc.) regardless of the nature, location and prevalence of the tumor process.

scholarly journals A relapse of POEMS syndrome presenting as acute kidney injury

2019 ◽  
Vol 12 (8) ◽  
pp. e228894
Author(s):  
David Owen Rees ◽  
David Hywel Thomas ◽  
Nilima Parry-Jones ◽  
Gareth Roberts
Keyword(s):  
Acute Kidney Injury ◽  
Peripheral Neuropathy ◽  
Rare Disease ◽  
Blood Test ◽  
Renal Involvement ◽  
Kidney Injury ◽  
Poems Syndrome ◽  
Skin Changes ◽  
Monoclonal Protein ◽  
History Of

Peripheral neuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes (POEMS) syndrome is a rare disease, and only in a minority of cases, causes an impairment of kidney function. Here, we describe a case of a 55-year-old man with a history of POEMS syndrome who presented with acute kidney injury following a routine blood test. On further investigation, a relapse in POEMS syndrome was diagnosed, uniquely isolated to renal involvement.

Cutaneous paraneoplastic syndrome in a cat with thymoma

2013 ◽  
Vol 41 (04) ◽  
pp. 255-259 ◽  
Author(s):  
J. Karaś-Tęcza ◽  
R. Lechowski ◽  
A. Rodo ◽  
I. Dolka ◽  
O. Gójska-Zygner
Keyword(s):  
Paraneoplastic Syndrome ◽  
Histopathological Examination ◽  
Skin Condition ◽  
Radiographic Examination ◽  
Post Mortem ◽  
Ventral Part ◽  
Soft Tissue Density ◽  
Skin Changes ◽  
Exfoliative Dermatitis ◽  
Costs Of Treatment

SummaryFeline cutaneous paraneoplastic syndrome is a rare disorder associated mainly with pancreatic carcinoma and thymoma. In this report the authors describe the case of a 12-year-old cat with paraneoplastic exfoliative dermatitis associated with thymoma. Lateral radiographic examination of the chest showed a small subtle soft tissue density in the ventral part of the first and second intercostal space, which together with skin changes suggested thymoma. Because of pain associated with the skin condition, costs of treatment and the risk associated with surgical treatment, the owner chose euthanasia of the cat. Post-mortem examination revealed a tumour which was diagnosed as thymoma by histopathological examination.

scholarly journals Riedel's thyroiditis: diagnostic difficulties (clinical case and brief review)

2021 ◽  
pp. 166-171
Author(s):  
A. A. Rybakova ◽  
N. M. Platonova ◽  
T. V. Soldatova ◽  
N. V. Tarbaeva ◽  
T. S. Panevin ◽  
...  
Keyword(s):  
Drug Therapy ◽  
Clinical Case ◽  
Clinical Performance ◽  
Fine Needle Biopsy ◽  
Morphological Description ◽  
Positive Trend ◽  
Riedel’S Thyroiditis ◽  
Compression Syndrome ◽  
Diagnostic Difficulties ◽  
Riedel's Thyroiditis

Riedel's thyroiditis is a rare disease characterized by chronic fibrosis. Clinical performance of this disease is dense stony goiter, which can poorly be displaced during palpation. The overgrowth of the goiter can lead to the development of compression syndrome. To diagnose we need to made fine needle biopsy and made the final diagnose according to its results or according to the morphological description of the postoperative material. An important step in the diagnosis of Riedel's thyroiditis is the determination of serum IgG and IgG4 to exclude an IgG4-associated disease. Treatment of this disease includes drug therapy, which is based on glucocorticosteroids administration or surgical treatment when develops compression syndrome. This article presents a clinical case of a patient with Riedel's thyroiditis; the main complaints were associated with the growth of goiter and the development of compression syndrome. In this regard, patient underwent surgery on the thyroid gland, and after this we get final diagnose. Due to feeling unwell, drug therapy with glucocorticosteroids was prescribed, against the background of which we noted a positive trend.

scholarly journals Classical Hodgkin Lymphoma Presenting with Severe, Recurrent Hypothermic Episodes

2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Jonas Juul Hansen ◽  
Hans Beier Ommen ◽  
Lars Christian Gormsen ◽  
Francesco Annibale d’Amore ◽  
Peter Martin Hjørnet Kamper
Keyword(s):  
Complete Remission ◽  
Hodgkin Lymphoma ◽  
Autonomic Neuropathy ◽  
Paraneoplastic Syndrome ◽  
Adverse Outcome ◽  
Specific Treatment ◽  
Classical Hodgkin Lymphoma ◽  
Cyclic Pattern ◽  
Present Patient ◽  
Severe Hypothermia

We report a case presenting with recurrent episodes of severe hypothermia preceding the diagnosis of Hodgkin lymphoma. The episodes of hypothermia were accompanied by other symptoms of autonomic dysfunction, mainly hypotension, which could be caused by autonomic neuropathy as part of a paraneoplastic syndrome. In comparison with previous reports describing an association between the presence of hypothermia and an adverse outcome, the present patient has responded well to lymphoma-specific treatment and is currently in an ongoing complete remission. Due to the peculiar cyclic pattern of the hypothermic episodes presented in this case, we hypothesize whether intermittent release of disease-related chemo- and cytokines could be a plausible pathogenetic explanation.

Clinical case of semilobar holoprosencephaly

2021 ◽  
Author(s):  
А.В. Серёжкина ◽  
И.Г. Хмелевская ◽  
Н.С. Разинькова ◽  
Т.А. Миненкова ◽  
И.И. Жизневская ◽  
...  
Keyword(s):  
Clinical Case ◽  
Fatal Outcome ◽  
Clinical Manifestations ◽  
Specific Treatment ◽  
Stable Condition ◽  
Horseshoe Kidney ◽  
Structural Defects ◽  
Surgical Interventions ◽  
Ultrasound Diagnostics ◽  
The Brain

Поскольку непосредственные этиологические факторы аномалии развития не известны, в работе выделены основные группы причин, способствующих формированию пороков развития головного мозга. Большое значение отводится ранней диагностике пороков развития, которая позволяет своевременно решить вопрос о возможности пролонгирования беременности, что определяется видом порока, совместимостью с жизнью и прогнозом в отношении постнатального развития. В исследовании рассматриваемого порока развития большую роль играют такие современные методы, как пренатальная ультразвуковая диагностика, нейросонография, рентгеновская компьютерная и магнитно-резонансная томография головного мозга, имеющие достаточно высокую информативность. Указаны сроки гестации, позволяющие выявить структурные дефекты головного мозга. Медико-генетическое консультирование помогает выявить риск появления больного потомства. Проведена дифференциальная диагностика семилобарной с другими формами голопрозэнцефалии. Также отмечены возможные клинические проявления рассматриваемой нозологии. В данной статье представлен клинический случай семилобарной голопрозэнцефалии, диагностированной у мальчика в возрасте 1 месяц. При поступлении мать предъявляла жалобы на срыгивания и периодическое беспокойство сына. Объем и результаты обследования ребенка изложены ниже. Выявлена сопутствующая патология в виде пупочной грыжи, врожденной аномалии развития мочевой системы: подковообразная почка; водянки яичек и головчатой формы гипоспадии. После проведения курса поддерживающей терапии пациент был выписан в стабильном состоянии. В настоящее время специфическое лечение голопрозэнцефалии отсутствует. Оперативные вмешательства на головном мозге проводятся редко ввиду тяжести состояния больных, в связи с чем лечение данной патологии возможно только с помощью хирургической коррекции симптомов. Длительная дыхательная и кардиоваскулярная дисфункция предопределяет летальный исход заболевания. Since the direct etiological factors of developmental abnormalities are not known, the main groups of causes contributing to the formation of brain malformations are identified in the work. Great importance is given to the early diagnosis of malformations, which allows us to timely resolve the issue of the possibility of prolonging pregnancy, which is determined by the type of defect, compatibility with life and the prognosis for postnatal development. In the study of the considered malformation, such modern methods as prenatal ultrasound diagnostics, neurosonography, X-ray computer and magnetic resonance imaging of the brain, which have a sufficiently high informative value, play an important role. The terms of gestation are indicated, which make it possible to identify structural defects of the brain. Medical and genetic counseling helps to identify the risk of the appearance of sick offspring. The differential diagnosis of semilobar with other forms of holoprosencephaly was carried out. Possible clinical manifestations of the nosology under consideration are also noted. This article presents a clinical case of semilobar holoprosencephaly diagnosed in a boy at the age of 1 month. Upon admission, the mother complained of regurgitation and periodic anxiety of her son. The scope and results of the child's examination are set out below. Concomitant pathology was revealed in the form of an umbilical hernia, a congenital abnormality of the development of the urinary system: a horseshoe kidney; dropsy of the testicles and a cephalic form of hypospadias. After a course of maintenance therapy, the patient was discharged in a stable condition. Currently, there is no specific treatment for holoprosencephaly. Surgical interventions on the brain are rarely performed due to the severity of the patients ' condition, and therefore, the treatment of this pathology is possible only with the help of surgical correction of symptoms. Prolonged respiratory and cardiovascular dysfunction determines the fatal outcome of the disease.

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