scholarly journals Systemic mastocytosis: Case report with literature review

2012 ◽  
Vol 140 (1-2) ◽  
pp. 100-103
Author(s):  
Mirjana Mitrovic ◽  
Maja Perunicic-Jovanovic ◽  
Aleksandra Sokic-Milutinovic ◽  
Sladjana Andrejevic ◽  
Nada Suvajdzic

Introduction. Mastocytosis is a clonal neoplastic disorder of the mast cells. The clinical signs and symptoms of mastocytosis are heterogeneous ranging from indolent disease with a longterm survival to a highly aggressive neoplasm with survival of about 6 months. Systemic mastocytosis (SM) is characterized by mastocyte infiltration of one or more organs, with or without skin involvment. Case Outline. The presented patient presents a highly challenging diagnostic and therapeutic case. A 46-year-old man was referred to our Centre due to the 7-year-long history of hepatosplenomegaly and mild thrombocytopenia. Ultrasound examination showed hepatosplenomegaly (liver 170 mm; spleen 200 mm), platelet count was 90C109/L, serum tryptase level was elevated and bone marrow biopsy showed infiltration with mast cells (CD117, CD25 and mast cell tryptase positive). Our patient was diagnosed with aggressive systemic mastocytosis (SM) according to WHO Classification (2008), although the clinical course of the disease was indolent, without complications for more than 7 years. Because of the ?intermediate? course, this patient was referred to as smouldering or intermediate SM and was not treated with cytostatics. Conclusion. Utilizing the established criteria, indolent SM can be discriminated from the aggressive subvariants of SM in most cases. However, a small group of patients, like our case belongs to the ?grey zone?. Therapeutic approach to these patients is individual and prognosis is uncertain.

1976 ◽  
Vol 4 (5) ◽  
pp. 423-428
Author(s):  
H A Gaafar

In a 1-year evaluation of the indirect fluorescent antibody test for gonorrhea, 16,127 specimens from low-and high-risk groups were examined, and the results were compared with culture, history of exposure to a known patient, and clinical signs and symptoms. The results confirmed the usefulness of the indirect fluorescent antibody test for gonorrhea as a screening procedure, especially for women in the low-risk group. The test is more economic and probably more sensitive than culture for mass screening of asymptomatic women.


2019 ◽  
Vol 13 (1) ◽  
Author(s):  
Farnoosh Razmara ◽  
Zahra Ghoncheh ◽  
Ghazal Shabankare

Abstract Background A traumatic bone cyst is an uncommon nonneoplastic lesion of the jaws that is considered as a “pseudocyst” because of the lack of an epithelial lining. This lesion is particularly asymptomatic and therefore is diagnosed by routine dental radiographic examination as a unilocular radiolucency with scalloped borders, mainly in the posterior mandibular region. The exact etiopathogenesis of the lesion remains uncertain, though it is often associated with trauma. Case presentation We report three Persian cases of traumatic bone cyst with different clinical and radiographic features, and we present a review of the literature to further discuss diagnostic and treatment challenges. Only one of the three patients reported a history of trauma, and despite the usual signs and symptoms of the lesion, extension of the defect to the ramus, swelling of the lingual cortex, and their unusual presence in the anterior mandible were noted in these patients. Conclusions Because features of this cyst can be varied, careful history taking and radiographic evaluation alongside the clinical signs and symptoms have a very significant role in definitive diagnosis, appropriate treatment, and accurate assessment of prognosis.


2017 ◽  
Vol 7 ◽  
pp. 6 ◽  
Author(s):  
Charu Chanana ◽  
Nishant Gupta ◽  
Itisha Bansal ◽  
Kusum Hooda ◽  
Pranav Sharma ◽  
...  

Vaginal bleeding in the first trimester has wide differential diagnoses, the most common being a normal early intrauterine pregnancy, with other potential causes including spontaneous abortion and ectopic pregnancy. The incidence of ectopic pregnancy is approximately 2% of all reported pregnancies and is one of the leading causes of maternal mortality worldwide. Clinical signs and symptoms of ectopic pregnancy are often nonspecific. History of pelvic pain with bleeding and positive β-human chorionic gonadotropin should raise the possibility of ectopic pregnancy. Knowledge of the different locations of ectopic pregnancy is of utmost importance, in which ultrasound imaging plays a crucial role. This pictorial essay depicts sonographic findings and essential pitfalls in diagnosing ectopic pregnancy.


Author(s):  
Abdolali MOSHFE ◽  
Arash ARIA ◽  
Najme ERFANI ◽  
Ali JAMSHIDI ◽  
Bahador SARKARI ◽  
...  

Background: In the current study, we described the epidemiological features, clinical presentation, diagnosis and management of patients with suspicion of fascioliasis in Kohgiluyeh and Boyer-Ahmad Province in southwest of Iran.    Methods: Overall, 56 patients with suspicion of fascioliasis, based on their clinical signs and symptoms that referred to Clinic of Internal Medicine in Yasuj city, from 2014 to 2016 were enrolled. Demographic data, history of eating aquatic local plants, the chief complains, and laboratory findings were recorded for each patient. Stool samples were obtained from each case for detection of Fasciola eggs. Moreover, blood samples were taken from each patient and evaluated for detection of anti-Fasciola antibodies by an indirect ELISA. Patients who defined as having fascioliasis were treated with triclabendazole and were followed for at least three months for clinical improvement. Results: Serological test was positive in 5 patients. Of these 5 cases, three cases had a history of ingesting raw aquatic vegetables. The main clinical signs and symptoms in positive cases were; abdominal pain (60%), epigastric pain (40%), anemia (60%), and dermal pruritus (20%). Hypereosinophilia was seen in all of 5 positive cases. No Fasciola egg was found in stool specimens of any of the patients. The fascioliasis cases were treated by triclabendazole and clinical symptoms disappeared in all of 5 cases. Conclusion: Our observation further confirmed Yasuj district as a human endemic area for fascioliasis in Iran. The study also highlighted the importance of clinical features together with eosinophilia, as key parameters, in the diagnosis of human fascioliasis. Clinicians need to be aware of this disease and should keep in mind fascioliasis when hypereosinophilia present in patients in such endemic areas.


2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Kedar R. Mahajan ◽  
Amity L. Roberts ◽  
Mark T. Curtis ◽  
Danielle Fortuna ◽  
Robin Dharia ◽  
...  

Cryptococcus neoformanscan cause disseminated meningoencephalitis and evade immunosurveillance with expression of a major virulence factor, the polysaccharide capsule. Direct diagnostic assays often rely on the presence of the cryptococcal glucuronoxylomannan capsular antigen (CrAg) or visualization of the capsule. Strain specific phenotypic traits and environmental conditions influence differences in expression that can thereby compromise detection and timely diagnosis. Immunocompetent hosts may manifest clinical signs and symptoms indolently, often expanding the differential and delaying appropriate treatment and diagnosis. We describe a 63-year-old man who presented with a progressive four-year history of ambulatory dysfunction, headache, and communicating hydrocephalus. Serial lumbar punctures (LPs) revealed elevated protein (153–300 mg/dL), hypoglycorrhachia (19–47 mg/dL), lymphocytic pleocytosis (89–95% lymphocyte, WBC 67–303 mg/dL, and RBC 34–108 mg/dL), and normal opening pressure (13–16 cm H2O). Two different cerebrospinal fluid (CSF) CrAg assays were negative. A large volume CSF fungal culture grew unencapsulatedC. neoformans. He was initiated on induction therapy with amphotericin B plus flucytosine and consolidation/maintenance therapy with flucytosine, but he died following discharge due to complications. Elevated levels of CSF Th1 cytokines and decreased IL6 may have affected the virulence and detection of the pathogen.


2013 ◽  
Vol 26 (1) ◽  
pp. 30-34 ◽  
Author(s):  
C. Gagliardo ◽  
F. Martines ◽  
F. Bencivinni ◽  
G. La Tona ◽  
A. Lo Casto ◽  
...  

We present a case of an elderly woman with no history of audiological disease with sudden onset of visual and hearing deficits associated with systemic clinical signs. On examination she had impairment of right CNs from V to X. CT and MR imaging demonstrated a cystic vestibular schwannoma with a rare intralesional fluid-fluid level correlated to a recent bleed. We include high quality MR images to show the acute impairment of the cranial nerves next to the tumour after acute bleeding. Our case report includes a voxel-based morphometry (VMB) analysis of the tumour that, as far as we know, has never been done before for such a tumour. VBM analysis was performed to calculate the hypothesized volume changes after the acute bleed which likely resulted in a sudden increase in the overall size of the tumour resulting in atypical clinical signs and symptoms due to the establishment of a mechanical conflict with the adjacent cranial nerves.


2020 ◽  
Vol 4 (2) ◽  
pp. 67-68
Author(s):  
Vimalraj Padayatchiar Govindan ◽  
Parag Madhukar Dhakate ◽  
Ayush Uniyal

Adult male non-descript bred dog presented with a history of circling motion, frequent head nodding, crusty muzzle, muscle twitching, seizure, vomiting, inappetence, coughing, inability to walk, watery pustular discharge from eyes and blood stained left ears and on further examination of foot pads showed thick, crusty or hardened sole and based on the clinical signs and symptoms, provisionally diagnosed as Canine distemper and advised euthanasia due to close geographic onset of the disease epidemic and to prevent Canine distemper-associated deaths in wild canids and felids.


2021 ◽  
Vol 23 (6) ◽  
pp. 1271-1284
Author(s):  
D. Sh. Macharadze

The present review considers the role of mast cells (MC) and tryptase levels in various pathological conditions in children and adults. The main causes of hypertryptasemia are presented, as well as a list of the most important stimuli that can cause activation of MC. Cliical allergologists should focus their clinical practice on the patients with anaphylaxia and suspected MC activation syndromes. Moreover, hypertryptasemia (> 20 ng/ml) is considered an additional diagnostic criterion for systemic mastocytosis, according to the WHO recommendations. As a rule, the level of tryptase is constantly elevated in most patients with systemic mastocytosis, whereas it is undergo changes in acute IgE-mediated hypersensitivity reactions. In cases of anaphylaxia, tryptase concentration should be determined in the patients during the first hours following onset of acute allergic reaction, and 24-48 hours later. Recommendations are given for determining tryptase levels in blood serum of the patients (basal and peak values), and algorithms are provided for usage of these indexes in various diseases. The article also provides the assessed threshold values of tryptase when diagnosing anaphylaxia, MC activation syndromes, and a novel disorder – alpha-tryptasemia. In the diagnosis of hereditary alpha-tryptasemia, as well as MC activation syndromes (primary and secondary), clinical manifestations in the patient and time dynamics of tryptase levels should be taken into account. The accumulated experience allowed to consider, first of all, frequency of severe allergic reactions (most often as anaphylaxia) in the patients with suspected MC activation syndromes. The syndrome of MC activation is characterized by excessive release of their granule contents without signs of clonal proliferation, which in many cases may be due to gene allele duplication, especially, TPSAB1 α-tryptase gene. For patients with hereditary alpha-tryptasemia, the most characteristic manifestations are represented by vegetative-vascular dystonia (orthostatic tachycardia), joint hypermobility, etc. Hence, determination of tryptase level (especially in time course) should be given more attention in the practice of clinicians. Difficulties with interpretation of the results arise in cases when tryptase concentration remains within normal range (up to 11.4 ng/ml), and the patient has clinically evident mastocytosis, or MC activation syndromes. If the patient has a history of anaphylaxia, especially after bites of hymenoptera insects, the TC activation syndromes should be excluded.


Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2289-2289 ◽  
Author(s):  
Kenneth V.I. Rolston ◽  
Scott A. McConnell ◽  
Jack Brown ◽  
Kenneth C. Lamp

Abstract Infections occurring during neutropenia in cancer patients (pts) frequently involve Gram-positive pathogens and are complicated by antibiotic resistance. Outcomes for infections due to either methicillin-resistant or -susceptible Staphylococcus aureus (MRSA, MSSA) treated with vancomycin are considered suboptimal by many clinicians (Sakoulas G. J Clin Microbiol2004;42:2398–402). New effective agents are needed for treatment. Data were collected retrospectively as part of an ongoing registry (Cubicin Outcomes Registry and Experience; CORE, 2005 and 2006 program years). Data were collected on demographic and microbiologic characteristics; prior, concomitant and follow-up antibiotics; DAP regimen; and clinical data. Outcomes were assessed at the end of DAP therapy by the investigator who, in most cases, was the infectious disease physician involved in the treatment of the patient. The definitions for clinical outcome were: Cure - clinical signs and symptoms resolved (and/or) no additional antibiotic therapy necessary (or) negative culture reported at the end of therapy; Improved - partial resolution of clinical signs and symptoms (and/or) additional antibiotic therapy warranted at the end of therapy; Failure - inadequate response to therapy: worsening or new/recurrent signs and symptoms (or) need for a change in antibiotic therapy (or) positive culture reported at the end of therapy. Pts reported with neutropenia during DAP, a history of cancer, and evaluable for outcome (cure, improved, failure) were analyzed. Eighty-four pts had a history of cancer and had neutropenia during DAP treatment, of these, 72 (86%) were evaluable for DAP outcome. Sixty-five of 72 (90%) pts had either cure (n=40, 56%) or improved (n=25, 35%) as an outcome. Success rates (cure plus improved) stratified by the lowest WBC during DAP were; 29/34 (85%) for < 100 cells/m3, 26/28 (93%) for 100–499 cells/m3, and 10/10 (100%) for 500–1000 cells/m3, P=0.32. The median (range) time to clinical response in those successfully treated (data reported in 47 pts) was 4 days (1–21). Fifty-nine of 72 (82%) had a hematological malignancy. Fifty-eight percent were male, 22% were ≥66 yrs old, 7% had an initial CrCl <30 mL/min, 18% received DAP in an ICU. Eighty-eight percent of pts received antibiotics before DAP, most frequently with vancomycin (83%), cefepime (17%), and linezolid (16%). The most frequent reason for switching to DAP was prior clinical failure / resistance (54%). The major infections being treated with DAP were bacteremia (76%), UTI / pyelonephritis (10%), and uncomplicated skin and skin structure (6%). Seventy-four percent of pts with a WBC < 100 cells/m3 had a bacteremia. The most common Gram-positive pathogens reported were vancomycin-resistant enterococci (52%), coagulase-negative staphylococci (21%), and S. aureus (14%; 7/11, 64% were MRSA). The median (range) initial DAP dose was 6.0 mg/kg (3–7). The median (range) DAP duration of therapy was 13 days (1–86). Nineteen percent of the pts received DAP as an outpatient at some time during their therapy. There were 37 adverse events (AE) reported in 20 (28%) pts; however, only three AEs in 3 pts (4%) were assessed as possibly related to DAP. The data from the registry provide useful information about the clinical characteristics of neutropenic cancer patients treated with DAP for primarily bacteremia. Most of the pts were bacteremic and there was no difference in outcome regardless of the degree of neutropenia. These data require confirmation via prospective clinical trials.


Primer on Multiple Sclerosis, second edition is designed to be a practical guide to the basic science and clinical manifestations of multiple sclerosis. It is intended primarily for neurologists and other health care professionals who treat persons with this disease. The book starts with a review of the history of multiple sclerosis and the basic genetics, immunology, electrophysiology, and pathophysiology that are central to the disease. It then reviews the common and uncommon clinical signs and symptoms of multiple sclerosis and the management of these conditions. The latest diagnostic strategies are presented. There is extensive coverage of approved and experimental disease-modifying therapies, including algorithms to assist clincians in choosing these therapies. Complementary and alternative therapies that are popular among persons with multiple sclerosis are examined. New additions to this edition include a chapter for nursing health care professionals, and updates on therapeutics. Unique to this book are the chapters on the legal, psychosocial, and vocational issues that often present challenges for person with multiple sclerosis, topics that typically are not covered in standard texts.


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