scholarly journals Haematological Malignancies in Systemic Sclerosis Patients: Case Reports and Review of the World Literature

2017 ◽  
Vol 2017 ◽  
pp. 1-12 ◽  
Author(s):  
M. Colaci ◽  
D. Giuggioli ◽  
C. Vacchi ◽  
C. Ferri

Background. The association of systemic sclerosis (SSc) and haematological cancers was reported in a large number of case reports and cohort studies, describing SSc patients with highly heterogeneous clinical pictures. Objective. We reviewed the literature to better describe SSc patients with haematological malignancies. Methods. SSc cases complicated by haematological malignancies described in the world literature were collected; other 2 cases referred to our centre were reported. Results. One hundred-thirty SSc subjects were collected from 1954 up to date. The mean age of patients at cancer diagnosis was 56.1 ± 16.7 years; 72% of patients were females. In 60% of cases, the diagnosis of haematological malignancy was described within 5 years of SSc diagnosis. In 7.8% of cases, coexistence of Sjögren’s syndrome or other autoimmune disorders was cited. Sixty-six cases with lymphoma (in the majority of cases B-cell neoplasms), 28 with leukaemia (chronic lymphocytic form in 9), 14 with multiple myeloma plus one solitary IgM plasmocytoma, and 16 with myeloproliferative disorders were found. No specific SSc subsets seem to be related to haematological malignancies. Conclusions. We remarked the importance of clinical work-up in SSc, in order to early diagnose and treat eventual occult haematological malignancies, especially during the first years of the disease.

2012 ◽  
Vol 127 (1) ◽  
pp. 67-69 ◽  
Author(s):  
T Al Balushi ◽  
J Z Naik ◽  
M Al Khabori

AbstractObjective:We present an extremely rare case of congenital cholesteatoma in identical twins.Method:Case report of congenital cholesteatoma in identical twins, and a literature review, are presented.Results:Both cases presented to the ENT out-patient clinic, but with different clinical pictures. Both were managed surgically.Conclusion:Congenital cholesteatoma presents as a whitish mass in the middle ear, with an intact tympanic membrane. It is a rare entity comprising between 3.7 and 24 per cent of total cholesteatoma cases. The key to its diagnosis is the absence of previous ear infection, ear surgery and local trauma. To our best knowledge, this paper represents the first report in the world literature of congenital cholesteatoma in identical twins.


2010 ◽  
Vol 41 (3) ◽  
pp. 453-461 ◽  
Author(s):  
M. Begum ◽  
P. J. McKenna

BackgroundThe nosological status of olfactory reference syndrome (ORS) is a matter of debate and there is uncertainty as to what treatments are effective.MethodThe world literature was searched for reports of cases of ORS. Clinical, nosological and therapeutic information from cases meeting proposed diagnostic criteria for the disorder was summarized and tabulated.ResultsA total of 84 case reports (52 male/32 female) were found. Age of onset was <20 years in almost 60% of cases. Smell-related precipitating events were recorded in 42%. Most patients could not smell the smell or only did so intermittently. Authors of the reports expressed reservations about the delusional nature of the belief in slightly under half of the cases. Over two-thirds were improved or recovered at follow-up, with the disorder responding to antidepressants and psychotherapy more frequently than to neuroleptics.ConclusionsORS is a primary psychiatric syndrome that does not fit well into its current classification as a subtype of delusional disorder, both in terms of its nosology and its response to treatment.


Medicine ◽  
1941 ◽  
Vol 20 (2) ◽  
pp. 145-210 ◽  
Author(s):  
Wm. F. ASHE ◽  
H. R. PRATT-THOMAS ◽  
C. W. KUMPE

1989 ◽  
Vol 7 (5) ◽  
pp. 669-678 ◽  
Author(s):  
P R Cohen ◽  
M E Grossman ◽  
L Almeida ◽  
R Kurzrock

Tripe palms are characterized clinically by thickened velvety palms with pronounced dermatoglyphics. We describe two patients with triple palms and pulmonary tumors, and review the 77 patients with idiopathic- and malignancy-associated tripe palms reported in the world literature. The majority (94%) of published cases of tripe palms occurred in patients with cancer; only five patients showed no evidence of an associated malignancy. Tripe palms were frequently seen in conjunction with acanthosis nigricans (77% of cases), although they can occur alone (23% of cases). In cancer patients with tripe palms alone, the most common underlying neoplasm was pulmonary carcinoma (53% of cases), whereas patients with both tripe palms and acanthosis nigricans frequently had gastric (35% of cases) or pulmonary (11% of cases) carcinomas. A wide variety of other solid tumors have also been observed. Importantly, in over 40% of patients, tripe palms were the presenting feature of a previously undiagnosed malignancy. Therefore, all patients with tripe palms should be evaluated with a full diagnostic work-up for an associated malignancy, particularly lung or gastric carcinoma.


2009 ◽  
Vol 123 (10) ◽  
pp. 1169-1173 ◽  
Author(s):  
M P Kos ◽  
B J van Royen ◽  
E F David ◽  
H F Mahieu

AbstractObjective:We report two cases in which dysphagia and aspiration, caused by anterior cervical osteophytes, were so severe that surgical resection was performed.Method:Case reports and a review of the world literature concerning dysphagia caused by anterior cervical osteophytes, in regard to pathogenesis, diagnosis and treatment.Results:Two patients, aged 71 and 70 years, had long-standing, slowly progressive dysphagia and aspiration; one patient had recurrent episodes of aspiration pneumonia as a result. Both patients were diagnosed on videofluoroscopy with large bony anterior cervical osteophytes. Immediate relief of symptoms was obtained after resection of the osteophytes via an anterolateral, extrapharyngeal approach. Anterior cervical osteophytes are relatively common in the elderly, although not frequently diagnosed, and are mostly seen in cases of diffuse idiopathic skeletal hyperostosis. If therapy is indicated it is mainly conservative; resection is rarely needed.Conclusion:In patients with anterior cervical osteophytes, surgical treatment is indicated only for selected cases with large, bony osteophytes and severe symptoms.


1993 ◽  
Vol 107 (4) ◽  
pp. 363-367 ◽  
Author(s):  
Gwyneth Owen ◽  
Jeremy Berry ◽  
Philip Bicknell

AbstractA female infant nine months of age presented with a bilobed swelling at the base of her tongue. Histological examination showed it to be a hamartoma of the tongue. Lingual hamartomas are rare. There are fifteen case reports of lingual hamartoma in the world literature. Our case is presented and the previous case reports are reviewed.


2011 ◽  
Vol 125 (11) ◽  
pp. 1176-1180 ◽  
Author(s):  
H Joshi ◽  
B A Woodworth ◽  
A S Carney

AbstractObjective:To propose radiofrequency coblation as a potential treatment modality for mild to moderate epistaxis in patients with hereditary haemorrhagic telangiectasia.Method:Case reports and review of the world literature concerning coblation and other treatment modalities for epistaxis in patients with hereditary haemorrhagic telangiectasia.Results:Effective epistaxis control was achieved in four out of five cases of hereditary haemorrhagic telangiectasia. In the fifth case, we struggled to achieve haemostasis due to disease severity.Conclusion:Radiofrequency coblation is a novel technique, which was found to be a safe, effective, quick and well tolerated treatment option for epistaxis management in patients with hereditary haemorrhagic telangiectasia.


Author(s):  
Anna C. Jansen

Malformations of cortical development (MCD) constitute a group of brain disorders which are mainly genetic in origin. MCD form an important cause of cerebral palsy, intellectual disability and refractory epilepsy. Despite considerable progress which has been facilitated by advances in the fields of neuroimaging and genetics, the high degree of phenotypic and genotypic heterogeneity associated with MCD continues to hamper etiological diagnosis and cousneling of numerous patients and families. The first section of this manuscript is a plea for detailed clinical phenotyping in MCD and reviews the differential diagnosis and clinical work-up based on six clinical case reports. The second part provides a review of personal highlights in the field of MCD-research, and ends with an outlook to the joint efforts of international and interdisciplinary collaborations, which will hopefully result in better care for patients with MCD and their families.


2006 ◽  
Vol 121 (1) ◽  
pp. 65-67 ◽  
Author(s):  
P Homøe ◽  
J Rosborg

Objective: We report an extremely rare case of family clustering of cholesteatoma.Method: Case reports and a review of the world literature concerning cholesteatoma and heredity are presented.Results: The family consists of parents and seven siblings of whom the mother and three sons have been surgically treated for cholesteatoma. All cholesteatomas in the family are acquired and all have a history of otitis media. Cholesteatomas occur with an incidence of 5/100 000 in Greenland, corresponding to two to three new cholesteatoma patients per year among the 57 000 inhabitants of Greenland. The family is very exceptional and interesting for further research concerning heredity in the pathogenesis of acquired cholesteatoma.Conclusion: To our knowledge this is the first report in the world literature of family clustering of acquired cholesteatoma. This case indicates that hereditary factors interplay with other factors in the pathogenesis of cholesteatoma.


2010 ◽  
Vol 76 (11) ◽  
pp. 1198-1204 ◽  
Author(s):  
Christopher P. Michetti ◽  
Emily Smeltzer ◽  
Samir M. Fakhry

Splenic injury is a rare complication of colonoscopy. Most literature on the topic is case-report based. Our objective was to perform a comprehensive analysis of characteristics of splenic injury due to colonoscopy from available published reports in the world literature, to compare and contrast this entity with that of traumatic splenic injury, and provide recommendations for management based on the analysis. We reviewed the PubMed database without restrictions using the terms splenic trauma after colonoscopy, splenic rupture from colonoscopy, splenic injury following colonoscopy, and splenic complications of colonoscopy, and also reviewed the references from the resulting publications. Retrieved manuscripts (case reports, reviews, and abstracts) were reviewed by two authors, and data extracted for 15 specific characteristics of each patient reported using a standardized data collection tool. Data were analyzed using descriptive statistics. Splenic injury due to colonoscopy is extremely rare as reported in published literature. The majority of patients that seek medical attention have delayed symptoms, and most require splenectomy. Subcapsular hematoma is the most common injury pattern seen. Selection criteria for operative management may be extrapolated from management guidelines for traumatic splenic injury, although nonoperative failure rates are higher for splenic injury due to colonoscopy than for trauma.


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