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Biomedical subjects

A Filiotou

Publications and source records attributed to A Filiotou.

12 recordsLinked to original sources
PubMed2004

Type I Gaucher disease with severe skeletal destruction, extraosseous extension, and monoclonal gammopathy.

Extraosseous extensions of Gaucher-cell deposits simulate malignant diseases. We describe a 65-year-old male with type-I Gaucher disease, confirmed by low leukocyte glucocerebrosidase activity, high plasma chitotriosidase, and N370/L444P gene-mutations, who had severe skeletal involvement, IgG-kappa monoclonal gammopathy, and a soft-tissue mass within the left iliac muscle. Bone marrow biopsy showed heavy infiltration by Gaucher cells, and histopathology of the excised extraosseous mass revealed infiltration by Gaucher cells. Thus, malignant diseases were excluded and the diagnosis of an extraosseous Gaucher-cell extension was well documented. Our case is reported because it is very interesting and unique in the literature.

Aged↗
PubMed2001

Multiple myeloma in sickle cell syndromes.

Multiple myeloma (MM) is rare among patients with sickle cell syndromes (SCS). We describe six Greek sickle cell patients aged 56 to 65 years: five haemoglobin Sbeta+thalassaemia (HbSbeta+ (thal), one sickle cell anaemia (HbSS), who developed MM (three IgGkappa, one IgGlambda, one IgAkappa, and one IgGK-IgAK (biclonal). Our HbSbeta+thal cases, represent the first reported association of this entity with MM. Generalized bleeding diathesis, stroke, grand mal seizures, bone marrow necrosis and other clinical manifestations due to hyperviscosity aggravated by sickle cell vasoocclusion were treated by plasmaphereses and exchange blood transfusions. The increase of mean survival in SCS patients due to the current medical facilities may have an impact on the incidence of MM among them, if a pathogenetic link between the two conditions exists. All our patients carried a diagnosis of cholelithiasis which may predispose to MM; two of them progressed from a monoclonal gammopathy of undetermined significance (MGUS) to MM. Further studies are needed in order to understand the relationship between SCS and MM.

Adult↗
PubMed1999

Systemic AL amyloidosis in Gaucher disease. A case report and review of the literature.

Chronic Gaucher disease [GD] in association with systemic AL amyloidosis is extremely rare. We describe a 46-year-old Greek male with chronic GD confirmed by low glucocerebroside activity in fibroblasts and N370S/L444P mutations at the cerebrosidase gene, who also had systemic AL amyloidosis diagnosed by bone marrow diffuse plasmacytosis, serum monoclonal IgA-lambda, severe total proteinuria with monoclonal IgA-lambda, Bence-Jones-lambda and amyloid deposits in bone marrow, liver, spleen and kidney biopsy specimens. Treatment with melphalan and prednizolon has dramatically decreased both levels of serum M-IgA and proteinuria and also improved the clinical symptoms of amyloidosis. He died from restrictive cardiac disease 30 months after the diagnosis of amyloidosis. Previously reported cases of GD in association with AL amyloidosis are reviewed.

Amyloidosis↗
PubMed1995

Gastroscopic findings in Mediterranean Kaposi's sarcoma (non-AIDS).

The frequency and degree of gastrointestinal involvement in patients with Mediterranean Kaposi's sarcoma (non-AIDS), a newly recognized form of Kaposi's sarcoma, is unknown. Eighty-seven patients with Mediterranean Kaposi's sarcoma proven by skin and/or nodal biopsy underwent endoscopic study of the upper gastrointestinal tract. Of these, 71 (81.6%) had gastrointestinal lesions. All these patients had lesions in the stomach. Additional lesions were detected in the esophagus in 19 patients and in the proximal duodenum in 8 patients, whereas additional lesions in both the esophagus and duodenum were identified in 2 patients. The lesions were classified into 4 types according to their size, shape, and color. Most types of lesions showed characteristic discoloration, but lesions with the appearance and color of normal mucosa that histologically were shown to be Kaposi's sarcoma were also identified. The high prevalence of gastrointestinal involvement in patients with Mediterranean Kaposi's sarcoma (non-AIDS) suggests that an endoscopic examination of the upper gastrointestinal tract may be useful in non-AIDS-related forms of Kaposi's sarcoma.

Adult↗
PubMed1995

HLA in familial and nonfamilial Mediterranean Kaposi's sarcoma in Greece.

Fifty-four (54) unrelated patients with Mediterranean Kaposi's sarcoma (MKS) and 8 patients members of 4 unrelated families with familial MKS were serotyped for HLA-A,B and DR antigens. The diagnosis was histologically confirmed and all patients were negative for anti-HIV antibodies. An increased frequency of HLA-B18 (44.4% vs 14.2% in the controls, p < 0.001, RR = 4.8) and HLA-DR5 (57.6% vs 37.2% in the controls, p < 0.025, RR = 2.29) was observed in the group of patients with MKS. Seven (7) of the 8 family members with FMKS possessed HLA-DR5, and the affected members in the 3 families shared a common haplotype which included HLA-DR5. These findings support the hypothesis that genetic factors linked to HLA-DR5 antigen may contribute to the pathogenesis of MKS.

Adult↗
PubMed1984

Histocompatibility antigens HLA-A, -B, -DR in Greek patients with Kaposi's sarcoma.

Thirty-two Greek patients with histologically documented Kaposi's sarcoma, aged 46 to 82 years, were typed for HLA-A, B and DR antigens. None of them was homosexual and they had not been subjected to any immunosuppressive therapy. The study revealed a significant increase of HLA-DR5 (53.1% vs. 21.4%, R.R. 4.1) and a decreased frequency of HLA-DR1 (3.3% vs. 16.6%, R.R. 0.16). An increased frequency of HLA-B18 was also noted (43.7% vs. 20.7% R.R. 2.96). These results indicate that the same positive association with HLA-DR5 antigen is observed in Greek patients as in other patients of Mediterranean origin and support the view that HLA linked factor(s) may have a role in the development of the disease.

Aged↗