Cardiac death after autologous stem cell transplantation (ASCT) for treatment of systemic sclerosis (SSc): no evidence for cyclophosphamide-induced cardiomyopathy.

In patients with systemic sclerosis (SSc) treatment-related mortality after autologous stem cell transplantation (ASCT) appears to be increased as compared to patients with hematological malignancies. In our phase I/II study on ASCT in autoimmune diseases a patient with SSc died on day 2 after ASCT. Here we report the results of the autopsy which revealed advanced pulmonary and cardiac fibrosis as the most probable cause of death.

Grading of tumors and tumorlike lesions of bone: evaluation by FDG PET.

Unlabelled: Clinical diagnosis of skeletal tumors can be difficult, because such lesions compose a large, heterogeneous group of entities with different biologic behaviors. The aim of this prospective study was to assess the value of PET in grading tumors and tumorlike lesions of bone.

Methods: Two hundred two patients with suspected primary bone tumors were investigated using FDG PET.

Langerhans cell deficiency in reticular dysgenesis.

Reticular dysgenesis is a rare inherited immunodeficiency characterized by the lack of blood monocytes and neutrophils and low lymphocyte counts, contrasting with normal red blood cell counts and normal or decreased platelet counts. Whether dendritic cells or macrophages, both of which derive primarily from blood monocytes, are affected in this condition remains unknown. We studied 7 patients with reticular dysgenesis.

Fluorodeoxyglucose positron emission tomography of soft tissue tumours: is a non-invasive determination of biological activity possible?

Since musculoskeletal tumours comprise a large heterogeneous group of entities with different biological behaviour, clinical diagnosis of such lesions can be very difficult. The aim of this prospective study was to assess the usefulness of 2-[F-18]-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (PET) in the non-invasive evaluation of soft tissue tumours. One hundred and two patients with suspected soft tissue neoplasms were investigated by FDG-PET.

Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas.

Unlabelled: Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Large haemangiomas may cause trapping of platelets and coagulation disorders known as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening thrombocytopenia, anaemia and intravascular coagulation.

[Color Doppler controlled needle biopsy in diagnosis of soft tissue and bone tumors].

In a prospective study we investigated 168 patients with musculoskeletal tumors, including 71 sarcomas, by core needle biopsy using the high-speed device Autovac. Monitoring with colour-coded duplex sonography allowed a well-aimed puncture of smaller or deeply localized lesions and also permitted the discrimination of necrotic and viable parts of the tumor. Adequate material for histologic diagnosis including grading and determination of tumor subtype was obtained from soft tissue sarcomas, soft tissue metastases, malignant lymphomas, plasmacytomas, and osteolytic skeletal secondaries.

Clonal origin of tumor cells in a plexiform neurofibroma with LOH in NF1 intron 38 and in dermal neurofibromas without LOH of the NF1 gene.

LOH at the NF1 locus was investigated in 38 neurofibromas of 26 NF1 patients. Only in one of these tumors LOH was observed. In this plexiform neurofibroma of a NF1 patient with a constitutional one base-pair insertion in NF1 exon 4b, a non-random X-inactivation pattern was found, strongly suggesting a clonal origin of the tumor cells.

[A case of sarcoidosis of the mastoid].

Background: Sarcoidosis is a common granulomatous systemic inflammatory disease mainly affecting the interstitial lung tissue and perihilar lymph nodes. Since its first description by Bernier 1889 its etiology has remained obscure.

Method: Sarcoidosis of the right middle ear in a 38-year-old woman was diagnosed by biopsy.

Histomorphological examination of endoluminal stent grafting in the descending aorta in a sheep model.

Experiments were designed to examine the wound healing characteristics at the aorta-endograft interface. Thoracic aneurysms were induced in sheep and excluded by endovascular placement of a selfexpanding stent graft (Corvita Endovascular Graft). After a follow-up of 1, 4, or 12 weeks sheep were sacrificed and the corresponding segments of the aorta were subjected to histological examinations.

Epstein-Barr-virus-associated lymphoproliferative syndrome in severe combined immunodeficiency: establishment of a lymphoblastoid cell line as an in vitro model for biological and therapeutic studies.

Patients with primary or secondary immunodeficiency are at high risk for B cell lymphoproliferative syndromes (LPS) that are generally Epstein-Barr virus (EBV)-associated. We established a cell line, termed JuWa, from an immunoblastic lymphoma that developed in a child with severe combined immunodeficiency. JuWa cells were representative of the original lymph node as shown by a similar IgH gene rearrangement pattern.

Intestinal cytomegalovirus disease in immunocompromised patients may be ruled out by search for cytomegalovirus DNA in stool samples.

Cytomegalovirus (CMV) PCR from stool specimens was adopted as a diagnostic tool for patients with suspected CMV colitis. After being established, the method was evaluated in 17 AIDS patients and 19 other immunocompromised patients by comparison of PCR results with clinical, histological, and microbiological or virological data. CMV PCR was positive in 4 symptomatic patients with proven CMV colitis and negative in 15 of 16 patients without characteristic histopathology.

[Inflammatory pseudotumors of the lung and trachea].

Inflammatory pseudotumors (synonym: plasma cell granulomas) of the lung and trachea are a group of non-neoplastic lesions of unknown etiology which may occur at any age. The complex histomorphology und proliferative capacity of these pseudotumors may result in diagnostic difficulties during intraoperative frozen section analysis. Four cases of inflammatory pseudotumors of the respiratory tract (three pulmonal, one tracheal pseudotumors) are reported.

A fatal infection with Alternaria alternata and Aspergillus terreus in a child with agranulocytosis of unknown origin.

Alternaria alternata and Aspergillus terreus were isolated from cutaneous nodules in a 5-year-old girl with agranulocytosis of unknown origin. Histopathological examination supported the diagnosis of an infection with two opportunistic moulds. Aspergillus terreus was also isolated from the secretions of the maxillary sinuses of the patient.

Hepatitis C virus infection is a risk factor for liver failure from veno-occlusive disease after bone marrow transplantation.

The contribution of hepatitis C virus (HCV) infection to liver disease after bone marrow transplantation (BMT) was retrospectively evaluated in 61 patients treated with BMT. HCV genome, as well as antibodies to HCV, was analyzed in sera collected before and serially after BMT. Six patients had been infected with HCV before BMT and three patients acquired the infection during or shortly after BMT.

A symptomatic papillary fibroelastoma of the left ventricle removed with the aid of transesophageal echocardiography.

A papillary fibroelastoma, causing amaurosis fugax and paresis of the right arm, was detected by echocardiography as a free floating mass in the left-ventricular outflow tract. Based on the exact localization of the tumor by intraoperative transesophageal echocardiography the initial indication for ventriculotomy was disregarded and an atraumatic transvalvular approach was chosen.

Experimental Yersinia enterocolitica infection in euthymic and T-cell-deficient athymic nude C57BL/6 mice: comparison of time course, histomorphology, and immune response.

To elucidate the role of T lymphocytes in primary infection with Yersinia enterocolitica, we investigated the elimination rate of this pathogen, the histomorphology of tissue lesions, and the immune responses of athymic T-cell-deficient C57BL/6 nude mice and their euthymic littermates after parenteral infection with Y. enterocolitica of serotype O:8. While a low inoculum of 3 x 10(2) Y.

The cellular immune response against Yersinia enterocolitica in different inbred strains of mice: evidence for an important role of T lymphocytes.

Resistance of mice against infection with Yersinia enterocolitica has been shown to be related neither to the Ity locus coding for resistance against infection with Salmonella typhimurium and other pathogens nor to the H-2 locus. From other mouse infection models, e.g.

Immunohistological characterization of the cellular immune response against Yersinia enterocolitica in mice: evidence for the involvement of T lymphocytes.

Previous work from this laboratory has demonstrated that cloned T lymphocytes from spleens of Yersinia-infected mice can transfer immunity against Y. enterocolitica into naive animals. In this study, we investigated the cellular immune response to parenteral infection of Yersinia-resistant C57 BL/6 mice with the highly virulent Y.

[Idiopathic ulcer of the small intestine].

The authors report on a female patient, 32 years old, suffering for eight years from recurrent and progressive abdominal pain, combined with chronic anemia, due to iron deficiency. Neither anamnesis and clinical course, nor technical examinations elucidated the etiology. Finally an explorative laparotomy with segmental resection of the small intestine led to diagnosis and healing of the rare "idiopathic small bowel ulcer".

[Severe combined immune defect. Presentation of exfoliative dermatitis with eosinophilia and lymphadenopathy].

Background: We report on 9 infants with severe combined immunodeficiency (SCID), who additionally showed signs of Omenn syndrome with an exfoliative dermatopathy, alopecia, enlarged lymph nodes, a hepatomegalia and a striking blood eosinophilia. The immunological evaluation revealed the characteristic abnormalities of SCID with cellular and humoral immunodeficiency. All patients however had the unusual finding of mature T cells in the peripheral blood.

Peripheral dentinogenic ghost cell tumor.

A case of dentinogenic ghost cell tumor, that has originated peripherally in the jaw, is presented and the literature reviewed with particular reference to the origin of the tumor. The total number of central and peripheral cases reported in the English literature is 10 and although mucosal infiltration is common, peripheral origin of the neoplasm could be verified in only 3 cases.

[Cytostatic-induced stomatitis].

45 patients with oral cancer preoperatively received regional intraarterial chemotherapy (RIAC). All patients developed stomatitis or glossitis limited to the region of cytostatic perfusion. Between 1 and 19 days (median 4 days) after RIAC the tumor was removed by hemiglossectomy, partial resection of the floor of the mouth etc.

Gonadal agenesis in a 46,XY female with multiple malformations and positive testing for the sex-determining region of the Y chromosome.

A full-term 46,XY female newborn presented with respiratory failure due to a right-sided diaphragmatic hernia. During surgical repair, exploration revealed isolated dextrocardia and hypoplasia of the right lung. Neither gonads nor wolffian or müllerian structures could be palpated.