[Reminiscences on the occasion of the Whipple's disease centenary: a summary paper]. / Reminiscence k stoletému výrocí objevu Whippleovy nemoci: souborný referát.

The disease referred to eponymically as Whipple's disease (WD) in medical literature was thoroughly described by the American physician and pathologist George Hoyot Whipple (1878-1976) in 1907 and given a temporary denomination of "intestinal lipodystrophy". According to literature, WD is rare, but its precise incidence has not yet been established. Familial incidence of the disease is acknowledged, and its immunogenetic pathogenesis is assumed. The incidence ofWD is prevailingly observed in middle-aged men (mean age 55), exceptionally at child age - the ratio being 3 to 6 for men and women, respectively. 1. Clinical diagnosis is based on symptoms in the GIT region and, in rare cases, on extraintestinal symptoms. Clinical symptomatology includes: abdominal pain with persistent diarrhoea (steatorrhoea), symptoms typical of malabsorption connected with weight loss, fevers, polyarthritic symptoms, swollen lymph nodes and, in part of patients, skin hyperpigmentation. Anaemia and hypoalbuminaemia (reduced IgA) are typically detected in laboratory tests. Rarer extraintestinal symptoms of the disease are of a diverse nature: cardiac lesions, cerebral lesions, ocular symptoms, conspicuous or even tumour-like enlargement of lymph nodes, lesions of the hemopoietic system. The clinical course ofWD is of progressive or remittent nature and the disease is fatal without treatment. Long-term therapy with antibiotics, especially a combination oftetracyclines (doxycyclin) and corticoids (dexametazone), or chloramphenicol in case of cerebral lesion, have a significantly positive effect on the course and prognosis of WD. From the point of view of pathology, WD is a multisystem infectious disease (Tropheryma whipplei) primarily affecting the GIT (39, 47, 52, 103) or different extraintestinal locations. Due to the known diversity of clinical symptoms, no clinical-diagnostic standard has been established for WD. Differential diagnosis includes different multisystem diseases, primarily malignant lymphomas (especially Hodgkin's disease). From the pathogenetic point of view, we can either assume the effect of a particular cytokine (or TNFalpha) activating macrophage phagocytosis or, if its production is normal, a disorder or defect of the respective receptor in the macrophage cellular membrane. The identification of "Whipple's bacteria" - Tropheryma whipplei - gen. nov. et sp. nov. was made possible by modern molecular biology research methodologies. Its cultivation allows both for the acquisition of the specific antibody and of detailed knowledge of its genoma (PCR, 16S rRNA sequencing).

[The subacute angiohypertrophic myelomalacia Foix-Alajouanine--a rare disease?]. / Subakutní angiohypertrofická myelomalacie Foix-Alajouanine--raritní jednotka?

The subacute angiohypertrophic myelomalacia Foix-Alajouanine was first described by French authors in 1926 and belongs to the wider category of CNS vascular malformations. Both individual casuistics and larger sets of patients with this disease can be found in literature scarcely. We are presenting three necroptic cases of subacute angiohypertrophic myelomalacia (Foix-Alajouanine) that were mistakenly diagnosed clinically, once as metastatic lesion of spinal canal, once as amyotrophic lateral sclerosis, and once as inflammatory lesion of spinal cord. The histologic finding is dominated by myelomalacia of varying extent with varicosely dilated, constricted or even occluded arterialized both extra- and intramedullary veins, sporadically coupled with secondary thrombi. Clinical diagnostics is difficult and requires spinal angiography. According to literature data, it is presumable that this disease is not as rare as it may seem at first sign.

[Diagnosis of lymphoproliferative diseases from bone marrow biopsies]. / Zkusenosti s diagnostikou lymfoproliferativních onemocnení v biopsii kostní drene.

The cytological, histological and immunophenotypical markers of lymphoproliferative diseases are evaluated in the collection of 950 trepanobiopsies, and correlated with types of extramedullary malignant lymphoma. The diagnostic significance of tumor infiltrates topography and basic rules of the differential diagnoses of malignant lymphomas in the bone marrow biopsies are discussed.

Foreign material as a cause of choledocholithiasis.

BACKGROUND AND STUDY AIMS: There are sporadic reports concerning foreign material as a cause of choledocholithiasis. Gallstones obtained endoscopically from the biliary duct were analyzed to determine their structure and the presence of foreign material. The possible correlation between foreign material in gallstones and clinical data was investigated. PATIENTS AND METHODS: Gallstones were examined qualitatively using a crystallographic analysis by X-ray powder diffraction, and the foreign material which was found was subjected to morphological analysis using polarization microscopy. Patients' clinical data were obtained from clinical records, physical examination and documentation. RESULTS: Out of a group of 54 patients, foreign material was found in CBD stones of six individuals (11.1%). Four of these patients were from the group who had undergone a previous cholecystectomy (29/54, 13.8%), and surgical suturing material was found in these four. On the other hand, in the group who had not undergone the operation, foreign material was found in two patients out of 25 (8%); in one case it was cellulose and in the other case the fibre was not identified. CONCLUSIONS: Foreign material can account for lithiasis in the biliary duct. In contrast to present reports in the literature, we have proved that this is possible even in patients without previous surgical intervention in the pancreatobiliary region.

[Prion diseases. Subacute spongiform encephalopathies in humans and animals (neural "anthropo- and zooprionoses")]. / Aktuální problémy prionových nemocí. Subakutní spongiformní encefalopatie lidí a zvírat (neurální "antropo- a zooprionózy").

General review concerning current problems of subacute spongiform encephalopathies (Prion-diseases) in humans and in animals. The work is based on the aetiological conception of "Prion" as an allosteric conformational variant of the Prion-protein with autocatalytic "infectious" properties. The therm "Anthropoprionosis" and "Zooprionosis" are recommended by the author for human and animal Prion-diseases. Actual medical problems of various Prion-diseases are discussed in more detail, particularly those of aetiopathogenesis, genetics and molecular-biological principles in Prion-replication as well as the consensual neuropathological and clinical diagnostical criteria. Neuropathological findings are evaluated based on personal experience of diagnosed and/or consulted CJD-cases. On the ultrastructural level the morphological proof of abnormal secondary phagosomes in neurons of the CNS with accumulation of protein material ("prionosomes") is documented. Finally, the risks of CJD and the infectious potential of various organs and contaminated materials are also mentioned. In the methodical part, safety precautions in handling histological material from stereotactic biopsies and necropsies of brain are described, including safety techniques of autopsy in cases of CJD.

[Dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos)]. / Dysplastický gangliocytom mozecku (Lhermitte-Duclos).

In a surgical specimen from a 33-year old woman with features of expansive cerebellar symptomatology, dysplastic gangliocytoma of the cerebellum (Lhermitte-Duclos) was diagnosed by histology. The lesion was characterized by typical proliferation of dysplastic neuronal elements in the cortical granular and Purkinje cell layers. Immunohistology showed focal positivity of synaptophysin in some cells, thus confirming their neuronal origin. In electron microscopy, peculiar features of ER in some of dysplastic cells was also noted. Genetic examination proved familiar incidence of Cowden's disease in several members. Chromosomal examination of the patient (karyotype 46, XX) was without abnormalities.

Immunoreactivity of new antibodies anti-p53 and anti-MDM-2 in paraffin embedded tissue samples.

Detection of various epitopes of the p53 and MDM-2 proteins, using new antibodies was performed on formalin-fixed and paraffin-embedded tissue samples from breast cancer and compared with results obtained using well-characterized antibodies. The results show that the distribution of positive nuclei and intensity of staining varies significantly depending on the antibody used, as well as on the microwaving procedure. Antibodies DO-14, DO-13 and SMP-14 have very good characteristics and are available for immunohistochemical analysis of p53 and MDM-2. Our results indicate, that immunohistochemical expression of p53 and MDM-2 is a not stable and unitary phenomenon and from this point of view a single antibody is not sufficient for its determination. Since, characteristics of p53 and MDM-2 molecules can vary from one sample to the next, panel antibodies capable of determining a wide range of wide type and mutant conformations must be used.

[Malignant B-monocytoid mantle zone lymphoma]. / Maligní lymfom plást'ové zóny, B-monocytoidní typ.

Three cases of B-monocytoid malignant lymphoma were studied by histology, immunohistology and electron microscopy. Two of them were primarily extranodal (in parotid gland and palatine tonsil), the third was in lymph nodes. All tumours were characterized by patients' long survival without signs of generalization.

Expression of estrogen receptors and estrogen-induced proteins in tumors of "hormone non-dependent tissues".

Estrogen receptors (ER), progesterone receptors (PR) and some other estrogen-induced proteins (EIP) were found in the cells of salivary gland carcinomas, thyroid carcinomas, in gliomas and in various farther neoplasms as well as in some normal tissues. The positivity was demonstrated in both females and males. The results indicate that steroid hormones and their receptors could play, both in normal and tumorous tissues, more important biological role than expected so far.

Experimental nonspecific immunostimulation by the Propionibacterium acnes vaccine.

The immunostimulation effect of the selected Propionibacterium acnes strain 16073 was described. The optimal conditions for the preparation of the P. acnes vaccine and the nonspecific immunostimulation effects of the vaccine on various experimental infections were evaluated.

Preliminary experimental experiences with xenotransplantations.

In our xenotransplantation experiments has been a major aim to induce specific tolerance. The results from the meeting of the donor's and the recipient's immunocompetent systems already in the period of embryonic development and/or the influence of methylprednisolone are described.

"Nude rabbit" with aplasia of B-lymphoid structures.

An original observation of the randombred nude rabbit with aplasia of B-lymphoid structures (absence of the follicles in lymph nodes and spleen, no Peyer's plaques). The thymus gland and the T-dependent structures in the spleen and lymph nodes were-on the contrary-well preserved. The nude skin of this genetic rabbit mutant contained large numbers of hair follicles showing almost intrafollicular retention of hairs with their subsequent dysplasia at the subepidermal and ostiopilar level.