Immunoproliferative small-intestinal disease. An immunohistochemical study.

We performed a detailed histological and immunohistological study on both fresh-frozen and paraffin-embedded tissue from full-thickness jejunal biopsy specimens taken from three patients with immunoproliferative small-intestinal disease (IPSID). In all three patients, the mucosal infiltrate consisted of "centrocyte-like" (CCL) cells forming lymphoepithelial lesions and plasma cells. In one patient, the mucosal infiltrate was strikingly follicular. Immunohistochemistry showed alpha 1 heavy chain, but no light chain, in the perinuclear space and cytoplasm of the CCL cells and in the plasma cells. In two patients, the plasma cells (but not the CCL cells) also contained alpha 2 heavy chain. In the case showing a follicular pattern, the extrafollicular CCL cells and most of the cells within the mucosal follicles expressed alpha 1 heavy chain, but a minor and variable population of cells expressed polytypic IgM. The dendritic reticulum cells stained for alpha 1 (but not alpha 2) heavy chain, mu chain, and both light chains. In all cases, the CCL cells did not stain for common acute lymphoblastic leukaemia antigen (CALLA); in the follicles, CALLA negative cells displaced a residual CALLA-positive population to the periphery and merged with the CALLA negative cells outside the follicles. These findings confirm the homology between IPSID and low-grade B-cell "Western" lymphomas arising in mucosa-associated lymphoid tissue; they suggest that the follicular pattern sometimes seen in these lymphomas is caused by selective colonization of reactive follicles by CCL tumor cells.

Light chains in Mediterranean lymphoma.

Four cases of Mediterranean lymphoma, in two of which serum alpha chains had been identified, were investigated with immunohistochemical techniques. In addition to alpha chains of alpha 1, subclass, monotypic light chains were identified in infiltrating tumour cells in all four patients and in plasma cells in two. The pattern of staining was in keeping with loss of light chain occurring with plasma cell differentiation and strongly suggested that the plasma cells and tumour cells shared a common clonal origin. In two patients concentrations of dendritic reticulum cells were identified in the tumour. These results support the suggestion that Mediterranean lymphoma is a tumour of follicle centre cells which undergoes plasma cell differentiation as a result of exposure to lumenal antigen.

Immunoglobulin gene rearrangement in immunoproliferative small intestinal disease (IPSID).

Analysis of DNA from the mucosal tissue of three patients with immunoproliferative small intestinal disease (IPSID) and alpha chain disease, two of whom had early stage disease responsive to antibiotics, showed monoclonal heavy and light chain gene rearrangements in all cases. These findings suggest that IPSID is neoplastic even in its early stages, but that the neoplastic cells respond to normal stimuli. Monoclonal lymphoid populations could not be detected in circulating lymphocytes from these patients, which raises the possibility that the circulatory pathways of lymphocytes derived from human gut associated lymphoid tissue may not necessarily parallel those in experimental animals.

Complete appendicular inversion: the "inside-out" appendix. An unusual presentation of Crohn's disease. A case report and review of the literature.

Intussusception of the appendix into the caecum is an uncommon condition and the diagnosis is rarely made preoperatively. Specific anatomic requirements as well as pathological conditions that cause irritation or increased peristalsis should be present for this event to occur. A case is discussed in which the diagnosis was made preoperatively by barium enema and in which the lead point was a large inflammatory polyp due to Crohn's disease. It appeared in this case that inflation at the time of the barium enema resulted in reduction of the intussusception.

Immunoproliferative small intestinal disease: a study of 13 cases with alpha heavy-chain disease.

The pathology of 13 cases of immunoproliferative small intestinal disease (IPSID) associated with alpha-heavy-chain disease--one an apparent non-secretor and another with localized infiltration--is described. Four cases exhibited immunohistological light-chain monotypia. In one of these, evolution of a light-chain negative cell population was observed over a 7-year period. In the intestine, centrocyte-like cells produced lympho-epithelial lesions in 11 cases and enlarged lymphoid follicles in three. In lymph nodes, perifollicular infiltration was observed in 11 cases and abnormal follicles in six. Of three patients with high-grade lymphoma at presentation, one died untreated at 2 months, and two are alive at 34 and 91 months. Of 10 patients with low-grade disease at presentation, two died--one at 76 months, the other after transforming to high-grade lymphoma at 73 months. Eight patients with low-grade disease are alive, an average of 67 months after presentation. Four of five conservatively treated low-grade cases (including three in remission) showed evidence of monoclonality at presentation (light-chain monotypia in two and gene rearrangement in two), while two of the five exhibited DNA aneuploidy. It is concluded that IPSID with alpha-heavy-chain disease is neoplastic in all its stages and is a variant of mucosa-associated lymphoma. The role of centrocyte-like cells and the response to conservative therapy are discussed.

Etomidate as a rectal induction agent. Part I. A preliminary study in rats.

Etomidate (Hypnomidate; Janssen) 1,25% in sterile water was given rectally on 100 occasions to 50 male Long-Evans rats in doses ranging from 4 mg/kg to 12 mg/kg. The onset and duration of ataxia and hypnosis (i.e. loss of righting ability) were recorded. Ataxia was observed in all rats, even at the lowest dose levels. The lowest hypnotic dose was 6 mg/kg, when 2 out of 5 rats lost their righting ability. In all 50 rats given 8 mg/kg or more hypnosis occurred within 4 minutes (range 2-4 minutes, average 3,3 minutes), from which they recovered within an average of 10,4 minutes. The duration of hypnosis and time to full recovery from ataxia were dose-dependent. The rectal hypnotic dose of etomidate in rats has been found to be approximately ten times the documented intravenous dose. No mortality was recorded despite the rectal administration of ten times the intravenous lethal dose (LD50) for rats. Histological examination of the rectal and colonic mucosa showed that etomidate 1,25% in sterile water (pH 3,5) caused no significant mucosal change, but undiluted etomidate 12,5% (pH 1,8) caused haemorrhagic necrosis. We conclude that rectal etomidate 1,25% in sterile water is an efficient, predictable, evanescent and safe method of inducing anaesthesia in rats and warrants further investigation in clinical anaesthesia in children.

Dermal and intravascular fasciitis. Unusual variants of nodular fasciitis.

Three cases with characteristic features of nodular (pseudosarcomatous) fasciitis arising in the dermis are described. This is only the second report documenting a dermal location for this condition. One of the three cases also showed features of intravascular fasciitis. A fourth case of intravascular fasciitis arising in periocular tissue is included. The differential diagnosis of dermal fasciitis includes benign fibrous histiocytoma, pyogenic granuloma, peripheral nerve tumors, cutaneous smooth muscle tumours, spindle cell carcinoma, spindle cell melanoma, dermatofibrosarcoma protuberans and atypical fibroxanthoma.

Malakoplakia. A case report and review of the renal manifestations and immunopathology.

A case of renal malakoplakia associated with prednisone therapy is reported; clinical recovery had taken place 6 months later, after nephrectomy and cessation of corticosteroid administration.

Heterotopic thyroid tissue in the heart.

Heterotopic thyroid tissue, presumably due to an embryological developmental abnormality, was discovered incidentally within the right atrial wall of an elderly woman who underwent coronary arterial aortic saphenous vein bypass grafting. As far as we are aware, this is only the third patient with thyroid heterotopia of the heart to be documented.

Randomized comparison of R1 and R2 gastrectomy for gastric carcinoma.

The contention that the R2 radical gastrectomy for localized and potentially curable gastric carcinoma may be superior to gastrectomy without lymphadenectomy (R1) was assessed by randomized trial. Five years after commencement 403 patients have been evaluated at surgery and only 43 (11 per cent) found eligible (S0-2, P0, H0, N0-1), 22 of whom underwent R1 and 21 R2 gastrectomy. Seven patients had final histological stages in excess of the protocol. The R2 group had a longer operating time (P less than 0.005), a greater blood transfusion requirement (P less than 0.005), a longer hospital stay (0.05 greater than P greater than 0.025) and required reoperation in four cases. There were no postoperative deaths. Four patients have died from the disease in the R1 group and five in the R2 group, there being no difference in the probability of survival at a median follow-up of 3.1 years. The small proportion of patients suitable for radical R2 surgery, the high associated morbidity and the fact that survival advantage has yet to be proven in trial suggest that this procedure should not yet be performed outside of controlled clinical trials.

Primary oesophageal rhabdomyosarcoma.

A case is presented of a rhabdomyosarcoma of the oesophagus with a description of the cytology, light microscopy, and immunocytochemical findings and a discussion of spindle cell tumours occurring at this site. Cytologically, large bizarre shaped pleomorphic cells were seen in which desmin was demonstrated in order to confirm the diagnosis after destaining a Papanicolaou stained slide and restaining it with antibody to desmin.

Lymphocytic gastritis in nonulcer dyspepsia.

The prevalence of lymphocytic gastritis, a specific form of chronic gastritis characterized by infiltration of gastric superficial epithelium with T lymphocytes, has been established in nonulcer dyspepsia. Among a population sample of 586 patients at risk for gastric carcinoma, 0.83% of patients with nonulcer dyspepsia and 1.63% of patients with chronic active gastritis showed lymphocytic gastritis. Among routine gastric biopsies from 5130 patients, only five cases met histological and immunohistochemical criteria of lymphocytic gastritis.

Case management to enhance HIV risk reduction among users of injection drugs and crack cocaine.

Case management is being applied to increasingly diverse client populations. This article describes the application of a field-based, service-broker model of case management with a population of injection drug users and crack cocaine users within the context of an AIDS risk-reduction program. The major problem encountered involved client engagement. The feasibility of implementing an effective case management program with active users of injection drugs and crack is questionable so long as involvement with such drugs is cast primarily as a criminal justice problem.

Myospherulosis: further ultrastructural observations.

The changes of myospherulosis were observed in masses from the buttocks of three patients. Ultrastructural examination of parent bodies and spherules from two patients revealed some spherules to contain dense bodies and filaments. Spherules had a double electron dense wall: the outer layer had a thickness corresponding to that of a cell membrane. Incubation of packed red blood cells with tetracycline ointment produced a similar electron dense deposit on the inner aspect of the cell membrane of the erythrocytes. No filaments or parent bodies were seen. Our findings support the hypothesis that myospherulosis represents altered red blood cells. The filaments seen in our patients may represent polymers of haemoglobin. It is suggested that parent bodies may be derived from histiocytes.

The nutritional consequences and neoplastic potential of juvenile polyposis coli.

Case reports of three teenage patients with nonfamilial diffuse juvenile polyposis are presented to illustrate the considerable morbidity and significant growth retardation that may occur. Although generally considered to have no malignant potential, histologic findings revealed features ranging from adenomatous change to severe dysplasia. It is suggested that a conservative approach may not always be appropriate in this condition.

Abdominal tuberculosis: still a potentially lethal disease.

The findings in a 4-yr survey of 82 patients with abdominal tuberculosis are described and compared with those encountered in previous surveys. Fourteen cases of intestinal, 11 of mesenteric-lymphnodal, and 57 of peritoneal tuberculosis were identified. The disease occurred essentially in patients living under worsening socioeconomic conditions, and 51 of them had associated pulmonary tuberculosis. Symptoms and clinical findings were again nonspecific, but newer imaging, endoscopic, and other invasive procedures were helpful in establishing a definite diagnosis. In addition, adenosine deaminase determination showed great promise as a noninvasive diagnostic procedure in patients with tuberculous ascites. The six hospital deaths in the series highlight the hazard of potentially lethal delays in early diagnosis and treatment, even in centers with a high awareness of the disease.

Hydatid cyst in the head of the pancreas with obstructive jaundice.

We report a rare case of obstructive jaundice caused by an intrapancreatic hydatid cyst in a 17-year-old black girl. Ultrasonography and computed tomography demonstrated the obstructing cyst in the head of the pancreas. Cyst aspiration produced clear fluid with a low amylase content and no hydatid hooklets or protoscolices. Pancreaticoduodenectomy was performed for a presumed cystic neoplasm of the pancreas, but histology showed the true diagnosis. Pancreatic hydatidosis should be considered in the differential diagnosis of obstructing pancreatic cysts in the appropriate epidemiological setting.