Autopsies in HIV: still identifying missed diagnoses.

This study reviews the deaths and autopsies carried out over 23 years, 1983-2005, in a British Infection Unit in HIV patients. Of 115 HIV patients known to have died, we obtained data on 93%. Of this 80% were male, median age 38 (25-68) years; 83% were Caucasian; 12% Black African. Major risk factors were men who have sex with men, 52%; heterosexual in Africa, 17%; and injecting drug use, 8%. The commonest diagnosis pre- and post-autopsy diagnosis was pneumonia. Changes in diagnoses in the 38% who underwent autopsy were high (we requested autopsy in 50%). Primary diagnosis changed in 70%, and 36% of all opportunistic infections were missed. This included six of nine cytomegalovirus, all tuberculosis and 75% of Kaposi's sarcoma. Lymphoma was overdiagnosed. Thus, despite excellent resources, the majority of primary diagnoses were wrong, suggesting inadequacy of current diagnostics. To improve these and improve both epidemiological data and future management autopsy should be considered for all deaths.

Cystic neoplasms of the exocrine pancreas.

The increasing use of radiological imaging has led to greater detection of small and asymptomatic cystic lesions of the pancreas. Most are resectable, but not all are neoplastic. This review provides an update on the histopathology, immunohistochemistry, molecular biology, pathogenesis and management of cystic neoplasms of the exocrine pancreas. These include the serous, the mucinous cystic, the intraductal papillary mucinous and the solid pseudopapillary neoplasms. Recently reported variants are described and very rare cystic variants of other pancreatic epithelial and mesenchymal neoplasms are briefly mentioned.

Sedaghatian congenital lethal metaphyseal chondrodysplasia--observations in a second Iranian family and histopathological studies.

In 1980, Sedaghatian described in two brothers and one sister a neonatally lethal disorder associated with slight rhizomelic limb shortness, mild platyspondyly, and severe metaphyseal dysplasia. Here data are presented on another Iranian infant with the Sedaghatian syndrome who died on day 4 and was found to have histologic evidence of severe epimetaphyseal dysplasia. The occurrence in children of both sexes in one instance, born to normal parents who were first cousins, and currently apparent confinement of the disorder to Iranians suggests that the Sedaghatian syndrome is an autosomal recessive trait with high gene frequency in Iranians. This may be a more complexly pleiotropic syndrome than suggested by the roentgenograms, since one of Sedaghatian's patients also had "microphthalmia, asymmetry of ears, depressed nasal bridge, broad nose, short neck, prominent sternum, and short lower extremities."

High prevalence of human immunodeficiency virus infection in children with thalassemia exposed to blood imported from the United States.

PIP: 38.5% or 10 of 26 children with thalassemia who had received blood transfusion have tested positive for HIV at the Hamad General Hospital, Doha, Qatar. Blood for transfusion was obtained from commercial suppliers in Miami, Florida, since late 1970. Since December 1985 all blood for transfusion has been tested for HIV for ELISA kits from Abbott Laboratories, Chicago, Ill, and confirmed by Western Blot. These children ranged from 3-13 years old, mean 7.7, and had received an average of 63.5 transfusions. All had been given at least 30 transfusions. Children testing negative for HIV averaged 35.0 lifetime transfusions. 4 of the affected children had persistent generalized lymphadenopathy, but no other symptoms were recorded.^ieng

Localized Leishmania lymphadenitis. Immunohistochemical studies.

Tissues from 10 patients with localized leishmania lymphadenitis were stained by immunoperoxidase technique for leishmanian antigens, immunoglobulins (Ig), complement, components, alpha 1-antitrypsin, and fibrin and by routine and chloroacetate esterase techniques. Lymph nodes with leishmania lymphadenitis showed a granulomatous process with a varying degree of necrosis, leishmanian amastigotes, abundant plasma cells, and focal fibrosis. Leishmanian antigens stained by rabbit anti-Leishmania tropica antiserum was detected in lymph nodes with granulomatous lymphadenitis (eight cases examined) and in a skin lesion but not in a lymph node showing only nonspecific hyperplasia. Positive antigen staining was seen in leishmanian amastigotes, granulomata, and (in three cases) vascular smooth muscles. Dominant plasma cell classes were IgG and IgE. Macrophages and granulomata stained positively for IgG and IgE (but not for IgM or IgA), faintly for C3, and strongly for alpha 1-antitrypsin. Mast cells were few. IgG and IgE may be involved in a specific way in this condition; formation of immune complexes with leishmanian antigens leads to immunologically mediated lysis of the host macrophages. This finding is important for the understanding of the pathogenesis of leishmania lymphadenitis and for effective elimination of the parasites.

Immunoglobulin levels in infantile pneumocystosis.

Two hundred and twelve determinations of IgA, IgG, and IgM were performed in 50 infants during an epidemic of interstitial plasma cell pneumonia. Criteria for diagnosis are discussed. The immunoglobulin levels in pneumocystic, non-pneumocystic, and normal American infants are compared. An analysis of the findings in individual cases reveals a time-related immunoglobulin response, which helps to elucidate the pathogenicity of the disease.

"Localized" leishmania lymphadenitis: a light and electron microscopic study.

Nineteen cases of "localized" leishmania lymphadenitis without any evidence of visceral leishmaniasis are reported. Fifteen males and 4 females aged 5 to 30 years have presented with localized lymphadenopathy of up to 3 months duration. The disease has a seasonal incidence of late summer to mid-winter. Cutaneous leishmaniasis even when present, usually was overlooked. Lesions of cutaneous leishmaniasis were absent in 3; healed in 1; small, similar to insect bites in 5; classic in 3; lupoid in 1 and unknown in 6 patients. Serum leishmanial antibody determination by IFAT performed on 12 cases were positive. Toxoplasma serology was negative. Histological picture of one lymph node biopsy showed an (anergic) intact histiocytic response characterized by thousands of intracellular amastigotes, no necrosis and inconspicuous plasma cells. In 17 biopsies the picture was that of histiocytic granulomata with varying degrees of necrosis, moderate numbers of amastigotes in several foci, fibrosis and varying numbers of plasma cells. One biopsy from a lupoid case shows numerous epitheloid granulomata, no organisms, no necrosis and inconspicuous plasma cells. Electron microscopy has been performed on 8 biopsies to confirm leishmania amastigotes. Differential diagnosis from toxoplasmosis and cat-scratch disease is discussed. Histological types of responses in the lymph nodes are comparable to those described in cutaneous leishmaniasis: an anergic response with intact macrophage granuloma, a histiocytic response with necrosis, and a lupoid type of response with epithelioid granulomas.

Histological spectrum of cutaneous leishmaniasis due to Leishmania tropica.

Skin biopsies from 117 cases of acute and 23 cases of lupoid leishmaniasis were studied. Of the 117 acute cases, anergic macrophage response were seen in 44, diffuse necrosis in 9 and focalized necrosis in 11 biopsies; the remaining 53 biopsies showed scanty or no parasites with a few to abundant epithelioid cells probably representing post necrotic changes before complete healing of the lesions. The 23 biopsies of lupoid leishmaniasis showed rather well organized epithelioid granulomata surrounded by lymphocytes, inconspicuous plasma cells, no amastigotes and no necrosis. Lupoid cases showed strong delayed hypersensitivity with leishmanin and low serum antibody titres. It appears that the lesions of urban cutaneous leishmaniasis spend a long time in the anergic phase. Partial destruction of parasites by activated macrophages together with gradual elevation of antibody levels prepares the appropriate antigen-antibody ratio for optimum development of necrosis at PI3 leading to effective elimination of parasites. Plasma cells appear to be important in the induction of necrosis and inhibition of epithelioid granulomata. A significant inverse relationship has been found between plasma cells and epitheloid cells. The delayed hypersensitivity observed in lupoid leishmaniasis is probably the result of a poor humoral response evidenced by inconspicuous plasma cells and low serum antibody titres.

Arterial fibrodysplasia: a regional cause of peripheral occlusive vascular disease.

The authors studied 17 cases of symptomatic arterial fibrodysplasia involving peripheral arteries without concomitant renovascular hypertension in Iranian males (age range: twenty-two to forty-five years). Affected arteries were most commonly the femoral, iliac, and popliteal, but in 2 cases the axillary and brachial arteries were also affected. Three cases were progressive. Differential diagnosis of arterial fibrodysplasia is discussed. It is concluded that this entity is the most common cause of peripheral occlusive vascular disease in younger Iranian males.

Verruciform xanthoma of the penis.

Verruciform xanthoma is a rare pathologic entity with a predilection for the oral mucosa. A case of verruciform xanthoma involving the penis, the second reported in this location, is presented.

Spindle cell hemangioendothelioma in association with epithelioid hemangioendothelioma.

A forty-two-year-old man presented with a twelve-year history of a multilobular, firm, but nonulcerated tumor (6 by 4.5 cm) of the dorsum of the right foot. He had capillary telangiectasia of both ankles but no lymphedema. Results of tests for human immunodeficiency viruses I and II were nonreactive. The tumor showed a complex combination of features of spindle cell hemangioendothelioma, epithelioid hemangioendothelioma, and areas suggestive of a possible well-differentiated (lymph) angiosarcoma. The tumor was completely excised with minimal healthy margin. There was no evidence of local or distant lesion at one-year follow-up examination.

Rhinosporidiosis: immunohistochemical and electron microscopic studies.

Sixteen biopsies of rhinosporidiosis (15 nasal and one conjunctival) from 16 Southern Indian male immigrant workers showed mucosal lymphoplasmacellular infiltrates together with transepithelial elimination of nodular bodies and destruction of some late stage nodular bodies in histiocytic granulomata with central neutrophilic microabscesses. Early nodular bodies were immunohistochemically positive for alpha 1-AT, alpha 1-ACT, CEA, S100, fibronectin, amyloid-p-component, IgG, IgA, C1q and C3. Electron microscopy showed organized concentric lamellated bodies in early nodular bodies and not in end-stage nodular bodies which contained mostly amorphous electron dense materials. Structures formerly regarded as 'sporangia' and 'spores' are believed to be lysosomal bodies loaded with indigestible residues to be cleared via transepithelial elimination or segregated/destroyed by secondary immune/granulomatous responses.

Glomus tympanicum chemodectoma: unusual radiological findings.

A 64-year-old Qatari female, with a one-year history of right otorrhoea and deafness, had a reddish-white mass projecting into the right ear canal, through the tympanic membrane, that proved histopathologically to be a paraganglioma. Computerized tomography (CT) of the temporal bones showed extensive destruction of the right mastoid bone, the posterior ear canal wall, and the sinus plate, with total disruption of the ossicles, simulating a malignant tumour, which is unusual for a middle ear paraganglioma.

Inflammatory pseudotumor of the spleen: a case report.

An inflammatory pseudotumour of the spleen mimicking a lymphoma clinically is reported. Immunohistochemical studies and the characteristic polymophous inflammatory and fibroblastic infiltrates confirm the lesion as benign.

Kikuchi's lymphadenitis in Qatar.

The clinicopathologic features of five cases of Kikuchi's lymphadenitis (histiocytic necrotizing lymphadenitis without granulocytic infiltration) occurring in Qatar are presented. There were three females and two males aged from 18 to 36 years with a mean of 29 years. Three were Indians and two were Qataris. Cervical lymph nodes were affected in all cases while one patient also had inguinal and femoral lymph node enlargement. Three patients achieved complete remission within three months. The differential diagnosis both clinically and histologically, included malignant lymphoma, toxoplasmosis, tuberculosis, leprosy, Yersinia and viral infections. To avoid errors in diagnosis, especially that of malignant lymphoma, attention must be paid to the location of the lesion, composition of the cellular infiltrate and the typical fibrinoid type of necrosis in the background.

Small, flat colorectal cancers in the UK population: an analysis of resected specimens.

OBJECTIVES: To establish the prevalence of small, flat carcinomas in surgically resected colon. To determine whether tumour morphology influences stage at presentation. METHOD: 1763 surgically resected colorectal cancers from one UK centre excised between 1995 and 2004 were examined. Age 69 years, (42-90), M:F equal. Sixty-one tumours < or =20 mm across were identified. Slides were reviewed by a consultant histopathologist and classified using Japanese Research Society Classification, JRSC and TNM staging. Fisher's exact test was used for analysis. RESULTS: In 61 small cancers, 64% (39/61) showed flat morphology and 33% (20/61) polypoid. Two lesions were unclassifiable. Prevalence was 2.2% of all resected colorectal cancers. More T1 tumours at presentation were polypoid, (30% vs. 8%; P = 0.033). T3 tumours were more likely to be flat than polypoid, (49% vs. 20%; P = 0.016). Infiltration into musclaris mucosa occurred in 77% (30/39) flat tumours. Rates of metastases were high in both groups, (30% polypoid vs. 39% flat, not significant). CONCLUSIONS: The prevalence of small, flat cancers in resected specimens in the UK concurs with that of Japanese studies. Small, flat cancers should be staged carefully because of high rates of T3/4 disease. The results support the theory of accelerated carcinogenesis in flat cancers.