Mucin-producing signet ring cell adenoma of the thyroid.

Signet ring cell adenoma of the thyroid, though rare, is well documented. This change is chiefly due to intracellular accumulation of thyroglobulin that appears mucinous. Awareness of this entity is important as it may closely simulate a metastatic mucin-secreting signet ring cell carcinoma. Although the mucinous material in signet ring cells has been reported to stain positive with thyroglobulin, in some cases it may not be so. We herein describe a rare case of a 46-year-old man who was hypothyroid and the mass removed from the thyroid showed a mucin-producing signet ring cell adenoma of the thyroid.

Inflammatory pseudotumor of lymph nodes presenting as pyrexia of unknown origin.

Inflammatory pseudotumor (IPT) is an uncommon benign disorder characterized by proliferation of spindle cells, inflammatory cells, and small vessels. The IPT of lymph nodes is a rare cause of lymphadenopathy that usually affects one or two nodal groups. We describe a 27-year-old male presenting with generalized lymphadenopathy, hepatosplenomegaly and fever for 1 year. Histologic examination of lymph node revealed few remnant lymphoid aggregates with marked sclerosis and numerous anastomosing blood vessels in lymph node parenchyma. Interspersed in between the fibrotic bands was a polymorphic infiltrate composed of lymphocytes, plasma cells, eosinophils, and immunoblasts. Also, many histiocytes, multinucleated giant cells some being Langhan's type and at places forming ill-defined granulomas were observed. The presence of granulomas and Langhan's type of giant cells can lead to a misdiagnosis of tuberculosis as was also done in the present case. It is thus not only important to be aware of this rare benign pathologic entity while dealing with a case of lymphadenopathy, but also consider it in the differential diagnosis of granulomatous disease.

Pathology and pathogenesis of rheumatic heart disease.

Cardiovascular disease is on the rise. In India and other developing countries, rheumatic heart disease (RHD) continues to be a major public health problem and contributes to significant cardiac morbidity and mortality. RHD in the juvenile age group namely juvenile mitral stenosis is a variant which is unique to the Indian subcontinent. Severe valve deformities lead to high morbidity and mortality. Despite various measures no appreciable decline in prevalence of RHD has been documented. At autopsy, mitral valve was most commonly affected either alone or in combination with aortic and tricuspid valves. Both functional and organic involvement of tricuspid valve was documented. It has been convincingly demonstrated that molecular mimicry between Streptococcus pyogenes antigen and human proteins lead to autoimmune reactions both humoral and cell mediated causing RF/RHD. Heart tissues namely the valves, left atrial appendage (LAA) and myocardium reveal variable amounts of infiltration by lymphocytes. Significant endocarditis and valvulitis is observed in these cases. CD4+ T cells are most likely the ultimate effectors of chronic valve lesions in RHD. They can recognize Streptococcal M5 protein peptides and produce various inflammatory cytokines such as TNF-alpha, IFN-gamma, IL-10, IL-4 which could be responsible for progressive fibrotic valvular lesions. Cardiac myosin has been defined as a putative autoantigen recognized by autoantibodies of RF patients. Cross reactivity between cardiac myosin and group A beta hemolytic Streptococcal M protein has been adequately demonstrated. Cardiac myosin has been shown to produce myocarditis in rats and mice. Valvulitis/ endocarditis has been observed in excised LAA, cardiac valves and in hearts at autopsy from cases of RHD. The disease predominantly affects the valvular endocardium culminating in crippling valve deformities. Endocardial infiltrate and their migration into the valve substance has been elegantly demonstrated in rats and mice. Immune responses against cardiac myosin lead to valvular heart disease and infiltration of the heart by Streptococcal M protein reactive T lymphocytes. Mitral valves showed various degrees of calcification. An interesting observation is the nature of calcification in diseased/distorted valves in RHD. Recent studies indicate that calcification is not merely an inactive, "dystrophic" process but involves a regulated inflammatory process associated with expression of osteoblast markers and neoangiogenesis. Increased plasma osteopontin levels correlated with severity of mitral valve calcification. Further evidence of inflammation is supported by high levels of advanced oxidation protein products and high sensitive C-reactive protein in plasma detected in patients with RHD. Presence of inflammatory cells and increased expression of several cytokines in cases of "end stage" RHD reflects a possible subclinical, ongoing insult/injury to some unrecognized antigenic stimulus by beta hemolytic Streptococcal antigens that have sensitized/primed the various target tissues and which further culminate in permanent valve deformities.

Bacteriological evaluation of conjunctiva, contact lens storage cases and solutions during contact lens wear.

A total of 90 subjects were investigated in this study, out of which 51 constituted the contact lens wearing group and 39 constituted the control group who had never worn contact lenses. Out of 51 contact lens wearing group, 41 were asymptomatic lens wearer subjects and 10 were symptomatic lens wearer subjects having some complications. All the subjects of contact lens wearing group had worn soft contact lenses on daily wear basis for a duration of 7 days to one year. In total 33 subjects were culture positive and remaining 57 culture negative. Staphylococcus epidermidis was isolated equally from asymptomatic and control group, however, significantly more gram negative organisms such as Pseudomonas aeruginosa and Serratia marcescens were isolated from the symptomatic lens wearers. P. aeruginosa and S. marcescens could also be isolated from lens storage cases. S. marcescens was the organism most commonly isolated from the contact lens solutions. Further, the results showed that both P. aeruginosa and S. marcescens adhere to injured cornea only and not to normal cornea when microscopic study of adherence of bacteria to uninjured and injured ex vivo mouse corneas was done.

Post-kala-azar dermal leishmaniasis: a histopathological study.

BACKGROUND: Post-kala-azar dermal leishmaniasis follows an attack of visceral leishmaniasis and is caused by the same organism, i.e. Leishmania donovani. METHODS: In the present study, biopsy specimens from hypopigmented macules, nodules or plaques of 25 patients clinically diagnosed as PKDL were evaluated for epidermal and dermal changes and for the presence or absence of Leishmania donovani bodies (LDBs). RESULTS: The hypopigmented macules showed a patchy perivascular and periappendageal infiltrate with no demonstrable LDBs in any of the biopsies. In the nodular and plaque lesions, the infiltrate was diffuse, beneath an atrophic epidermis (74%) and follicular plugging (95.6%) was seen in most biopsies. The infiltrate consisted of lymphocytes, histiocytes and plasma cells in decreasing order of presence. LDBs could be demonstrated in only 10 (43.5%) biopsy specimens from nodular and plaque lesions and were never numerous. CONCLUSIONS: Histopathological features of PKDL are elucidated and discussed.

An experimental design for induction of non-specific aortoarteritis.

BACKGROUND: An attempt was made to induce aortoarteritis in mice by using various antigens. METHODS AND RESULTS: The Swiss mice were immunized with eight different antigens and were grouped A to G. Group H served as control. The mice were then bled at 1st, 2nd, 4th, 6th and 8th month interval post-immunization for estimating antibody titer. Then the mice were sacrificed and the heart, aorta and kidney were taken out and processed for hematoxylin-eosin staining. There was gradual increase in the antibody titer from 1st month till 4th month within all the experimental groups (A-G), when compared with control group H. The titer started falling sharply from 6th month post-immunization. However, the control group H did not show much variation. When each individual group was compared separately with control group H, the significant statistical value was obtained. Histopathological examination revealed mild inflammation (+) in kidney by 2nd month, moderate inflammation (++) by 6th month, extensive inflammation (+++) by 8th month and alteration in the normal parenchyma of kidney by 8th month. CONCLUSIONS: The histopathological changes brought out through antigens were more pronounced by 8th month following injection of tunica media, tunica adventitia, tunica intima and aorta collagen as compared to that of standard collagen and mouse aorta injections.

Mucosal and peri-orificial involvement in post-kala-azar dermal leishmaniasis.

BACKGROUND AND AIMS: Lesions of post-kala-azar dermal leishmaniasis (PKDL) usually affect the skin. Uncommonly, the involvement of oral and genital mucosae has been reported. METHODS: Twenty five patients clinically diagnosed as post-kala-azar dermal leishmaniasis were studied for periorificial and mucosal lesions. Clinical examination, skin smears and biopsy were done for the patients with periorificial or mucosal lesions. RESULTS: Out of 25 patients of PKDL, seven patients had lesions on the oral and/or genital mucosa. Three cases had oral lesions; two had only genital lesions and three patients had both sites involved. All the patients were having skin lesions elsewhere too either as nodules and/or plaque or macules. Conclusion: While examining a case of PKDL, mucosal involvement must also be examined carefully.

Therapeutic trial of sodium antimony gluconate alone and in combination with ketoconazole in post-kala-azar dermal leishmaniasis.

BACKGROUND: Drugs used in PKDL include parenteral sodium antimony gluconate (SAG), amphotericin-B, pentamidine, and ketoconazole (KTZ). SAG is the most effective one. Given alone, SAG has to be given for a long duration, leading to poor patient compliance and treatment failure. This study was carried out to compare the effectiveness of SAG alone and a combination of SAG and KTZ for sixty days. METHODS: Ten patients of PKDL were included in the study. Five patients (Group A) were given SAG intravenously, in the dose of 20 mg/kg per day and five (Group B) were given SAG (intravenously 20 mg/kg per day) and KTZ (200 mg twice daily orally). Both treatment regimens were given for sixty days. RESULTS: In Group A, the nodules and/or plaques showed approximate 80-85% clinical improvement, and macules showed 25-30% improvement. In group B (SAG + KTZ), there was 90-95% clinical improvement in the nodules and/or plaques and 25-30% in macules. CONCLUSION: This study suggests the therapeutic superiority of the combination treatment regimen in a shorter duration but is not conclusive as the number of patients was low. Further trials are recommended.

Cardiac myxoma with glandular elements: a histologic, histochemical, and immunohistochemical evaluation.

Epithelial differentiation in cardiac myxoma is a rare phenomenon. Out of 104 surgically excised specimens, we studied 3 cases of cardiac myxoma with glandular differentiation. All the cases had well formed glands in addition to the myxoma cells lying in a myxoid background. Detailed histochemical and immunohistochemical studies suggest that the epithelial islands in cardiac myxoma show an enteric phenotype.

Isolated cardiac aspergillosis.

A 40-year-old man, a known case of Wolff-Parkinson-White syndrome, was admitted to the hospital in an unconscious state. In spite of medical treatment, the patient died within two hours of admission. At autopsy, the deceased was found to have aspergillosis involving the interatrial septum, aortic valve and root of the aorta. The rest of the organs were unremarkable. The patient did not show any obvious signs of being immunocompromised. We report this case of isolated cardiac aspergillosis in an apparently healthy individual.

Fatal atypical mycobacterial infection in a cardiac transplant recipient.

A 37-year-old female underwent heart transplantation for giant cell myocarditis. The patient died within three-and-a-half months of cardiac transplantation. Postmortem specimens from the heart and lung showed multiple necrotizing granulomas with numerous acid-fast bacilli. Polymerase chain reaction done on both the postmortem samples confirmed the presence of atypical mycobacterial infection. This fatal case of atypical mycobacteriosis in a cardiac transplant patient is reported for its rarity.

Non-myxomatous cardiac tumours: twenty-year experience.

Eighty-eight patients underwent surgery for various cardiac tumours from January 1978 to June 1998 at our Institute. Seventy-seven tumours were myxomas, 10 were non-myxomatous and one was secondary cardiac tumour. Case records of the patients with non-myxomatous primary cardiac tumours and one secondary tumour were reviewed. Six of these primary tumours were benign and four, malignant. Age of the patients ranged from 26 days to 47 years. Among patients (3 children, 8 adults) with non-myxomatous primary cardiac tumours, dyspnoea on exertion was the commonest symptom and was the cause of presentation in seven out of 11 patients. Of the eight adults, six were in New York Heart Association functional class II/III and two in class IV. Echocardiographic diagnosis was possible in all the patients. Complete excision of the tumour was possible in all benign and two of the four malignant tumours. Incomplete resection was done in the secondary tumour. Of the six benign tumours, three were rhabdomyomas and one each of fibroma, haemangioma and lipoma. The malignant tumours were one each of fibrosarcoma, angiosarcoma, unclassified sarcoma and malignant mesothelioma. The secondary tumour was a malignant thymoma. Follow-up ranged from 1 to 10 years (mean 7.2 years). Of the patients with benign tumours, four out of six are alive; one patient died on the first post-operative day and one lost to follow-up. Two of the four patients with malignant cardiac tumours died, one was lost to follow-up and one is alive two years after surgery. The patient with secondary malignant thymoma to the superior vena cava was lost to follow-up three months after an uneventful recovery from surgery.

Appraisal of histogenesis of cardiac myxoma: our experience of 78 cases and review of literature.

Cardiac tumours are uncommon. Of these, myxomas have generated significant interest, not only because it is the commonest cardiac neoplasm but also because of controversial theories regarding its histogenesis. We encountered 78 cases of cardiac myxoma in our centre between 1976 and 1997. These included 73 sporadic cases and five cases from a single family of mother, daughter and son. The familial cases had recurrent, biatrial cardiac myxomas. Histopathologic analysis, immunohistochemical study, electron microscopic evaluation and DNA ploidy analysis done in some of these cases revealed evidence in support of the neoplastic nature of this entity. This communication sums up our observations and literature related to the histogenesis of cardiac myxoma.

Congenital gingival granular cell tumor--a case report.

Presented here is a case report of a congenital granular cell tumor commonly known as congenital epulis. It has been found to be ten time more common in females as compared to males. The most common presenting problem is that of difficulties in feeding and respiration. Surgical excision is indicated in case the above problems are present. In the absence of any respiratory or feeding difficulties it is advisable to wait for spontaneous regression.

Root development in relation to impacted mesiodens.

Presented here is a case of 7 years 6 month old child with an apically impacted mesiodens in relation to the developing root of right upper central incisor. The removal of mesiodens was deferred in order to avoid disturbance in the natural development of the upper central incisor. Serial X-rays are presented and case is discussed.

DNA ploidy and proliferative index of cardiac myxoma.

Cardiac myxoma is the commonest tumour of heart. The histogenesis of this lesion is controversial as its origin has often been debated between the thrombogenic and neoplastic theories. We analysed DNA ploidy and proliferation indices of 30 cardiac myxomas which include 25 sporadic and five familial cases by image cytometry and proliferating cell nuclear antigen immunostaining. Of the 25 cases, 18 were aneuploid, three diploid and four tetraploid. Four of the five familial cases including the recurrent lesions were aneuploid. Poor tissue preservation precluded ploidy analysis in one familial case. The proliferation index of the sporadic cases ranged from 0.4 to 36.1 percent. The familial cases showed proliferation index between 10.2 and 22 percent. In addition to cardiac myxoma, proliferation index was assessed in 10 cardiac thrombi where it ranged from three to 58 percent. This study suggests that cardiac myxoma can be best interpreted as a neoplasm with a slow growth potential.

Enzyme-linked immunosorbent assay and immunoblot study in Takayasu's arteritis patients.

Takayasu's arteritis or non-specific aortoarteritis is an inflammatory and stenotic disease of the aorta of questionable aetiology. Immunopathogenic mechanism, the precise nature of which is uncertain, is often suspected to be one of the basic causes of this disease. The present study was designed to estimate the antiaorta antibody titre in Takayasu's arteritis patients and to further locate the antigen in the vessel wall. Thirty clinically and angiographically proven cases of Takayasu's arteritis patients with appropriate controls were studied. Antiaorta antibody titres were estimated using Enzyme-Linked Immunosorbent Assay method. The controls included patients of vascular diseases other than Takayasu's arteritis, autoimmune diseases like rheumatoid arthritis and systemic lupus erythematosis and normal healthy individuals. Absorbance value at 492 nm at a dilution of 1:500 of the patients' sera was expressed as the antiaorta, antibody titre. There was significant difference (p < 0.005) between the mean value of the antibody titre in patients (0.471 +/- 0.073) and patients of other vascular diseases (0.209 +/- .056); autoimmune diseases (0.143 +/- .024); and, controls (0.108 +/- 0.012). Collagenase treatment of the aorta resulted in the fall of the antibody titre of aortitis patients (0.162 +/- 0.036) suggesting that the collagen might be one of the components responsible for autoantigenecity of aorta resulting in aortitis. The aortic extract was further subjected to 10 percent sodium dodecyle sulphate-polyacrylamide gel electrophoresis and immunoblot was done with Takayasu's arteritis patients' sera as well as controls' sera. The sera in 80 percent of Takayasu's arteritis patients immunoprecipitated a protein of molecular weight 45,000 (45 kilodalton) whereas only 15 percent patients of autoimmune disease group showed precipitation band though of lower molecular weight. Normal human sera gave no immunoprecipitation band. The precise nature of the antigen still needs to be identified.

Effect of endothelial cells on the lymphoproliferative response in Takayasu's arteritis.

Takayasu's arteritis, also known as 'non-specific aortoarteritis' is an inflammatory disease of the aorta and its major branches. It also involves the pulmonary artery. The aetiology of the disease is not known so far. Abnormalities of the endothelial cells in terms of their structure and function are seen in the pathology of a number of diseases affecting the blood vessel wall. However, involvement of the endothelial cells in non-specific aortoarteritis is not known. In an effort to identify the role of endothelial cells in the pathogenesis of Takayasu's arteritis, peripheral blood lymphocytes isolated from the blood of patients suffering from Takayasu's arteritis were cultured in the presence of endothelial cells alone and in the presence of mitogens concanavalin-A and phytohaemagglutinin-P. The peripheral blood lymphocytes of patients with Takayasu's arteritis showed a significantly decreased blastogenic response to the mitogen concanavalin-A when cultured in the presence of endothelial cells. Our result thus suggests that endothelial cells may probably induce an inhibitory effect on the lymphocytes in patients with Takayasu's arteritis.

Pathology of cardiac transplantation--the initial experience.

BACKGROUND: Cardiac transplantation has recently been started in India at the All India Institute of Medical Sciences, New Delhi. All transplants are monitored by right ventricular endomyocardial biopsies. This study describes our initial experience with the histopathological assessment of post-transplant endomyocardial biopsies. METHODS: Fifty-four endomyocardial biopsies from 8 transplanted hearts were reviewed. At least 3 haematoxylin-eosin and Masson trichrome stained sections were available on each biopsy. Special stains and immunohistochemistry were done as required. Biopsies were graded for rejection using the grading system of the International Society for Heart and Lung Transplantation. Infection, 'Quilty' effect, biopsy site, ischaemia and reperfusion injury were also assessed. RESULTS: There was no evidence of rejection in 34 biopsies. Eleven biopsies showed mild rejection. Only 9/54 biopsies from 4 of the transplant recipients had clinically significant grades of rejection. Biopsy sites were present in 13/54 biopsies. Ischaemia was present in the first biopsy after transplantation in 4 recipients. There was no evidence of viral or parasitic infection. 'Quilty' lesions were seen in 14/54 biopsies from 5 transplant recipients. Four of these 5 recipients had 'Quilty' lesions on more than 1 biopsy. CONCLUSIONS: The low rejection rate at our centre is consistent with reports from around the world. Endomyocardial biopsy has been a safe, convenient and useful method for diagnosing rejection and monitoring the postoperative course of cardiac transplants at our centre.