Atypical hemolytic uremic syndrome with membranoproliferative glomerulonephritis.

Atypical hemolytic uremic syndrome (aHUS) associated with membranoproliferative glomerulonephritis (MPGN) is an uncommon clinical presentation, especially in children. We report a 8-year-old-boy who presented like aHUS but the kidney biopsy showed MPGN type 1.

F-18 flourodeoxyglucose positron emission tomography/computed tomography findings in a case of hepatosplenic T-cell lymphoma.

T-cell lymphoma (TCL) is a biologically diverse and uncommon group of lymphoid malignant diseases. Compared with its B-cell counterparts, TCL is notably more difficult to diagnose and manage owing to its rarity and biologic heterogeneity. Hepatosplenic TCL is an extremely rare subtype of TCL. A 37-year-old Indian male presented to his physician with swelling and pain in left hypochondrium. Clinical examination revealed pallor, icterus and massive splenomegaly. His blood examination revealed pancytopenia. His bone marrow biopsy was suggestive of lymphoma. Whole body F-18 flourodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scan revealed diffuse increased metabolic activity in massively enlarged spleen, liver and bone marrow. There was no evidence of metabolically active lymphadenopathy anywhere in the body. Guided by the FDG PET/CT findings, a liver biopsy was advised. Liver histopathology revealed the presence of hepatosplenic TCL. A final diagnosis of hepatosplenic TCL with liver, spleen and bone marrow involvement was made. Even though rare, hepatosplenic TCL should be considered as a possible diagnosis in patients presenting with similar clinical picture and FDG PET/CT scan findings.

Ultrastructure of the H. pylori microbe in individuals having macrocytosis and B12 deficiency.

The incidence of Helicobacter pylori gastritis is high in India and the number of individuals with vitamin B12 deficiency is also large. An association has been found between these two factors. It is necessary to determine whether H. pylori infection may be a factor in the causation of B12 deficiency and whether it is associated with any morphological changes on ultrastructural examination. A cohort-based study has been performed, which includes 505 young asymptomatic males. These cases have been investigated for presence of H. pylori and macrocytosis. The study confirms an association between H. pylori infection and B12 deficiency. It is recommended that H. pylori infection be looked for in subjects having macrocytosis of unknown etiology.

Microvillous inclusion disease--an ultrastructural diagnosis: with a review of the literature.

Microvillous inclusion disease is a rare disorder of infancy associated with protracted diarrhea. This malady reveals distinct ultrastructural changes. The surface enterocytes of the duodenum show vesicles lined with microvilli and the surface microvilli are poorly formed. The authors present one case of microvillous inclusion disease with a review of the literature.

Extraskeletal chondrosarcoma of labium majus.

Extraskeletal myxoid chondrosarcoma (ESMC) is a rare tumor seen more often in men. It is seen to arise from soft tissue of lower extremity or buttocks. We report a case of soft tissue swelling of left labium majus in a 66-year-old female. Patient underwent wide excision with uneventful postoperative course. Histopathology of specimen confirmed it to be ESMC. Patient refused adjuvant therapy. Followup of 1 year has shown her to be disease- and symptom- free. Only two cases arising from vulva have been reported in literature . This is the third case and first from Indian subcontinent. A brief review of clinical features, diagnosis, treatment, and outcome of patients with extraskeletal chondrosarcoma is presented.

Banded collagen in the kidney with special reference to collagenofibrotic glomerulopathy.

BACKGROUND: The normal glomerular basement membrane, composed of type IV collagen, plays an important function in the process of filtration. Rarely, type III, type I, or type V collagen is seen in the glomerulus, resulting in three different types of non-immune mediated glomerulopathies recognized thus far. These are characterized by deposition of banded collagen fibers in the glomerulus. METHODS: The authors reviewed 4934 kidney biopsies submitted over the past 5 years. Five of these revealed the presence of banded collagen in the glomeruli. CONCLUSION: Combined clinical and ultrastructural examination has led to a definitive diagnosis. These diseases exhibit indolent progression and as yet do not have specific treatment.

Desmoplastic non-infantile ganglioglioma: a low-grade tumor, report of two patients.

Desmoplastic infantile gangliogliomas (DIGG) are low-grade (WHO Grade I) tumors of early childhood rarely occurring in older children and young adults. The mainstay of treatment is surgical. We report two older children with DIGG, both showing atypical radiology. One case was treated with radiation. The recurrence treated with only surgical excision showed excellent response to treatment.

IgM nephropathy in adults: incidence and correlation with electron microscopic features.

IgM nephropathy is characterised on light microscopy (LM) by variable features of normal glomeruli to mesangial hypercellularity; and immunofluorescence (IF) deposits of LgM. Our aim was to study the incidence of IgM nephropathy in adults with primary glomerular disease, with correlation to electron microscopy (EM) features. All adults presenting with proteinuria glomerular hematuria underwent renal biopsy. We excluded patients with systemic diseases and post-infectious glomerulonephritis. All the specimens were evaluated by LM, IF and EM. Our series had 146 cases. Of the 42 cases diagnosed on LM as minimal change disease, mesangial deposition of IgM was present in 11 cases. In addition there were seven cases of mesangioproliferative glomerulonephritis with mesangial IgM deposition. Thus, there were a total of 18 cases of IgM nephropathy (12.3%). Only six of these 18 cases showed typical electron dense deposits in the mesangium on EM. We feel that IgM nephropathy is probably a separate pathological entity, comprising 12.3% of all adults with primary chronic glomerulopathy. Electron dense deposits are seen in only about a third of these cases.

Cancer and AIDS.

The occurrence of cancer as an AIDS defined disease is a subject that has received scant attention in the Indian medical establishments and lay public. It is important to know that the concept of acquired immunodeficiency syndrome was ushered in with reports of rare forms of cancers in HIV infected subjects. In the developed countries 34% of AIDS patients suffer from cancer, a cancer that is very aggressive, resistant to treatment and often fatal. On the other hand, incidence of cancer in patients infected with HIV virus is only 3%-4% in the Indian population. Nearly all patients with AIDS in India are victims of tuberculosis and opportunistic infections. Among the various cancers reported in the Indian population Kaposi's sarcoma is very rare indeed. AIDS associated malignant tumours tend to be more anaplastic and disseminate fairly early. The object of this review is to increase awareness of the various aspects of cancer and AIDS. There is an urgent need to improve gathering of epidemiological data and to direct research effort to explain a very strikingly low incidence of cancers in Indian subjects as compared to that in the West (prevalence of 4% versus 34% among HIV infected patients).

Hemangiopericytoma in the trigone of the lateral ventricle--case report.

A 40-year-old male presented with hemangiopericytoma in the lateral ventricle manifesting as headaches persisting for 6 months associated with vomiting and visual obscurations for one month. Computed tomography and magnetic resonance imaging of the brain showed a large tumor in the trigone of the right lateral ventricle. The highly vascular tumor was completely excised. The histological diagnosis was hemangiopericytoma. Hemangiopericytoma is rarely located in the lateral ventricle and is difficult to differentiate from meningioma by neuroimaging methods.

Late splenic metastasis after curative resection for oesophageal carcinoma.

The spleen is an unusual site of distant metastasis from solid tumours. While contiguous involvement of the spleen may occur in tumours arising from the stomach, pancreas or colon; the spleen as the seat of distant metastasis is a rare occurrence. We report herewith one such instance of metastatic involvement of the spleen in an operated case of carcinoma oesophagus.