A rare case of haemolytic disease of newborn with Bombay phenotype mother.

We are reporting a rare case of severe hemolytic disease of newborn (HDN) with Bombay phenotype mother. A retrospective study of a case with severe haemolytic disease of newborn with Bombay phenotype mother was done. Blood grouping, antibody screening, and lectin study was done on the blood sample of the baby and mother to confirm the diagnosis. Hematological and biochemical parameters were obtained from the hospital laboratory information system for the analysis. Blood group of the baby was A positive, direct antiglobulin test was negative. Blood group of the mother was confirmed to be Bombay phenotype, Hematological parameters showed all the signs of ongoing hemolysis and the bilirubin level was in the zone of exchange transfusion. Due to the unavailability of this rare phenotype blood unit, baby was managed conservatively. Anticipating the fetal anemia and HDN with mothers having Bombay phenotype and prior notification to the transfusion services will be of great help in optimizing the neonatal care and outcome.

Linear accelerator: a reproducible, efficacious and cost effective alternative for blood irradiation.

BACKGROUND: The dedicated devices for blood irradiation are available only at a few centers in developing countries thus the irradiation remains a service with limited availability due to prohibitive cost. OBJECTIVE: To implement a blood irradiation program at our center using linear accelerator. MATERIALS AND METHODS: The study is performed detailing the specific operational and quality assurance measures employed in providing a blood component-irradiation service at tertiary care hospital. X-rays generated from linear accelerator were used to irradiate the blood components. To facilitate and standardize the blood component irradiation, a blood irradiator box was designed and fabricated in acrylic. Using Elekta Precise Linear Accelerator, a dose of 25 Gy was delivered at the centre of the irradiation box. Standardization was done using five units of blood obtained from healthy voluntary blood donors. Each unit was divided to two parts. One aliquot was subjected to irradiation. Biochemical and hematological parameters were analyzed on various days of storage. Cost incurred was analyzed. RESULTS: Progressive increase in plasma hemoglobin, potassium and lactate dehydrogenase was noted in the irradiated units but all the parameters were within the acceptable range indicating the suitability of the product for transfusion. The irradiation process was completed in less than 30 min. Validation of the radiation dose done using TLD showed less than ± 3% variation. CONCLUSION: This study shows that that the blood component irradiation is within the scope of most of the hospitals in developing countries even in the absence of dedicated blood irradiators at affordable cost.

Evaluation of placenta in foetal demise and foetal growth restriction.

AIM: The study objective was to evaluate the pathological changes of the placenta in foetal death and foetal growth restriction and to find correlation of the findings with clinical causes. SETTING AND DESIGN: Prospective study at a tertiary care hospital. MATERIAL AND METHODS: Gross and histopathological examinations of the placentae were carried out in pregnancies with foetal demise (IUD) and Foetal Growth Restriction (FGR). STATISTICAL ANALYSIS: SPSS, version 11.5. RESULTS: Placentae of twenty seven women with foetal demise and of equal number of women with foetal growth restriction were studied. Placental weight was less than 10(th) percentile in 61.5% women in IUD group and in 93% women in the FGR group. Gross examination of placentae showed abnormalities in 12 (44%) women of IUD group and in 16 (59%) women of FGR group. Histopathological abnormalities were observed in 74.1% women of the IUD group and in 66.7% women of FGR group. Placental histopathology correlated with clinical risk factors in 60% women of IUD group and in 40% women of FGR group. Among the women with no clinically explainable cause for IUD and FGR, 86% and 57% had placental histopathological abnormalities respectively. CONCLUSION: The histopathological abnormalities of the placenta can be used to document the clinical causes of foetal demise and growth restriction; it may explain the causes in cases of clinically unexplained foetal demise and foetal growth restriction.

Desmoplastic small round cell tumour in a young woman with widespread metastasis and peritoneal caking.

Desmoplastic Small Round Cell Tumour (DSRCT) is a rare, highly aggressive, mesenchymal tumour that arises from the peritoneal cavity. It is commonly seen in adolescent and young adult males and its occurrence in females is uncommon. We are reporting here a rare case of DSRCT in a young woman, which clinically masqueraded as an ovarian malignancy, with metastasis to liver, lung, spleen and peritoneum. The cytologic findings, Histomorphological and immunohistochemical features have been discussed, with a brief review of literature.

Multiple neural fibrolipomas with macrodactyly.

Neural fibrolipoma is an uncommon tumor-like lesion that involves the upper extremity and usually arises in the median nerve. It is associated with macrodactyly in one-third of the cases. A 3-year-old girl presented with increasing size of fingers of both the hands since birth. Clinical examination revealed macrodactyly of two fingers of the right hand and three fingers of the left. Surgical reduction was performed and microscopy of the biopsy specimen established the diagnosis of neural fibrolipoma. Knowledge of the clinicopathological features is necessary for accurate diagnosis and treatment of this rare entity.

PCR for M. tuberculosis in tissue samples.

BACKGROUND: The presence of granuloma, visualized in histopathology for diagnosing tuberculosis in tissue samples, is not a specific finding. Moreover, histopathological examination of tissue sections needs one to two weeks for final reporting. A rapid and sensitive method is therefore needed for detection of Mycobacterium tuberculosis in these paucibacillary tissue samples. METHODOLOGY: A PCR-assay specific for IS6110 was evaluated for 104 different tissue samples in comparison to histopathology that was considered gold standard. RESULTS: PCR showed 74.1% sensitivity and 96.1% specificity. False positive and false negative results were observed in three (2.88%) and seven (6.73%) samples, respectively. Positive agreement between histopathology and PCR was observed as 0.737, indicating substantial good agreement between two tests. CONCLUSIONS: PCR can be used for early diagnosis of tuberculosis in tissue samples that can help to initiate timely anti-tubercular treatment and prevent progression to irreversible changes.

Retroperitoneal ganglioneuroma with lymphnode metastasis: a case report.

Ganglioneuroma is a benign, most differentiated tumor of neural crest origin. These tumors though benign, can very rarely metastasize to regional lymphnodes or to distant sites. We report a case of a 52 year old lady who presented to the surgical OPD with dyspeptic symptoms of 2 months duration. Ultrasonography and CT scan of the abdomen showed a homogenous mass in the region of the right adrenal gland, extending between the inferior vena cava and the aorta. A clinical and radiological diagnosis of an adrenal tumor was made. Microscopically, the tumor histology was that of a ganglioneuroma (schwannian stroma-dominant) mature type. The lymph node attached to the tumor showed metastasis, the metastatic foci having the same morphology as the primary tumor. The right adrenal gland was free of tumor. The case is illustrative due to its clinical presentation mimicking an adrenal incidentaloma, and a rare phenomenon of a benign tumor exhibiting completely differentiated lymph node metastasis.