A 6 day old newborn presented with fever, lethargy, poor suck and mild hepatosplenomegaly, features suggestive of sepsis. Septic work up was negative. Peripheral smear revealed hyperleukocytosis, thrombocytopenia and presence of atypical cells. Bone marrow examination revealed increased myeloblasts. Other causes of leukemoid reaction were ruled out and a final diagnosis of congenital leukaemia was made.

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Background: In myeloproliferative disorders, occurrence of Janus kinase 2 [JAK2] mutation is a significant factor in pathogenesis in polycythemia rubra vera (PRV) and Essential thrombocythemia (ET). We studied the frequency of JAK2 mutation in patients with PRV and ET and to compare clinical and laboratory features of patients positive and negative for mutation. Patients and Methods: Clinical and laboratory features of PRV and ET patients (WHO criteria) from 1997 to 2004 were included. After morphological diagnosis, presence or absence of JAK 2 mutation was done during follow up and follow up details were recorded. Results: A cohort of 39 patients (ET-17, PRV -14, ET- PRV-8) were identified .The mean age of entire cohort was 55.5+14.5 years. Comparison of clinical and laboratory features showed JAK2 positive patients were older by a decade, had higher frequency of splenomegaly and higher values for hemoglobin (16.5+2.6 gm/dl) and neutrophil counts (14.5+4.8 thousand/μl).During the course of follow up (6 to 106 months; mean 25.5+28.6 months) frequency of thrombotic events in both groups (p value >0.05) was same, though time for occurrence for thrombotic event was shorter in JAK 2 positive patients, which is noteworthy. Conclusions: The identification of JAK 2 mutation probably defines a sub entity in ET with aggressive behavior as evidenced by splenomegaly, higher total counts and transformation to PRV (n=6/8). The onset of JAK2 V617F mutation probably heralds progression to PRV.

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Extrophy of the bladder is a rare congenital anomaly with an incidence of about 1/50000 newborn. Malignant potential of ectopic mucosa is high. Sixty percent of cancers in ectopic bladder occur during fourth and fifth decade of life, majority being adenocarcinoma (95%). Here we report a case of squamous cell carcinoma of ectopic urinary bladder in a 74-year-old male patient. In view of locally advanced disease, he received neoadjuvant chemotherapy with poor response. He underwent total cystectomy with ileal conduit diversion and continues to be alive and disease free at 36 months interval. The case is being reported on account of its rarity. Pertinent literature is being reviewed.

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Wilms' tumour is most common malignant renal tumour of childhood. Rarely, it may be seen in the adults. We report a 35 year old male presenting with flank pain. Abdominal CT scan revealed a right renal mass and a clinical diagnosis of renal cell carcinoma was made. Nephrectomy was performed, and a final diagnosis of adult Wilms' tumour was made. This case in being reported on account ot its rarity. Pertinent literature is being reviewed.

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Background: Radiation with concurrent chemotherapy (weekly cisplatin) is currently standard of care for locally advanced cervical cancer. Gemcitabine, a pyrimidine analogue is a potentially radio-sensitizing drug. We compared cisplatin and gemcitabine in the treatment of locally advanced cervical cancer. Methods: 90 patients with locally advanced squamous cell cancer of the cervix (stage IIB-IVA) were randomized to receive either cisplatin 40mg/m² weekly or gemcitabine 150 mg/m² weekly (45 patients in each arm) along with external beam radiation (50Gy in 25# over 5 weeks). This was followed by three insertions of high dose radiation (HDR) intracavitary brachytherapy one week apart. Results: At a median follow up of 13months, 25 (55.56%) patients were in complete response (CR) in the cisplatin arm compared to 22 patients (48.89%) [p=0.67] in gemcitabine arm. 10 patients (22.22%) in cisplatin arm had either died or lost to follow up compared to 11 patients in gemcitabine (24.44%) arm. Nausea/vomiting was higher in cisplatin arm. Diarrhea, skin reaction and hematological toxicity were more in gemcitabine arm. Conclusion: Cisplatin seems to be a better option than gemcitabine when used concurrently with radiation for locally advanced cervical cancer both in terms of response and toxicity.

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p53, besides being a tumor suppressor protein, is a transcription factor with a powerful activation domain. Mutation(s) in this gene is a frequent event in human carcinogenesis. Several of these mutations lead to loss of its function as transcription factor. Proteases are thought to play an important role in tumor invasion and metastasis by degradation of extra cellular matrices and basement membranes. The function of protein-coding genes is carried out by the protein product, it is important to study p53-dependent expression at the protein level. There are many factors which regulate the expression of proteases in the cell, however here in this review we have focused on the p53 gene and its regulatory effect on the expression of proteases.

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Unusual manifestations often lead to misdiagnosis of patients with acute lymphoblastic leukemia (ALL). We describe two children with ALL having unusual associations. The first case presented with pseudohypopyon after 18 months into her maintenance therapy whereas the other case masqueraded as juvenile rheumatoid arthritis at presentation.

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Merkel cell carcinoma is a rare neuroendocrinal tumour of the skin. It is noted for its typical aggressive nature unlike other skin tumours. Because of the rarity, the treatment is not standardised. Radiotherapy in the postoperative setting is effective in improving the loco regional control. A case of merkel cell carcinoma treated with postoperative radiotherapy is reported. Pertinent literature is reviewed.

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