Congenital Thrombotic Thrombocytopenic Purpura: An Unusual Case of Microangiopathic Haemolytic Anaemia in a Newborn.

We present an extremely rare case of hyperbilirubinemia with rapid progression leading to bilirubin encephalopathy in term neonate. Despite early recognition and intervention, death occurred as a total serum bilirubin reached 25 mg/dl. It was a case of Coomb’s negative microangiopathic haemolytic anaemia in a newborn period which is autosomal recessive inheritance i.e. Upshaw-Schulman Syndrome. (Congenital thrombotic thrombocytopenic purpura) characterised by numerous schistocytes on peripheral blood smear, thrombocytopenia , increased reticulocyte count, increased bilirubin and LDH level. This rare disease is often misdiagnosed especially in newborn baby. So we present this case not only for its variety but also for to create more awareness among pathologist and paediatrician as treatment protocol entirely differ.

Pseudomyxoma peritonei presenting as sub-acute large bowel obstruction.

A case of pseudomyxoma peritonei, a rare mucin-secreting tumour in the peritoneum, in a 30-year-old male and presenting as sub-acute large bowel obstruction, is reported here with review of the literature.

Meckel Gruber Syndrome--a case report.

Meckel Gruber Syndrome is a rare syndrome inherited as Mendelian autosomal recessive condition. The affected infant usually has a large occipital encephalocoele associated with renal cysts and sometimes polydactyly. The prognosis is poor. The affected child is still born or dies early in infancy. If diagnosis is done by prenatal ultrasound examination termination of pregnancy can be done.

Nodular and diffuse fibrous proliferation of tunica vaginalis--a case report.

A case of nodular and diffuse fibrous proliferation (NDFP) of the tunica vaginalis testis occurring in a 40 year male is described. Immunohistochemistry confirmed fibroblastic origin. Histogenesis & differential diagnosis of this lesion is considered. Simple excision of this lesion is curative. The lesion is very rare but it is important that both surgeons & pathologists become aware of this entity to avoid radical orchiectomy in young patients.