Shameem Shariff

Squamous cell carcinoma arising from congenital lymphedema

Congenital lymphedema is a rare type of primary lymphedema occurring at birth or developing shortly later. Primary lymphedema can be classified according to whether it is familial or sporadic. The primary congenital familial lymphedema is also known as Milroy’s disease. Majority of primary cases are sporadic type. Chronic lymphedema can be secondary to infections, surgery with lymph node excision, trauma, lymphadenectomy, radiotherapy, filarial infection, and so on. It is recognized that a variety of malignant tumors can arise in chronic congenital or acquired lymphedema; the most documented associations are lymphangiosarcoma, basal cell carcinoma, lymphoma, malignant melanoma, and Kaposi’s sarcoma. A total of 13 cases of squamous cell carcinoma arising from chronic (primary or acquired) lymphedema have been reported, and only 3 cases of congenital lymphedema presented with squamous cell carcinoma as reported. A 32-year-old young male presented with chronic unilateral left lower limb lymphedema of 28 years duration. In addition, he had a 3-month history of a fungating cutaneous lesion on the lateral side measuring 2 cm × 1 cm in size. Fine-needle aspiration cytology was performed on the later mass, and a diagnosis of angiosarcoma was made. At histopathology, the appearances did not confirm angiosarcoma. However, an impression of carcinoma was made as squamous cells were observed in sheets. Immunohistochemistry was performed using markers for CD31, factor VIII (FVIII), and MiB. The epithelial marker cytokeratin was positive for squamoid cells and MiB index of 75%. The vascular markers FVIII and CD31 were negative, thus ruling out angiosarcoma. The final diagnosis was given as infiltrating squamous cell carcinoma in chronic lymphedema.

A Clinico - Pathological Study of Hemophilia in Rural Set up of Karnataka

Haemophilia is the most common inherited coagulation disorders, with X linked recessive inheritance, affecting the males w hile females are the carriers of the disease. Haemophilia A and Haemophilia B are the commonest form of Haemophilia encountered and they result from defect in Factor VIII and Factor IX gene respectively. A clinico hematological study with suspected coagula tion disorder was conducted over a period of two years, from June 2008 to July 2010. Of the 76 patients visited OPD, based on clinical presentation and family history, 50 cases were categorized as Hemophilia A or B after laboratory investigations. Majorit y (41) of the cases were categorized as Hemophilia A & only 7 cases were Hemophilia B. The mean age group of the patients was 2.87 years with an age of onset ranged between 3