Lipoid proteinosis in two Indian brothers - a case report from South Bengal

Lipoid proteinosis is a rare autosomal recessive disorder with variable phenotype, caused by defect in extracellular matrix protein-1 and is characterized by deposition of periodic acid-Schiff-positive, diastase resistant material in skin, mucous membrane and internal organs. There are only few reports regarding lipoid proteinosis in literature and in this part of the world. Here, we report a case of lipoid proteinosis in a 29-year-old male with positive family history and widespread distribution involving skin and internal organs. Histopathological finding was consistent with clinical diagnosis of lipoid proteinosis

Neonatal Screening for genetic blood diseases

Previous neonatal screening in 1986 showed that the incidence of sickle cell disease [SCD] is 2.1% and [SCT] is l1%. Since 1984 the Ministry of Health [MOH] instituted a prevention campaign. The incidence has been falling gradually since then. To update the national data on the incidence of SCD among the newborns and to compare it with the previously available data. All Bahraini newborns delivered at the [MOH] maternity hospitals for a period of three months from February to April 2002 were targeted. Cord blood samples were analyzed by HPLC. Two thousand newborns constituted the study population five were excluded. Eighteen were found to be affected with SCD with an incidence of 0.9%. SCT was found in 325 [16.3%]. G6PD deficiency was found in 18% of males, and 10% of females. Parental age distribution and consanguinity were documented. Bahrain has for the first time recorded less than 1% babies with SCD

Spectrum of malignant lymphomas in Bahrain. Leitmotif of a regional pattern

To determine the clinicopathologic features of malignant lymphomas in Bahraini patients. A retrospective hospital-based study was conducted. All new cases of malignant lymphoma diagnosed during the period January 1996 to December 2001 at the Salmaniya Medical Complex in Bahrain were included in the study. Seventy-two cases met the inclusion criteria. This included 24 [33.3%] cases of Hodgkin's disease [HD] and 48 [66.7%] cases of Non-Hodgkin lymphomas [NHL]. A young age at presentation [median 20 years] mixed cellularity histology, lack of extra nodal involvement and rare marrow involvement characterized HD. The majority of NHL showed diffuse high or intermediate grade lesions. A high number of primary extra nodal neoplasms [41.7% of NHL] and frequent involvement of the gastrointestinal tract with Helicobacter pylori-associated gastric lymphomas were notable features among NHL cases. Immunohistochemical staining in 30 cases showed 26 cases [86.7%] of B cell and 4 cases of T cell origin. The study highlights common features that distinguish malignant lymphoma reported from countries of the Arabian Gulf region. This pattern distinguishes them from the disease encountered in the Western world

Agnogenic myeloid metaplasia with cutaneous granulocytic sarcomas

A case of agnogenic myeloid metaplasia that transformed to acute myeloblastic leukaemia terminally, is described. The acute phase was heralded by the development of cutaneous tumours, which are rarely seen in this condition