von Willebrand's disease with gastrointestinal telangiectasia.

A 30-year old woman presented with recurrent and massive bleeding from gastrointestinal tract. Her coagulation profile revealed von Willebrand's disease and her endoscopic examination showed extensive telangiectasia localized to the gastrointestinal tract. The association of Von Willebrand's Disease and telangiectasia is extremely rare and hence the report.

Comparative study of oral versus injectable vitamin K in neonates.

One hundred term exclusively breast fed babies weighing more than 2.5 kg were evaluated to determine the efficacy of various modes and doses of Vitamin K to prevent hemorrhagic disease of newborn (HDN). The babies were grouped into four categories of 25 each: Group A--1 mg Vitamin K intramuscular (Menadione sodium disulphite) at birth; Group B--0.5 mg Vitamin K intramuscular; Group C--1 mg Vitamin K orally, and group D--no Vitamin K. The prothrombin index was estimated in all babies between 36-72 hours of age. The results revealed a prothrombin index in Groups A, B, C and D as 94.98 +/- 7.64%, 95.08 +/- 9.91%, 92.51 +/- 10.10% and 80.39 +/- 15.90%, respectively. The differences between Groups A, B and C were insignificant. However, Group D, prothrombin index was significantly reduced as compared with the other three groups. It is, therefore, concluded that oral Vitamin K is as effective as injectable Vitamin K and its usage is recommended in our country to reduce complications and costs of parenteral therapy.

Frequency of hepatitis B, C and D and human immunodeficiency virus infections in multi-transfused thalassemics.

Of forty multi-transfused thalassemia patients (26 males, 14 females; mean age 8.1 +/- 5.3 years, range 1-35) with no clinical or biochemical evidence of liver disease, HBsAg, anti-hepatitis C virus and anti-human immunodeficiency virus antibodies were present in 18 (45%), 7 (17.5%) and 1 (2.5%) cases respectively. Three of the 18 (16.7%) HBsAg positive patients were anti-delta antibody positive. Our results indicate that more than 50% of multi-transfused thalassemia patients show serological evidence of one or more of hepatitis B, C and D and human immunodeficiency virus infection.

PIP: Transfusion of whole blood or blood components is the mainstay of treatment in patients with beta-thalassemia and hemophilia. Owing to the scarcity of reports regarding the frequency of transfusion-transmitted hepatitis virus infections in thalassemia patients, the frequency of such infections was studied in India in 40 multi-transfused thalassemia patients (26 males, 14 females; mean age 8.1 +or- 5.3 years, range 1-35) with no clinical or biochemical evidence of liver disease. The enzyme-linked immunosorbent assay (ELISA) technique (Abbott) was used for all tests. The patients had received an average of 80 units (range 10-250) of blood. A majority of these units had been screened for hepatitis B surface antigen (HBsAg) using RPHA. HBsAg antibodies were present in 18 (45%), antihepatitis C virus (HCV) in 7 (17.5%), and antihuman immunodeficiency virus in 1 (2.5%) case, respectively. Of 18 HBsAg positive patients, antidelta and anti-HCV antibodies were present in 3 and 4 patients, respectively; 1 patient had both the antibodies. 4 of 40 (10%) patients had evidence of both hepatitis B virus (HBV) and HCV infection. In a US study, the frequencies of HBsAg and anti-HBs positively among thalassemics were 4.5% and 43.5%, respectively. In contrast, 90% of hemophiliacs show serological evidence of HBV infection. Routine screening of blood donors by CEP or RPHA technique was started in the hospital blood bank 7 years ago. The sensitivity of these techniques is much lower than that of RIA and ELISA and a majority of the patients has received initial blood transfusions before HBsAg screening was started. The study indicated that more than 50% of multi-transfused thalassemia patients showed serological evidence of one or more HBV, HCV, HDV, and HIV infection. Thus, screening of blood units for HBV, HCV, and HIV infections to be used for thalassemic patients and vaccination of thalassemic patients against hepatitis B is imperative.

Experience with bone marrow trephine biopsy.

One hundred and ten bone marrow trephine biopsies were studied from January 1987 to July 1989, using Zenker's acetic acid as fixative and routine paraffin embedding. Trephine biopsies were useful in differential diagnosis of cytopenias, especially when bone marrow aspiration was hypocellular or a dry tap, and in staging of lymphomas and multiple myeloma.