utility of red cell discriminant function [DF] in screening for trait

This study was conducted to determine the frequency of minor in adult males at a tertiary referral center by using discriminant function [DF] and to compare mean MCV [mean corpuscular volume] and mean RBC [red blood cell] count in minor and non thalassaemia minor group. This was an observational cross-sectional study. Place and Duration of Study: This study was conducted at the Clinical Laboratory, Agha Khan University Hospital from 1[st] August 2003 to 31st October 2003. Blood samples were taken from normal individuals in EDTA and were run on Coulter STKS. The indices that were taken into consideration were haemoglobin, RBC count and MCV, DF was calculated by formula as proposed by England and Fraser. Discriminant Function = MCV- [5xHaemoglobin]-RBC-3.4. If the value was less than zero; it was interpreted as suspected thalassaemia minor. Mean MCV and mean RBC count in thalassaemia minor and iron deficiency anaemia were calculated and compared. A total of 1270 individuals were included in the study. In 70 individuals the red cell indices were found to be low their DF was calculated and thalassaemia minor was suspected in 49 individuals and this was confirmed with haemoglobin electrophoresis in 25 patients. Mean MCV was almost the same in both thalassaemia minor and iron deficiency anaemia. However, mean RBC count was relatively higher in case of thalassaemia minor. Discriminant function can be a good screening tool for evaluating patients with low red cell indices. However further and confirmation with haemoglobin electrophoresis is required for labeling a patient as having thalassaemia minor

Hairy Cell Leukemia: clinical presentation and long term follow up after treatment with 2-Chlorodeoxyadenosine [2-CdA]

The aim of the study was to the clinical features and long term follow up after treatment with Cladarbine in a tertiary care hospital. Seven patients with hairy cell leukemia were diagnosed between January 1990 till December 2003. Diagnosis in all the patients was established by bone marrow aspirates and trephine biopsy along with TRAP. In two patients the diagnosis was supplemented by flowcytometry and in another two patients by splenectomy. Six patients were male while one was female. Mean age was 47.7 years [range 36-64]. Most common presenting features were pallor and weakness [n=5]. All patients had splenomegaly. Blood count at presentation revealed that one patient had bicytopenia, two had isolated thrombocytopenia, and three had pancytopenia. Treatment responses were evaluable in seven patients. Complete response was seen in six patients [85.7%]. One patient died after two months due to sepsis while 3 [50%] patients relapsed. Those who relapsed received another course of CDA and have maintained remission with a median duration of response of 48 months [20-48]. From this small series we can conclude that CDA is an effective treatment for HCL and even it works very well in relapsed cases.

Anaphylactic reaction after Intramuscular injection of Cyanocobalamin [Vitamin B12]: a case report

report a case of anaphylactic reaction to intramuscular injection of cyanocobalamin. This 52-year-old lady was diagnosed as a case of megaloblastic anemia secondary to dietary vitamin B12 deficiency. She had severe anaphylactic reaction after the parenteral administration of cyanocobalamine. Later she received oral vitamin B12 with no adverse effects. The purpose of this case report is to draw attention to the hypersensitive reaction to injectable vitamin B12, which is rarely seen. This could be due to sensitization to the vitamin B12 molecule itself or an IgE mediated reaction. We concluded that anaphylactic reaction to vitamin B12 is a rare but serious side effect and it should be kept in mind while the drug is being administered to the patient, especially via the parenteral route

Familial homocystinuria

Two cases of siblings diagnosed as cases of familial homocystinuria are reported. Both the cases have classical presentation of familial homocystinuria including history of dislocation of lens of the right eye. Brother had history of psychomotor retardation while sister had a significant history of deep vein thrombosis. Levels of plasma homocysteine were elevated and urinary homocysteine was positive in both the cases

Frequency of irregular red cell alloantibodies in patients with thalassemia major: a bicenter study

To provide frequency and distribution pattern of various types of irregular red cell alloantibodies in patients with thalassemia major. This is a descriptive study conducted at two centers from January to December 2001. Purposive sampling was done and all patients diagnosed to have thalassemia major were included in the study. Antibody identification was carried out on serum employing commercial two-cell panel using standardized blood bank methods. If patients were found to have an irregular red cell alloantibody then the antibody identification was performed using 16 panel cells. A total of ninety-seven patients were included in the study. Fifty-three patients were males and 44 females. Mean age was 10.6 years. Irregular red cell alloantibodies were found in 9 [9.2%]. Mean age of patients who developed red cell alloantibody was 11.9 years. Three [33.3%] patients developed anti-K while two [22.2%] had non-specific antibody. One patient each developed anti-D [11.1%] and anti-E [11.1%]. Two had anti-D [11.1%] and anti-C while the other one [11.1%] developed anti-E and anti-K. We concluded that there is relatively high rate of alloimmunization in our set of patients when compared to data from our region. We also suggest that red cell alloimmunization should not be overlooked in patients receiving regular blood transfusions