Waldenström macroglobulinemia presenting with neurological symptoms after chemotherapy

No abstract available.

Extensive marrow infiltration in epidural Ewing sarcoma

No abstract available.

Rhesus and Kell phenotyping of voluntary blood donors: foundation of a donor data bank

Objective: To assess the Rhesus [Rh] and Kell [K] phenotype of voluntary blood donors and lay foundation of a data bank of voluntary blood donors

Study Design: Cross-sectional study

Place and Duration of Study: Blood Bank, The Aga Khan University Hospital, Karachi, in the year 2014

Methodology: Voluntary blood donors were inducted after taking written informed consent. Three -5cc of EDTA anticoagulated blood sample was taken to phenotype red cells for C, c, E, e, and Kell antigens using antisera. [DiaMed- Switzerland]

Results: Hundred blood donors were included in the study. ABO blood groups of the donors were: O [37%], B [31%], A [21%] and AB [11%]. Ninety-seven percent were Rh D positive while 3% were Rh D negative; 'e' antigen had the highest frequency [99%], while 'E' antigen was the least frequent [19%]. The most common probable Rh phenotype was R1R1 [[DCe/DCe] in 44 [44%]. In the Kell system, all the donors [100%] had phenotype of K-k+

Conclusion: The most common blood group was O +ve. The pattern of Rhesus antigen expression and phenotype found in this study was concordant to that reported previously from Asia. However, there was a much lower frequency of K antigen

Therapeutic outcomes of older patients with acute myeloid leukemia

To evaluate the therapeutic outcomes of acute myeloid leukemia [AML] in elderly patients. This study was conducted at the Aga Khan University Hospital, Karachi, Pakistan over 11 years from January 1997 to August 2008. This was a descriptive case series study. We investigated the impact of disease biology and various treatment protocols on the outcome in this population. A total of 55 evaluable patients [>60 years of age] were diagnosed with AML including 34 [61.8%] males and 21 [38.2%] females. The median age was 67 years [range 60-86 years] at the time of presentation. The AML was preceded by myelodysplastic syndrome in 15 [27.2%] patients. High-risk cytogenetics were observed in 3 [5.4%] patients. Forty patients received palliative treatment while only 15 received chemotherapy. Of the last group with primary AML [n=10], there were 2 remitters, one showed resistant disease while 8 had induction death. The overall mean survival was 75.1 days [95% confidence interval: 46.7-103.5 days] in all patients. There was no survival advantage in patients treated with chemotherapy versus those conservatively treated. We found high mortality among aged patients with AML in our setting. Patients receiving chemotherapy were extremely intolerant to toxic drugs and succumbed earlier than patients receiving palliative care only

Is manual reticulocyte count a reliable option for under resourced countries?

To establish the credibility of manual reticulocyte counts without compromising the quality of care, and to evaluate the degree of acceptability of manual reticulocyte counts in terms of accuracy and cost effectiveness in comparison with two automated haematology analyzers. Visual reticulocyte enumeration was evaluated for comparability, within-batch precision and costing with respect to Coulter STKS and Gen S haematology analyzers. The results of reticulocyte estimation for 80 samples as obtained by 3 modes were correlated using Pearson's correlation coefficient [r] which were computed as 0.884, 0.875, and 0.793 for manual-Gen.S, Gen.S-STKS and manual-STKS respectively thus showing positive association of these results. STKS had the CV of 10.4% and was more precise compared to Coulter Gen.S [CV=11.6%] while manual counts showed the least precision with a CV of 19.8%. The cost per test was calculated to be $ 0.11 for manual technique in contrast to $0.45 for Gen S and $1.09 for STKS. Visual counting of reticulocytes can be used as a reliable tool for estimating reticulocytes in resource strained countries as it is not only cost effective but can also efficiently discriminate between high and low reticulocyte ranges which are required for sound clinical judgment

RhnuII: a rare blood group phenotype

Rh[nuII] phenotype is a rare blood group characterized by the lack of expression of all Rh antigens [D, C, c, E and e] on the red cells. The phenotype is further classified into the regulator and amorph type based on underlying genetic defect. The clinical significance of its recognition is that such patients suffer from Rh[null] syndrome associated with osmotically fragile red cells called stomatocytes with subsequent chronic haemolytic anaemia of varying degree. Another importance is that such subjects readily form alloantibodies on exposure to Rh antigens. We report herein rare Rh[null] phenotype in a young pregnant female which was detected as a part of routine antenatal work-up for red cell antibody screening and identification

Diagnostic tool for glanzmann's thrombasthenia clinicopthologic spectrum

To platelet aggregometry and describe the clinical spectrum of Glanzmann`s thrombasthenia diagnosed by platelet aggregometry. A case-series. This study was carried out at the clinical laboratories at the Aga Khan University Hospital, Karachi from January 2003 to January 2006. All patients irrespective of age and gender presenting with bleeding symptoms and having normal platelet count were evaluated. Demographic details, relevant clinical history along with results of complete blood count, bleeding time and platelet aggregation studies were retrieved through computerized data base and evaluated for the diagnosis of Glanzmann`s thrombasthenia. During the study period, 50 out of 2317 patients [2.2%] were diagnosed as Glanzmann`s thrombasthenia by platelet aggregometry with male to female ratio of 0.85:1 and median age of 10.2 years [ranging from 3 months to 27 years]. Common symptoms were epistaxis, oral and gingival bleed, bleeding from minor cuts and trauma that were observed in 46% of the patients; while 18%, 8% and 10% of them also complained of bruising, hematuria and bleeding per rectum respectively. Majority i.e. 86% had a bleeding time greater than 10 minutes. All patients had received blood or blood products for their bleeding episodes. Platelet aggregometry is a useful diagnostic modality for the assessment of Glanzmann`s thrombasthenia. The disorder presents with muco-cutanoeus bleeding and was found to be a common cause of bleeding in our setup

Rotavirus as the etiological agent of diarrhea in hospitalized children

Diarrheal diseases are a significant cause of mortality in children under five years of age and rotavirus is considered to be one of the major causes of childhood gastroenteritis. As it is a sig-nificant contributor to childhood mortality in developing countries, WHO recommends epidemiological studies regarding rotavirus especially in developing countries. As it is a vaccine preventable disease these researches can have a significant impact on the future recommendation of vaccines. To identify rotavirus in the stool analysis of children less than five years of age, presenting with acute diarrhea. To describe the age distribution and clinical features of rotavirus gastroenteritis. Hospital based, cross-sectional study. Analysis of 40 fecal samples was done for rotavirus by immunochromatography; rotavirus was identified in 6 [15%] samples. The majority of thechildren with rotavirus gastroenteritis were younger than 2.5 years. The children who tested positive for rotavirus had a better nutritional status than the children who were negative for the virus. No significant difference was seen in the duration of diarrhea and of hospitalization in the two groups. Diarrhea is a major public health problem in developing countries. Rotavirus as the etiological agent was detected in 15% of cases in the present study, which is in close approximation to several other studies from Pakistan. Still large-scale studies are required to determine the prevalence of rotavirus gastroenteritis that can have important implications for rotavirus vaccination. Meanwhile our strategy s-hould focus on good sanitation and clean drinking water to save our children from this democratic disease

Appropriate and inappropriate use of fresh frozen plasma

To analyze the current situation of use and misuse of fresh frozen plasma [FFP] in various clinical situations. This was a cross sectional study done at Liaquat National Hospital [LNH] during a period of 4 months from December 2002 to March 2003. About 300 file records of those patients who received fresh frozen plasma were studied. Each file record was checked for the diagnosis of the patient, coagulation profile and doctor's trigger for blood transfusion. The indications of FFP were checked according to guidelines set by British Committee of Standards and Haematology. Of 1486 units of FFP that were transfused to 300 patients, it was observed that 78.6% [1169 / 1486] of FFP was appropriately transfused while the remaining 21.3% [317/1486 units] was used without any supportive evidence. Although majority of the plasma was used appropriately, a considerable volume of plasma was also wasted

Erythropoietin induced hypertension

Hypertension has frequently been reported in association with Erythropoietin [EPO] use In-patients with renal failure. However, the relation between the two is complex, as chronic renal failure itself is associated with numerous cardiovascular complications including hypertension. The present clinical trial was undertaken to appreciate this unwanted effect of EPO in 24 hemodialysed patients with end stage renal disease. Out of them, 12 were hypertensives already receiving anti-hypertensive medication at the start of EPO therapy while rest were normotensive. These patients were randomly divided into group A and B, each had 12 patients [6 hypertensive and 6 normotensive]. Group A received EPO in dose of 40 units/kg body weight subcutaneously twice a week while group B was given double dose. The blood pressures of all the patients were noted before and during 10 weeks of EPO treatment, their hematocrit, hemoglobin, iron profile and blood chemistry were also monitored. Only 3 hypertensive patients receiving 80 a/kg EPO showed worsening of their blood pressures. No significant pressure changes were observed in normotensives with either EPO dosage or in hypertensive patients receiving low dose EPO. Our study showed that low dose EPO therapy could effectively be used to reverse anemia in renal failure patients without significantly altering their blood pressures. However, a minority of already hypertensive patients might show a worsening of their blood pressures with higher doses of EPO

Overview of normal hemostasis and bleeding disorders

Abnormal bleeding or bruising is frequently encountered in the clinical practice of dermatology. For the correct diagnosis and hence appropriate management of a bleeding patient, it is essential that a clinician must have a sound knowledge of pathophysiology of normal hemostasis as well as physical and laboratory evaluation of such patients