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

Clinical characteristics, outcome and early induction deaths in patients with acute promyelocytic leukaemia: a five-year experience at a tertiary care centre

<p><b>INTRODUCTION</b>Acute promyelocytic leukaemia (APL) is a distinct clinical and biological subtype of acute myeloid leukaemia. APL is notorious for causing early death during induction therapy, resulting in induction failure. The aim of our study was to report the clinical characteristics, outcome and early induction deaths with regard to patients with APL seen at our hospital.</p><p><b>METHODS</b>This was a retrospective study carried out at Aga Khan University Hospital, Karachi, Pakistan. Patients aged > 15 years diagnosed with APL within the period September 2007-September 2012 were included in the study.</p><p><b>RESULTS</b>Within the study period, 26 patients were diagnosed with APL based on morphology and the detection of t(15;17)(q24.1;q21.1) and promyelocytic leukaemia-retinoic acid receptor alpha (PML-RARA). The male to female ratio was 1:1. The median age of the patients was 41 (range 16-72) years. In all, there were 13 (50.0%) high-risk patients, and early induction death rate was 61.5%. Causes of early induction deaths (n = 16) included haemorrhage in 7 (43.8%) patients, differentiation (ATRA) syndrome in 7 (43.8%) and infection in 2 (12.5%). The survival rate among patients who survived the early period was 70% at 42 months. The relapse rate was 30%.</p><p><b>CONCLUSION</b>Early induction death rate was very high in patients with APL. The most common cause of early induction death in our study was haemorrhage. Outcome among patients with APL was found to be better among those who survived the initial period.</p>

Chromosomal abnormalities in primary myelodysplastic syndrome

To determine the frequency of cytogenetic abnormalities in patients diagnosed as primary myelodysplastic syndrome [MDS] using conventional karyotyping. Case series. The Clinical Laboratory. The Aga Khan University Hospital, Karachi, between January 2006 - June 2012. Patients of all ages and either gender who fulfilled WHO criteria for MDS were included. Cytogenetic analysis was conducted at the time of diagnosis. Patients who had secondary MDS were excluded from analysis. Chromosome identification and karyotype description was done according to the International System for Chromosome Nomenclature [ISCN, 1995] and described as frequency percentage. Out of the 122 cases of MDS, 71 patients had their karyotype done at the time of diagnosis, including 42 males [59.2%] and 29 females [40.8%] with median age of 60 years. Forty one [57.7%] showed normal karyotype and 30 [42.3%] showed clonal karyotypic abnormalities at diagnosis. Out of which 14 [19.7%] had single, 11 [15.5%] had complex and 6 [8.5%] had double cytogenetic abnormalities. The common abnormalities found were: trisomy 8 in 7 cases [9.9%], -7/del [7q] in 3 cases [4.2%], -Y and complex 5q in 2 cases [2.8%] each, complex trisomy 8, del 11q, inversion 9, trisomy 19 and del 20q were found in 1 case [1.4%] each. Other abnormalities were found in 11 cases [15.5%]. Trisomy 8 was the most common disorder/abnormality found in this study population followed by the complex cytogenetics

T-cell large granular lymphocytic leukemia: 4 cases

No abstract available.

Application of the dermatology life quality index in clinical trials of biologics for psoriasis / 中国结合医学杂志

The significance of patient-reported outcomes (PROs) is increasingly being acknowledged and quality of life (QOL) has become an integral element of the assessment of overall burden of disease. Psoriasis has been known for its major impact on patients' QOL and various generic, dermatology-specific and psoriasis-specific self-administered psychometric instruments have been used over the years to assess the QOL of psoriasis patients. However, the Dermatology Life Quality Index (DLQI) is the most widely used QOL measure among these measures in psoriasis-related clinical trials. A number of topical and systemic treatments have been used in the management of psoriasis and lately biologics have emerged as a new and promising treatment modality for difficult-to-treat psoriasis. The evidence on the efficacy of these agents has been growing dramatically with QOL being used as one of the primary outcome measures in many clinical trials. The aim of this paper is to give an overview of the use of the DLQI as an outcome measure for assessing the QOL impact of biologics on psoriasis patients. Furthermore, the efficacy of five commonly used biologics has been compared in terms of their ability to improve the QOL assessed by the DLQI. This review has revealed that there is a variation in the efficacy of various biologics in terms of QOL improvement with the mean reduction in the DLQI scores being highest for ustekinumab 90 mg (mean DLQI score reduction=9.5), followed by infliximab (8.5), etanercept 50 mg, twice a week (7.7), adalimumab (6.3), and alefacept (4.0).

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

Additional chromosomal abnormalities in Philadelphia-positive chronic myeloid leukemia

The emergence of non-random chromosomal abnormalities is a well-recognized occurrence in chronic myeloid leukemia [CML] and detection of these abnormalities is important in prognostic stratification. The frequency and types of additional chromosomal abnormalities in CML patients has not been determined in our region. We conducted a descriptive, prospective study of additional chromosomal abnormalities in patients with an established diagnosis of Philadelphia-positive CML from May 2001 to June 2007. Cytogenetic studies were repeated every three months with the conventional G-banding technique and described according to the international system for Human Cytogenetic Nomenclature. All patients received imatinib mesylate. In 219 patients with Philadelphia-positive CML, 34 [15.5%] [median age, 38 years] developed 51 additional chromosomal abnormalities. Five cases had variant translocations prior to starting imatinib; the remaining 29 cases acquired chromosomal abnormalities after starting imatinib, including 8 cases that received prior interferon-alfa. Twenty-one patients were in chronic phase, 10 in accelerated phase and 3 were in blast crisis. Trisomy 8 was the most frequent abnormality followed by random chromosomal abnormalities and variants of the Philadelphia chromosome. The overall frequency of additional chromosomal abnormalities was similar to that in previous reports. Early identification of these abnormalities may help in adapting to a more appropriate therapeutic approach

Significance of cytogenetic abnormalities in acute myeloid leukaemia

To evaluate the role of karyotype in acute myeloid leukaemia [AML] as a predictor of response to induction chemotherapy. A cross-sectional study was carried out at the department of Pathology and Oncology, Aga Khan University Karachi from January 2003 to January 2005. Newly diagnosed patients with denovo AML admitted to the hospital were included in the study. Diagnosis of AML was based on FAB criteria, immunophenotyping and cytogenetic studies. They were treated according to standard protocols [combination of anthracycline and cytarabine -3+7] and those who had acute promyelocytic leukaemia additionally received all- trans retinoic acid [ATRA]. A total of 56 patients were enrolled, 4 were excluded due to inadequate cytogenetic analysis and the remaining patients entered the study protocol. There were 32 males and 20 females with mean age of 31.3 years [range 9 months to 73 years]. Thirty-five [67.3%] patients had normal karyotype while 17 [32.7%] were found to have cytogenetic abnormalities. Eleven patients did not receive treatment at our hospital. Half of the [51.2%] patients out of remaining 41 achieved complete remission on bone marrow examination after receiving induction chemotherapy. In favourable risk group 3/3 [100%] achieved complete remission [CR] while 15/32 [46.9%] in intermediate risk group and 3/6 [50%] in unfavourable risk group. There was low CR rate in patients with high white cell counts. The frequency of cytogenetic abnormalities in AML and response to induction chemotherapy was low when compared with international data possibly due to the small sample size. However, there was a clear difference in CR rates between favourable and unfavourable risk groups

Fludarabine induced autoimmune haemolytic anaemia in a patient with chronic lymphocytic leukaemia

Autoimmune haemolytic anaemia following fludarabine is an uncommon complication and previously treated patients are at higher risk. We describe a case of 57- year old lady with chronic lymphocytic leukaemia; she received intermittent courses of alkylating agents and purine analogue, fludarabine. Reintroduction of fludarabine for her relapsing disease induced autoimmune haemolytic anaemia. Numbers of cases have been reported regarding autoimmune haemolytic anaemia following fludarabine administration, but none have been published from our part of the world. Normally T-cell suppresses autoreactive lymphocytes that can produce autoantibodies. Suppression of T-cells by fludarabine, in addition to the underlying disease process appears to be a contributory factor for autoimmune haemolytic anaemia

Maternal and fetal complications of antiphospholipid syndrome: a case report with long-term follow-up

We report a case of a 26- year old woman having antiphospholipid syndrome in pregnancy with long-term follow up. She presented with recurrent miscarriages, venous thrombosis, avascular necrosis of femoral head, mid-cerebral artery infarction and skin ulcers. Antiphospholipid syndrome is a recognized disorder of pregnancy. Diagnosis requires a high index of suspicion when evaluating women with recurrent pregnancy losses and vascular thrombosis. A low dose aspirin combined with heparin can reduce morbidity and improves the pregnancy outcome

Role of ICT malaria immunochromatographic test for rapid diagnosis of malaria

To evaluate the sensitivity and specificity of immunochromatographic test [ICT] malaria p.f/p.v using microscopy as the gold standard diagnosis. Five hundred and sixty patients of both sexes and all age groups with clinical suspicion of malaria were studied. Venous blood was collected for microscopy and ICT. Thick and thin films prepared and stained with Leishman's stain were examined. ICT malaria test was performed and interpreted according to manufacturer's instructions. Data was analyzed using Epi-6. A total of 560 cases were studied, 339 males and 221 females with age ranges between 2 to 73 years. Seventy two [12.85%] cases had parasitaemia [with or without sexual forms]. On microscopy 65 [11.6%] cases had asexual-stage parasitaemia and 7 [1.25%] cases had P. falciparum gametocytes only. Thirty two cases were infected with P. falciparum, 29 with P. vivax and 4 had mixed infection. For P. falciparum the ICT was 97.0% sensitive, 98.3% specific, with positive predictive value [PPV] of 78.0% and a negative predictive value [NPV] of 99.8%. For P. vivax the sensitivity was only 89.7%, specificity 97.9%, PPV was 70.3% and NPV 99.4%. Our results are in concordance with previous studies. Rapid tests though expensive are simple to perform and effective diagnostic tools of malaria. They can be used selectively, though microscopy remains the gold standard diagnosis, economical and accurate if performed by skilled technologists

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

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

Acute myeloid leukemia following regression of sarcoidosis: a case report

This report describes Acute Myeloid Leukemia [AML] occurring in a 46 years old woman previously diagnosed to have Sarcoidosis. There was no evidence of Sarcoidosis at the time of diagnosis of AML. Although the association is well recognized, a cause and effect relationship between the two diseases is not fully established. A brief review of the literature is presented. Case Reports: A 46 years old lady presented to the emergency room with a history of high grade fever, exertional dyspnoea and generalized weakness for the past 4 weeks. She had undergone coronary artery bypass grafting [CABG] five years back. A year before the current presentation, she had presented with a history of fever, dry cough and anorexia. Examination had been unremarkable, except for an ESR of 32 mm/hr and bilateral hilar lymphadenopathy on chest X-ray. She was treated with standard anti-tuberculosis therapy [ATT] empirically. Subsequent to a lack of response to ATT and cultures for Acid Fast Bacilli remaining negative, Angiotensin Converting Enzyme [ACE] levels were found to be elevated to 59 IU/L [normal 8-52 IU/L]. A lymph node had also appeared in the left supraclavicular region by this time; excisional biopsy of which revealed non-caseating granuloma. ATT was discontinued and the patient was started on oral steroids. Within the next two months she became asymptomatic and the chest X-ray showed a complete regression of hilar Lymph nodes. During her current admission, she was found to be febrile, pale, icteric with hepatosplenomegaly, but no lymphadenopathy Examination of the cardiovascular, respiratory and the central nervous system were unremarkable. Her laboratory data revealed a haemoglobin of 6.3 g/dl, total leukocyte count of 121x 109/L with 88% blast cells, and a platelet count of 24x109/L. Bone marrow aspirate revealed Auerrod containing blast cells which constituted 90% of the total nucleated cells. 80% of the cells showed reactivity to Sudan Black. The patient was diagnosed to have AML. The chromosomal analysis revealed a 46 XX karyotype. Serum chemistries revealed a BUN of 7 mg/dl; creatinine 1mg/dl; Na 140 mEq/L; K 2mEq/L; total bilirubin 3.6 mg/dl; ALT 11 IU/L; alkaline phosphate 52 IU/L; LDH 5487 IU/L; and uric acid 7.2 mg/dl. The ACE levels were within normal limits. The chest Xray showed evidence of previous sternotomy and no lymphadenopathy. She was started on induction chemotherapy consisting of cytosine arabinoside 100 mg/m2 for 7 days and mitoxantrone 12 mg/m2 for three days. Hematological remission was documented on the 29th day of induction treatment. Bone marrow biopsy did not reveal a granuloma or fibrosis

Case of pelvic relapse in a child suffering from acute lymphoblastic leukemia

We describe here a case of an eight years old child suffering from acute lymphoblastic leukemia. She developed pelvic infiltration of leukemic cells while in bone marrow remission and receiving maintenance chemotherapy. She also developed leukemic infiltration of Central Nervous System and died of complications resulting from massive pelvic relapse. With greater number of children in bone marrow and CNS remission, the issue of possible greater predisposition to extramedullary replase has been discussed. The need for greater vigilance towards pelvic surveillance has been stressed

Factor XIII deficiency in Pakistan

Patients with undiagnosed haemostatic defects seen at the Aga Khan Hospital and Fatimid Blood Transfusion Centre during the period of 7 years [1985-1992] were screened with routine tests including bleeding time [BT], whole blood clotting time [CT], platelet count, activated partial thromboplastin time [APTT], prothrombin time [PT] and 5 molar urea test. Nine patients had a positive 5 molar urea test indicating factor XIII deficiency. Rest of the screening tests were normal in these patients. High incidence of consanguinity was observed in affected families. Clinical features included excessive bleeding from umbilical stump, bruising, post-traumatic bleeding, epistaxis, melaena and intracerebral bleeding. All the patients were treated with fresh frozen plasma and cryoprecipitate