An audit of blood cross-match ordering practices at the Aga Khan University Hospital: first step towards a Maximum Surgical Blood Ordering Schedule

Objective: In the absence of an explicit maximum blood order policy, ordering for blood transfusion is frequently based on subjective anticipation of blood loss instead of evidence based estimates of average requirement in a particular procedure. This study was done to assess current practice and the feasibility of a prospective randomized work to develop practice guidelines.METHOD: We audited transfusion data for elective surgical procedures in our hospital during the last 2 years. Cross-matched to transfused ratio (C/T ratio) and Transfusion Index (Ti) for each of the elective surgical procedures was performed during the study period. C/T ratio is used as a measure of the efficiency of blood ordering practice. It should ideally be between 2 and 2.5. We compared our results with the ideal.Results: Data was analyzed for 32 elective surgical procedures in 2131 patients. Majority (2079) (97.56%) of the patients had C/T ratios higher than 2.5. Only 12 in 450 (21.11%) patients, had a Transfusion Index (Ti) higher than 0.5. There were 13 procedures in which both C/T ratio was greater than 2.5 and Ti less than or equal to 0.5.CONCLUSION: In vast majority of elective surgical procedures routine cross match is not necessary. We propose a draft Maximum Surgical Blood Ordering Schedule (MSBOS). It provides guidelines for frequently performed elective surgical procedures by recommending the maximum number of units of blood to be cross-matched preoperatively. Implementation of MSBOS will result in about 60% reduction of cost to the patients

Cyclosporin induced fatal rhabdomyolysis in a young patient with acquired aplastic anemia: a case report

Cyclosporin is used as an immunosuppressive agent in the management of a variety of autoimmune disorders1 and in patients of organ transplant to suppress host immunity and prevent rejection.2 It is now well established that acquired aplastic anemia is secondary to immune mediated destruction of stem cells in the bone marrow3 and hence Cyclosporin is used as a first line immunosuppressive drug alone or in combination with anti lymphocyte globulin in the management of aplastic anemia.1 Rhabdomyolysis is a rare side effect of Cyclosporin.4 Here we describe a case of fatal rhabdomyolysis in a young patient who received cyclosporin for treatment of aplastic anemia

Epidemiological features of aplastic anaemia in Pakistan

Objective: To complete the data on the demographic features of patients diagnosed to have aplastic anemia at a single institution over a 7.5 years period. Methods: Demographic information was retrieved from the patients medical records retrospectively as well as prospectively of those patients who presented with features of aplastic anaemia. Their diagnosis was confirmed by performing a complete blood count and bone marrow trephine. Results: One hundred and forty four patients were diagnosed to have aplastic anemia; there were 106 males and 38 females. Their ages ranged from 2 to 75 years, with a median of 17 years, 112 (77.7%) patients were below the age of 30 years. Severe aplastic anemia (SAA) was seen in 74 (51.4%), very severe (VSAA) in 24 (16.7%) and non-severe aplastic anemia (NSAA) in 46(31.9%) patients. No obvious cause could be established for 74.3%. Thirteen patients admitted using drugs known to cause AA and one was a radiographer (9%). Out of 44 patients tested, 7 (15.9%) were found to have either hepatitis B virus markers or antibody to hepatitis C at the time of diagnosis of AA. However it was difficult to establish a cause and effect relationship with either drugs or viruses. Conclusion: Aplastic anaemia is found to occur mostly severe aplastic anaemia (JPMA 51:443,2001)

Prevalence of hepatitis C virus in lymphoproliferative disorders

Objective: To study the prevalence of hepatitis C virus in lymphoproliferative disorders. Methods: A case control prospective study was performed on 143 patients with lymphoproliferative disorders and 29 patients with non-hematological malignancies were taken as controls. All the patients in both groups were analyzed for various risk factors for infection with hepatitis C virus and were tested for the presence of hepatitis C virus antibody (anti HCV), cryoglobulins and rheumatoid factor antibody. Hepatitis C viremia was documented by detection of HCV RNA by polymerase chain reaction. Results: There was no significant difference for risk factors for hepatitis C virus infection in both the groups except for the increase in number of surgical procedures being carried out in the control group. There was no significant difference in the presence of rheumatoid factor antibody in both the groups and cryoglobulins were not positive in any individual. Five percent patients with lymphoproliferative disorders and 3.4% with non-hematological malignancies were positive for anti HCV. HCV RNA was detected in 29.2% cases and 31.0% in controls. Conclusion: There was no association between hepatitis C virus infection and lymphoproliferative disorder in our population. However, further studies are required from this region to establish any causal relationship between hepatitis C virus infection and lymphoproliferative disorde

Long-term outcomes of acute myeloid leukemia in adults in Pakistan

Objective: To describe the long-term outcomes of Acute Myeloid Leukemia (AML) and to study clinico­pathological features at presentation, morphological subtypesand remission rates. Methods: Demographic information, response to therapy and survival of patients (\u3e14 years of age) admitted between January 1988 to August 1996 with acute myeloid leukaemia was retrieved and analysed. Results: Seventy-four patients were admitted with a diagnosis of AML during the study period. There were 43 males and 31 females. Age ranged between 15 and 70 years with a mean age of 38 years. The most common presenting feature was fever (67.5%) and the morphological subtype according to French-American-British Group (FAB) criteria was M4. Fifty-five patients received treatment and were evaluable for response and outcomes. Thirty-six (65.4%) patients had complete remission. Sixteen (29.1%) died during the first 28 days after starting induction chemotherapy. The median survival was 11 months. Six (11%) patients (4 females, 2 males) are surviving beyond 4 years (long-term survivors).Conclusion: Our study suggests that the long-term outcomes of adults with AML are comparable to what has been reported in the literature for patients who do not receive bone marrow transplants (JPMA 52:482;2002)

Impaired splenic function in systemic amyloidosis: diagnostic importance of peripheral blood film

We describe case of a 40 year old male, who went on to developed systemic amyloidosis 3 years after the diagnosis of chronic renal failure. The diagnosis of systemic amyloidosis was suspected upon a routine examination of periheral blood film showing features of hyposlenism. We would like to highlight the importance of examination of pheripheral blood film in patients with renal failure for the diagnosis of extensive systemic amyloidosis which occasionally leads to functional hyposplenism, recognized by the presence of abnormal red cell morphology and reduced splenic uptake on isotope scan1

Hematologic and cytogenetic findings in eleven chronic myelogenous leukemia patients treated with imatinib mesylate at a tertiary care hospital

Objective: To evaluate the response of Imatinib mesylate in patients with myeloid leukemia in chronic ,accelerated and blast phase. Methods:Eleven patients with established diagnosis of chronic myeloid leukemia were treatedwith Imatinib mesylate. Adverse events were documented with regular follow ups. Hematological and cytogeneticresponses were assessed according to established criteria. Patients with zero percent Philadelphia positivemetaphases were labeled as complete cytogenetic response while patients with 1% to 35% Philadelphia positive metaphases were termed as partial responders. Results: Of 11 cases there were 7 males and 4 females with a mean age of 39.5 years and median age 51 years(range 21-69). Male to female ratio was 7:4. Median follow-up was 34 weeks (range 8-78). Four patients werein blast crisis, 1 in accelerated phase and remaining six patients were in chronic phase. All patients achieved hematological response. Cytogenetic response was present in six patients, 3 were responders and the remainingwere non responders. Two patients achieved complete cytogenetic response and one patient had partial cytogenetic response. Both patients with complete cytogenetic response relapsed in twelve weeks time. Conclusion: Imatinib mesylate is a drug with curative potential and can be used as a first line drug in the management of CML, however at present the cure rate is unknow

Genetic diversity of beta-thalassemia mutations in Pakistani population

Background: beta-thalassemia is one of the most common inherited single gene disorder in Pakistan. It is characterized by reduced or absent beta-globin gene expression resulting in abnormal maturation and survival of red blood cells. Due to high prevalence of this disease in the local population, it has become important for the health care providers to encourage people to utilize laboratory facilities for carrier and prenatal genetic testing. Objective: To study the frequency of beta-thalassemia mutations in Pakistani population. Setting: A tertiary care teaching hospital. Methods: Blood samples of 72 couples and chorionic villus (CV) biopsy specimen collected at the Aga Khan University Hospital, Karachi were tested by Amplified Refractory Mutation Systems (ARMS) for the 12 most common mutations in the beta-globin gene. Results: Out of 72 chorionic villus biopsy specimen analyzed, 17 (23%) had mutations in both alleles of the beta-globin gene. Homozygosity was identified in 6 CV samples, whereas 11 CV specimens were diagnosed as double heterozygous. Almost 60% of the CV biopsies showed mutations in one allele and were diagnosed as carriers. IVSI-5 (G-C) was the most common mutation identified in this study. It was found in 53% of the subjects and was represented equally in all the ethnic groups except Pathans. Several regional and ethnic differences were observed in the distribution of common mutations, for example in Pathan families Fr 8-9 (+G) mutation was most prevalent. In addition, variation in the distribution of mutations was also observed between the Northern and the Southern regions. Conculsion: This study indicates that in Pakistan, the five most common mutations are IVS1-5 (G-C), IVS1-1 (G-T), Fr 41-42 (-TTCT) Fr 8-9 (+G) and deletion 619 bp. An important factor contributing to high incidence of thalassemia is the unawareness among people about the available diagnostic facilities for the prenatal diagnosis in Pakistan. Strict implementation of collective measures including carrier identification, genetic counseling and prenatal diagnosis are required for preventing beta-thalassemia in Pakistan

Frequency of fab subtypes in acute myeloid leukemia patients at Aga Khan University Hospital Karachi

BACKGROUND: Acute myeloid leukemia (AML) is a heterogeneous disease. Therefore, various parameters are needed to classify this disease into subtypes, so that specific treatment approaches can be utilized effectively. The commonly used method for diagnosis and classification is based on FAB criteria using morphology and cytochemical stains. For some of the categories, immunophenotyping is necessary. The aim of present study is to determine the frequency of various sub types in acute myeloid leukemia using FAB criteria in our population. This will aid in the correct diagnosis of acute leukemia and hence proper management of the patients. MATERIALS AND METHODS: This is descriptive case control study conducted at Aga Khan University Hospital from January 1999 to December 2000. The total number of subjects was 116 that included both adults and children. The patients were diagnosed on the basis of bone marrow morphology using FAB classification. Cytochemistry was done in all cases, while immunophenotyping was considered only in those cases that were found to be problematic. RESULTS: Among 116 patients, 70 were males and 46 were females with male to female ratio 1.5:1. The age ranged between 6 months to 85 years with a mean age of 32 years. AML-M4 was the predominant French-American-British (FAB) subtype (36.2%) followed by M2 (30.25%), M3 (10.4%), M1 (8.7%), M0 (7.7%), M5a (3.5%), M5b (2.5%) and M6 (0.8%). CONCLUSIONS: The most common FAB subtype observed in our study was Acute myelomonocytic leukemia (M4) which is in accordance with studies reported from Saudia Arabia and a previous study reported from our institution. However,other national and international studies have reported Myeloblastic Leukemia with maturation (M2) as the predominant subtype of AML

Polycythemia vera and idiopathic erythrocytosis: comparison of clinical and laboratory parameters

Objective: To evaluate the various clinical and laboratory parameters of Polycythemia vera and idiopathic erythrocytosis in order to differentiate between two entities at the Aga Khan University Hospital. Methods: Twenty six patients of polycythemia vera and 34 patients of idiopathic erythrocytosis were analyzed with respect to clinical features and laboratory findings. Results: Patients with idiopathic erythrocytosis were males with a mean age of 41 years and no splenomegaly. Patients with polycythemia were older males and females with splenomegaly, red cell count of mor than 6.5 million/cmm, haematocrit 55%, leucocytosis, thrombocytosis and low erythropoietin level. Conclusion: Based on the above-mentioned findings, we suggest that polycythemia vera and idiopathic erythrocytosis are separate entities and the diagnosis of these can be made on the basis of clinical and laboratory parameter