Institutional-based tumor registry of hematopoietic malignancies: A 4 years' preliminary report from Karachi.

BACKGROUND: Pakistan has a population of over 198 million making it the world's sixth populous country. However, operational population-based cancer registries in Pakistan are lacking. Limited data are available based on institutional or Karachi Cancer Registry from Karachi; however, no exclusive registry for hematological malignancies is established till date. Hence, we decided to conduct a database analysis to determine the frequencies of various hematological cancers in our tertiary care center in Karachi. PATIENTS AND METHODS: Retrospective assessment of 366 patients presented to Hematology Department, Liaquat National Hospital and Medical College from May 2012 to May 2016 with confirmed diagnosis of hematological cancers was performed. Data were retrieved from hospital-based tumor registry. All pro forma was filled by qualified medical research officer, which included demographic data as age, gender, history, disease type, and its stage at presentation. RESULTS: Male gender was predominant and accounted for 69.9% (n = 256) and female accounted for 30.0% (n = 110) of the total cases with the male-to-female ratio of ~2:1. The mean ages of female and male patients were 42.9 ± 20.7 and 49.7 ± 18.1 years with the median age of 45 and 51.5 years, respectively. In males, the three most frequent malignancies were chronic myeloid leukemia (CML) (16%), acute lymphoblastic leukemia (ALL), and acute myeloid leukemia (AML) (12.8% of each). In females, AML was the most common cancer accounting for 21.8% followed by CML (18.1%) and ALL (10%). CONCLUSIONS: Our institution-based tumor registry indicates CML and AML were the most common cancers in males and females, respectively, followed by ALL. Chronic lymphoid leukemia, unlike in the West, is seen infrequently in our population. National and provisional hematological tumor registry will be beneficial for future health planning and research.

Pattern of bone marrow involvement in non Hodgkin's lymphoma classified according to WHO classification: Report of a developing country Pakistan.

BACKGROUND: Limited information is available from developing countries in patients of non-Hodgkin's lymphoma (NHL). Hence, we reviewed the demographical profile along with bone marrow infiltration pattern in patients with NHL presented at Liaquat National Hospital and Medical College. MATERIALS AND METHODS: In this descriptive study, adult patients with NHL were enrolled from January 2011 to December 2015. RESULTS: One hundred and Eighty-four histopathologically confirmed cases of NHL were identified. There were 139 males and 45 females, with a male-to-female ratio of 3:1. The mean age was 48.5 ± 16.0 years with the median age of 50 years. B-symptoms were present in 80.4% of patients. Lymph node enlargement was present in 71.1% of the cases. One hundred and sixty-eight patients had B-cell lymphoma (91.3%) and 16 patients had T-cell lymphoma (8.6%). Overall 158 (85.8%) patients had aggressive lymphoma. The frequency of bone marrow infiltration in our NHL patients was found to be 31.5%. Pattern of infiltrate was diffuse (14.6%) predominantly followed by interstitial (6.5%) and paratrabecular (5.4%) types of infiltration. The least common was nodular infiltrate accounted in 9 (4.8%) patients. CONCLUSIONS: B-cell lymphoma is more frequent than T-cell lymphoma. Younger age, aggressive lymphoma, and predominance of B-symptoms are more frequently seen. Diffuse bone marrow infiltration is more common in our set up probably because of a relatively late presentation in our patients.

Internal quality control of blood products: An experience from a tertiary care hospital blood bank from Southern Pakistan.

INTRODUCTION: Internal quality control (IQC) is the backbone of quality assurance program. In blood banking, the quality control of blood products ensures the timely availability of a blood component of high quality with maximum efficacy and minimal risk to potential recipients. The main objective of this study is to analyze the IQC of blood products as an indicator of our blood bank performance. METHODS: An observational cross-sectional study was conducted at the blood bank of Liaquat National Hospital and Medical College, from January 2014 to December 2015. A total of 100 units of each blood components were arbitrarily chosen during the study. Packed red cell units were evaluated for hematocrit (HCT); random platelet concentrates were evaluated for pH, yield, and culture; fresh frozen plasma (FFP) and cryoprecipitate (CP) were evaluated for unit volume, factor VIII, and fibrinogen concentrations. RESULTS: A total of 400 units were tested for IQC. The mean HCT of packed red cells was 69.5 ± 7.24, and in 98% units, it met the standard (<80% of HCT). The mean platelet yield was 8.8 ± 3.40 × 109/L and pH was ≥6.2 in 98% bags; cultures were negative in 97% of units tested. Mean factor VIII and fibrinogen levels were found to be 84.24 ± 15.01 and 247.17 ± 49.69 for FFP, respectively. For CP, mean factor VIII and fibrinogen level were found to be 178.75 ± 86.30 and 420.7 ± 75.32, respectively. CONCLUSION: The IQC of blood products at our blood bank is in overall compliance and met recommended international standards. Implementation of standard operating procedures, accomplishment of standard guidelines, proper documentation with regular audit, and staff competencies can improve the quality performance of the transfusion services.

Insight into donor deferral pattern based on peripheral blood counts: An experience from South Pakistan.

BACKGROUND: Donor deferral owing to anemia is one of the major causative factors of temporary donor rejection, which is preventable and treatable. The basic knowledge about frequency, types, and severity of anemia among donors will help plan a strategy to promote donor recruitment and overall national health. OBJECTIVE: The objective of this study was to provide the predonation deferral rate of the healthy blood donors based on peripheral blood counts and second to determine the types of anemia along with its severity. MATERIALS AND METHODS: Prospective records of all the reported donors were collected from January 2014 to December 2015 at Liaquat National Hospital, Karachi, Pakistan. Donor samples were analyzed on an automated hematology analyzer. RESULTS: Overall, 36,954 potential donors reported to the blood bank, out of which 33,853 were selected and 3101 were deferred, which makes the deferral rate of 8.39%. Majority of donors (n = 2663 [7.20%]) were deferred based on peripheral blood counts. Based on peripheral count, anemia (91.8%) represents the major cause of deferral, followed by raised total leukocyte count (3.7%) and polycythemia (3.3%), and thrombocytopenia (1.0%) was the least potential cause. Microcytic-hypochromic anemia was found in 58.5% of the donors followed by normocytic and macrocytic anemia in 38.9% and 2.4%, respectively. Mild anemia was seen in 78.2% followed by moderate and severe anemia in 20.5% and 1.18%, respectively. CONCLUSION: A high prevalence of anemia among blood donors signifies deteriorating health status not only in donor population but also in general population. This situation calls for more concerted efforts as otherwise it would lead to decreased blood donor pool.

Clinico-hematological Profile of 184 Patients with Non-Hodgkin's Lymphoma: An Experience from Southern Pakistan.

BACKGROUND: Non-Hodgkin lymphoma (NHL) is a diverse group of lymphoma comprises of divergent tumors with paradoxical clinical behaviors and potential difference in response to therapy. We conducted a data-base analysis on NHL patients to evaluate the clinico-epidemiological features and WHO spectrum of NHL in Pakistani patients. MATERIALS AND METHODS: This descriptive study was conducted over a period of 5 years from January 2011 to December 2015 at Hematology department of Liaquat National Hospital. All NHL cases were diagnosed by morphology on H&E sections and Immunohistochemical profile according to WHO classification of lymphoid neoplasms. RESULTS: 184 histopathologically confirmed cases of NHL were identified. There were 139 males and 45 females, with a male to female ratio of 3: 1. The mean age was 48.5±16.0 years with the median age of 50 years. B symptoms were present in 80.4% of patients. Lymph node enlargement was present in 71.1% of the cases. 168 patients ad B-cell lymphoma (91.3%) and 16 patients had T-cell (8.6%) lymphoma. Overall 158 (85.8%) patients had aggressive lymphoma. Histopathologically, Diffuse large B-cell lymphoma constituted major subtype in 67.9%, followed by follicular lymphoma in 7.6% patients. Marginal lymphoma in 3.8%patients, 3.2% patients had mantle cell, 2.7% patients of anaplastic large cell and 2.1% patients each for Burkitt's lymphoma and T-cell rich lymphoma. In T cell neoplasm, peripheral T cell lymphoma and adult T cell lymphoma are the main variants accountable in 4.3% and 3.2% respectively. CONCLUSIONS: B cell lymphoma is more frequent than T cell lymphoma with diffuse large B-cell lymphoma being the commonest NHL. Our analysis shows that clinicopathological features of NHL are comparable to published data. However, aggressive lymphoma and predominance of B symptoms are more frequently seen.

Clinico-Hematological Findings for Classical Hodgkin's Lymphoma: an Institutional Experience.

BACKGROUND: Classical Hodgkin's lymphoma (cHL) is a B-cell lymphoid neoplasm characterized by a distinctive biological behavior with potentially curable disease characteristics. It is an uncommon hematological malignancy which primarily affects younger individuals. The rationale of this study was to determine its clinico-hematological profile along with stage strati cation in Pakistani patients. MATERIALS AND METHODS: In this descriptive study, adult patients with Hodgkin's lymphoma were enrolled from January 2010 to December 2014. RESULTS: Sixty two histopathologically con rmed cases of cHL were identified. There were 42 males and 20 females, with a male to female ratio of 2:1. The mean age was 29.7±13.8 years with the median age of 30. B symptoms were present in 72.5% of patients. Histopathologically, the mixed cellularity type constituted 62.9% of cases, followed by nodular sclerosis in 25.8%, lymphocyte predominant in 9.6% and lymphocyte depleted in 1.6%. Stages I and II were present in 43.5% of patients at disease presentation, with 56.4% in stages III and IV. CONCLUSIONS: Our analysis shows that clinico-pathological features of Hodgkin's lymphoma in Pakistan are comparable to published data. Mixed cellularity is the commonest histological variant and advanced stage at presentation are common findings in our patients.

Adverse Reactions in Allogeneic Blood Donors: A Tertiary Care Experience from a Developing Country.

OBJECTIVES: Fragmented blood transfusion services along with an unmotivated blood donation culture often leads to blood shortage. Donor retention is crucial to meet the increasing blood demand, and adverse donor reactions have a negative impact on donor return. The aim of this study was to estimate adverse donor reactions and identify any demographic association. . METHODS: We conducted a prospective study between January 2011 and December 2013. A total of 41,759 healthy donors were enrolled. Professionally trained donor attendants drew blood and all donors were observed during and following donation for possible adverse events for 20 minutes. Blood donors were asked to report if they suffered from any delayed adverse consequences. . RESULTS: Out of 41,759 blood donors, 537 (1.3%) experienced adverse reactions. The incidence was one in every 78 donations. The mean age of donors who experienced adverse events was 26.0±6.8 years, and all were male. Out of 537 donors, 429 (80%) developed vasovagal reaction (VVR), 133 (25%) had nausea, 63 (12%) fainted, 35 (6%) developed hyperventilation, 9 (2%) had delayed syncope, and 9 (2%) developed hematoma. Arterial prick, nerve injury, cardiac arrest, and seizures were not observed. Donors aged less than < 30 years and weighing < 70 kg were significantly associated with VVR, hyperventilation, and nausea (p < 0.005). Undergraduates and Urdu speaking donors also had a significant association with fainting and nausea, respectively (p < 0.05). . CONCLUSION: The prevalence of adverse events was low at our tertiary center. A VVR was the predominant adverse reaction and was associated with age and weight. Our study highlights the importance of these parameters in the donation process. A well-trained and experienced phlebotomist and pre-evaluation counseling of blood donors could further minimize the adverse reactions.

Acute Lymphoblastic Leukemia in Adults - an Analysis of 51 Cases from a Tertiary Care Center in Pakistan.

BACKGROUND: Acute lymphoblastic leukemia (ALL) is a malignant disease in which early lymphoid precursors proliferate and replace the normal hematopoiesis. It has distinctive clinical and biological features. In respect to adult ALL, available data from Pakistan are limited. Therefore we reviewed the demographical and clinico- hematological profiles along with FAB stratification of adult patients with ALL presented at our hospital. MATERIALS AND METHODS: In this cross sectional study, 51 adults (≥15 years) patients with ALL were enrolled from January 2010 to December 2014. RESULTS: The mean age was 23.8±12.9 years with the median age of 18.0 years. The male to female ratio was 2:1. The major complaints were fever (60.7%), generalized weakness (47.0%), overt bleeding (19.6%) and weight loss (13.7%). Physical examination revealed lymphodenopathy as a predominant finding detected in 43.1% followed by splenomegaly and hepatomegaly in 23.5% and 21.5%, respectively. The mean hemoglobin level was 9.0±2.75g/dl with a mean MCV of 82.2±15.4 fl, a mean total leukocyte count of 31.1±64.0x109/l, a mean ANC of 2.1±3.0 x109/l and a mean platelet count of 71.7±85.7x109/l. According to FAB classification, 47.1% were L1 type, 45.1% L2 and 7.8% L3 variant. CONCLUSIONS: Clinico-pathological features appeared comparable to published data. Febrile illness associated with lymphodenopathy was the commonest presentation. FAB classification revealed a predominance of ALL-L1 variant in Pakistani adult patients with ALL.

Multiple Myeloma: a Retrospective Analysis of 61 Patients from a Tertiary Care Center.

BACKGROUND: Multiple myeloma (MM) is an acquired clonal B-cell malignancy which primarily affects elderly individuals with an annual incidence of approximately 1% of all malignancies. Our aim is to study demographic and clinicopathological features of adult Pakistani MM patients at presentation. MATERIALS AND METHODS: This single centre retrospective study extended from January 2010 to December 2014. Data were retrieved from the patients' maintained records on predetermined performa. RESULTS: Overall, 61 patients were diagnosed at our institution with MM during the study period. There were 43 males and 18 females. Age ranged between 34 and 81 years with a mean of 56.1±12.8 and a median of 57 years. The male to female ratio was ~2:1. Common presenting complaints included fatigue (81.9%), backache (80.3%) and bone pain (67.2%). Physical findings revealed pallor (44.2%) as a presenting clinical feature. The mean hemoglobin value was 8.9±1.7g/dl with a mean MCV of 85.3±11.0 fl. Severe anemia with hemoglobin <8.5 gm/dl was seen in 40.9%. The mean total leukocyte count was 8.9±8.2x109/l, the ANC was 5.0±3.1x109/l and the mean platelet count was 188.4±150.6x109/l. CONCLUSIONS: MM in Pakistani patients is seen in a relatively young population with male preponderance. The majority of patients present with symptomatic anemia and backache to seek medical attention. However, clinico-pathological features appear comparable to the published literature.

Frequency and Pattern of Bone Marrow Infiltration in Classical Hodgkin's Lymphoma: Experience from Southern Pakistan.

BACKGROUND: Hodgkin's lymphoma (formerly, Hodgkins disease) is a potentially curable malignancy with distinctive biological behavior and specific clinical characteristics. Limited information is available from developing countries for patients with classical Hodgkin's lymphoma (cHL). Therefore we reviewed the demographical and clinico-hematological profiles along with bone marrow infiltration patterns in adult patients presenting at Liaquat National Hospital and Medical College. MATERIALS AND METHODS: In this cross sectional study, 62 adult (≥15 years) patients with cHL were enrolled from January 2010 to December 2014. RESULTS: The mean age was 29.7±13.8 years with a median of 30 years. The male to female ratio was 2:1. B symptoms were present in 72.5% of patients and lymph node enlargement in 85.4%. The frequency of bone marrow infiltration in our cHL patients was found to be 27.4%, the pattern being predominantly focal followed by diffuse. The mean hemoglobin was 9.4±1.9g/dl with a mean MCV of 78.1±7.9 fl, a mean total leukocyte count of 10.9±20.6x109/l and a mean platelet count of 241.6±150.1x109/l. CONCLUSIONS: Our analysis shows that clinico-pathological features of cHL in Pakistan are comparable to published data. Peripheral lymphodenopathy associated with B symptoms is the commonest presentation. Bone marrow involvement is more common in our setup as patients usually presented at an advanced stage of disease.

International Scoring System in Symptomatic Multiple Myeloma: Experience from a Tertiary Care Center.

BACKGROUND: Symptomatic multiple myeloma (MM) is an acquired B-cell malignant proliferation of anti- body secreting plasma cells, characterized by end organ damage due to monoclonal immunoglobulin secretion. The aim of this study wa to determine the stage stratification according to an international scoring system in adult Pakistani MM patients at presentation. MATERIALS AND METHODS: This single centre retrospective study extendedfrom January 2012 to December 2015. Data were retrieved from the departmental maintained records. RESULTS: A total of 39 patients were diagnosed at our center with MM during the period of the study, 25 males and 14 females. Age ranged between 36 and 81 with a mean of 54.5±14.8 and a median of 57 years. Common presenting complaints included fatigue (80.9%), backache (79.3%) and bone pain (66.2%). Overall, 9 patients were in ISS stage I (23%), 12 were in stage II (30.7%) and 18 were in stage III (46.1%). Out of the total, 29 (74.3%) had kappa immunoglobulin andthe remaining 10 (25.6%) had lambda type myelomas. IgG myeloma was commonest, seen in 26 (66.6%) followed by IgA in 11 (28.2%) with non secretory myeloma in one (2.5%) and light chain disease also in one patient (2.5%). CONCLUSIONS: MM in Pakistani patients is seen in a relatively young population with male predominance. Primarily patients are symptomatic and risk stratification revealed a predominance of advanced stage III disease in our setting.

Spectrum of the WHO Classification De Novo Myelodysplastic Syndrome: Experience from Southern Pakistan.

BACKGROUND: Myelodysplastic syndrome (MDS) is a clonal disorder of hemopoeitic stem cells, characterized by infective hematopoiesis, peripheral cytopenias along with hypercellularity of marrow and marked dysplastic features. Our aim was to study the spectrum of the WHO classification in adult Pakistani patients with MDS at disease presentation. MATERIALS AND METHODS: This retrospective descriptive study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Patient data were retrieved from the maintained archives. RESULTS: Overall, 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean of 57.6±17.4 years. The male to female ratio was 1.7:1. According to the WHO classification, 53.3% had refractory cytopenia with multilineage dysplasia, 22.2% had refractory cytopenia with unilineage dysplasia, 4.4% each had refractory anemia with excess of blasts-1 and II and 15.5% had MDS unclassified. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20% of patients. Hemoglobin was <10 g/dl in 41 (91.1%). Pancytopenia and bicytopenia were noted in 18 (40%) and 14 (31.1%) respectively. CONCLUSIONS: MDS in our patients presents at a relatively young age. Refractory c ytopenia with multilineage dysplasia was the dominant disease variant in our setting.

Somatic JAK-2 V617F Mutational Analysis in Polycythemia Rubra Vera: a Tertiary Care Center Experience.

BACKGROUND: Polycythemia rubra vera (PV), being a primary polycythemia, is caused by neoplastic proliferation of erythroid, megakaryocytic and granulocytic lineages which result in panmyelosis. PV patients have a somatic acquired mutation in the Janus kinase (JAK2) pathway, rendering cell proliferation independent of the normal regulatory mechanisms that regulate erythropoiesis. The rational of this study was to determine the prevalence of the JAK-2 V617F mutation in Pakistani patients with PV. MATERIALS AND METHODS: In this cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. Patients were diagnosed based on WHO criteria for PV. All were screened for G-T point mutation (V617F) in the JAK2 gene on chromosome 9 by an allele specific PCR. RESULTS: The mean age was 53.4±9.31 years (range 36-72) and the male to female ratio was 2:1. The frequency of JAK2 V617F positivity in our PV patients was found to be 92.3%. Overall 30.7% of patients were asymptomatic and remaining 69.3% presented with symptomatic disease. The mean hemoglobin was 18.1±1.9g/dl with the mean hematocrit of 55.6±8.3%. The mean total leukocyte count was 12.8±7.1x109/l and the platelet count was 511±341.9x109/l. A positive correlation of JAK2 V617F mutation was established with high TLC count (P=0.01). No correlation of JAK2 V617F could be established with age or gender (P>0.05). CONCLUSIONS: The JAK2 V617F mutation frequency in our PV patients was similar to those reported internationally. Screening for the mutation in all suspected PV cases could be beneficial in differentiating patients with reactive and clonal erythrocytosis.

Adult Primary Myelodysplastic Syndrome: Experience from a Tertiary Care Center in Pakistan.

BACKGROUND: Primary myelodysplastic syndrome (MDS) is an acquired clonal disorder of myeloid progenitor cells, characterized by peripheral cytopenias in the presence of hypercellular marrow with dysplastic features. Our aim was to study the demographical and clinicopathological features of adult Pakistani patients with MDS at disease presentation. MATERIALS AND METHODS: This single centre study was conducted at Liaquat National Hospital and Medical College, extending from January 2010 to December 2014. Data were retrieved from the patient archives. RESULTS: Overall 45 patients were diagnosed at our institution with de novo MDS during the study period. There were 28 males and 17 females. Age ranged between 18 and 95 years with a mean age of 57.6±17.4 years and median of 64 years. The male to female ratio was 1.7:1. The main presenting complaints were generalized fatigue (60%), fever (33.3%), dyspnea (15.5%), bleeding (13.3%) and weight loss (11.1%). Examination was unremarkable in 42.2% of patients. Physical examination revealed pallor in 37.7%, followed by petechial and purpuric rashes in 20%. The commonest laboratory finding was anemia (hemoglobin <10 g/dl in 41 (91.1%) patients. Out of these, 27 (60%) patients had normocytic anemia, followed by macrocytic (22.2%) and microcytic (8.8%). CONCLUSIONS: Primary MDS in Pakistani patients demonstrates a male preponderance. The proportion of anemic patients was high in our series with predominance of normocytic anemia. However, other clinico-hematological features appear comparable to published data.

Clinico-Epidemiological Profile of Patients with Polycythaemia Rubra Vera - a Five Year Experience from a Tertiary Care Center.

BACKGROUND: Polycythaemia rubra vera (PV) is a Philadelphia chromosome negative myeloproliferative neoplasm characterized by increased red cell production, independent of the mechanisms that regulate normal erythropoiesis. The aim of this study was to analyze the clinico-epidemiological profile of Pakistani patients with PV. MATERIALS AND METHODS: In this retrospective cross sectional study, 26 patients with PV were enrolled from January 2010 to December 2014. They were diagnosed based on WHO criteria. RESULTS: The mean age was 53.4±9.31 years (range 36-72) and the male to female ratio was 2:1. Overall 30.7% of patients were asymptomatic. In symptomatic patients, major complaints were headache (30.8%), abdominal discomfort (23.1%), blurred vision (15.3%), pruritus (11.5%) and vascular incidents (11.5%). Physical examination revealed plethoric face and splenomegaly as predominant findings, detected in 34.6% and 30.7%, respectively, with the mean splenic span of 15.9±2.04cm. The mean hemoglobin was 18.1±1.9 g/dl with the mean hematocrit of 55.6±8.3%. The mean total leukocyte count was 12.8±7.1x109/l and the platelet count 511±341.9x109/l. Mean erythrocyte sedimentation rate was 3.5±1.22mm/hr. Serum lactate dehydrogenase, serum creatinine and uric acid were 552.7±309.2, 0.8±0.17 and 6.60±1.89 respectively. CONCLUSIONS: PV in Pakistani patients, unlike in the West, is seen in a moderately young population. The disease is frequently seen in male gender and primarily patients present with symptoms related to hyperviscosity.

Biochemical Markers of Bone Turnover in Patients with ß-Thalassemia Major: A Single Center Study from Southern Pakistan.

Objectives. Skeletal complications in ß-homozygous thalassemic patients are uncommon but often debilitating, even amongst children and adolescent patients with well maintained transfusion and chelation therapy. The aim is to evaluate the biochemical markers of bone turnover in regularly transfused thalassemic patients and its possible correlations with demographic data and hematological and biochemical markers. Methods. In this prospective cross-sectional study, 36 ß-thalassemia major patients were enrolled from March 2012 to March 2014. All patients underwent complete blood counts, LFTs, serum ferritin, serum calcium, phosphorus, serum albumin, alkaline phosphatase, 25-OH vitamin D, and parathormone (PTH) levels. Results. There were 17 males and 19 females with mean age of 12.56 ± 5.9 years. Hypocalcemia and hypophosphatemia were seen in 66.6% and 19.4%, respectively, while 25-OH vitamin D deficiency was present in 72.2% of thalassemic children and adolescents. Hypoparathyroidism was seen in 13.8% while hyperparathyroidism was detected in 8.3% of patients. There was direct correlation between serum phosphorus and ferritin levels (P < 0.05). No correlation was found between indirect bilirubin and skeletal parameters, calcium and parathyroid hormone (P > 0.05). Conclusions. Biochemical profile is significantly altered in patients with ß-thalassemia major and bone associated biochemical abnormalities like hypocalcaemia, 25-OH vitamin D deficiency, and hypophosphatemia are not uncommon in Pakistani patients with thalassemia major.

Trends of Venereal Infections among Healthy Blood Donors at Karachi.

BACKGROUND: To determine the prevalence and trends of venereal infections, including the Human Immunodeficiency Virus (HIV) and treponema pallidum in healthy blood donors over a decade. MATERIALS AND METHODS: This cross-sectional descriptive study conducted at the Blood Bank of Liaquat National Hospital, extended from January 2005 to December 2014, in which 148268 healthy donors were enrolled. Screening for HIV was done by Microparticle Enzyme Immunoassay and Chemiluminescent immunoassay techniques. Syphilis screening was done by Treponema pallidum particle agglutination and Chemiluminescent Immunoassay methods. RESULTS: Out of the 148268 total donors, 147684 (99.6%) were replacement donors and remaining 584 (0.4%) were voluntary donors with a mean age of 29.09 ± 7.04 years. Overall, there were 147939 (99.8%) males and 329 (0.2%) females. A total of 1536 (1.03%) donors were reactive. Among the replacement donors, 1535 (1.03%) were found to be reactive for HIV and treponema pallidum. Of these, 172 (0.11%) donors were positive for HIV, while 1363 (0.91%) were positive for treponema pallidum. All reactive donors were male (99.94%), except for one female (0.06%). The prevalence of HIV and syphilis were higher in the younger age group (18 - 30 years). Out of 584 voluntary donors, only one (0.1%) donor was positive for HIV, while all donors were negative for treponema pallidum. The positive rates among replacement and voluntary donors were 1.03% and 0.1%, respectively. Co-infectivity was observed in two (0.001%) donors. CONCLUSION: The prevalence of venereal infection was higher in replacement donors as compared with voluntary donors. The prevalence of syphilis in our series was high compared with earlier studies. It emerges that syphilis positivity rate appears to escalate; however, HIV infection remains static over a decade. Higher prevalence in younger donors is alarming and adversely affects the prospective blood donation pool. Stringent donor selection, emphasis on voluntary donation, and vigilant donors screening are highly recommended to ensure the maximum safety of blood recipient.

Demographic and Clinical Characteristics of Adult Acute Myeloid Leukemia--Tertiary Care Experience.

BACKGROUND: Acute myeloid leukemia (AML) is an acquired clonal frequent malignant disorder of myeloid progenitor cells. Our aim was to study demographical and clinicopathological features of adult Pakistani AML patients at presentation. MATERIALS AND METHODS: In this single centre study extending from January 2010 to December 2014, data were retrieved from the patient records with a predetermined performa and analyzed with SPSS version 22. RESULTS: Overall 125 patients were diagnosed at our institution with de novo AML during the study period. There were 76 males and 49 females (ratio 1.5:1), with an age range between 15 and 85 years and a mean age of 38.8±20.1 years. The major complaints were fever (72.8%), generalized weakness (60%), bleeding (37.6%) and dyspnea (12%). Physical examination revealed pallor in 56.8%, splenomegaly and hepatomegaly in 16% and 12.8%, respectively, and lymphodenopathy in 10.4%. The mean hemoglobin was 8.19±2.12g/dl with a mean MCV of 86.0±9.83 fl, a mean total leukocyte count of 43.1±68.5x109/l, an ANC of 3.09±6.66x109/l and a mean platelet count of 62.3±78.6x109/l. CONCLUSIONS: AML in Pakistani patients is seen in a relatively very young population with male preponderance, compared with the west. However, clinico-pathological features appear comparable to published data.

Acute Myeloid Leukemia: Clinical Spectrum of 125 Patients.

BACKGROUND: Acute myeloid leukemia is an acquired clonal heterogeneous stem cell disorder. Hence, various parameters are sought out to categorize this disease into subtypes, so that as a consequence specific treatment modalities can be offered. Conventionally, the practically used method for classification utilizes French American British (FAB) criteria based on morphology and cytochemistry. The aim of present study was to determine the current spectrum of AML sub types in patients in Karachi. MATERIALS AND METHODS: This single centre cross sectional study was conducted at Liaquat National Hospital, Karachi, extending from January 2010 to December 2014. Data were retrieved from archives were analyzed with SPSS version 22. RESULTS: A total of 125 patients were diagnosed at our institution with de novo AML during five years period, 76 males and 49 females. Median age was 34.5 years. AML-M1 was the predominant FAB subtype (23.2%) followed by M2 (18.4%), M3 and M4 (16% each), M0 (14.4%), M5 (7.2%), M6 (3.2%) and M7 (1.6%). CONCLUSIONS: AML in Pakistani patients is seen in a relatively young population. The most common FAB subtype observed in our study was acute myeloblastic leukemia, without maturation (M1).

Serum fasting lipid profile in children and adolescents with ß-thalassaemia major in southern Pakistan.

OBJECTIVE: Serum fasting lipid profile has been studied in various clinical spectrum of Beta (ß)-thalassaemia syndrome. Premature cardiac impairment in thalassaemia major appears primarily due to iron accumulation and oxidative injury; however it might be a sequel of abnormal lipoprotein concentrations. The rational of this study is to analyse the serum fasting lipid profile in cardiovascular disease free ß-thalassaemia major (ß-TM) patients. Relationships with age, gender, haematological parameters, liver enzymes and serum ferritin were observed. METHOD: Fasting serum lipid levels, liver function test (LFT), complete blood count (CBC) and serum ferritin were measured in 36 patients with homozygous ß-TM from March 2012 to March 2014. Patients were stratified into two groups, age≤15 and >15 years, to determine the possible lipid profile distinction in relation to age. RESULTS: 17 were males and 19 were females, with median age of 12.0 years. The mean total cholesterol (TC) and triglyceride (TG) were 5.01±1.32 and 8.36±5.28 mmol/L respectively. High TG was detected in 36.1%, while high density lipoprotein cholesterol (HDL) and low density lipoprotein cholesterol (LDL) were markedly low, 0.98±0.51 and 2.35±1.22 mmol/L respectively. No statistically significant difference was noted between the two age groups. The median TC to HDL ratio (TC:HDL) was elevated, 5.7 (4.0). We established significant correlation of total bilirubin with TC (r=-0.4), HDL (r=-0.5) and LDL (r=-0.4) (P<0.05). CONCLUSION: Dyslipidaemia in ß-TM patients is irrespective of age and gender including low HDL and high TC:HDL, whilst high TC:HDL may contribute as a significant risk marker for future cardiac events in these patients.