Response of Imitanib Meysylate in patients with gastrointestinal stromal cell tumour

In this study, the response of Imitanib Mesylate in patients with Gastro-intestinal stromal cell tumour (GIST) was assessed. GIST results from a mutation in one of the receptor protein tyrosine kinases. Imitanib Mesylate, a tyrosine kinase inhibitor, has emerged as a promising new treatment for GISTs. Total 16 cases were reviewed. Diagnosis was based on biopsy and immunohistochemistry. Response assessment was done using CT scans, at a median duration of 4 months. The median age of the patients was 52 years, Majority were male (n=14). Most common presenting complaint was abdominal pain (n=7). Commonest primary site was stomach (n=7), Liver was the most common organ involved in metastasis (n=8). All patients received Imitanib 400 mg orally, once a day. No mortality was reported during median follow up time of 28 months on Imitanib. There was also either radiological remission (n=5) or response (n=5), in about half of the patients without any serious side effects

Pleural effusion as a manifestation of multiple myeloma

Multiple myeloma is a clonal B-cell malignancy, characterised by proliferation of plasma cells and secretion of paraproteins. These plasma cells accumulate predominantly in the bone marrow; rarely, they invade other areas, especially the thorax. Myeloma presenting with a pleural effusion is rare and reported in only 6% of patients with myeloma. Such patients generally present late and have a poor prognosis. Here, we describe a patient presenting with a lung mass, renal failure and a massive unilateral pleural effusion due to multiple myeloma who was treated successfully

Clinico-pathological profile and outcomes of patients with polycythaemia vera, essential thrombocythaemia and idiopathic myelofibrosis: a tertiary care center experience from southern Pakistan

Background: The “Philadelphia Negative Classic Myeloproliferative Neoplasms” include polycythaemia vera (PV), essential thrombocythaemia (ET) andidiopathic myelofibrosis (IMF). These three disorders share several clinical and laboratory features including JAK2 V617F mutation. Our objectives were to determine the clinicoat hological profile and outcomes of Pakistani patients with polycythaemia vera (PV), essential thrombocythaemia (ET) and idiopathic myelofibrosis (IMF) in order to have an insight regarding behaviour of these conditions. Methods: A retrospective analysis of all the cases of PV, ET and IMF diagnosed at our institute from January 1995 to December 2013 was performed.Age, gender, clinical presentation, laboratory investigations, treatment provided and duration of follow up were included for analysis. Appropriatestatistics were utilized for calculation of data. Results: A total of 58 patients were diagnosed as PV, ET or IMF during the study period. Male to female ratio was 1.1:1Forty five percent (n=2) patients came to medical attention due to abnormal laboratory results, 3 had cerebrovascular events, 3 had pruritus, and 1 patient each with gangrene and BuddChiari syndrome. Haemorrhag was not seen in any patient. Sixty percent (n=35) patients were treated with phlebotomy, hydroxyurea and aspirin alone or in combination. None of the patients transformed to myelofibrosis (MF) ormyelodysplasia (MDS) during the mean (±SD) followupperiod of 57.2±50 months.One patient with ET transformed to acute myeloid leukaemia9 yearsafter the diagnosis. Conclusions: This study demonstrated a relatively more benign form of PV, ET and IMF with lesser frequency of symptoms, good response to treatment andless likelihood of transformation to MF, MDS or AM

Autologous hematopoietic stem cell transplantation-10 years of data from a developing country

Intensive chemotherapy followed by autologous stem cell transplantation is the treatment of choice for patients with hematological malignancies. The objective of the present study was to evaluate the outcomes of patients with mainly lymphoma and multiple myeloma after autologous stem cell transplant. The pretransplant workup consisted of the complete blood count, an evaluation of the liver, kidney, lung, and infectious profile, chest radiographs, and a dental review. For lymphoma, all patients who achieved at least a 25% reduction in the disease after salvage therapy were included in the study. Mobilization was done with cyclophosphamide, followed by granulocyte colony-stimulating factor, 300 g twice daily. The conditioning regimens included BEAM (carmustine, etoposide, cytarabine, melphalan) and high-dose melphalan. A total of 206 transplants were performed from April 2004 to December 2014. Of these, 137 were allogeneic transplants and 69 were autologous. Of the patients receiving an autologous transplant, 49 were male and 20 were female. Of the 69 patients, 26 underwent transplantation for Hodgkin\u27s lymphoma, 23 for non-Hodgkin\u27s lymphoma, and 15 for multiple myeloma and 4 and 1 for Ewing\u27s sarcoma and neuroblastoma, respectively. The median age ± SD was 34 ± 13.1 years (range, 4-64). A mean of 4.7 * 10⁸ ± 1.7 mononuclear cells per kilogram were infused. The median time to white blood cell recovery was 18.2 ± 5.34 days. Transplant-related mortality occurred in 10 patients. After a median follow-up period of 104 months, the overall survival rate was 86%. High-dose chemotherapy, followed by autologous stem cell transplant, is an effective treatment option for patients with hematological malignancies, allowing further consolidation of response

Chromosomal abnormalities in primary myelodysplastic syndrome

Objective: To determine the frequency of cytogenetic abnormalities in patients diagnosed as primary myelodysplastic syndrome (MDS) using conventional karyotyping. Study Design: Case series. Place and Duration of Study: The Clinical Laboratory, The Aga Khan University Hospital, Karachi, between January 2006 - June 2012. Methodology: 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. Results: 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%). Conclusion: Trisomy 8 was the most common disorder/abnormality found in this study population followed by the complex cytogenetics

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

Frequency and outcome of graft versus host disease after stem cell transplantation: A Six-Year Experience from a Tertiary Care Center in Pakistan

Objective: The objective of this study was to evaluate the frequency and outcome of graft versus host disease after stem cell transplantation for various haematological disorders in Pakistan. Materials and Methods. Pretransplant workup of the patient and donor was performed. Mobilization was done with G-CSF 300 mu g twice daily for five day. Standard GvHD prophylaxis was done with methotrexate 15mg/m(2) on day +1 followed by 10mg/m(2) on days +3 and +6 and cyclosporine. Grading was done according to the Glucksberg classification. Results. A total of 153 transplants were done from April 2004 to December 2011. Out of these were allogeneic transplants. There were females and males. The overall frequency of any degree of graft versus host disease was 34%. Acute GvHD was present in patients while had chronic GvHD. Grade II GvHD was present in patients while grade III and IV GvHD was seen in patients each. Acute myeloid leukemia and chronic myeloid leukemia were most commonly associated with GvHD. The mortality in acute and chronic GvHD was 8.8% and 12% respectively. Conclusion. The frequency of graft versus host disease in this study was 34% which is lower compared to international literature. The decreased incidence can be attributed to reduced diversity of histocompatibility antigens in our population

Outcome of match related allogeneic stem cell transplantation procedures performed from 2004 till 2011

We present our initial experience of allogeneic stem cell transplant procedure performed between April 2004 and August 2011 for various haematological disorders. All patients with non-malignant and malignant haematological disorders with HLA matched donors were selected after pre-transplant workup. Ninety seven patients underwent the procedure. Most common indications for transplant were aplastic anaemia in n=34 (35%), followed by beta-Thalassemia major in n=21 (21.6%) and chronic myeloid leukemia in n=11 patients (11.3%). Primary graft failure present was present in 2.06%. Incidence of graft versus host disease (GvHD) in our patients was 34%. After median follow-up of five years the overall survival was 71.3% with a mean survival time of 51.2±3.3months

Diagnostic Accuracy of Positron Emission Tomography-Computed Tomography (PET-CT Scan) In Detecting Bone Marrow Involvement in Patients with Diffuse Large B cell Lymphoma

Objective: To evaluate the diagnostic accuracy of positron emission tomography combined with CT scan (PET-CT Scan) in detecting bone marrow involvement in patients with diffuse large B-cell lymphoma, keeping bone marrow biopsy as gold standard. Methodology: From November 2017 to May 2018, a cross sectional validation study was carried out at the Aga Khan University in Karachi Department of Oncology's Section of Clinical Hematology. The study comprised a total of 112 patients who were identified as having diffuse large B cell lymphoma after a lymph node was implicated was histopathologically examined. All patients had a PET-CT scan and bone marrow biopsy technique as part of the staging workup. With bone marrow biopsy acting as the gold standard, the diagnostic efficacy of a PET-CT scan for identifying bone marrow involvement was evaluated. Results: Of 112 patients, there were 71(63.39%) males and 41(36.61%) females. The mean age was 45.09±17.36 years. The mean duration of diagnosis was 17.19±6.02 days. Through biopsy, bone marrow involvement was identified in 40 (35.7%) cases. Through a PET-CT scan, bone marrow involvement was identified in 47 (41.9%) cases. The PET- CT scan in comparison with bone marrow biopsy for detecting bone marrow involvement in patients with DLBCL had a sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy of 95%, 87.7%, 80.85%, 96.92% and 90.18% respectively. Conclusion: PET-CT scan can accurately detect bone marrow involvement in patients with DLBCL so it can be used in most patients instead of invasive bone marrow biopsy procedure for staging of DLBCL patients