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Infective endocarditis is rarely caused by Burkholderia cepacia. Pseudomonas putidahas not been reported to cause infective endocarditis so far. This is the first case of infective endocarditis being reported, that is caused by Pseudomonas putidaand Burkholderia cepaciain an immunocompetent host with no predisposing factors. Aortic valve replacement surgery was carried out and antibiotics were given, to which the patient responded well and recovered.

The aim of this study was to determine the frequency of various clinico-haematological features in patients suffering from paroxysmal nocturnal haemoglobinuria (PNH). It was an observational study carried out from October 2008 - January 2016. All the patients of PNH, diagnosed on the basis of clinical and laboratory findings and confirmed by CD55 and CD59 deficiency on red cells by means of flow cytometry, were included in the study. A total of 22 patients were diagnosed which included 18 (81.8%) males and 4 (18.1%) females. Median age was 27 years. Pallor, fever, fatigability and haemoglobinuria were the most common clinical features. Pancytopenia was seen in 13 (59.09%) and hypocellular marrow was found in 14 (63.6%) patients. One patient presented with Budd Chiari syndrome.

Objective: To analyze factors associated with survival, rejection and graft versus host disease in aplastic anaemia patients undergoing allogeneic haematopoietic stem cell transplantation (SCT) from HLA matched sibling donors., Study Design: Analytical study., Place and Duration of Study: Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan from July 2001 to June 2010., Methodology: Consecutive aplastic anaemia (AA) patients undergoing haematopoietic stem cell transplantation from HLA-matched sibling donors at this centre were included in this study. Potential factors affecting overall survival, rejection, disease-free survival and graft versus host disease were analyzed. Survival analysis was done by Kaplan-Meier method. Cox regression model was applied for multivariate analysis., Results: Ninety male and thirty-five female patients with AA were included in the study. Median age was 18 years. Conditioning regimens used were cyclophosphamide (Cy) plus antilymphocyte globulin (ALG) or antithymocyte globulin (ATG), fludarabine (FLU) +Cy+ATG, Campath 1-H +Cy in 89, 30 and 6 cases respectively. GVHD prophylaxis used was ciclosporin (CSA) plus prednisolone and short methotrexate in 81 while 44 received CSA plus prednisolone. At a median follow-up of 1185 days OS and DFS were 84% and 78% respectively. Factors associated with better OS were male sex, Flu/Cy/ATG conditioning and use of bone marrow as stem cell source., Conclusion: Flu/Cy/ATG conditioning regimen, bone marrow as stem cell source and CSA, prednisolone and short methotrexate regimen were associated with better survival in AA.

Neutropenic fever is an important cause of morbidity and mortality during therapy of acute myeloid leukemia. Patients with acute myeloid leukemia are at increased risk of acquiring vancomycin resistant enterococcal infection and its treatment remains problematic. Vancomycin therapy for more than 7 days is usually associated with inducible vancomycin-resistant enterococcal infections. We report a case of vancomycin resistant enterococcal sepsis in a patient of acute myeloid leukemia.

A 42 years old male with relapsed diffuse large B-cell lymphoma was given second-line chemotherapy followed by reduced intensity allogeneic stem cell transplantation from HLA matched brother. Twelve weeks posttransplant, his disease relapsed evidenced by the appearance of lymphoma cells in the peripheral blood and declining donor chimerism. Donor lymphocyte infusion was given that induced complete lymphoma remission. The patient is well 3 years posttransplant with his disease in complete remission.

Objective: To determine post-transplant survival in chronic myeloid leukaemia patients undergoing allogeneic stem cell transplant., Study Design: Longitudinal, descriptive study., Place and Duration of Study: Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan, between April 2002 and August 2007., Methodology: All patients of chronic myeloid leukaemia in chronic phase having HLA identical donor and age under 55 years, normal hepatic, renal and cardiac functions with good performance status were selected. Patients in accelerated phase or blast crisis, poor performance status, impaired hepatic, renal, cardiac functions or pregnancy were excluded. Survival was calculated from the date of transplant to death or last follow-up according to Kaplan-Meier and Cox (proportional hazard) regression analysis methods., Results: Thirty seven patients with chronic myeloid leukaemia underwent allogeneic stem cell transplant from HLA identical sibling donors. Thirty two patients were male and five were females. Median age of patients was 28 years. All patients and donors were CMV positive. Post-transplant complications encountered were acute GvHD (Grade II-IV) (n=13, 35.1%), chronic GvHD in 18.9% (n=7), Veno Occlusive Disease (VOD) in 5.4% (n=2), acute renal failure in 2.7% (n=1), haemorrhagic cystitis in 2.7% (n=1), bacterial infections in 40.5% (n=15), fungal infections in 16.2% (n=6), CMV infection in 5.4% (n=2), tuberculosis in 5.4% (n=2), Herpes Zoster infection 2.7% (n=1) and relapse in 2.7% (n=1). Mortality was observed in 27% (n=10). Major causes of mortality were GvHD, VOD, septicemia, CMV infection and disseminated Aspergillosis. Overall Disease Free Survival (DFS) was 73% with a median duration of follow-up of 47.4 +/-12 months. DFS was 81% in standard risk and 54.5% in high-risk group., Conclusion: Results of allogeneic stem cell transplant in standard risk group CML patients were good and comparable with other international centres, however, results in high-risk CML patients need further improvement, although, number of patients in this group is small.

Objective: To compare survival in Acute Promyelocytic Leukemia (APL) patients treated with or without All-Trans Retinoic Acid (ATRA)., Study Design: Longitudinal, comparative study., Place and Duration of Study: The Armed Forces Bone Marrow Transplant Centre (AFBMTC), Rawalpindi, Pakistan from May 2001 to April 2007., Methodology: All consecutive newly diagnosed patients of acute promyelocytic leukemia, treated at Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan, between May 2001 and April 2007, were included and given chemotherapy according to availability of ATRA. Diagnosis was confirmed on morphology/ karyotyping/ molecular analysis. Eligibility criteria included confirmed morphologic diagnosis and/or by demonstration of t(15;17) and/or PML/RAR proportional to re-arrangement, no prior chemotherapy, normal hepatic and renal function, Eastern Cooperative Oncology Group (ECOG) performance status of 0 - 2 and no contraindications to ATRA (history of sensitivity to Vit. A or other retinoids). All patients having history of cardiac failure (LVEF < 50) and arrhythmias, ECOG performance status 3 and 4, relapse / refractory disease, ALT twice normal values, serum creatinine > 150 micromol/L and pregnancy were excluded from this study. Survival was calculated from the date of chemotherapy to death or last follow-up according to Kaplan-Meier and Cox (Proportional hazard) regression analysis methods., Results: During the 6 years study period, 31 newly diagnosed patients with acute promyelocytic leukemia received treatment at AFBMTC. Seventeen patients received anthracycline-based remission induction and consolidation chemotherapy, while 14 received ATRA-based remission induction, consolidation and by two years maintenance therapy. Overall Survival (OS), Disease Free Survival (DFS) and mortality were 29.4%, 29.4% and 70.6% respectively in 17 patients who received anthracycline based chemotherapy, whereas in patients who received ATRA-based chemotherapy OS, DFS and mortality was 71.4%, 64.2% and 28.6% respectively. Major causes of mortality were septicemia and chemotherapy related toxicity., Conclusion: Response to ATRA-based chemotherapy in patient cohort was better as compared with anthracycline based chemotherapy (71.4% vs. 29.4%) in terms of survival and mortality.

Aplastic anaemia is characterized by severe compromise of haematopoiesis and hypocellular bone marrow. Haemorrhagic episodes in patients with aplastic anemia occur usually secondary to thrombocytopenia and require frequent support with platelet concentrates and other blood products. Infection with dengue virus (particularly dengue sero type-2 of South Asian genotype) is associated with dengue haemorrhagic fever. Dengue infection further worsens the disease process in patients with aplastic anaemia due to uncontrolled haemorrhagic diathesis and major organ failure, which may prove fatal in these already immunocompromised patients, if not treated in time. Recent epidemics of dengue haemorrhagic fever has not only affected the southern region of our country but also spread to other areas of the country. With this background, we report a case of aplastic anaemia complicated by dengue haemorrhagic fever who achieved successful engraftment after allogeneic stem cell transplantation from sibling brother and is having normal healthy post transplant life.

This case report describes a patient with severe aplastic anaemia, who developed Guillain Barre Syndrome (GBS) 10 weeks after allogeneic haematopoietic stem cell transplantation (HSCT) from HLA-matched sibling-younger sister. GBS was preceded by pneumonia, herpes labialis and oral candidiasis a week earlier. Treatment with ventilatory management, intravenous human immunoglobulin (IVIg) and antimicrobials resulted in smooth recovery in thirty-one days.

Objective: To assess magnitude of tuberculosis (TB) in patients suffering from various haematological malignancies and stem cell transplant (SCT) recipients., Design: Descriptive study., Place and Duration of Study: Oncology Department, Combined Military Hospital, Rawalpindi, and Armed Forces Bone Marrow Transplant Centre, Rawalpindi, from July 2001 to December 2002., Patients and Methods: Patients suffering from various haematological malignancies treated between July 2001 and December 2002 were included in the study. The hospital records and out-patient follow-up charts were reviewed for demographic information, diagnosis, clinical presentation, laboratory investigations, radiological and pathological examinations, sites involved in TB, methods of diagnosis, number and type of anti-tuberculosis drugs given and response to treatment., Results: During the study period a total of 213 (including 25 allogeneic stem cell transplant (SCT) recipients) patients with different haematological disorders were treated. Out of these, 34, including 4 SCT recipients developed tuberculosis. Overall frequency of TB was 16 %. Median age of TB patients was 33.5 years (range 8-80 years). Median time between diagnosis of haematological disorders and tuberculosis was 21 weeks. Sites of involvement by TB were lung (18), disseminated (6), lymph node (5), pleura (2), spine (2) and pericardium (1). Three of the patients died of TB; one undiagnosed, second with multi-drug resistant TB and the third soon after the start of anti-tuberculosis treatment while remaining 31 cases responded to anti-tuberculosis treatment., Conclusion: Tuberculosis is a major problem in immunocompromised patients and there is need to establish guidelines for TB chemoprophylaxis in our setup.

Objective: To evaluate and compare the response between food supplemented with iron in powdered and iron in syrup forms for the treatment of iron deficiency anemia in children aged 1-5 years., Design: Quasi-experimental study., Place and Duration of Study: This study was conducted at Combined Military Hospital, Multan, from January 2000 to December 2000., Patients and Methods: Consecutive 200 cases of iron deficiency anemia, aged 1-5 years, were included in the study. Diagnosis was based on history, physical examination, complete blood counts (CBC) and serum ferritin levels. The patients were randomized to receive either iron in syrup form (Group A) or equivalent doses of iron powder sprinkled over food (Group B). The patients were followed up with hemoglobin estimation (Hb) and reticulocyte response at 2 weeks, 4 weeks and 6 weeks. CBC and serum ferritin were repeated at 6 weeks., Results: Over half (51%) of the patients were between 1-2 years of age. One hundred thirty-two were males and 68 females. Most of the patients belonged to poor socioeconomic class. The iron in powder form was better tolerated than iron syrup as this group witnessed fewer episodes of gastrointestinal disturbances. The rise in mean Hb level after 6 weeks of treatment in group A and B was 1.6 g/dl and 1.9 g/dl respectively. Hemoglobin rise in group B was more than group A but this was statistically non-significant (p > 0.05). There was small but significant (p < 0.05) rise in serum ferritin in both the groups. There was no significant difference between the two groups for response to the two forms of iron administration., Conclusion: The powdered form of iron is a cost-effective and better tolerated method of iron administration in children and can be considered as an alternate option for the treatment of iron deficiency anemia in children.

The majority of stem cell recipients rely on indwelling central venous catheters situated in superior vena cava or right atrium. Semi-permanent tunneled silicone rubber Hickman catheters are widely used to provide durable central venous access for patients undergoing stem cell transplantation. A case of 5 years old child with diagnosis of severe aplastic anemia is reported. The patient received peripheral blood stem cells (PBSC) and had successful engraftment with complete hematological recovery. He had Hickman catheter embolism in the pulmonary circulation following unsuccessful attempt to remove the line. The catheter was successfully removed by midsternostomy operation. The child is normal with sustained remission on day +218 post stem cell transplant.