lenalidomide induced toxic epidermal necrolysis and del [5q]: co-occurrence of rarities

Primary myelofibrosis [PMF] is a clonal, BCR-ABL1 negative myeloproliferative neoplasm characterised by splenomegaly, leukoerythroblastic peripheral blood picture and bone marrow fibrosis. Different cytogentic abnormalities are documented in PMF which have impact on clinical outcome and overall survival. Del 5q31 is documented in only 0.8% of PMF patients and is associated with poor outcome and increased risk of progression to acute leukemia. Anemia with del 5q responds frequently to lenalidomide treatment. We are reporting case of a middle-aged male who presented with constitutional symptoms, myelofibrosis; and calreticulin type 2 mutation was present. His cytogenetics showed del 5q positivity. He was started on lenalidomide but developed toxic epidermal necrolysis, resultantly lenalidomide was stopped. Skin eruptions are a known entity in patients with lenalidomide therapy; but to date, there is no reported case of lenalidomide induced toxic epidermal necrolysis [TEN] in patients with myelofibrosis

Outcome of allogeneic haematopoietic stem cell transplantation in aplastic Anaemia

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. Analytical study. Armed Forces Bone Marrow Transplant Centre, Rawalpindi, Pakistan from July 2001 to June 2010. 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. 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. 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

Improvement in the pattern of advice of investigations following rationalization campaign

To assess the magnitude of change in advising pattern of laboratory and radiological investigations during and after campaign emphasizing rational use of these tests. Comparative cross sectional study. Combined Military Hospital Peshawar, Twenty seven months. The laboratory investigation analysis were Blood Glucose, Thyroid Function Tests, Liver Function Tests [LFT], Renal Function Tests [RFT] and Cardiac Markers and radiological tests were X-Rays Chest, Skull, Wrist and Plain Abdomen, Obstetric Ultra Sound [USG] and CT Scan. The awareness was done by Lectures, handouts, feedback advice on test reports and personal communications to the Medical Officers, Nursing Officers and Paramedical Staff. The data was analyzed through Quarterly Returns, records of laboratory and radiology retrieved from Hospital Information System and departmental registers. In some laboratory investigations there was a significant improvement in the advisory pattern e.g. blood glucose fasting instead of random, ALT instead of whole LFT, electrolytes not included in every RFT and abolishing of AST and LDH from cardiac marker profile and TSH only as screening test. In Radiology there was improvement in the rationality of X-Ray chest, X-Ray skull, X-Rays wrist and obstetric USG. CT scan data could not be compared because CT Scan equipment became out of order during the study. There was also reduction in expenses incurred on irrational investigations. A dynamic ongoing process of awareness and realization campaign for medical professionals is required in any public sector medical institute for cultivating a culture of rational use of investigations

Clinico-epidemiological pattern of cutaneous leishmaniasis in armed forces personnel fighting war against terrorism in Khyber Pakhtunkhwa Province and FATA regions

Cutaneous leishmaniasis [CL] is endemic in various regions of Khyber Pakhtunkhwa [KPK] province and Federally Administered Areas [FATA]. Troops deployed in these regions are at an increased risk of acquiring the disease as compared to the native population. To determine clinical and epidemiological pattern of CL in armed forces personnel serving in endemic areas of CL in KPK and FATA. This observational/descriptive study was conducted at CMH, Peshawar from January, 2010 to June, 2010. All patients of any age reporting in skin outdoor with clinical diagnosis of CL were enrolled in the study and all were subjected to skin slit smears for Leishman-Donovan [LD] bodies and skin biopsies were also taken in all cases to observe histopathological features. Patients in whom clinical diagnosis was not supported by laboratory diagnosis, were excluded. Clinical and epidemiological data was recorded and finally analyzed by using descriptive statistics. Out of 172 initially enrolled cases, 2 were excluded from the study as their clinical diagnosis was not supported by laboratory findings. All patients were young males [deployed armed forces personnel]. Their ages ranged from 18 to 43 [mean age: 27.4 years]. Number of lesions ranged from 1-11. Multiple lesions were seen in 41.2% cases only. Size of lesions ranged from 1-13 cm. 70.6% of lesions were seen on upper and lower limbs followed by head and neck region 27.6%, and trunk and abdomen 1.8%. Morphological patterns seen were crusted plaques, psoriasiform plaques, nonhealing ulcers, erythematous infiltrated nodules and papules. Majority of the cases were from the regular army units deployed in the areas in recent past [71.8%] as compared to 28.2% of native troops of Frontier Corps [FC]: 3% deployment of regular troops against 0.5% of FC troops. CL is common in soldiers serving in KPK province and FATA regions of Pakistan. Armed forces personnel who moved from central Punjab and deployed in these areas for operations against terrorists are at much higher risk of acquiring the disease as compared to the native troops. This risk can be decreased by implementing effective precautionary measures and education of the soldiers

Pattern of injuries inflicted on troops fighting, on the western front

To determine the pattern of fatal and non fatal injuries in soldiers and officers during the present war on Western front. Descriptive study with partly retrospective data collection. The study was carried out at Combined Military Hospital [CMH] Peshawar, the tertiary care centre for Pakistan Armed Forces serving FATA and NWFP from March 2004 to May 2009. Data of non-fatal injuries was collected by noting down the wounds inflicted on injured soldiers and officers evacuated from forward areas while the record of non-fatal injuries was noted from the hospital papers prepared for each patient. Due to reasons of confidentiality the analyses is based on percentages only, while actual figures can be provided by the authors on appropriate security cleared requests. The Lethality Index [LI] of wounds, calculated by dividing the fatal injuries by the total injuries, was 18% during these six years. Out of the total fatal injuries in all six years highest number occurred in 2008 [40%] while LI was highest in 2005 [25%]. Only a small number of patients [1.86%], who were evacuated alive, died in the hospital. Fifty one percent soldiers received multiple [>two] fatal injuries. Head [46%] and Chest [44%] were the commonest sites of fatal injuries while limbs were the commonest sites of non-fatal injuries. Gun shot wounds were the commonest [68%] mode of fatal and non-fatal injuries. Head and chest injuries are the commonest sites of fatal injuries, while limbs injuries constituted the major portion of the non-fatal injuries indicating potential areas in need of improved protective

EDTA contamination in laboratory specimens-effect of an awareness campaign

To measure the effects of awareness campaign on the frequency of EDTA contamination. A cohort study. The study was carried out in Combined Military Hospital, Peshawar, from October to December 2008. A cohort of 200 doctors, nurses and paramedical staff involved in sample collection for laboratory investigations were selected for this three phased study. In all samples with plasma potassium level>6.0 mmol/L, plasma Ca++ was measured for one month before, during and after a campaign of correct filling order of sample tubes for two weeks. Renal function test and LDH were also measured to rule out chronic renal failure and in-vitro haemolysis, respectively. Fisher's exact test was used for comparison of frequency of hyperkalemia. There was a significant decrease [75%, p<0.01] in the cases of spurious hyperkalaemia and hypocalcaemia. EDTA contamination is a common and important source of pre-analytical error which can be prevented to some extent by education of medical and nursing staff