Fludarabine induced immune thrombocytopenia in a patient with CD5 positive B cell chronic lymphocytic leukemia

Fludarabine is a purine nucleoside analogue, which inhibits DNA synthesis by inhibiting DNA polymerase and ribonucleoside reductase.1 It affects both dividing and non- dividing cells.2 Fludarabine possesses proven efficacy in the treatment of a variety of indolent B cell lymphoproliferative disorders including chronic lymphocytic leukemia3, low-grade non-Hodgkin\u27s lymphoma4 and Waldenstrom macroglobulinemia.5 It is also a part of conditioning regimes in non-myeloablative bone marrow transplantation.6 The common side effects include myelosuppression, immunosuppression, and neurologic toxicity.7 The rare side effects are immune mediated hemolytic anemia8 and thrombocytopenia.9 Here we describe a case of a middle-aged lady who was diagnosed as B cell chronic lymphocytic leukemia and developed immune mediated thrombocytopenia following oral Fludarabine

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

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

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

Anaphylactic reaction after intramuscular injection of cyanocobalamin (vitamin B12): a case report

We report a case of anaphylactic reaction to intramuscular injection of cyanocobalamin. This 52-year-old lady was diagnosed as a case of megaloblastic anemia secondary to dietary vitamin B12 deficiency. She had severe anaphylactic reaction after the parenteral administration of cyanocobalamine. Later she received oral vitamin B12 with no adverse effects. The purpose of this case report is to draw attention to the hypersensitive reaction to injectable vitamin B12, which is rarely seen. This could be due to sensitization to the vitamin B12 molecule itself or an IgE mediated reaction. We concluded that anaphylactic reaction to vitamin B12 is a rare but serious side effect and it should be kept in mind while the drug is being administered to the patient, especially via the parenteral route

Clinico-pathologic features of chronic myeloid leukemia and risk stratification according to sokal score

To compile the clinical and haematological parameters of chronic myeloid leukemia (CML) and risk stratification according to Sokal score in our population. A descriptive analysis. The Aga Khan University Hospital, during the period from August 1997 to August 2005. All patients with diagnosis of chronic myeloid leukemia treated as outpatient in haematology clinic, or admitted in haem-oncology wards in The Aga Khan University Hospital were included. Records were retrospectively analyzed for clinicopathologic features. Risk groups were assigned as per Sokal scoring. During the 8 years study period, 245 patients with chronic myeloid leukemia were analyzed, the median age of presentation was 35 years (range 11-73); with male to female ratio 1.69:1. At the time of diagnosis, 178 patients (72.6%), 48 (19.7%) and 17 (7.8%) of patients were in chronic, accelerated and blast phase of the disease respectively. Abdominal fullness was the frequent clinical presentation followed by fever. Laboratory parameters revealed mean hemoglobin 10.0 g/dl (range 4.6-15), mean total leukocyte count 168 x 10(9)/L (35-959) and mean platelets 408 x 10(9)/L (range 30-2335). The mean size of spleen at the time of presentation was 9.2 cm below the left subcostal margin. A large number of patients (46.2%) were in high risk group according to Sokal score i.e. \u3e 1.2. CML occurred more commonly at younger age in this series. Most of these patients were in high risk group according to Sokal scor

Corrosion mechanisms of 304L NAG in boiling 9M HNO3 containing Cr (VI) ions

In this research, the mechanisms of end-grain corrosion of 304L NAG tubes in boiling 9M HNO3-containing Cr (VI) ions are reported to sustainably manage the corrosion of nuclear fuel reprocessing plant components. Specific heat treatments were applied to as-received specimens to produce phosphorus and/or sulphur intergranular segregation. End-grain corrosion on heat-treated specimens and the effect of a Cr (VI) concentration on a 304L NAG tube (as-received) were investigated. It has been reported that an increase in Cr (VI) ions leads to the acceleration of end-grain corrosion due to high electrochemical potential. After systematic heat treatments on the 304L NAG specimens, it is concluded that the primary causes of heat-induced end-grain corrosion are phosphorus or sulphur segregation to the grain boundaries. The key findings of this research are highly significant in terms of understanding the corrosion mechanisms and controlling the end-grain corrosion of NAG steel in boiling HNO3 environments. This research will help to sustainably reduce power plant maintenance costs and will have a significant impact on the delivery of long-term, clean, secure, and tenable energy

Hairy cell leukemia: clinical presentation and long term follow up after treatment with 2-chlorodeoxyadenosine (2-CdA)

The aim of the study was to the clinical features and long term follow up after treatment with Cladarbine in a tertiary care hospital. Seven patients with hairy cell leukemia were diagnosed between January 1990 till December 2003. Diagnosis in all the patients was established by bone marrow aspirates and trephine biopsy along with TRAP. In two patients the diagnosis was supplemented by flowcytometry and in another two patients by splenectomy. Six patients were male while one was female. Mean age was 47.7 years (range 36-64). Most common presenting features were pallor and weakness (n=5). All patients had splenomegaly. Blood count at presentation revealed that one patient had bicytopenia, two had isolated thrombocytopenia, and three had pancytopenia. Treatment responses were evaluable in seven patients. Complete response was seen in six patients (85.7%). One patient died after two months due to sepsis while 3 (50%) patients relapsed. Those who relapsed received another course of CDA and have maintained remission with a median duration of response of 48 months (20-48). From this small series we can conclude that CDA is an effective treatment for HCL and even it works very well in relapsed cases

Diagnostic tool for Glanzmann\u27s thrombasthenia clinicopathologic spectrum

OBJECTIVE: To platelet aggregometry and describe the clinical spectrum of Glanzmann\u27s thrombasthenia diagnosed by platelet aggregometry. STUDY DESIGN: A case-series. PLACE AND DURATION OF STUDY: This study was carried out at the clinical laboratories at the Aga Khan University Hospital, Karachi from January 2003 to January 2006. PATIENTS AND METHODS: All patients irrespective of age and gender presenting with bleeding symptoms and having normal platelet count were evaluated. Demographic details, relevant clinical history along with results of complete blood count, bleeding time and platelet aggregation studies were retrieved through computerized data base and evaluated for the diagnosis of Glanzmann\u27s thrombasthenia. RESULTS: During the study period, 50 out of 2317 patients (2.2%) were diagnosed as Glanzmann\u27s thrombasthenia by platelet aggregometry with male to female ratio of 0.85:1 and median age of 10.2 years (ranging from 3 months to 27 years). Common symptoms were epistaxis, oral and gingival bleed, bleeding from minor cuts and trauma that were observed in 46% of the patients; while 18%, 8% and 10% of them also complained of bruising, hematuria and bleeding per rectum respectively. Majority i.e. 86% had a bleeding time greater than 10 minutes. All patients had received blood or blood products for their bleeding episodes. CONCLUSION: Platelet aggregometry is a useful diagnostic modality for the assessment of Glanzmann\u27s thrombasthenia. The disorder presents with muco-cutaneous bleeding and was found to be a common cause of bleeding in our setup