فهرست مطالب natasha ali

A-64-year old male presented with cough, weight loss, and maculopapular rash for 15-20 days. On examination, he was found to have cervical lymphadenopathy and splenomegaly. His leukocyte count was 62.1x109/L, platelets were 1169x109/L and LDH was 816 IU/L. Peripheral blood film showed a leukoerythroblastic picture with thrombocytosis. He was started on hydroxyurea and allopurinol. Subsequently, bone marrow evaluation was done which depicted increased lymphoid cells with an M:E ratio of 4:1. Cellular areas exhibited an increase in myeloid precursors along with prominent lymphoid cells and abundant megakaryocytes. Immunohistochemistry showed an increase in B-lymphocytes. Grade MF-2 reticulin fibrosis was noted. Overall findings suggested essential thrombocythemia (ET). On flow cytometry, CD45-positive lymphoid cells population was 31% and showed reactivity to Pan-B-markers with lambda light chain restriction. Janus Kinase (JAK) 2 mutation was detected while BCR-ABL1 translocation was negative. A diagnosis of ET progressing to myelofibrosis and mature B-lymphoproliferative disorder was made. Hydroxyurea and allopurinol were stopped while ruxolitinib was introduced and 2.5 years later he remains stable on this treatment.

Allogeneic hematopoietic stem cell transplantation is a potentially curative treatment modality for hematological malignancies. We evaluated the outcome of patients suffering from hematological malignancies، including acute leukemias، chronic myeloid leukemia and myelodysplastic syndrome after allogeneic transplantation. All patients having hematological malignancies with HLA identical sibling donors who underwent allogeneic transplantation were included. Pre-transplant workup consisted of complete blood counts، evaluation of liver، kidneys، lungs، infectious profile، chest x-ray، paranasal sinus roentgenograms and dental review. Donors were given G-CSF at a dose of 5-10 μg/kg/twice daily for five days prior to harvest. The conditioning regimens included cyclophosphamide، busulfan and total body irradiation. A total of 41 allogeneic transplants were performed for hematological malignancies from April 2004 to December 2012. There were 31 males and 10 females. Median age ± SD was 28 ± 11. 7 years (range 8 – 54 years). A mean of 7. 7x108±1. 5 mononuclear cells/kg were infused (range: 6. 2-9. 2x108/kg). The median time to white cell recovery was 19±4 days (range: 15-23 days). Transplant related mortality was 19. 5%. The median overall survival was 53. 6 months. Overall survival at a median follow up of 37 months was 67%. Allogeneic stem cell transplantation is an effective treatment option in patients with hematological malignancies. Our outcomes are comparable with results from neighboring countries as well as the western world.