PPT-Inherited bone marrow failure syndromes

Josu de la Fuente Departments of Paediatrics and Haematology Imperial College London 25 of paediatric and 10 of young adult patient presenting with aplastic anaemia Fanconi Anaemia Median age of presentation 7 years of age. Normal adult Bone marrow in old age Neonatal bone marrow Bone Marrow Cellularity Cellularity Age Site hemopoietic cellsfat Neonate All bones liver spleen 1000 Child Most bones 7030 Adult Axial skeleton 5050 Old age Axial skeleton 3070 brPage 2br Cel By. . SOMESHWAR.K. M.PHARM.. II - SEMESTER . . . Department of Pharmaceutics. University College of Pharmaceutical Sciences,. KAKATIYA UNIVERSITY. and more. skeleton. made up of. bones – cartilage – ligaments – tendons. bones . are a major part of the skeleton and . provide. support. – organs, teeth, viscera. protection. – brain, CNS, marrow, viscera. Brian Boulmay. , . MD. Bone Marrow Failure . Ineffective marrow-poeisis is the final endpoint of many diseases. . Congenital. Acquired . Genetic. Environmental or iatrogenic causes. Congenital marrow failure can present at any age.. Lauren . Cosolo. , RN, BScN, MN. Outline. Review bone marrow failure and disease. Discuss . Myelodysplastic. syndrome, pathophysiology, clinical presentation, diagnosis, treatment. Discuss Aplastic Anemia, . Department of Pathology and Laboratory Medicine. Located 2-3655. Hours of Operation: . 8am-6pm M-F . 8am-5pm Saturday. . Follow a . Bone. Marrow from collection to diagnosis. Laboratory. Testing. : are genetic(AR) disorders in . globin. chain production. . In individuals with β. -thalassemia. , there is either: . complete. absence of β-globin gene production . (β. 0. -thalassemia). Pancytopenia. Pancytopenia. . is a reduction in the blood count of all the major. cell lines – red cells, white cells and platelets. It has several causes. which . can be broadly divided into . decreased bone marrow . Ventricular arrhythmia with structural abnormality. Idiopathic ventricular arrhythmia. Inherited arrhythmia syndromes. Idiopathic Ventricular Arrhythmia. Inherited Arrhythmia Syndromes. Inherited Arrhythmia Syndromes. 59 Procedure Name of the Fellow: Successfully completed a bone marrow biopsy simulation course ? YES NO N/A I. Procedure Preparation: □ List indications . □ Check labs, medication Fibrosis Figure Legend: Figure 1 Dense fibrous tissue surrounded by areas of angiectasis is present in bone marrow in a male F344/N male rat from a chronic study. Figure 2 Higher magnification of Fig - tion (Figure 1). Skeletal aging is characterized as a gradual loss of bone mass due to an excess of bone resorption that is not matched by new bone formation. There are two major types of bone Dr. Ali Khazaal Jumaa. F.I.B.M.S (Internal Medicine). F.I.B.M.S (Clinical Hematology). Gross anatomy and histology. Bone marrow is a jelly-like substance that fills the cavity left by the trabecular network of bone. It accounts for about 4 – 5% of the total body weight of an individual. It is responsible for producing platelets, lymphocytes, erythrocytes, granulocytes and monocytes.. Part 2. Wendy Blount, DVM. Slide Preparation. Pipette flecks out of the petri dish and put on glass slides immediately. Elevate one end of the slide to let extra blood run . off. Slide Preparation. Pipette flecks out of the petri dish and put on glass slides immediately.