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Blood Disorder (Hematologic Disease)

Blood Disorder (Hematologic Disease). Presented by : Jackie Peter D11A012 Khirrol Nizam D11A014 Lee Joy Yoong D11A015. Types of Hematologic Disease. Anaemias (presented by Josephin and Herlina ) Hemoglobinopathies (congenital abnormality of Hb or Hb synthesis)

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Blood Disorder (Hematologic Disease)

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  1. Blood Disorder(Hematologic Disease) Presented by : Jackie Peter D11A012 KhirrolNizam D11A014 Lee Joy Yoong D11A015

  2. Types of Hematologic Disease • Anaemias (presented by Josephin and Herlina) • Hemoglobinopathies (congenital abnormality of Hb or Hb synthesis) • Cytopenia (decrease in no. of blood cells) • Myeloproliferative disease (increase in no. of blood cells) • Coagulopathies (disorders of bleeding and coagulation • Hematological malignancies (cancer that affect blood, bone marrow, or lymph nodes) • Hemochromatocies (hereditary disease that causes Fe overload

  3. Cytopenia and Hemoglobinopathies JACKIE PETERD11A012

  4. Outline • Cytopenia • definition • Types of Cytopenia • Clinical correlations 2.Hemoglobinopaties • Definition, causes, examples • Clinical correlations

  5. 1. CYTOPENIA DEFINITION: Cytopenia is a reduction in the number of blood cells

  6. TYPES OF CYTOPENIA

  7. CLINICAL CORRELATION

  8. 2. HEMOGLOBINOPATHIES • DEF: congenital abnormality of the hemoglobinmolecule or of the rate of hemoglobin synthesis CAUSES • production of abnormal hemoglobin • deficiency of hemoglobin synthesis. MAJOR EXAMPLES • sickle cell disease • thalassemia.

  9. Clinical correlationsHEMOGLOBINOPATHIES • SICKLE CELL ANAEMIA • THALASSEMIA

  10. Sickle cell anaemia

  11. Thalassemia • Thalassemia describes a group of inherited disorders characterized by reduced or absent amounts of hemoglobin, the oxygen-carrying protein inside the red blood cells. • β-thalassemia is an inherited disorder caused by mutations in the β-globin chain of haemoglobin that lead to ineffective production of red blood cells and profound anaemia

  12. Myeloproliferative disorder&Coagulophaty

  13. Myeloproliferative disorders • Agroup of conditions that cause blood cells (platelets, white blood cells, and red blood cells) to grow abnormally in the bone marrow • Increased numbers of cells • Myeloproliferative disorders include: • Polycythemia vera • Essential thrombocytosis • Primary or idiopathic myelofibrosis aka myelosclerosis • Chronic myelogenous leukemia (CML)

  14. Myeloproliferative disorders • Polycythemiavera - occurs when the bone marrow produces too many blood cells, especially red blood cells. More than 95% of people with polycythemiavera carry the blood mutation JAK2V617F. • Essential thrombocytosis - occurs when the body produces too many platelet cells, which help blood to clot. Clots can block blood vessels leading to heart attack or stroke.

  15. Myeloproliferative disorders • Primary or idiopathic myelofibrosis aka myelosclerosis - occurs when the bone marrow produces too much collagen or fibrous tissue in the bone marrow. This reduces bone marrow's ability to produce blood cells • Chronic myelogenous leukemia (CML) - cancer of the bone marrow that produces abnormal granulocytes, a type of white blood cell, in the bone marrow.

  16. Myeloproliferative disorders Causes • overproduction of one or more types of cells • Genetics - some people with CML have an abnormally shortened chromosome known as the Philadelphia chromosome • Environment - may result from an overexposure to radiation, electrical wiring, or chemicals • Cats - commonly associated with Feline Leukemia Virus (FeLV) infection or from recovering from panleukopenia or hemobartonellosis infections

  17. Myeloproliferative disorders Symptoms • Lethargy • Weakness • Anemia • Recurrent infections • Pale mucous membranes • Weight loss • Enlargement of liver and spleen (except essential thrombocytosis)

  18. Myeloproliferative disorders Diagnosis • detect an enlarged spleen during a routine physical examination • Blood tests - to find abnormal types or numbers of red or white blood cells. • detect anemia and leukemia. • Bone marrow biopsy - sample of bone marrow may be taken after blood tests. It can show the presence of abnormal types or numbers of red or white blood cells and may detect certain types of anemia and cancer in the marrow. • Cytogenetic analysis - views blood or bone marrow are viewed under a microscope to look for changes in the chromosomes.

  19. Myeloproliferative disorders Treatment • Polycythemiavera - owerred blood cell count by removing blood, called phlebotomy or treatment with medication, called myelosuppressivetherapy • Essential thrombocytosis - treat symptoms, when present, with medications • Primary myelofibrosis - treat symptoms, when present, with medications and blood transfusion • CML - targeted therapy, chemotherapy, biologic therapy, high-dose chemotherapy with stem cell transplant, donor lymphocyte infusion (DLI), surgery.

  20. Coagulopathy • is a condition in which the blood’s ability to clot is impaired • cause prolonged or excessive bleeding, which may occur spontaneously or following an injury or medical and dental procedures

  21. Coagulopathy Causes • missing or has low levels of one or more of clotting factors • platelet levels are low • Inherited or genetic disorders • Chronic disease, severe infection, and the use of certain medications • Anticoagulant drug use, such as heparin or warfarin (Coumadin) • Disseminated intravascular coagulation - serious disorder in which the proteins that control blood clotting become abnormally active • Idiopathic thrombocytopenic purpura or thrombotic thrombocytopenic purpura (rare conditions in which small blood clots form throughout the body, resulting in low platelet levels) • Long-term use of antibiotics • Severe liver disease • Viral infection • Vitamin K deficiency

  22. Coagulopathy Symptomps • excessive bleeding that may occur spontaneously or following an injury, surgery, or other incident • Abnormal menstrual bleeding • Bleeding of the mouth and gums • Easy bleeding or bruising • Frequent nosebleeds

  23. Coagulopathy Treatments • draw blood samples for laboratory testing • Aminocaproic acid (Amicar), which prevents the breakdown of blood clots and may be used to limit bleeding in the mouth during dental procedures • Birth control (oral contraceptive) pills, which may help control excessive menstrual bleeding in women with coagulopathy • Desmopressin acetate (DDVAP or Stimate), which may be used to release blood-clotting proteins stored in the body in cases of mild to moderate coagulopathy • Plasma-derived factor concentrates and recombinant factor concentrates, which replace missing clotting factors

  24. Hematological Malignancies&Hemachromatocies

  25. Hematological Malignancies • Myelomas • Lymphomas • Plasmacytoma • Leukemias

  26. Myeloma Myeloma • Cancer of plasma cells, where plasma cells collection accumulate at bone marrow, interferes production of RBC. • S & S : Hypocalcaemia (causes renal failure), bone pain at spine and ribs, lesions at bone, anaemia, immune deficiency • Causes : damage to gene causes the loss of control to proliferation of B cells and secretion of plasma

  27. Lymphomas • Cancer of lymphocytes, • S & S : Anorexia, dyspnea, fatigue, fever (due to unknown origin), itching, night sweats, lymphadenopathy • By WHO, its divisible into : • B cell lymphomas • T cell lymphomas • NK cell lymphomas

  28. Plasmacytoma • Malignant plasma cell tumour growing within soft tissues (ST form) or within the skeleton (skeletal form) • S & S : Paraproteinemia (excessive amount of single monoclonal gammaglobulin) Treatment : • Skeletal form : Radiotherapy • Soft tissue form : Resection

  29. Leukaemia's • Type of cancers with abnormal increase of immature white blood cells • S & S : Blood clotting mechanism disturbed, dysfunctional WBC, enlarged spleen and liver, frequent infection, WBC attack other body cells • Divisible to : acute leukaemia and chronic leukaemia, myelogenous and lymphocytic

  30. Definition • Acute Leukemia : rapid increase in the numbers of immature blood cells. • Chronic Leukemia : by the excessive build up of relatively mature, but still abnormal, white blood cells. Takes months or years to progress, the cells are produced at a much higher rate than normal, resulting in many abnormal white blood cells

  31. Lymphocytic L. & Myelogenous • Myelogenous : cancerous change takes place in a type of marrow cell that normally goes on to form red blood cells, some other types of white cells, and platelets • Lymphocytic : cancerous change takes place in a type of marrow cell that normally goes on to form lymphocytes

  32. Hemachromatocies • iron overload with a hereditary/primary cause, or originating from a metabolic disorder • S & S : cirrhosis (chronic liver disease characterized by replacement of liver tissue by fibrosis, scar tissue and regenerative nodules), arthritis, joint and bone pain, testicular failure, tanning of skin, cardiomyopathy

  33. Treatment • Deferoxamine, deferasirox, deferiprone (chelating agent) • Regular bloodletting (blood draws) by phlebotomies

  34. Thank you ^^

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