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HEMATOLOGY

HEMATOLOGY. Bone marrow function. Hematopoiesis Antibody producing plasma cell differentiation Monitoring hematopoietic cell quality Important key nutrients: iron folic acid

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HEMATOLOGY

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  1. HEMATOLOGY

  2. Bone marrow function • Hematopoiesis • Antibody producing plasma cell differentiation • Monitoring hematopoietic cell quality • Important key nutrients: iron folic acid vitamin B12 regulatory hormones (EPO, CSF-s) interleukins

  3. Signs and symptoms of hematological diseases • Factors • Red blood cells • Leukocytes • Coagulation system • Alterations • Change of the amount • Change in the function

  4. Anemia • Decrease in red cell mass or hemoglobin content of blood below the physiologic need • Not a disease itself! A clinical sign! • Analysis of anaemia • Background mechanisms • loss of red blood cell - bleeding • lack of red blood cell production • excessive red blood cell damage

  5. History • Family history: anemia, splenomegaly, jaundice splenectomy • Bleeding tendency in the family • Diet, alcohol intake • Pregnancy, menorrhagia • Drugs • Chronic diseases • Malnutrition, malabsorption • Transfusion, iron or other therapy against anemia

  6. Anemia • Signs and symptoms vary with the rapidity of onset: • Rapid (bleeding or brisk hemolysis) • cardiovascular compensatory reactions: tachycardia, postural hypotension, vasoconstriction in the skin and extremities, dyspnea on exertion, faintness, even shock • Slowly developing anemias (ie.nutritional deficiency, chr.bleeding, hemolysis etc.) • there is time for compensation • the patient remains asymptomatic for a long time

  7. Anemia • Mild: often asymptomatic • Moderate: symptoms on exertion • Severe: symptoms on rest heart failure

  8. Anemia – non cause-specific symptoms • fatigue • dizziness, vertigo, headache, tinnitus • increased irritability, difficult concentration • sleeping disturbancies • cold intolerance • exertional intolerance: Hb<75g/l : resting heart rate, stroke volume  • dyspnea • complaints of local vascular diseases  (angina pectoris, cerebral ischaemia, intermittent claudication)

  9. Anemia - physical findings Non cause-specific • Pallor of skin and mucous membranes • causes: Hb and blood redistribution from the skin • colors • greyish: malignancy • lemon-like: hemolysis, B12 deficiency • Tachycardia • Hyperkinetic precordium • Systolic murmur (reversible)

  10. Anemia - physical findings Cause-related • Jaundice hemolysis • Hepatosplenomegaly - e.g.hemolysis • Lymphadenopathy - lymphomas, autoimmune diseases • Cheilosis (fissura)iron deficiency • Koilonychia (spoon-shaped nails) iron def. • Beefy red smooth tongue (Hunter-glossitis) - pernicious anemia • Neuropathy - pernicious anemia • Rectal digital examination - bleeding

  11. Polycythemias • Hyperviscosity • Decresed cerebral blood flow tinnitus, lightheadedness, dizziness, stroke • Congestive heart failure • Thrombosis • Increased cell turnover • Gout (due to hyperuricaemia) • Itching • In polycythemia vera • Thrombocytosis • Hemorrhage

  12. Polycythemias • Primary: polycythemia vera myeloproliferative disorder • other cell lines are affected (leukocytosis, thrombocytosis) • hepatosplenomegaly • EPO level: low • Secondary: • hypoxia EPO production • chr. pulmonary diseases • morbid obesitiy (Pickwick’s syndrome) • high altitude • EPO overproduction: tumors

  13. Leukocyte disorders • Granulocytes (neutrophil, eosinophil basophil) • Monocytes and tissue macrophages • Lymphocytes

  14. Functions of leukocytes • Maintaining host defences against disease • Killing microorganisms • Digestion of tissue debris • Releasing cytokines and mediators • Activation of the immune system

  15. Leukocyte diseases • Benign • Leukocytosis > 10 G/L • Leukocytopenia < 4 G/L • Agranulocytosis absolute granulocyte count < 1 G/L • Malignant • Leukemias • Lymphomas

  16. Acute leukemias • Agressive immature hemopoietic cell proliferation, without differentiation (hiatus leucemicus in the blood smear) • Subtypes: • ALL (acute lymphoblastic leukemia) • ANLL (acute non lymphoblastic leukemia) or AML (acute myeloblastic leukemia)

  17. Acute leukemia syndrome • Susceptibility to infections - serious infections • Anemia due to bone marrow infiltration and bleeding • Thrombocytopenia - bleeding tendency purpuras, petechiae, mucosal bleeding • Organ infiltration • In ALL: lymphadenopathy, splenomegaly

  18. Chronic leukemias • Uncontrolled expansion of premature hemopoietic cells which are able to differentiate • Subtypes: • Chronic myelogenous leukemia (CML or CGL) a myeloproliferative disorder • Chronic lymphocytic leukemia (CLL) a malignant lymphoma

  19. Diseases of the lymphoid system • Normal lymph nodes • Non palpable or <1 cm, except in the inguinal region where can be 0,5-2 cm sized lymph nodes normally • Small lymph nodes can be remain after infection • Significant: one or more new nodes >1 cm (which can’t explain by a previously recognised cause) • New lymph nodes in older age more significant • children more likely respond with lymphoid hyperplasia • lymphyadenopathy under 30 years: 80% beningn over 50 years: 40% beningn

  20. Lymph node characteristics • Location • Number (single, multiple, matted together) • Size • Tenderness • Consistency (hard, rubbery, soft) • Mobility • Skin reactions above the lymph node

  21. Malignant lymphomas • Arise in lymph nodes or extranodal lymphoid tissue • Subtypes: • Hodgkin’s disease • Non Hodgkin’s lymphomas

  22. Hodgkin’s disease • Mostly young adults • Asymptomatic lymphadenopathy • often cervical • sometimes mediastinal or abdominal • Hepatomegaly, splenomegaly • Fever, night sweats, loss of weight • Diagnosis: lymph node histology (Reed-Sternberg cells) Staging

  23. Non Hodgkin’s lymphomas • Older patients • More diffuse lymph node involvement • General symptoms:fever, weight loss, night sweat • Frequent extralymphatic involvement (50%: bone marrowanemia, tct-penia) • Special forms: CLL Skin infiltration (T-cells) MALT-lymphoma Multiple myeloma

  24. Coagulation disordersBleeding tendency, thrombosis, embolism Factors • platelets (number, function) • coagulation factors (amount, function) • surface of the blood vessels, circulation

  25. Bleeding - history • History of common hemostatic stresses: • gum extraction, minor surgeries • menstruation, childbirth • injuries • Family history of bleeding tendency

  26. Bleeding • Primary hemostatic defect (platelets) • occurs immediately after trauma • from superficial sites (skin, mucous membranes, nose, rarely from gastrointestinal, genitourinary tract) • purpuras, petechiae • ecchymoses • bleeding time • Secondary hemostatic defect (coagulation) • delayed occurence (hours, days) • from deep sites (joints, subcutaneous tissues, muscles, retroperitoneum, body cavities, cerebrum) • hematomas • hemarthroses • coagulation time 

  27. Bleeding - physical findings • From capillaries: purpuras, petechiae small, superficial,dermal or mucosal pinpoint hemorrhages characteristic for platelet disorders • From small arterioles and venules: • ecchymoses subcutaneous blood collections- bruises • hematomas deeper, palpable • in platelet and coagulation disorders • Hemarthros - bleeding into joints characteristic for coagulation disorders (mostly hemophilia) leads to chronic joint deformity

  28. Thrombosis and embolism • Virchow’s triad • Hypercoagulability • Circulation disturbance- congestion • Pathological endothelial surface

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