HEMATOLOGICAL DISORDER describes the blood disorders

It's all about blood disorders

1. Hematological disorder By Kamran javed & Shahzad riaz

2. objectives Anemia Sickle cell anemia Thalasemia Iron deficiency anemia Hemophilia Von willebrand disease

3. anemia Definition; “Decease in the HB level by decrease production or by increase bleeding or destruction” Levels of HB; Male= 13.5-------16.5g/dl female=12.5------15g/dl Child=14-----------19g/dl New born=15-----20g/dl

4. Symptoms Shortness of breath particularly on exercise Weakness Skin (pallor) Palpitation Headache Angina pectoris Cardiac failure Fatigue Confusion Visual disturbances due to retinal haemorrhages

5. Body compensatory mechanisms Impaired supply of O2 to following organs then; Heart( increase sympathetic activity increase C.O) Muscle, Skin ( pallor in color) Kidney ( erythropoietin release) Brain (increase sympathetic activity) etc

6. Consideration in anesthesia Pre-oxygenate the patient with 100% oxygen Minimize drugs which decrees C.O Factors leading to leftward shift of Oxy- Hb dissociation curve hyperventilation causes hypocapnia , metabolic alkalosis which leads to Oxy- Hb dissociation curve.

7. Consideration in anesthesia Monitoring should be aimed at assessing the adequacy of perfusion and oxygenation of vital organ Use that type of anestethetics which have lower O2 consumption

8. clinical treatment Iron supplement Erythropoietin Vit -b 12 Blood transfusion

9. Blood transfusion criteria Hb = 6g/dl Chronic anemia Hb =6 -10g/dl Major cardiac surgies Hb <8g/dl Excess blood loss upto 30-40% 30% blood loss required crystalloid solution 15% blood loss don’t required

10. Types of anemia Self study?

11. Sickle cell anemia Definition; “decrease in Hb due to change in RCS shape due to mutation in DNA sequence” Genetic disease cause by the disturbance in the sequence of gene. Sickle cell patient don’t get malaria Types of SCA; 1.Sickle cell anemia 2.Scikle cell Trail

12. Hb genetics Our Hb contain these chains;(normal) Hgb A ( α2,β2) 98% Hgb A2 ( α2,δ2) 1.5- 3.2% Hgb F ( α2,γ2) <1% I n sickle cell ; (abnormal) 1.Sickle cell ( HbS 95% & HbA 5%) 2.Sickle cell trail( HbS 30% & HbA 70%)

13. pathophysioogy Hemoglobin A; in which at chromosome no 6 “GULTAMIC ACID” is present. Hemoglobin S; in which at chromosome no 6 “VALINE” is present HbS is formed by the change in the sequence of amine acid at chromosome no 6 from HbA to HbS . HbS is unstable and cause oxidative damage & endothelial injury

14. pathophysiology Due to the presence of HbS in the RBC the chains make PROTUSION like shape of the cell and many chains are alined up and make another long chain in the cell and the structure are called FIBROUS CHAIN These fibers twist and turn and get right and finally take the shape wich is know as SICKLE CELL

15. symptoms Shortness of breath (pulmonary hypertension) Lung and heart injury Fatigue Chronic Renal failure Palpitation Leg ulcer Headache Mostly anemic signs and symptoms

16. syptoms Vaso -occlusive crisis( thrombos ) Cerebral infarction MI Splenic sequestration crisis. Hemolytic crisis

17. Consideration of anesthesia Preoperative; Hb should be well maintained Patient should be well hydrated Infection should be control Major surgeries are avoided

18. Consideration of anesthesia Intra operative; Avoid hyperthermia and hypothermia bcz its lead oxydissociation cure to leftward Use that type of anesthetics which have decrease O2 consumption Acidosis Hypotension and hypovolumia Even mild degree of hyperthermia will lead the patient to hypoxia Promote mild alkalosis bcz it prevent scikilng of the RBCs.

19. Consideration of anesthesia Post operative; Same as intra operative But remember one thing that this type of patients would not survive prolong in post operative …they have high chance of mortality

20. diagnosis Sickle cell test CBC HB electrophoresis DNA analysis

21. Thalasemia Definition; “ it is an inherited disorder in which decrease production of Hb due to decrease production of globulin chains “ Causes; Mutation Heredity

22. Types of thalasemia Alpha Thalasemia (alpha gene defected); one mutated gene two mutated genes three mutated genes four mutated genes Beta Thalasemia (beta gene defected); One gene mutated B- thalacemia intermediate two genes mutated

23. Signs Fatigue Weakness Pale or yellowish skin Facial bone deformities Slow growth Abdominal swelling Dark urine

24. anesthesia considerations Following drugs are avoid bcz they hemolysis or some of them cause inhibition of erythroposis ; Prilocain Nitropruside Penicillin Aspirne Vit -k

25. anesthesia considerations Laryngeal procedure(ETT) would difficult bcz of its facial bone deformalities Aspiration risk is high bcz of acities Drugs should be chosen careful bcz patient would be underweight Spleenomegaly Thinning of cortical bone → potentially difficult regional secondary to vertebral destruction Decrease dose of muscle relaxant bcz of its muscle strength

26. diagnosis CBC Smear test Hb electrophoresis Marrow test Iron, follic acid and billurobin would be high in this patient

27. Iron deficiency anemia Definition; “ nutritional deficiency of iron cause anemia” It is microcytic-hypocromic Chronic blood loss also lead to iron deficiency anemia bcz from diet only 10% of iron is absorbed from the Gut

28. Pathophysiology this disease consist of 3 stages 1 st stage; (iron deplition ) Only stored iron is enough Anemia is not occur Only ferritin level is decreases

29. Patho [ hysiology 2 nd stage; ( iron deficient erythroposis ) Stored iron is get clear Serum iron deficient In effected erythropoisis (new RBCs are microcytic slightly) Still anemia would not occurred

30. pathophysiology 3 rd stage; (iron deficiency anemia) Anemia occurred( microcytic-hypocromic ) Sign and symptoms are shown now.

31. Ppl who are at risk Female with prolong menses Purely vegetarians Pregnant women bcz iron metabolism is high specifically in 2rd trimester and absorption is not potent Chronic GIT bleeding Antacid taken ppl Vit c deficient ppl Ulcer patient Mal nutrition Young children Intestinitis Marrow disease

32. lab diagnosis Serum iron low Serum ferritin low Transferin saturation low Total iron binding capacity(TIBC) high

33. treatment Diet Medicinal Iron Blood Transfusion Removal of Cause

34. Anesthesia consideration Same as in anemia?

35. Bleeding disorder Bleeding disorders  are a group of  disorders  that share the inability to form a proper  blood  clot. Bleeding can result from either too few or abnormal platelets, abnormal or low amounts of clotting proteins, or abnormal blood vessels.

36. Signs and symptoms Bleeding gums Uncontrolled bleeding hemorrhages Heavy bleeding from small cuts or dental work Nosebleeds Heavy menstrual bleeding Excessive bleeding following surgery

37. Diagnosis of Bleeding Disorders a complete blood count (CBC), which measures the amount of red and white blood cells in your body a platelet aggregation test, which checks how well your platelets clump together a bleeding time test, which determines how quickly your blood clots to prevent bleeding

38. H emophilia Definition “It is a rare disorder in which your blood doesn't clot normally because it lacks sufficient blood-clotting proteins (clotting factors)” Hemophilia is classified as type A or type B, based on which type of clotting factor is lacking (factor VIII in type A and factor IX in type B) Hemophilia results from a genetic defect found on the X chromosome

39. Values of factor VIII Normal value is above the 25% But in hemophilic A patient which is going to procedure we keep this value up to 50% Classification of hemophilia A; Mild:  5-25%  Moderate: 1-5%   Severe: <1%

40. Treatment Injecting clotting factor concentrates into bloodstream. These injections can prevent or control excessive bleeding. Specially factor VIII fresh frozen plasma transfusions if you lack certain clotting factors. Fresh frozen plasma contains factors V and VIII, which are two important proteins that help with blood clotting.  DDVAP( despmoprisin ) it is a type of synthetic hormone which increase the production of factor VIII , von willebrand factor and tissue plasminogen activator(t-PA)

41. Treatment Half life of factor VIII in adults are 12 hours so repeated infusion of factor VIII will be needed to keep the level normal in body In severe hemophilia A patient factor VIII infusion therapy would be for 2 weeks approximately in order to avoid bleeding that could disrupt wound healing Fibrinolytic inhibitors such as ε- aminocaproic acid (EACA) and tranexamic acid, can be given as adjunctive therapy for bleeding from mucous membranes

42. Considerations in anesthesia Prioperative bleeding risk bleeding can be enter into enclosed spaces (joints, intracranium , pericardium, thorax) Coexisting viral infections secondary to transfusions: HIV, hepatitis (less now with recombinant products) Consider preoperative hematology consultation  Optimize factor activity(by giving it ffp etc) & coagulation profile in perioperative period FFP=10ml/kg Minimize perioperative blood loss; consider blood conservation strategies(plan A,B,C…..)

43. diagnosis aTTP PT Fibrinogen Test Specific clotting factor test For knowledge --> if your baby is Bleeding after circumcision of the penis goes on for a long time then be carefull .

44. Von Willebrand disease  (VWD) Definition; “it is a genetic disorder caused by missing or defective von Willebrand  factor(a clotting protein)” VWF binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug during the clotting process. Von Willebrand disease is classified into three different types (Types 1, 2, and 3) Type 1 is the mildest and most common form; Type 3 is the most severe and least common form.

45. Types of von Willebrand Type 1 VWD is found in 60%-80% of patients.  People with type 1 VWD have a quantitative deficiency of VWF. Levels of VWF in the blood range from 20%-50% of normal. The symptoms are usually mild. Type 2 VWD is found in 15%-30% of patients.  People with type 2 VWD have a qualitative deficiency in their VWF Type 3 VWD is found in 5%-10% of patients.  People with type 3 VWD have a severe quantitative deficiency of VWF. Symptoms are typically severe, and include spontaneous bleeding episodes, often into their joints and muscles.

46. signs Epitasis Easy bruising Menorrhagia ( sevear bleeding in mensis ) Gingival GI bleeding.

47. Diagnosis Von Willebrand factor antigen.  This test determines the level of von Willebrand factor in your blood by measuring a particular protein. Ristocetin cofactor activity.  This test measures how well the von Willebrand factor works in your clotting process. Ristocetin , which is an antibiotic, is used in this laboratory testing. Factor VIII clotting activity.  This test shows whether you have abnormally low levels and activity of factor VIII. Von Willebrand factor multimers .  This test evaluates the specific structure of von Willebrand factor in your blood, its protein complexes and how its molecules break down.  rapid platelet function assay (RPFA) platelet count

48. Anesthesia managment Desmopressin: It's a synthetic hormone It controls bleeding by stimulating your body endotheial cells to release more von Willebrand factor already stored in the lining of your blood vessel Dose= 0.3 μ g/kg. Don’t work on type 3? Replacement therapies.  infusions of prepared doses of concentrated blood-clotting factors containing von Willebrand factor and factor VIII Will this work? Contraceptives.  For women, these can be useful for controlling heavy bleeding during menstrual periods. Clot-stabilizing medications.  These anti- fibrinolytic medications such as aminocaproic acid and tranexamic acid they can help stop bleeding by slowing the breakdown of blood clots.

49. Anesthesia considerations Same as in hemophillia