Purpura and leukemia DOC-20240122-WA0071..pptx

Disease condition and management

1. Purpura , leukemia

2. PURPURA DEFINITION Purpura is a bleeding disorder characterized by petechiae & ecchymosis which may be due to either deficiency in number or quality of platelets or defect in vascular endothelium Purpura occurs due to bleeding from capillaries, under the skin

3. Types Purpura is of two types Idiopathic Thrombocytopenic Purpura Anaphylactoid Purpura

4. Idiopathic Thrombocytopenic Purpura Definition Idiopathic Thrombocytopenic Purpura, the most common acquired thrombocytopenic disorder in children is a hemorrhagic condition involving skin, mucous membrane & internal organs. It is most likely an autoimmune response of the body Incidence: 3-7 yrs of age

5. Pathophysiology Normally the platelets are formed from megakaryocytes in bone marrow In 60% cases of purpura , the cause is autoimmune, with antibodies against platelets Most often these antibodies are against platelet membrane glycoproteins and are of immunoglobulin G ( lgG ) type After binding of the antibody to the platelet surface, circulating antibody coated platelets are recognized by the Fc receptors or splenic macrophages, ingested and destroyed leading to platelet deficiency

6. Clinical Features The child with Purpura may present with: Petechiae (prominently over legs) Ecchymosis Bleeding from gums Epistaxis Anemia (proportional to bleeding) internal hemorrhage evidenced by hematemesis , melena and hematuria Hemarthrosis (bleeding in joints) Low grade fever If platelet count goes below 5000/mm3,intracranial or intracerebral hemorrhage may occur as a complication.

7. Diagnostic Evaluation Diagnosis is based on the following laboratory tests: Platelet count : platelet count may be below 20,000/mm3 (normal count is 1.5-4.5 lakhs /mm3) Bleeding time and clotting time may be increased Peripheral blood smear -Giant forms and deeply stained platelets are seen Bone marrow examination: Abundant platelet precursors are seen in bone marrow

8. Management There is usually no need of treatment for patients with a platelet count above 50,000/mm3. A count below 20,000/mm3 is an indication for treatment includes- 1 . Steroids The first line of treatment usually consists of steroids, which suppress the immune system. The dose and route of steroid administration is determined by platelet count and whether there is active bleeding. In emergency, infusion of dexamethasone or methyl prednisolone may be used. Once the platelet count has improved, the dose of steroid is gradually reduced while monitoring for relapses. Long term steroids are usually avoided, as they can cause numerous side effects like cataract, diabetes and osteoporosis.

9. 2.Anti D antibodies Another line of treatment for Rh - positive patients is the use of Anti D immunoglobulin, which is given intravenously 3.Steroid Sparing agents Immunosuppressants like mycophenolate , mofetil and azathioprine are being used now a days due to their effectiveness.

10. 4. Immunoglobulins Intravenous Immunoglobulins may be infused in some cases. It is costly and produces short term improvement. 5.Platelet Infusion Platelet infusion may be done in emergency situation, but is unsuccessful in producing long term platelets count increase.

11. ANAPHYLACTOID PURPURA Generalized systemic vasulitis of unknown etiology is known as Schonlein-henoch vasculitis . It is characterized by deposition of immune complexes containing antibody lgA . Anaphylactoid purpura is a systemic inflammatory disorder, seen primarily in children between 2-8 year of age. Boys are affected twice as frequently as girls.

12. Etiology Children who develop anaphylactoid purpura have an increased incidence of atopic disease and recent upper respiratory infiction . Although the exact etiology is unknown, the distinctive clinical manifestations suggest a hypersensitive immunologic reaction to Group-A streptococcus resulting in localized or widespread vascular damage. Possible precipitating causes include various drugs (penicillin aspirin), food (chocolate, milk, eggs) insect bite and bacterial or viral infections.

13. Pathophysiology Hypersensitive immunologic reaction leads to vasculitis of small blood vessels in upper dermis, GIT, synovial joints and renal glomeruli . Due to the inflammation of blood vessels, there is extravasation of erythrocytes petechial hemorrhage and other clinical features.

14. Clinical Features The onset of purpura may be acute or insidious. There is vasulitis in upper dermis, GIT, synovial membrane and renal glomeruli . Clinical features are follows: Anaphylactoid purpura usually begins with erythematous petechial rashes appearing symmetrically over the buttocks and lower extremities that may progress to large palpable ecchymotic areas.

15. Gastrointestinal symptoms include- Colicky abdominal pain Vomiting with or without hematemesis Intussusception Synovial membrane vasculitis leading to arthralgia is characterized by warm swollen and painful joints. Usually knee and ankle joint are affected

16. Renal manifestation include- Hematuria Proteinuria Hypertension Hemorrhagic complications involving the central nervous system include seizures, paresis and coma, but they occur rarely.

17. Diagnostic Evaluation Anaphylactoid Purpura is usually diagnosed based on typical skin, joint and kidney findings. Throat culture, urinalysis and blood test for inflammation and renal function are used to suggest the diagnosis.

18. Management Medical management of Schonlein-henoch vasculitis is essentially symptomatic, since there is no specific treatment Antibiotics are given if a specific bacterial infection is identified. Short term steroid therapy (usually prednisolone ) may be give to suppress acute manifestations and relieve joint and abdominal pain. Analgesics like Acetaminophen are give for pain relief.

19. Nursing Management Nursing management for patients with Purpura focus on these aspects: a. Minimize chances of trauma to the child and provide safe environment b. Control bleeding c. Administer the prescribed drugs

20. A.Minimize chances of trauma to the child Safe environment should be provided to the child to protect him from injury. The side rails of cradle should be padded. Avoid using hard tooth brush for the child. Use light mouth wash or glycerin swabs for cleaning the child's mouth. Provide soft and nutritious diet to the child. Make the child wear loose garments. Give smooth edged or soft toys to the child

21. Do not give intramuscular or intravenous injection and prevent frequent venipunctures. Avoid elective surgeries. B. Control bleeding First aid needs to be given during a bleeding episode. If these measured do not stop bleeding, other treatment will be needed, following first aid measure may reduce the amount of bleeding: R= Rest: Make the child lie down quietly until bleeding episode ends. I= Ice: Apply an ice pack on the bleeding area.

22. C= Compression: Apply pressure over the bleeding site. A bleeding join may be wrapped with an elastic compression bandage. E=Elevate: Position the child so that the bleeding area is raised. Raise the area above the level of heart if possible.

23. Administration of Prescribed Drugs In order to relive sever joint pain, Acetaminophen is administered. Corticosteroids such as prednisolone may be give as prescribed by the physician to minimize hemorrhagic manifestations. Monitor the patient for side affect of steroid therapy like cataract, increased body weight, hyperglycemia, etc.

24. Prognosis The prognosis of Purpura is good if adequate supportive care is give and complications (such as intracranial hemorrhage) are prevented . Most of the patients recover within 2-3 months of onset.

25. LEUKEMIA

26. Introduction Leukemia is the most common type of cancer in children. All cancers begin in cells of the body, and leukemia is a cancer that begins in blood cells. Normally, cells grow and divide to form new cells as the body needs them. When cells grow old, they die and new cells take their place. Sometimes, this process does not work right. In cancer, new cells form when the body does not need them, and old cells do not die when they should.

27. LEUKEMIA Definition Leukemia (Greek leukos "white"; aima "blood") is a cancer of the blood or bone marrow characterized by an abnormal increase of blood cells, usually leukocytes (white blood cells). Leukemia is defined as uncontrolled neoplastic proliferation of leucocyte precursors.

28. Incidence Leukemia is the most common malignancy of children less than 15 years of age. The Peak incidence is at 4 years of age. Incidence in males is greater than in females. It is twice more common in white than black children.

29. Etiology The exact cause of leukemia is unknown but several etiologic factors have been identified including: Viruses like Human papilloma virus, Epstein-Barr virus Radiations Exposure to chemicals and drugs like benzene and dilantin Familial predisposition chromosomal abnormalities

30. Classification

31. Leukemia can be classified according to predominant cell type and level of cell maturity as: Lympho : It means leukemia involving the lymphoid series Myelo : It means leukemia of myeloid (bone marrow origin) series Blastic : It involves immature cells Cytic : It involves mature cells

32. Unknown aetiology Somatic mutation in gene Deactivate tumour suppressor gene Malignant transformation of lymphoblast in the bone marrow Uncontrolled proliferation of lymphoblast in bone marrow Lymphoblasts replace the normal marrow element

33. Uncontrolled proliferation of leucocyte precursors Competition for nutrients, infiltration of organs & replacement of normal cells by leukemic cells Bone marrow reticuloendothelial Central nervous Generalized Dysfunction system system hypermetabolism RBCs WBC Platelets Enlarged liver, Leukemic Cellular lymph nodes & meningitis starvation Anemia Infection Hemorrhage Spleen

34. Acute Lymphoid Leukemia(all) Acute lymphoid or acute lymphocytic leukemia is the most commonly diagnosed cancer in children, which accounts for 80% of all childhood leukemias . Many etiologic factors have been implicated, that might increased the risk of developing leukemia like viruses, irradiation, exposure to certain toxic chemical and drugs and a genetic predisposition. Acute lymphocytic leukemia develops when lymphoid cell line is affected.

35. Types of ALL A. T cell Leukemia It is seen in 10-15% cases of acute lymphocytic leukemia. It is seen in older children, particularly males. It has the following features- Mediastinal mass Hepatosplenomegaly High WBC count CNS involvement may be seen poor prognosis

36. B- cell leukemia it is seen in 1-2% children with ALL . It is an aggressive form and has poor prognosis Pre- B cell It has good prognosis and responds well to therapy Null cell leukemia it is the most common type of childhood ALL, occurring in 75% cases. It has better prognosis than other types.

37. ACUTE NON- LYMPHOID LEUKEMIA/ ACUTE MYELOID LEUKEMIA(AML) Acute non- lymphocytic leukemia is abnormal proliferation of monocytes and myelocytes in bone marrow. It is present in approximately 15% children with leukemia. It has a poor prognosis. It includes the following subtypes: a. Acute myeloblastic leukemia b. Acute promyelocytic leukemia c. Acute myelomonocytic leukemia d. Acute monocytic leukemia e. Acute erythrocytic leukemia

38. Clinical Features The clinical features of AML are: Recurrent chronic infections Fatigue Lymphadenopathy Hepatosplenomegaly Bone or joint pain Pallor Frequent bruising Gingival hypertrophy may be present

39. Feature of CNS involvement like headache, blurred vision, fundal hemorrhage and paresis. Other life threatening problem like sludging and clumping of leukemic cells in small cerebral capillaries and hyperuricemia Thrombocytopenia is also present.

40. Diagnostic Evaluation History Collection Physical Examination & Clinical manifestations Peripheral blood smear showing immature leucocytes frequently combined with low normal blood counts. Bone marrow Examination: A leukemic marrow is hypercellular , with 60-100% immature white blood cell reducing in normal marrow component . A finding of more than 25% of abnormal lymphoblasts is diagnostic

41. Blood investigations reveal an elevated serum uric acid level due to increased turnover of malignant cells. Radiologic studies are done to evaluate presence of mediastinal mass. Liver and renal functions tests are done to detect leukemic cells infiltration in liver and kidenys . Lumbar puncture is done to assess CNS involvement. Cytomorphologic , cytochemical and immunologic studies to categorize the disease into sub groups.

42. Management i . Treatment of leukemia involves systemic chemotherapy with or without cranial irradiation. A. chemotherapy: it is given in three phases- Induction Phase This is the phase that reduce leukemic cells to an undetectable level, a state known as remission. In remission, there is no evidence of leukemia on physical examination, bone marrow evaluation, peripheral blood smear, CSF examination or examination of other extramedullary sites. 95% children with leukemia achieve remission during induction, within 4 week.

43. Drugs used for induction in all are Predinisolone , Vincristine and L- Asparginase with or without in doxorubicin. In AML for induction drugs like Cytarabine ( ara -c) and Daunorubicin are used. Leukemic cells can cross the blood- brain barrier while most chemotherapy drugs, do not cross this barrier. Children with ALL receive CNS prophylaxis with chemotherapy drugs instilled intrathecally , into the cerebrospinal fluid space during lumbar puncture. Children who present with CNS disease also need radiation therapy.

44. Consolidation Phase The next phase of treatment is consolidation or intensification that aims at eradicating any residual leukemia cells. This phase of therapy begins once remission is attained. Treatment is directed at those anatomic sites which are protected to some extent from systemic chemotherapy like CNS (protected by blood- brain barrier) and testes (that lie outside the body) This phase involves prophylactic treatment of CNS with cranial irradition and or intrathecal administration of Methotrexate . The therapy usually consists of daily high dose radiation treatment for about 2 weeks or twice a week doses of Methotrexate 3 total of 5-6 injections.

45. Maintenance therapy Maintenance therapy aims at preventing recurrence and further reducing the number of leukemic cells. Maintenance therapy begins after successful completion of induction and consolidation phase. Drugs frequently used in maintenance therapy are 6-mercaptopurine and weekly doses of oral Methotrexate . During this phase weekly or monthly complete blood count is done, to evaluate marrow’s response to drugs. If WBC count goes below 2000/mm3 or toxic side effect occur, then therapy is temporarily stopped or dose is reduced.

46. B. Bone marrow transplantation Bone marrow transplant have been used successfully in treating some children with ALL and AML. In general bone marrow transplant is not recommended for children with ALL because of excellent result possible with chemotherapy. Marrow transplant is possible only when suitable donor is available. Prognosis after transplantation depends on type of leukemia. Long term survival after marrow transplant is seen in 25-50% cases.

47. Nursing Management Nursing management focuses on managing the problems of leukemia and side affects of chemotherapy. a. Management of Problem of Leukemia Infiltration of bone marrow with leukemia cells, leads to myelosuppression resulting in reduced RBC,WBC and platelet production. This ultimately leads to anemia, infections and chances of hemorrhage. Nursing management of these problems is an follows-

48. i . Management of Anemia Nurses should assess the leukemia children for anemia and its severity. Blood transfusion with packed red cells may be required to raise hemoglobin level above 10gm/dl. Nurse should take all precaution to prevent problem associated with blood transfusion like transmission of blood bone infection to the patient through transfusion of contaminated blood, transfusion reaction etc. Nurses should monitor the patient continuously during blood transfusion

49. ii. Prevention from Infections Infection is the most frequent casus of death in leukemia. Organisms most commonly infecting these children are varicella , herpes zooster , MMR and polio virus, protozoa like pneumocystis carnii and fungi like candida alibicans . Following measures must be taken to prevent infections- Broad spectrum antibiotics are used prophylactically Live vaccines should not be given to leukemic children, as they are already immuno compromised.

50. When the child is in hospital, universal precautions are used along with isolation, barrier nursing, strict hand washing and aseptic techniques. Fever is a sign of infection , so if fever occur blood urine, stool and nasopharyngeal cultures are done, to identify the cause and site of infection. An adequate protein and calorie intake provide the child with better host defenses against infection and increases tolerance to chemotherapy.

51. iii. Prevention and Management of Chemotherapy Most of the bleeding episode can be prevented by administration of platelet concentrates. Regular mouth care is essential since gingival bleeding is frequently seen The child may be provide with soft tooth brush or only mouth wash may be used to rinse the mouth. These children develop repeated episode of diarrhea, so they are prone to rectal ulceration. Provide perineal care after each loose stool and keep the area clean and dry. During a bleeding episode, the parents and child need emotional support. Children are kept away from activities that might cause injury or bleeding.

52. b. Management of side effect of chemotherapy and problems of irradiation Nausea and vomiting For mild to moderate vomiting, antiemetics like Promethazine ( phenargan ), chlorpromazine etc. are used. Metacloperamide is administered for severe vomiting. Antiemetics should be give before chemotherapy is started (30 minutes to 1 hour before chemotherapy) and then regularly at two hours interval upto 24 hours.

53. ii. Anorexia Loss of appetite because of chemotherapy and radiation therapy so: Give small frequent feeds to the child according to his likes. Give soft and easily digestible food to the child Serve the food in an attractive manner.

54. iii. Mucosal Ulceration As side effect of chemotherapy, the mucosa of gastrointestinal tract becomes ulcerated. Oral ulcers develop which make eating extremely uncomfortable so: Give bland, moist and soft diet to the child. Use soft tooth brush or cotton tipped applicator and mouth wash to clean the child’s mouth. Frequent mouth wash may be provide with normal saline. Local anesthetics like lidocaine can be used to anesthetize oral ulcers, before the child eats food. Liberal fluid intake is encouraged. Nasogastric feeds may be started in case of severe ulceration in mouth.

55. iv.Neuropathy Vincristine and vinblastin can cause various neurotoxic effect leading to foot drop, weakness and numbness of extremities and reduced bowel movements Use foot board to prevent foot drop in bed ridden children. The child suffers in from constipation. Regular bowel movement should be ensured by using stool softeners and laxatives. Also fluid intake of the child must be increased.

56. V.Hemorrhagic Cystitis The drug Cyclophosphamide leads to hemorrhagic cystitis. It can be prevented by: Liberal fluid intake. Motivate the child to void immediately on feeling the urge to urinate. Administer chemotherapy drugs in the morning, to allow for sufficient intake of oral fluids and frequent voiding or urination.

57. vi. Alopecia Hair loss occur because of chemotherapy and cranial irradiation, so:- Inform the parent and child about this side effect earlier. Encourage the parents to purchase a wig for child, before hair fall occur. Child’s hair should be cut short and he should be made to wear surgical cap to collect the fallen hair. Parents and child should be reassured that hair will grow again after the treatment stops.

58. vii. Mood changes Shortly after starting of steroid therapy, children experience mood changes which range from feeling of wellbeing and euphoria to depression and irritability. Parents should be made aware of these behavior changes.

59. C. Parents Support and Guidance Nurses should continually guide, support and help parent to adjust to this disease condition. Parents should be encourage to express their feeling, fear, grief and concerns. Provide emotional support to the parents continuously.

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