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What is the leading cause of death from disease in children past infancy?
- Neoplastic disorders (blood-forming)
- **almost half of cancer kids involve blood forming organs**
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Leukemias:
Most common, more frequent in males >1, peak onset 2-6 years
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Classification of Leukemias
Broad group of malignAnt diasease of bone marrow and lymph system
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Different types of Leukemias
A.L.L and AML, stem or blast cell leukemia
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Chromosome studies in leukemia
Kids with trisomy 21 have a 20x greater risk for getting ALL. -kids with more than 50 chromosomes on the leukemic cells have best prognosis
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Pathology of leukemia
Unrestricted proliferation of immature WBCs in blood forming tissues (liver and spleen) also causes low leukocyte counts
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Consequences of leukemia
Anemia from deceased RBCs, infection from neutropenia, bleeding tendencies from decreased platlet production, spleen liver and lymph glands infiltration, enlargement, and fibrosis.
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Diagnosis of leukemia
Based on history, physical manifestations, peripheral blood smear/CBC, bone marrow aspiration
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Therapeutic management of leukemia
Chemo, cranial irradiation sometimes
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4 phases of therapy in leukemia
Induction therapy(4-6 weeks, gets rid of cells), CNS prophylactic therapy(intrathecal chemo-spinal cord), intesification/consolidation therapy(prevents resistant leukemic clones and eradicates residual leukemic cells), & maintenance therapy to preserve remission
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Prevent complications of myelosuppression: ***
- 1)Infection: r/t to neutropenia. Most susceptible at time of diagnosis and relapse, during immunosuppressive therapy, and after prolonged antibiotic therapy. GCSF has reduced kids getting infections!**These kids should not get vaccines to protect them from dangerous childhood diseases bc of their compromised immune system.
- 2) Hemorrhage: preventable/controlled with platelet concentrates or platelet-rich plasma. Avoid skin punctures whenever possible bc bleeding sites become more easily infected with increases hemorrhage. Most common active bleeding is epistaxis and gingival bleeding, in which a platelet transfusion is necessary.
- 3) Anemia: May be intense initially from complete replacement of bone marrow by leukemic cells. During induction therapy, blood transfusion might be necessary.
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When is there increased susceptibility to infection? In leukemia....
Time of diagnosis and remission, during immunosuppressive therapy, after prolonged antibiotic therapy
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Chemotherapy side effects:
N+V, anorexia, mucosal ulceration, neuropathy, hemorrhagic cystitis, alopecia, moon face, mood changes.
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Allogenic bone marrow transplant
Matching histocompatable donor, very hard
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Umbilical cord blood stem cell transplantation
Rich source of hematopoietic stem cells, allows for partially matched
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Autologous BMT
Pts own marrow from disease free tissue, frozen and sometimes treAted to malignant cells
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Peripheral stem cell transplants (PSCT)
Type of autologous transplant, stimulates production of high number of stem cells then collect stem cells by an apheresis machine, stem cells seperated from whole blood, remaining blood cells and plasma return to pt after apheresis
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2 types of lymphomas
Hodgkin disease(15-19) and non-hodgkin lymphoma(<14)
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Hodgkin disease
Neoplastic disease in lymphoid system, often spreads to spleen liver bone marrow lungs and other tissues
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4 types of Hodgkin disease
Lymphocytic predominance, nodular sclerosis, mixed cellularity, lymphocytic depletion, accurate staging is basis for treatment and prignosis
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Classification of Hodgkin disease
***classification A: asymptomatic. Classification B: temp of 38 degrees or higher for 3 dAys, night sweats, unexplained weight loss of 10% or more in past six months
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Diagnostics of Hodgkin
Clinical manisfestations, lymph node biopsy, **presence of reed-sternberg cells***
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Therapeutic management of Hodgkin
Radiation, chemo, both
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Non-Hodgkin lymphoma
60% of kid lymphomas Are NHL; clinical apperance: diffuse rather than nodular, cell type undifferentiated, dissemination occurs eArly often and rapidly, mediastinal involvement
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Bone tumors
Osteosarcoma and Ewing sarcoma are85% of all primary malignant bone tumors. More common in males especially during accelerated growth rate of Adolescence(epiphyseal plate)
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Diagnosis of bone tumors
Rule out trauma or infection first, definitive based on radiologic studies, MRI for neuro, labs
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Osteogenic sarcoma
Most frequent malignant bone tumor in children, peaks around 10-25, predominantly in metaphysis of long bones, mainly legs(distal femur)
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Therapeutic management of osteosarcoma
Radical surgical resection or amputation, limb salvage procedure, chemo
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Nursing considerations of osteosarcoma
Preop preparation, support adolescent, body image concern, pain managing
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Ewing sarcoma
Second most common in kids and adolescence, rare in age >30. Arises in **marrow** especially in femur tibia humerous vertebrae scapula ribs
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Treatment of Ewing sarcoma
Radiation first approach, chemo with radiation, surgical resection. Prognosis best if no metastasis at time of diagnosis
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Nursing considerations of Ewing sarcoma
Nutritional concerns, helping family cope
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Rhabdomyosarcoma
Malignant neoplasm from undifferentiated mesenchymal cells in muscles, tendon, bursa and fascia. "rhabdo"=striated muscle
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Sites of Rhabdomyosarcoma
Orbit, nasopharynx, sinuses, middle eAr, retroperitineal area, perineum ( soft tissue cancer)
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Diagnostics of Rhabdomyosarcoma
Signs and symptoms depend on site of tumor and compression. Tumor in orbit-symptomatic early. In retroperitineal-minimal symptoms until large and invasive.
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Treatment and prognosis of Rhabdomyosarcoma
Highly malignant and frequent metastisis. Complete removal of tumor if possible. Radiation therapy. Chemo to shrink tumor. Long term chemo. Prognosis is verrry poor
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