Hematological And Oncological Disorders

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sickle cell disease- description

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1.hemoglobin(Hgb) is partly or completely replaced by abnormal sickle hemoglobin(hgbS)
2. insufficient o2 causes RBC's to assume a sickle shape, cell become rigid clumped together. thus obstructing capillary blood flow, this can cause a stroke

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sickle cell-description 2

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precipitate sickling include fever, emotional, physical stress, anything that increases body's need for o2, or alter transport of 02.
2. risk factors parents who have hetorozygous for HgbS or being black

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sickle cell- interventions

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1.adm. of meperidine(Demerol)is avoided bc of the risk of normeperidine induced seizures result of CNS toxicity.
2.

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sickle cell crisis- types 1-2

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vaso-occulsive crisis- caused by ischemia, infarction, stasis of bloodw clumping of cells in microcirculation(fever, painful swelling of hands feet joints, abd. pain)
splenic sequestration-this is caused by the pooling, clumping of bld in spleen(profound anemia, hypovolemia, hock)
aplastic crisis- diminished production & ^destruction of RBC triggered by viral inf. or depletion of folic acid(profound anemia and pallor)

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sickle cell - s/s

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stroke, paralysis, death
retinopathy, blindness, hemorrhage
avascular necrosis(shoulder), hepatomegaly, gallstones,
splenomegaly, splenic sequestration, autosplenectomy, hematuria, hyposthenuria,
abd. apin, dactylitis(hand foot syndrome) priapism, pain osteomyelitis, chronic ulcers

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iron deficiency anemia- description

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iron stores are depleted, decreased supply of iron for the manufacture of hgb in rbc.

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iron deficiency anemia- interventions

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1. do not give w milk or antacids decrease absorption
2. liquid iron preparations stain teeth take w straw
2. s/e of iron, black stools, constipation, foul aftertaste

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aplastic anemia- description

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deficiency of circulating erythrocytes due to arrested development of RBCs within bone marrow.
2. diagnosed thru bone marrow aspiration
3. tx bone marrow transplant(preferrably sibling)

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hemophilia- description

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bleeding disorders resilting from a deficiency of specific coagulation protein

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vonwilliebrand's disease- description

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hereditary bleeding disorder, deficiency or defect in protein called von williebrand's factor(vwf)
2. it is characterized tendency to bleed from mucous membranes.

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von willebrand's disease- s/s

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epistaxis, gum bleeding, easy bruising, excessive menstrual bleeding

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B- thalassemia- description

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an autosomal recessive disorder characterized by reduced production of globin chain in the synthesis of hemoglobin
2. highest among mediterranean descent.

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b-thaleassemia- s/s

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frontal bossing,maxillary prominence,
wide eyed, flatnose, greenish yellow skin tone
severe anemia, hepatosplenomegaly

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types of b-thalassemia

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t..minor- asymptomatic , silent carrier case
t..trait- produces a mild microcytic anemia
t..intermedia- manifested by splenomegaly and moderate to severe anemia
t..major- results in severe anemia that requires transfusion support to sustain life(cooley's anemia

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leukemia- description

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malignant exacerbationin the number of leukocytes.
2. cause is unknown appears to involve gene damage of cells thus leading cells from normal state to malignant.

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leukemia-s/s

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infiltration bone(fever, pallor, fatigue, aneroxia, hemorrhage, usually petechiae, bone joint pain, pathological fracture can occur.
infection, hepatomegaly, splenomegaly, decreased hgb and hematocrit

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leukemia-bleeding, chemotherapy

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bleeding(less than 20,000/mm may need transfusion
chemotherapy- mon for severe bone suppression, bld count will be extremely low during nadir(greatest bm supression)

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hodgkins disease- description

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malignancy of lymph nodes
2.causes; viral infections, previous exposure to alkalyting chemical agents

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hodgkin's disease s/s

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painless enlargement of lymph nodes
enlarged,firm, nontender, movable nodes in supraclavicular area.
non productive cough, abd. pain,
positive biopsy of lymph node(presence of reed stenberg cells) positive bone marrow biopsy)

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hodgkin's disease interventions

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stage 1-2 w/o mediastinal node involvement. tx is extensive external radiation.
2. more extensive disease, radiation in combo w multiagent chemotherapy.

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hodgkin's disease- main areas involvement

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waldeyer ring(by nose), cervical supraclavicular, mediastinal, spleen, para aortic and messenteric, lliac, liver, axillary,

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nephroblastoma(wilm's tumor)- description

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1. tumor of kidney
2. genetic inheritance
3. tx. surgery, chemotherapy w or w/o radiation

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nephroblastoma (wilm's disease)- interventions

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preop- place a sign on the bedside that reads do not palpate abdomen.

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neuroblastoma- description

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embroyonal tumor

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osteogenic sarcoma- description

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1. most common bone cancer in children

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osteogenic sarcoma- s/s

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localized pain at the affected site
palpable mass
pathological fractures at tumor site
progressively limited ROM

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brain tumors post op-

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1. mon for signs of ICP, hemorrhage, pooling of blood
2. mon temp, may be elevated bc involvement of hypothalamus or brainstem involvement during surgery
3. mon for colorless drainage from ears or nose.indicative of cerebrospinal fluid..report immediately