hematological disorder Flashcards
decrease platelet
thrombocytopenia (bleeding)
increase platelet
thrombocytosis(clot)
increase platelet
thrombocytosis(clot)
The most common hematology disorder is
Anemia
- is not a specific disease but a sign of underlying disorder
normal value of hemoglobin
M: 13.5-17.5
F: 11.5-15.5
sign and symptoms of Anemia
- weakness, fatigue, general malaise, pallor of skin and mucous membrane, tachycardia, dyspnea
what determine the size of RBC
Mean Cospuscular Volume
the normal Mean Cospuscular Volume RBC is
81-96
what determine how much iron is in the hemoglobin
Mean Cospuscular Hgb
the normal Mean Cospuscular Hgb is
33-36
normal size of MCV
normocytic
increased MCV
macrocytic(megaloblastic anemia)
Decreased MCV
Microcytic anemia
normal MCHC
normochromic
increased MCHC
hyperchromic
Decreased MCHC
hypochromic
Nursing diagnoses for anemia
Activity intolerance
Imbalanced nutrition
Ineffective tissue perfusion
Non-compliance with prescribed therapy
most common form of anemia, common among women especially menstruating and pregnant women
IRON DEFICIENCY ANEMIA
sign and symptoms of iron deficiency anemia
pallor of skin and mucous membranes,
increased PR, shortness of breath, palpitations,
dizziness, fatigue.
bone marrow is trying to make up by making baby RBC(reticulocytosis)
Labs: microcytosis, hypochromia
causes of iron deficiency
Inadequate intake of iron or absorption disorders.
Blood loss when the loss of iron component
Exceeds dietary intake i.E. G.I.
Blood loss from an ulcer, tumor, menstrual Loss
If you a 60 years old male comes with weakness, pallor, angular cheilosis what test would you do to determine iron deficiency anemia?
CBC and FOB( fecal occult bleeding)
GI bleeding among older patient can be….
colonic cancer
Clinical Manifestations of iron deficiency anemia
fatigue, weakness, epithelial changes such as brittle
ridged nails, smooth, red, atrophic tongue, angular cheilosis
Implementation fo iron deficiency anemia
Advise to take foods rich in iron: animal meats and organ meats (beef liver, chicken liver), beans (black beans, garbanzo), leafy green vegetables, raisins.
advice patient to eat foods high in fiber
vitamin c will enhance absorption
Oral iron
Ferrous sulfate, Ferrous gluconate, Fumarate; Iron is best absorbed on empty stomach
-stool will become dark in color
which iron supplement can be given in IV form?
Fumarate
Associated withchronic disease includingarthritis, infection, and malignancy, Rheumatoid Arthritis, serious infections, carcinoma and renal failure.
Characterized by hemoglobin levels of8-10 g/dl,
-Patients with this anemia have plentiful iron store but diminished utilization by the bone marrow
ANEMIA OF INFLAMMATION
Diagnosis of anemia of inflammation
marrow plentiful iron stores, ferritin normal or slightly increased, serum iron and TIBC lowered.
Normocytic, normochromic
Implementation for anemia of inflammation
Advise client on correction of underlying cause can lead to reversal of the anemia
Encourage intake of nutritious foods
medication for anemia of inflammation
synthetic erythropoietin (Epogen, Procrit)
caused by the disorders in the heme moeity of Hgb.
characterized by presence of trapped iron in the mitochondria resulting to diminished Hgb production.
Sideroblastic Anemias
Main characteristics of sideroblastic anemia is
morphologic findings ringed sideroblasts.
what are the 2 types of sideroblastic anemia
Hereditary Sideroblastic Anemia
Acquired Sideroblastic Anemia( most common)
a X-linked condition due to abnormality in pyridoxine metabolism congenital defect in the enzyme d-aminolevilinic acid (ALA) synthetase
Hereditary Sideroblastic Anemia
what are the causes of acquired sideroblastic anemia
lead, alcohol, isoniazid
Assessment of Sideroblastic Anemia
Hgb levels 8-10 g/dl
Normocytic, macrocytic, microcytic hypochromia,
Erythroid hyperplasia, Iron staining reveals ringed sideroblast
Implementations Sideroblastic Anemia
Avoid substances that contain lead such as paint, water in pipes
Administer prescribed pyridoxine to acquired form
Transfuse blood to congenital form.
Diminished synthesis of globin chain of Hgb, RBCs are more rigid leading to early destruction Characterized by severe microcytosis and hypochromia
Thallasemia
what are the types of thallasemia
Alpha-Thalassemia
Beta-Thalassemias (Cooley’s anemia)
deficient alpha-chain synthesis, usually due to deletion of gene
most prevalent among Asians and Middle Eastern.
Alpha-thalassemia
due to malfunction of -globin chain caused by -gene abnormality.
common among African descent
severe anemia, marked hemolysis, ineffective erythropoiesis
Can be fatal within the few years of life.
With early regular transfusion therapy growth and development through childhood are facilitated
Bata-thalassemia
Assessment for thalassemia
microcytosis, hypochromia, poikilocytosis
(bizarre shaped RBC)
Alpha-Thalassemia – Bart’s Hgb;
Beta-Thalassemia – HbF
Implementation for thalassemia
Client teaching on chronic blood transfusion.
Administer prescribed folic acid supplement.
Discuss purpose of iron chelators (Deferoxamine) to delay development of iron overload.
Transfuse RBC.
Patient teaching includes during preconception counselling.
characterized by RBC that exceed 100 micro m in size.
MEGALOBLASTIC ANEMIA
what is the main characteristics of MEGALOBLASTIC ANEMIA
Defective DNA synthesis
which vitamin deficiencies can cause megaloblastic anemia
Folic acid deficiency and B12
Rareamong those whoeat uncooked vegetable, alcoholics, pregnant women malabsorption disorders
Folic Acid Deficiency
strict vegetarians, malabsorption (Crohn’s Disease, gastrectomy, ileal resection
absence of IF can cause
Vitamin B12 deficiency
Clinical Manifestations of megaloblastic anemia
weakness, fatigue, pallor, jaundice
smooth, sore, red, atrophic tongue
mild diarrhea, angular cheilosis
Vitiligo, premature graying of hair often seen in Pernicious anemia, paresthesias in the extremities, ataxia (B12)
High methylmalonic and homocysteine levels more sensitive Vit B12 deficiency.
Test for presence of intrinsic factor and intestinal function to determine cause of Vit. B12
Schilling’s Test if it is normal the cause of the anemia will be Folic acid defeciency
Test for presence of intrinsic factor and intestinal function to determine cause of Vit. B12
Schilling’s Test if it is normal the cause of the anemia will be Folic acid deficiency
Implementation for megaloblastic anemia
Pay particular attention to ambulation.
Assess patient’s need for assistive devices
Ensure safety when position sense, coordination and gait are affected.
Bland, soft foods, small frequent meals for mouth and tongue soreness.
Client teaching on chronicity of disorder and need for monthly B12 injection
Instruct on importance of regular follow-up atrophic gastritis.
damage to the marrow with replacement by fat (aplasia) markedly reduced hematopoiesis
APLASTIC ANEMIA
APLASTIC ANEMIA can caused by
can be congenital or acquired,
infections, medications, chemical or radiation
it can also cause pancytopenia
Substances Associated with Aplastic Anemia
Analgesics
Antiseizure agents (Phenytoin, Triethadione)
Antihistamines
Antimicrobials
Antineoplastic agents
Antithyroid medications
Benzene (airplane glues, paint remover, dry cleaning solutions
Chloramphenicol
Gold compounds
Heavy metals
Hypoglycemic agents
Insecticides
Phenothiazines
Sulfonamides
Sedatives
Clinical Manifestations for aplastic anemia
Symptoms of anemia: fatigue, pallor,
dyspnea
Bleeding tendencies: Purpura, bruising,
Repeated infections such as sore throat,
cervical lymphadenopathies
Retinal hemorrhage
Bone marrow biopsy: aplastic marrow
replaced with fat.
Implementations for aplastic anemia
Assess for signs of bleeding, anemic and infections.
Offending agent should be discontinued.
Prepare client for possible BMT or PBSCT
Prepare client for immunosuppressive therapy (ant thymocyte globulin and cyclosporine)
Place client on isolation room
a severe hemolytic anemia due to inherited
sickle hemoglobin gene, common among
African descent
SICKLE CELL ANEMIA
Common causes of sickling
cold weather, dehydration, hypoxemia, acidosis
Clinical Manifestations of sickle cell anemia
Patients are always anemic with Hgb levels 5-11 g/dl.
Jaundice is characteristically and obviously in the sclerae
Enlarged bones of skull and face
Tachycardia, cardiomegaly due to chronic anemia
Organs commonly involved during infarction: spleen, lungs, CNS.
Unusually susceptible to infection especially to pneumonia
The first stage of sickle cell crisis is
Vaso-occulsive -tissue hypoxia, necrosis
the most affected organ due to Vaso-occulsive stage is
musculoskeletal- pain of the bone and muscle
aplastic crisis due to-
infection with human parvovirus, sudden massive drop of hemoglobin, absence of reticulocytes.
what is the most affected organ on sequestration crisis(when other organs pull the sickled cell)
spleen
Related to Abnormal Sickling and Vaso-occlusion
Painful Crisis
CVA
Acute and Chronic cardiopulmonary disease (e.g. Acute Chest Syndrome, cor pulmonale)
Priapism
Splenic autoinfarction
Skeletal changes (e.g. aseptic changes of the hip
Frequent complication among hospitalized SCD patients, Manifested by rapidly falling Hgb level, tachycardia, fever, bilateral infiltrateson X-ray
Commonly caused by bacterial infection
Acute Chest Syndrome
Implementations for sickle cell anemia
Manage pain- Distraction techniques, relaxation techniques, Administer analgesics
Joints preservation and muscle stimulation:
physical therapy, TENS
Prevent and manage infection-Place client on semi-Fowler’s position, Increase oral fluid intake, Administer prescribed medication, Assess for signs of DHN
Promote coping skills
Managing potential complications
Medical Management for sickle cell anemia
Hematopoietic Stem Cell Transplant
May cure SCD
Pharmacology Therapy
Hydroxyurea
Increases fetal Hgb which decrease formation of sickled cells
Transfusion therapy
extremely common X-linked disorder
Exposure to certain drugs (Sulfas,nitrofurantoin, Moxifloxacin, antimalarials)
results to denaturation of Hgb called Heinz bodies hemolysis of RBC
GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD) DEFICIENCY
protects individuals from falciparum malaria
common among Mediterranean and African descent
deficient individual are liable to oxidant stress, which occurs with infections and certain (e.g. sulfa drugs and quinine
GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD) DEFICIENCY
clinical manifestation of GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD) DEFICIENCY
patients are asymptomatic, normal Hgb and reticulocyte counts most of the time
patient may develop pallor, jaundice, hemoglobinuria, increased retic count
Heinz bodies
Implementations of GLUCOSE-6-PHOSPHATE DEHYDROGENASE (G6PD) DEFICIENCY
Stop the offending medication
Transfusion may be necessary for
severe hemolytic state.
Client education about the disease
and given a list of medications to
avoid
RBC survival is shortened due to destruction by antibodies
Antibodies attacks RBCs
Can also occur based on changes in body temperature (Coomb’s positive hemolytic anemia)
warm-body antibodies – 37’C
cold-body antibodies - 0’C
IMMUNE HEMOLYTIC ANEMIA
Clinical Manifestations for immune hemolytic anemia
anemia may be mild to severe
splenomegaly is the most common finding
hepatomegaly, lymphadenopathy
jaundice
Hgb, Hct, reticulocyte, (+) spherocytes (small, spherically shaped RBCs)
serum bilirubin
(+) Coomb’s Test or Direct antiglobulin test (DAT)
Implementation for immune hemolytic anemia
Client education regarding the disease.
2.Explain need for vaccination against pneumococcal infection (Pneumovax)
3.Administer corticosteroids as prescribed
4.Monitor for possible complications of corticosteroid therapy
5.Administer immunosuppressants (Cyclophosphamide, Azathioprine)
6.Prepare client for possible splenectomy
refers to increased volume of RBC’s
Hct level increased, may or may not increased WBC and platelets
Polycythemia
Aka Polycythemia Vera
Bone marrow autonomously synthesizes cells
independent of eythopoetin levels.
A true neoplasm of the marrow stem cells
> WBC, > platelets, >RBC can exceed 60%
over time bone marrow may become fibrotic (burnt out / spent phase) and may evolve into a metaplasia (AML)
PRIMARY POLYCYTHEMIA
Clinical Manifestations: of primary polycythemia
ruddy complexion
splenomegaly
headache, dizziness, tinnitus, fatigue, paresthesias, blurred vision ( BV)
angina, claudication, dyspnea, thrombophlebitis ( blood viscosity)
generalized pruritus (histamine release along with increased basophils)
increased RBC, increased WBC, increased platelets, erythropoietin N or slightly increased
O2 saturation of >92%
Implementations for polycythemia
Educate client on risk factors for thrombotic complications
Assess S/Sx of thrombocytosis.
TSB or cool bathing for pruritus.
Prepare client for Phlebotomy to keep Hct level within normal range.
initially once or twice weekly 500 ml
Instruct client to avoid Iron supplements.
Administer Allopurinol for increase uric acid level.
Administer Hydroxyurea to suppress marrow function as prescribed.
Administer Aspirin for erythromelalgia as prescribed.
due to excessive production of erythropoetin in response to hypoxia (COPD, cigarette smoking, cyanotic heart disease, high altitude, renal cell CA
SECONDARY POLYCYTHEMIA
A biopsy is the extraction of a very small amount of tissue, such as bone marrow, to definitively diagnose cell type and to confirm or rule out malignancy
Bone Marrow Biopsy
A bone marrow biopsy is commonly performed to diagnose
causes of blood disorders, such as anemia or thrombocytopenia; to diagnose diseases of the bone marrow, such as leukemia, and infection; or to stage lymphoma or other forms of cancer.
Pre-procedure of bone biopsy Nursing Implementations
Verify Consent
Administer a sedative if prescribed
Cleanse the site with an antiseptic solution. Maintain sterility of equipment and supplies.
Client education: test will last about 20 min
Post-procedure of bone biopsy Nursing Implementations
Apply pressure to the biopsy site to control bleeding.
Place a sterile dressing over the biopsy site. Maintain the client on bed rest for 30 to 60 min.
Monitor for manifestations of infection (fever, increased WBCs, pain, and swelling at the site) and bleeding.
Apply ice to the biopsy site to minimize bleeding and bruising.
Postprocedure discomfort is usually relieved by mild analgesics.
Avoid aspirin and other medications that affect clotting
Transfusion from compatible donor blood
STANDARD DONATION
The client’s blood is collected in anticipation of future transfusions (elective surgery). This blood is designated for and used only by the client.
Clients can donate up to 6 weeks prior to the scheduled surgery. is collected
AUTOLOGOUS TRANSFUSIONS
Sterile blood lost during a procedure is saved or retrieved into a device that filters and drains the blood into a bag for transfusion intraoperatively or postoperatively. Reinfusion must occur within 6 hr of salvaged blood collection
INTRAOPERATIVE BLOOD SALVAGE:
Platelet Transfusion
Platelets do not need to match the client blood type.
Platelet infusion bags contain 200 to 300 mL.
Must be transfused immediately infused once brought to the client’s room and given over 15 to 30 min using a special transfusion set with a small filter and short tubing.
Vital signs are taken before the infusion, 15 min after the infusion starts, and upon completion.
Plasma Transfusion
Plasma is frozen immediately following donation and is then in the form of fresh frozen plasma (FFP).
FFP is transfused as soon as the unit is thawed while clotting factors are still active.
The client can react to the FFP transfusion if the ABO compatibility
Infuse the unit of 200 mL of FFP rapidly over 30 to 60 min through a regular Y-set or straight filtered tubing is not matched
Washed packed RBCs
Infuse a unit of 200 mL over 2 to 4 hr.
Administer to a client who has a history of transfusion reactions or to a client who has had a hematopoietic stem cell transplant.
WBC Transfusion
Immunocompromised clients rarely receive WBC transfusions because of the risk for severe reaction.
If the client is receiving amphotericin B antibiotics, 4 to 6 hr should be between the administration of the antibiotic and the WBC transfusion because amphotericin B can hemolyze the WBCs.
Infuse WBCs suspended in 400 mL plasma over 45 to 60 min and vital signs are taken every 15 min. The presence of the provider may be required according to agency policy
An unregulated proliferation of leukocytes in the bone marrow
Acute forms, there can be infiltration of leukemic cells in other organs, such as the meninges, lymph nodes, gums, and skin
Leukemia
the onset of symptoms is abrupt, often occurring within a few weeks
Most leukocytes are undifferentiated cells or blasts
Death occurring within weeks to months without aggressive treatment
In chronic leukemia, symptoms evolve over a period of months to years
Majority of leukocytes produced are mature
Acute leukemia
Risk Factors for Leukemia
Exact cause is not fully known
Exposure to radiation or chemicals
Genetic disorders
Viral infections
Types of Leukemia
Acute Myeloid Leukemia
Acute Lymphocytic Leukemia
Chronic Myeloid Leukemia
Chronic Lymphocytic Leukemia
originates due to a series of genetic mutations in the myeloid Leading to development of abnormal blast cells
blast cells(i.e., immature leukocytes) continue to proliferate, they crowd out normal bone marrow production(thrombocytopenia, anemia, infection)
Most common form of leukemia
Most common cause death from leukemia
With treatment, patient survive an average of < 1 year.
ACUTE MYELOID LEUKEMIA (AML)
Affects stem cells that differentiates into all myeloid cells: monocytes, granulocytes (neutrophils, basophils, eosinophils) erythrocytes, platelets
peak incidence 60 years.
patients who are older and with more undifferentiated form have worse prognosis
ACUTE MYELOID LEUKEMIA (AML)
Risk factors ACUTE MYELOID LEUKEMIA (AML)
increasing age, exposure to benzene, pesticides, ionizing radiation, petroleum products, cancer therapy with alkylating agents, tobacco smoking, Down’s Syndrome,Trisomy 8
Clinical Features of ACUTE MYELOID LEUKEMIA (AML)
effects of acute leukemia is marrow infiltration by non-maturing, functionless blast cells
fatigue, pallor (due to anemia)
fever and infection (due to neutropenia)
petechiae, purpura, epistaxis (due to thrombocytopenia)
Clinical Manifestations of ACUTE MYELOID LEUKEMIA (AML)
splenomegaly, gum hypertrophy, bone pain
pancytopenia
increased uric acid, DIC
bone marrow biopsy: immature blast cells (>30%)
Suspected in patients with excessive WBC counts and splenomegaly
PBS reveal a spectrum of cell forms from mature PMN to immature blasts
There is uncontrolled proliferation of cells, the marrow expands into the cavities of long bones
Cells are also formed in the liver and spleen resulting to organomegaly
Characterized by abnormal chromosomal marker: Philadelphia chromosome
CHRONIC MYELOGENOUS LEUKEMIA
Patients diagnosed with CML have life expectancy of
3-5 years
on which phase of VML pt. will have have few symptoms, leukocytosis is detected by a CBC performed for some other reason
Chronic
Transformation from chronic to blast crisis
Fatigue, anemia, splenomegaly, or dyspnea, bone pain and may report fevers (without any obvious sign of infection) and weight loss.
accelerated phase
In this phase exhibit signs and symptoms that are more like AML
WBC count exceeding 100,000/mm³
Dyspneic, confused due to leukostasis (reduced blood flow due to excessive WBC
blast phase
75% to 80% of all cases are found in children, with peak incidence is 4 years of age
Boys are affected more often than girls
Results from uncontrolled proliferation of immature lymphoblasts cells; origin is in the lymphoid line
ACUTE LYMPHOCYTIC LEUKEMIA
ACUTE LYMPHOCYTIC LEUKEMIA associated with
Down’s Syndrome, Neurofibromatosis, Klinefelter Syndrome, Fanconi Anemia
Clinical Manifestations ACUTE LYMPHOCYTIC LEUKEMIA
Anemia
Bleeding
Infection
Pain from an enlarged liver, spleen as well as bone pain due to infiltration with leukemic cells.
CNS is frequent site of involvement
Headache, vomiting, meningismus
typically derives from a malignant clone of B lymphocytes, Agent Orange may be at risk for developing this disease, Many patients will have a normal life expectancy, Common among older adults.
Average age 71 years old
CHRONIC LYMPHOCYTIC LEUKEMIA
Clinical Manifestation of CHRONIC LYMPHOCYTIC LEUKEMIA
Many patients are asymptomatic and are diagnosed incidentally during routine physical examination
B Symptoms
Fever of at least 100.4’ F (38’C) that may come and go over several weeks that is not explained by an underlying infection (FUO)
Drenching night sweats
Unintentional loss of at least 10% body weight over the past 6 months
Clinical Manifestation of CHRONIC LYMPHOCYTIC LEUKEMIA
In the early stages: elevated lymphocytes >100, 000/mm3
Lymphadenopathy (lymphocytes are trapped in the lymph nodes); enlarged nodes can be very painful.
Hepatomegaly, splenomegaly then develops
Later stages
Anemia, infection bleeding
Medical Management of CHRONIC LYMPHOCYTIC LEUKEMIA
IV Immunoglobulin
PNA and Flu vaccine recommended
Avoid live vaccines
CML can potentially be cured with allogeneic Hematopoetic Stem Cell Therapy (HSCT) in otherwise healthy patients who are younger than 65 years
Goal for any type of Leukemia: Remission
neoplasms of lymphoid origin.
These tumors usually start in the lymph nodes but can involve lymphoid tissues in the spleen, GIT, liver or bone marrow.
LYMPHOMAS
A malignancy of the lymph nodes that originates in a single lymph node (unicentric) then spreads along the chain of nodes; has impressive cure rate
HODGKIN’S DISEASE
HODGKIN’S DISEASE is more common
among men with two peaks of incidence: early 20’s and the other after 50 years of age
Usually involves lymph nodes, tonsils, spleen, and bone marrow and is characterized by the presence of Reed-Sternberg cell in the nodes; it is the pathologic hallmark and essential criterion for diagnosis
HODGKIN’S DISEASE
possible causes of HODGKIN’S DISEASE
viral infections (Epstein-Barr Virus) and previous exposure to alkylating chemical agents
Clinical Manifestations of HODGKIN’S DISEASE
- Fever
- Pain, Malaise, fatigue, and weakness
- Night sweats, pruritus
- Loss of appetite and significant weight loss
- Anemia and thrombocytopenia
- Usually starts as painless enlargement of one or more lymph nodes on one side of neck (cervical, supraclavicular, mediatinal nodes)
Presence of Reed-Sternberg cell to nodes - Positive CT scan of the liver and spleen
- cough, pulmonary effusion
- jaundice
- abdominal pain, skeletal pain
- mild anemia (most common hematologic finding)
- increased ESR and copper level
- impaired cellular immunity i.e. absent reaction to skin testing, Candida infection
A heterogeneous group of cancers that originate from the neoplastic growth of lymphoid tissue
Lymph nodes from multiple sites may be infiltrated, as may sites outside the lymphoid system
Non-Hodgkin Lymphomas
Risk Factors Non-Hodgkin Lymphomas
Increased in patients who have immune deficiencies or autoimmune disorders
prior treatment for cancer
an organ transplant recipient
history of viral infections (e.g., Epstein–Barr virus, HIV, HHV8)
Clinical Manifestations Non-Hodgkin Lymphomas
May start as a painless swelling in one or more lymph nodes in the neck, axillary region, or groin
Splenomegaly
Compression effect of growing cancerous lymph node (ex. Chest pain with enlarging mediastinal mass)
Medical Management Non-Hodgkin Lymphomas
Indolent Type
Watchful waiting
Radiation therapy
Aggressive Type
Radiation + Chemotherapy
Large NHL tumor
Risk for Tumor Lysis Syndrome 12-72 hours after chemotherapy
Contents of tumor are released into the blood stream
A malignant proliferation of plasma cells and tumors within the bone
An excessive number of abnormal plasma cells and ultimately destroy bone; invasion of the lymph nodes, spleen, and liver occurs
MULTIPLE MYELOMA
The abnormal plasma cells produce and abnormal antibody (Myeloma/M protein or the Bence-Jones protein that is found in the blood and urine
MULTIPLE MYELOMA
Clinical Manifestations of MULTIPLE MYELOMA
Bone (skeletal) pain, (reported by 2/3 of patients)
Weakness and fatigue
Recurrent infections
Bence-Jones proteinuria and elevated serum
protein level
Osteoporesis (bone loss and the development
Anemia, thrombocytopenia and
granulocytopenia
Elevated calcium and uric acid levels
Renal failure, anemia
Spinal cord compression and
paraplegia
decreased WBC, decreased RBC, decreased platelets due to proliferation of cancer cells in the bone marrow; bone marrow biopsy will confirm diagnosis.
Implementation of MULTIPLE MYELOMA
Management of pain is very important for these patients.
Administer NSAIDs, opioids
2. Client education about activity restrictions
No lifting of weights more than 10 lbs
Proper use of body mechanics
3. Administer chemotherapy (primary treatment); corticosteroids are often combined with other agents such as melphalan, cyclophosphamide prescribed
4. Monitor for complications of corticosteroid, chemotherapy.
Prepare client for possible BMT, PBSCT
6. Maintain neutropenic and bleeding
precautions as necessary
7. Administer antibiotics as prescribed for infection
Monitor for signs and symptoms of bleeding, skeletal fractures.
8.Force fluids up to 3 to 4 liters a day, to reduce potential problems associated with hypercalcemia, hyperuricemia, and proteinuria
Encourage ambulation to prevent renal problems and to slow down bone resorption.
occurs most commonly in children and young adults
the syndrome is associated with streptococcal infections and drugs
AUTOIMMUNE (ALLERGIC) PURPURA (HENOCH-SCHONLEIN PURPURA)
Clinical Manifestation: of AUTOIMMUNE (ALLERGIC) PURPURA (HENOCH-SCHONLEIN PURPURA
Perivascular inflammatory lesions with serosanguineous leakage into the skin, submucosa, and serosa are the hallmark of the disease.
Lesions are symmetric and palpable usually found on distal extremities
Bowel lesions may cause gastrointestinal symptoms
Joints lesions can cause arthritis.
Management: of AUTOIMMUNE (ALLERGIC) PURPURA (HENOCH-SCHONLEIN PURPURA
No specific therapy is uniformly helpful.
Patients may respond to corticosteroid
therapy.
caused by Vitamin C deficiency, collagen synthesis is impaired, vessel walls with poor collagen, support are pliable and easily ruptured
SCURVY
Clinical Manifestations of scurvy
perifollicular petechiae, gum bleeding,
subperiosteal hemorrhage bleeding time is prolonged
Management of scurvy
Vitamin C 1 gm/day rapidly corrects all bleeding.
autosomal dominant disorder associated with abnormally thin vessel walls and impaired vascular contractility.
vessels are markedly pliable, liable to burst with trauma and unable to contract appropriately for hemostasis
HEREDITARY HEMORRHAGIC TELENGIECTASIA
Clinical Manifestations of HEREDITARY HEMORRHAGIC TELENGIECTASIA
small, nodular, lesions on the lips, face, ears, tongue and GIT mucosa; lesions blanch upon pressure.
bleeding is common, especially GIT bleeding Iron Deficiency Anemia
Recurrent hemorrhage
Multiple telengiectasias
Familiar occurrence
the most common cause of bleeding.
<100,000/l bleeding time begins to prolong
50,000 to 20, 000 /l petechia, purpura
<10, 000/l spontaneous bleeding
THROMBOCYTOPENIA
Mechanisms of thrombocytopenia
impaired platelet production
abnomal platelet pooling
increased peripheral destruction
Characterized by shortened platelet survival
Immune-mediated; sulfa drugs, SLE, pregnancy.
Anti-platelet antibodies bind to the platelets RES tissue macrophage ingest platelets destruction
IDIOPATHIC THROMBOCYTOPENIC PURPURA
Clinical Manifestations:Acute variant IDIOPATHIC THROMBOCYTOPENIC PURPURA
occurs in children between 2-6 years old usually following a viral infection.
resolves within 4-8 weeks
Chronic variant IDIOPATHIC THROMBOCYTOPENIC PURPURA
occurs in young adult women remissions and relapseseasy bruising, heavy mens mucocutaneous bleedingpetechiae on the extremities and trunk mucosal bleeding such as GIT, lungs, CNS excessive bleeding after trauma decreased platelet count, increased megakaryocytes
most common hereditary coagulopathy, accounting for 68-80% of such conditions.
is transmitted as classic X-linked trait (the disorder is carried by females and manifested by males)
HEMOPHILIA
HEMOPHILIA- A
CF VIII
HEMOPHILIA -B
CF IX
Clinical Manifestations of Hemophilia
Hemarthrosis i.e. knees, elbows, ankles, shoulders, wrists and hips
Patient often complain of pain and limitation of movements
Hematomas can be superficial (epistaxis) or deep (intracranial)
Excessive bleeding after minimal trauma, surgery
Implementations for hemophilia
Provide assistance with coping because it is chronic, places restriction on their lives.
Encourage client to be self-sufficient and maintain independence by preventing unnecessary trauma.
Extensive teaching about activity restrictions to diminish chances of hemorrhage.
Avoid aspirin, NSAIDs, herbs, OTC and alcohol.
Nasal packing should be avoided because frequently resumes when packing is removed.
Avoid all injections.
Invasive procedures should be minimized or performed after administration of factor replacement.
After surgical procedure, assess incision site frequently.
Transfuse factor VIII or IX as prescribed.
Caused by deficiency of von Willebrand factor (vWF) which is necessary for Factor VIII activity.
VWF is also necessary for platelet adhesion at the site of vascular injury
Bleeding is mixed in nature
VON WILLEBRAND’S DISEASE
Clinical Manifestations of VON WILLEBRAND’S DISEASE
Mucocutaneous i.e. nosebleeds, excessively heavy menses
Deep-tissue bleeding i.e. hemarthrosis, CNS bleeding
Implementation for VON WILLEBRAND’S DISEASE
Similar with Hemophilia
Transfuse cryoprecipitate (factor VIII, fibrinogen, factor XIII)
Tranfuse fresh frozen plasma
is not a disease but sign of underlying condition
Massive amount of tiny clots forms in the microcirculation.
As the platelets and clotting factors are consumed to form the microthrombi.
Paradoxical effect of excessive clotting is bleeding
DISSEMINATED INTRAVASCULAR COAGULATION
Risk Factors for DIC
Sepsis
Obstetric Complications (Abruptio Placenta)
Acute Hemolysis (transfusion reactions)
Trauma
Shock
Cancer (especially cancer and acute promyelocytic leukemia)
Allergic Reactions
Clinical Manifestations of DIC
bleeding from venipuncture sites, GIT, GUT
bleeding can range from occult internal bleeding to profuse hemorrhage from all orifices
organ dysfunctions i.e renal failure, pulmonary infarctions, as a result of microthromboses
progressive decrease in platelet count, clotting factors
increased PT, pTT, and FDP
