Problems of hemostasis

Report
PROBLEMS OF HEMOSTASIS
Megan McClintock, MS, RN
Fall 2011
THROMBOCYTOPENIA
Platelets < 150,000
 Causes – inherited or acquired (pg 678-679)
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Immune – ITP
Shortened circulation – TTP, DIC, HIT, splenomegaly
Turbulent blood flow – hemangiomaa, valve problems
Decreased production – chemotherapy, viral infxn,
sepsis, alcoholism, aplastic anemia, malignancy,
radiation
Food, Drug, Herbal causes – pg 679
IMMUNE THROMBOCYTOPENIC PURPURA
(ITP)
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Most common acquired thrombocytopenia
Autoimmune disease
Platelets survive < 8 days
Gradual onset with transient remissions
Tx
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Nothing if asymptomatic
Corticosteroids
Splenectomy
IVIG
Thrombopoietin receptor agonists
Platelet transfusions (only for life-threatening hemorrhage
Amicar (antifibrinolytic) for severe bleeding
Avoid ASA and other meds that affect platelets
THROMBOTIC THROMBOCYTOPENIC PURPURA
(TTP)
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Medical emergency!!!
Uncommon syndrome with clumping of platelets,
leading to microthrombi in small vessels
Almost always associated with HUS
Causes – idiopathic, drug toxicity,
pregnancy/preeclampsia, infxn, autoimmune
disorders
S/S – hemolytic anemia, thrombocytopenia, neuro
probs, fever with no infxn, renal probs
Tx
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Stop the underlying disorder, remove causative agent
Plasmapheresis daily
Corticosteroids
Immunosupressants (ie. Cytoxan, cyclosporine)
Splenectomy
No platelet transfusions!!!
HEPARIN-INDUCED THROMBOCYTOPENIA
(HIT)
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Patho – formation of abnormal antibodies that activate
platelets
Major complication is venous thrombosis (DVT, PE)
Other complications – stroke, kidney damage, MI
S/S – rarely have bleeding (platelets stay above 60,000),
new or worsening thrombosis, decreased platelet count
Tx – have to protect from thrombosis and not reduce the
platelet count further (no warfarin, no platelet transfusion)
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Stop heparin (even line flushes)
Maintain anticoagulation with direct thrombin inhibitors
Only start Coumadin if platelets > 150,000
For severe clotting, plasmapheresis, protamine sulfate,
thrombolytics, surgery to remove clots
No platelets
Never give heparin to them again
SIGNS AND SYMPTOMS
**Usually asymptomatic (unless platelets <
50,000)
 Bleeding (nosebleeds, gums, petechiae, purpura,
bruising)
 Prolonged bleeding after routine procedures
 May have normal PT and aPTT
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Complications
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Spontaneous hemorrhage (platelets < 20,000)
Internal bleeding
Vascular thrombosis
TREATMENT
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Platelet transfusions only if platelets < 10,000
OR active bleeding
Acquired (usually from another underlying
condition or therapy – ie. Leukemia)
Identify the cause and treat the disease
 Remove the causative agent
 If cause unknown, corticosteroids
 Platelet growth factor and thrombopoietin
(stimulates the bone marrow)
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NURSING CARE/TEACHING
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Discourage use of OTC meds that can cause
thrombocytopenia (ie. ASA, NSAIDs)
Teach pt to notify dr even for minor nosebleed, gum
bleeding, new petechiae
Avoid injections if possible
If injections unavoidable, use small-gauge needles for
injections and apply pressure for 5-10 minutes
Suppression of menses
Soft toothettes
No lemon glycerin swabs
Soft, bland, non-acidic foods
Use electric razor
Avoid high-impact activities
NURSING CARE/TEACHING (CONT.)
Blow nose gently
 Prevent constipation
 Do not pluck body hair
 No tattoos or body piercing
 No tampons
 Check with dr before invasive procedures (ie.
Dental cleaning, manicure, pedicure)
 Call dr for black BMs, black vomit or urine,
bruising, petechiae, bleeding, headache, change
in vision, stroke symptoms
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HEMOPHILIA AND VON WILLEBRAND
DISEASE
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X-linked recessive genetic disorder caused by
defective or deficient coagulation factor
*Hemophilia A (classic or Factor VIII deficiency)
 Hemophilia B (Christmas disease, Factor IX
deficiency)
 von Willebrand disease (deficiency of von Willebrand
coagulation protein)
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Hemophilia is transmitted by female carriers but
displayed almost exclusively in men (von
Willebrand is seen in both genders)
SIGNS & SYMPTOMS
Slow, persistent, prolonged bleeding from minor
trauma
 Delayed bleeding after minor injuries
 GI bleeding from ulcers and gastritis
 Subcutaneous hematomas that can lead to nerve
compression
 Hemarthrosis leading to joint injury/deformity
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TREATMENT
Preventive care!!!!
 Replacement of deficient clotting factors (not
FFP) with active bleeding and before surgery
 DDAVP (IV, SC, Intranasal) for minor bleeding
and dental procedures
 Antifibrinolytics (Amicar) with oral bleeding,
nosebleeds, menses
 Gene therapy (experimental)
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COMPLICATIONS
Development of inhibitors to factors VIII or IX
 Transfusion-transmitted infectious diseases (ie.
HIV, Hep B, Hep C)
 Allergic reactions
 Thrombotic complications with factor IX
 With von Willebrands, can develp alloantibodies
 Starting factor replacement too late
 Stopping factor replacement too soon
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Treat minor bleeding for 72 hours, longer for surgery
or traumatic injury
NURSING CARE/TEACHING
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Genetic counseling
Stop topical bleeding as quickly as possible (Gelfoam, fibrin
foam, thrombin)
Administer specific coagulation factor
If joint bleeding, totally rest the joint, pack in ice, treat
pain without NSAIDs/ASA, once bleeding stops, ROM and
PT; no weight bearing until healed
Watch for life threatening complications
Refer to local chapter of National Hemophilia Society
Immediate medical attention for severe pain/swelling of
muscles/joints, head injury, swelling in neck/mouth,
abdominal pain, hematuria, melena, skin wounds
Careful daily oral hygiene
Non contact sports only
Wear gloves when doing household chores
Wear a Medic Alert tag
DISSEMINATED INTRAVASCULAR
COAGULATION (DIC)
Complex systemic thrombohemorrhagic disorder
 Clotting is abnormally initiated and accelerated
using up all of the clotting factors and platelets
leading to uncontrollable bleeding (consumptive
coagulopathy)
 Not a disease, but a complication
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Always secondary to an underlying disorder (most
common is septic shock and trauma) (pg 687)
Almost always causes organ failure
 Can also have chronic DIC in which the body
compensates (seen in malignancies, autoimmune
diseases)
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SEQUENCE OF EVENTS
SIGNS & SYMPTOMS
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No well-defined sequence of events
Unexplained bleeding
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Pallor, petechiae, purpura
Oozing blood
Hematomas
Weakness
Malaise
Fever
Figure 31-9 (pg 687)
Respiratory – tachypnea, hemoptysis, orthopnea
GI – bleeding, abd distension, bloody stools
Urinary – hematuria
Neuro – vision change, dizziness, headache, change in LOC,
irritability
MS – bone/joint pain
Thrombotic s/s – cyanosis, necrosis, PE, ARDS, ECG changes,
paralytic ileus, oliguria
DIAGNOSIS & TREATMENT
D-dimer assay is the best test (increased)
 FSPs (fibrin split products) increased
 FDPs (fibrin degradation products) increased
 Schistocytes on blood smear
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Treatment is controversial
If chronic and no bleeding, no treatment
 If bleeding, provide support with blood products and
treat the primary disorder
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Only use platelets, cryoprecipitate, FFP if life-threatening
hemorrhage
If signs of thrombosis, use heparin (controversial)
NURSING CARE
Identify the development of DIC quickly
 Early detection of bleeding and microthrombi
 Administer blood and blood products correctly
 Same care as for those with thrombocytopenia
(see previous slides)
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NEUTROPENIA
Reduction in neutrophils (have to know the
absolute neutrophil count – ANC)
 ANC < 1000, severe if ANC < 500
 Even more important is the rapidity of the
decrease of the ANC, degree of neutropenia, and
the duration
 Causes – clinical consequence of disease, side
effect of certain drugs
 *Most common cause is iatrogenic from
chemo/immunosuppressants
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SIGNS & SYMPTOMS
Risk of infection from pathogens and normal body
flora
 Won’t have the normal signs of infection (ie.
Redness, heat, swelling)
 *Even low grade fever (100.4 F or 38 C) is critical
 *Minor c/o pain or symptoms (ie. Sore throat,
ulcers in the mouth, diarrhea, vaginal discharge,
shortness of breath, cough) must be reported
immediately
 Commonly infected with bacteria, fungi, viruses
 Must have a differential count to confirm
neutropenia
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NURSING CARE
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Monitor closely for infection and early septic shock
If fever 100.4 or higher, draw two blood cultures and
start IV antibiotics immediately (w/in 1 hour)
Watch for fungal infxn if neutropenia prolonged
GCSF to prevent neutropenia
*Handwashing!!!!
Private room (maybe HEPA filtration)
No uncooked meat, pepper, unwashed fruits/veggies
Avoid crowds (wear a mask in public areas)
Bathe or shower daily
Don’t garden or clean up after pets
No fresh flowers in the room
LEUKEMIA
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Malignant disorders affecting blood and bloodforming tissues
Cause – unknown, genetics and environment play a
role
Classified as acute or chronic (r/t cell maturity and
disease onset), also by type of leukocyte involved
(myelogenous or lymphocytic)
AML – much more common in adults, abrupt onset, very
sick
 ALL – most common type in kids, usu. have fever, can be
abrupt or insidious, may have CNS symptoms
 CML – have Philadelphia chromosome, usu. has a chronic
stable phase and then an acute, aggressive phase leading
to death
 CLL – most common type in adults, B cells, lymph node
enlargement, can lead to non-Hodgkin’s lymphoma
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SIGNS & SYMPTOMS
Vary (pg 695)
 Anemia
 Thrombocytopenia
 Neutropenia
 Late in disease can have:
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Splenomegaly
Hepatomegaly
Lymphadenopathy
Bone pain
Meningeal irritation
Oral lesions
DIAGNOSIS & TREATMENT
Peripheral blood and bone marrow exam
determines type and subtype
 LP, CT to look for leukemic cells outside of blood
and bone marrow
 Combination drug therapy to treat during all cell
cycles, minimize drug toxicity, decrease drug
resistance
 With CLL, watchful waiting
 If high WBCs (>100,000), leukapheresis and
hydroxyurea to prevent stroke
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CHEMOTHERAPY
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Induction – aggressive tx to destroy leukemic cells
and bring about remission
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Pts can get critically ill
Intensification – high dose therapy given immediately
after induction for several months
Consolidation – eliminate remaining leukemic cells
that may not be clinically/pathologically evident
Maintenance – given every 3-4 weeks (rarely done in
AML)
May also give corticosteroids, radiation, intrathecal
meds (ALL), may prepare for bone marrow transplant
or hematopoietic stem cell transplant
NURSING CARE
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Many physical and psychosocial needs
Fear of unknown, death
 Stress with the abrupt onset of disease
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Provide hope
 Be an advocate (help them understand, ask
questions, manage side effects)
 Care for neutropenia, thrombocytopenia, anemia,
skin, GI issues, nutrition, etc. (discussed in MS I)
 Be knowledgeable of the drugs
 Refer to support groups
 Encourage vigilant follow-up care
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HODGKIN’S LYMPHOMA
Overgrowth of Reed-Sternberg cells in the lymph
nodes
 Cause – unknown, but genetics, exposure to
occupational toxins, and infxn with EBV and/or
HIV play a role
 Originates in one location but can infiltrate other
organs
 S/S – insidious, painless enlargement of cervical,
axillary, or inginual lymph nodes; weight loss,
fever, night sweats (B symptoms); after ingestion
of alcohol have rapid onset of pain at site of
disease; can have other symptoms based on site
of disease
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DIAGNOSIS AND STAGING
Peripheral blood analysis (microcytic,
hypochromic anemia, leukocytosis)
 Lymph node biopsy
 Bone marrow exam
 Xray exam (defines sites of the disease)
 CT & MRI (staging the disease)
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TREATMENT & NURSING CARE
Intensive chemo
 Do not need maintenance chemo once a remission
is achieved
 Have a high risk of developing a secondary
malignancy in the future (ie. AML, nonHodgkin’s lymphoma, solid tumor)
 Prognosis is better than most cancers
 Nursing care is similar to leukemia
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NON-HODGKIN’S LYMPHOMA
Originate in B-cells or T-cells (B-cells more
common)
 Cause – unknown, more common with HIV, those
who have had immunosuppressants, chemo, or
radiation; EBV infection
 No hallmark sign, but always involve
lymphocytes and look a lot like leukemia
 Burkitt’s lymphoma is the most highly aggressive
 S/S – can originate outside the lymph nodes, can
spread unpredictably, and have widely
disseminated disease at time of dx; *painless
lymph node enlargement; may have B symptoms
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DIAGNOSIS, STAGING & TREATMENT
Very similar to Hodgkin’s lymphoma, but may do
more diagnostic studies since NHLs are more
disseminated
 Same staging as Hodgkin’s but more focus on the
histologic subtype
 Prognosis is not as good as for Hodgkin’s
 Tx – chemo, radiation
 *More aggressive lymphomas are more
responsive to treatment and more likely to be
cured
 Many pts relapse several times
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MULTIPLE MYELOMA
Cancerous plasma cells infiltrate the bone
marrow and destroy bone, produce abnormal
amts of immunoglobulin
 Usually develops in older, African American men
 S/S – insidious, skeletal pain is late in the
disease, diffuse osteroporosis, hypercalcemia,
Bence Jones proteins in the urine, high protein
levels leading to renal failure, anemia,
thrombocytopenia, neutropenia
 High levels of beta-microglobulin and low levels
of albumin are associated with poor prognosis
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TREATMENT AND NURSING CARE
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Seldom cured
Chemo with corticosteroids
Ambulation
Adequate hydration
Weight bearing
Biphosphonates given IV monthly
Radiation therapy
Allopurinol and Lasix
Be watchful and careful to prevent pathologic
fractures
Braces
NSAIS with opioids
Recognition and treatment of infection
Dialysis due to myeloma-induced renal failure
SPLENOMEGALY
When enlarged it doesn’t work as well and can
lead to a decrease in blood cells
 S/S – may be asymptomatic, may have abd pain,
early satiety
 Splenectomy is done to increase blood cell count
or for splenic rupture
 Care – can affect lung expansion, cause
significant pain, cause anemia,
thrombocytopenia, leukopenia
 Post splenectomy can have immunologic
deficiencies leading to a lifelong risk for infection;
recommend Pneumovax, influenza vaccine
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