PROBLEMS OF HEMOSTASISMegan McClintock, MS, RNFall 2011

THROMBOCYTOPENIA Platelets < 150,000 Causes – inherited or acquired (pg 678-679)

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) Most common acquired thrombocytopenia Autoimmune disease Platelets survive < 8 days Gradual onset with transient remissions Tx

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) 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

Stop the underlying disorder, remove causative agent Plasmapheresis daily Corticosteroids Immunosupressants (ie. Cytoxan, cyclosporine) Splenectomy No platelet transfusions!!!

HEPARIN-INDUCED THROMBOCYTOPENIA (HIT) 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) 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

Complications Spontaneous hemorrhage (platelets < 20,000) Internal bleeding Vascular thrombosis

TREATMENT 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)

NURSING CARE/TEACHING 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

HEMOPHILIA AND VON WILLEBRAND DISEASE

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) 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

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)

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

Treat minor bleeding for 72 hours, longer for surgery or traumatic injury

NURSING CARE/TEACHING 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 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)

SEQUENCE OF EVENTS

SIGNS & SYMPTOMS No well-defined sequence of events Unexplained bleeding

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

Treatment is controversial If chronic and no bleeding, no treatment If bleeding, provide support with blood products

and treat the primary disorder 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)

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

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

NURSING CARE 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 Malignant disorders affecting blood and blood-forming

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

SIGNS & SYMPTOMS Vary (pg 695) Anemia Thrombocytopenia Neutropenia Late in disease can have:

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

CHEMOTHERAPY Induction – aggressive tx to destroy leukemic cells

and bring about remission 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 Many physical and psychosocial needs

Fear of unknown, death Stress with the abrupt onset of disease

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

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

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)

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, non-Hodgkin’s lymphoma, solid tumor)

Prognosis is better than most cancers Nursing care is similar to leukemia

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

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

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

TREATMENT AND NURSING CARE 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