P ROBLEMS OF HEMOSTASIS Megan McClintock, MS, RN Fall 2011

T HROMBOCYTOPENIA Platelets < 150,000 Causes – inherited or acquired (pg ) 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

I MMUNE T HROMBOCYTOPENIC P URPURA (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

T HROMBOTIC T HROMBOCYTOPENIC P URPURA (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!!!

H EPARIN -I NDUCED T HROMBOCYTOPENIA (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

S IGNS AND S YMPTOMS **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

T REATMENT 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)

N URSING C ARE /T EACHING 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

N URSING 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

H EMOPHILIA 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)

S IGNS & S YMPTOMS 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)

C OMPLICATIONS 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

N URSING C ARE /T EACHING 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

D ISSEMINATED I NTRAVASCULAR C OAGULATION (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)

S EQUENCE OF EVENTS

S IGNS & S YMPTOMS 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

D IAGNOSIS & T REATMENT 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)

N URSING C ARE 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)

N EUTROPENIA 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

S IGNS & S YMPTOMS 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

N URSING C ARE Monitor closely for infection and early septic shock If fever 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

L EUKEMIA 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

S IGNS & S YMPTOMS Vary (pg 695) Anemia Thrombocytopenia Neutropenia Late in disease can have: Splenomegaly Hepatomegaly Lymphadenopathy Bone pain Meningeal irritation Oral lesions

D IAGNOSIS & T REATMENT 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

N URSING C ARE 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

H ODGKIN ’ S L YMPHOMA 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

D IAGNOSIS 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)

T REATMENT & N URSING C ARE 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

N ON -H ODGKIN ’ S L YMPHOMA 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

D IAGNOSIS, S TAGING & T REATMENT 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

M ULTIPLE M YELOMA 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

T REATMENT AND N URSING C ARE 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

S PLENOMEGALY 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