Module 8A: Chronic Hematologic Immunologic Conditions Part 1

A retrovirus replicated and overwhelms the CD4 cells and causes immunosuppression, making people more susceptible to infections.

Human immunodeficiency virus

What are risk factors for HIV?

sexually transmitted diseases

IV drug use

pregnant/breastfeeding women to child

What are the ways that HIV is transmitted?

Sexual transmission

Contact with blood perinatally

Contact with blood, semen, vaginal secretions, breastmilk

At what point do immune problems arise with HIV?

when CD4+ counts drop to less than 500 T cells/μL

In the acute infection phase (weeks 1-3) for HIV, what are symptoms like?

Mononucleosis-like symptoms; fever, soreness, swollen lymph nodes, sore throat, malaise, nausea/vomiting, headache, diarrhea, rash

During weeks 1-3 of an HIV infection, how infectious is the individual?

HIGHLY infectious

After week 3 of an HIV infection, what are symptoms like for weeks 3-3 months?

Asymptomatic

When do HIV patients typically become symptomatic again after being asymptomatic for weeks 3 - 3 months?

When CD4+ T cells drop to 200 - 500 cells/μL

What are symptoms an HIV patient may have when they get an infection after the asymptomatic phase?

fever, night sweats, diarrhea, headaches, fatigue

persistent shingles, herpes flares, bacterial/yeast infections

When is someone classified as having AIDS?

CD4+ count is less than 200 cells/μL

When the immune system becomes severely compromised due to HIV; severe infections, malignancies, wasting, cognitive changes

Acquired Immunodeficiency Syndrome

Enzyme linked immunosorbant Assay test; used to screen for HIV

ELISA

What is used to monitor HIV progression and provides a marker of immune function?

CD4+ cell count

What does a lower viral load suggest?

The HIV is less active

What may cause abnormal blood tests in HIV?

HIV itself, opportunistic diseases, or complications of therapy

What are examples of altered lab values that may be seen in a patient with HIV?

Decreased WBC counts, especially

• Lymphopenia

• Neutropenia

• Low platelet counts (thrombocytopenia)

• Anemia is associated with ART

• Altered liver function

If a patients CD4+ count is between 350-500 cells/μL, what may be seen?

increased respiratory illnesses or dermatologic symptoms (herpes)

If a patients CD4+ count is between 200-350 cells/μL, what may be seen?

increased infection, fatigue, fever, or severe bacterial infection

If a patients CD4+ count is below 200 cells/μL, what may be seen?

opportunistic and rarer infections such as PCP, cryptococcal meningitis, candida, CMV, toxoplasmosis

Occurs as CD4+ count increases and the immune system is able to respond to the presence of previously acquired opportunistic infections; response is an overwhelming inflammatory response

Immune reconstructive inflammatory response (IRIS)

What is the biggest benefit of early HIV diagnosis?

early ART initiation

What are the 3 things monitored in collaborative HIV treatment?

Monitor disease progression, immune

function, and symptom management

What are the main treatment goals for HIV?

Decrease viral load

Maintain and increase CD4+ count

Prevent HIV symptoms and OIs

Delay disease progression

Prevent transmission

What is the purpose of prophylactic medications in HIV?

reduce the risk of opportunistic infections such as toxoplasmosis and PCP

What is indicated for ALL HIV+ patients?

Antiretroviral therapy (ART)

What is critical to reduce viral resistance in HIV?

100% adherence to ART for lifetime

Although HIV patients should be kept up to date on immunizations, what vaccines are contraindicated?

Live virus vaccines

a combination of the drugs tenofovir and emtricitabine used to reduce the risk of acquiring HIV.

Truvada/pre-exposure prophylaxis (PrEP)

Common nursing diagnoses for HIV patients

Risk for infection

Imbalanced nutrition

Risk for fluid volume deficit

Anxiety r/t disease progression

Knowledge deficit r/t self care and disease process

What nursing interventions should be implemented with HIV patients?

Utilize universal precautions consistently

Administer ART as prescribed and on time

Provide nutritionally dense foods and small, frequent meals

Refer for social services evaluation

What patient educations should be provided for HIV patients?

Avoidance of high-risk behaviors that increase the risk of transmission

Adherence to the treatment regimen

Implementing infection-control precautions at home

Signs and symptoms to report to the healthcare provider urgently

Health maintenance needs

What may be causes of anemia due to decreased RBC production?

Deficient nutrients (iron, b12, folic acid), decreased erythropoietin, decreased iron availability

What may be causes of anemia due to blood loss?

Chronic issues such as a bleeding ulcer, colorectal cancer, or liver disease

Acute issues such as trauma, ruptured aneurysm, or GI bleed

What may be causes of anemia due to increased RBC destruction?

Sickle cell disease

Medications

Incompatible blood

Trauma

Anemia in which decreased formation of hemoglobin on the RBCs leads to inadequate oxygenation of the body's tissues, or hypoxia

Iron deficiency anemia

What groups is iron deficiency anemia most prevalent in?

non-caucasian americans of lower socioeconomic status

menstruating and pregnant women

How is iron deficiency anemia diagnosed?

through blood tests; CBC to check ferritin

What are causes for iron deificiency anemia

inadequate intake

malabsorption

blood loss

hemolysis

How is iron deficiency anemia treated?

Diet adjustments

Iron supplementation

What are physical manifestations of iron deficiency anemia?

pallor, Cheilitis, headaches, SOB, palpitations

What is megaloblastic (vit B12) anemia caused by?

decreased erythrocyte production

What are clinical manifestations of megaloblastic (vit B12) anemia?

• Neurological and psychiatric dysfunctions: peripheral

neuropathy

• Lhermitte sign

• Sore, red, beefy and shiny tongue

If someone is having new onset numbness/tingling, what kind of anemia might it be related to?

megaloblastic (vit B12) anemia

When there is neck flexion and the patient experiences an electric shock feeling down their spine; manifestation of megaloblastic (vit B12) anemia

Lhermitte sign

What is management for megaloblastic (vit B12) anemia?

Look at H&P and CBC, get on B12 supplementation

What is the most common cause of vitamin B12 deficiency?

pernicious anemia; due to absence of intrinsic factor

can also occur due to gastric bypass, small bowel resection

What is a gastric bypass patient on for the rest of their life typically?

vitamin B12 injections

What forms a complex pathway that is involved in DNA synthesis and is essential for cell maturation, embryonic neural tube development, and formation of heme for RBC maturation and synthesis of neurotransmitters

Folic acid

What are clinical manifestations of Megaloblastic Anemia caused by Folic Acid (Vit. B9) deficiency?

GI problems: stomatitis, cheilosis, dysphagia, flatulence, and diarrhea

What is management for Megaloblastic Anemia caused by Folic Acid (Vit. B9) deficiency?

identify etiology of the anemia and supplement folic acid

Primary or secondary disorder in which there is a mutation in the JAK2 gene, stimulating the overproduction of RBCs, WBCs, and platelets

Polycythemia

What is secondary polycythemia related to?

hypoxia

cardiopulmonary, COPD, OSA patients at risk!

What do increased RBCs in polycythemia cause?

blood becomes more viscous

What are clinical manifestations of polycythemia?

Headache, dizziness, weakness, splenomegaly, shortness of

breath, difficulty breathing when lying flat, and blurred vision

When is polycythemia often discovered?

during routine blood tests

What are the goals of treatment with polycythemia?

reduce blood hyperviscosity and prevent hemorrhage

Hereditary clotting disorder caused by females on the X chromosome

Hemophilia

Clotting factor VIII is deficient in...

Hemophilia A

Clotting factor IX is deficient in...

Hemophilia B/Christmas disease

Deficiency of von willebrand coagulation protein on Factor VIII; most common congenital bleeding disorder

Von willebrand disease

What can happen with dental work/toothbrushing in hemophilia patients?

uncontrollable hemorrhage

What can cause GI bleeding in hemophilia?

ulcers and gastritis

What are clinical manifestations of hemophilia?

• Slow, persistent, prolonged

bleeding

• Delayed bleeding after minor

injuries

• Uncontrollable hemorrhage with

dental work or toothbrush

• Epistaxis

• GI bleeding

Hematuria and potential renal failure

Ecchymosis and subcutaneous

hematomas

Compartment syndrome

Neurologic signs

Hemarthrosis

Bleeding in the muscles and joints; classic sign of hemophilia

Hemarthrosis

What is tested for in each type of hemophilia?

• Hemophilia A: clotting factor VIII

• Hemophilia B: clotting factor IX

• von Willebrand disease: vWF

What other labs should be checked with hemophilia?

PTT/APTT: will be prolonged

Platelets: will be normal

What is treatment of hemophilia A/B focused on?

Replacement of factor VIII for Hemo. A; factor IX for

hemophilia B

If a hemophilia pt has a minor injury, what will treatment consist of?

3 daily treatments

If a hemophilia pt has a major injury, what will treatment consist of?

7-10 days of treatment

What is the largest complication of hemophilia?

hemorrhage

Condition in which there is a decreased number of platelets below 150,000/mm3

Thrombocytopenia

What are 3 types of thrombocytopenia

• Immune thrombocytopenia (ITP)

• Thrombotic thrombocytopenic purpura (TTP)

• Heparin-induced thrombocytopenia (HIT)

What can cause thrombocytopenia?

decreased production in bone marrow, increased platelet destruction, increased consumption of platelets

Acquired thrombocytopenia due to decreased platelet production; lupus and infections such as HIV can contribute

Immune thrombocytopenia (ITP)

Uncommon kind of thrombocytopenia associated with hemolytic ureic syndrome; enhanced platelet aggregation causes microthrombi to deposit in arterioles and capillaries

Thrombotic thrombocytopenic purpura (TTP)

What can cause TTP?

Preeclampia, infection, drug toxicities, pregnancies, known autoimmunes such as lupus or scleroderma

Thrombocytopenia that occurs 5-14 days after initiating heparin therapy; suspected if platelet counts decrease by over 50% or to less than 150,000

Heparin-induced thrombocytopenia (HIT)

What is the major issue with HIT?

venous and arterial thrombosis

What are complications of HIT?

DVT and PE

What might a bone marrow aspiration and biopy be used for in thrombocytopenia?

to assess platelet production

What are diagnostic studies and labs for thrombocytopenia?

• History and physical exam

• Bone marrow aspiration and biopsy

• CBC with platelet count

• Specific lab test depending on type

What is the primary intervention when a patient is found to have HIT?

stop all forms of heparin administration

If someone has profound thrombocytopenia, what may be used to increase platelet counts by decreasing anti-platelet antibody production?

Glucocorticoids

When can warfarin be started in HIT patients?

when platelets reach 150,000

In severe cases what can improve thrombocytopenia?

Surgery: splenectomy or clot removal

Plasmapheresis

Why are platelet transfusions discouraged in HIT?

they can increase thrombus formation

What are approved anticoagulants for HIT?

direct thrombin inhibitor (e.g.,

Argatroban), indirect thrombin inhibitor (fondaparinux [Arixtra])

What is the major complication of thrombocytopenia?

hemorrhage, spontaneous bleeding

What should be implemented for thrombocytopenia patients?

bleeding precautions

Condition in which there is a disruption of the clotting cascade as a secondary problem; a serious bleeding AND thrombotic disorder

Disseminated intravascular coagulation

What can cause DIC?

injury, infection, or illness

shock, sepsis, malignancies, transfusion reactions

Why does bleeding happen in DIC if the problem is excessive thrombi forming?

when all the platelets and clotting factors are exhausted, causes spontaneous bleeding from nearly every oriface

What causes tissue necrosis in DIC?

multiple emboli, leading to multi system organ dysfunction

What are diagnostic testing and lab values seen in DIC?

Decreased fibrinogen level and elevated fibrin split product. Increased fibrin-degradation products – D-Dimer, prolonged aPTT and PT,

thrombocytopenia

What is the treatment for DIC?

Resolve underlying condition that causes DIC; administer blood, platelets, fresh frozen plasma, vitamin K, and cryoprecipitate

What should be given carefully via IV infusion in DIC, as it may or may not help?

heparin

What is a major complication of DIC?

hemorrhage; intravascular clotting has used up all the platelets

What are general nursing interventions for hemophilia, thrombocytopenia, and DIC?

• Minimize blood loss from lacerations or venipunctures

• Avoid intramuscular injections

• Avoid rectal temperatures, enemas, suppositories, douches

• Provide a safe environment (clutter-free)

• Use minimal inflation when assessing blood pressure

• Minimize/couple blood draws

What patient education should be provided for patients with hemophilia, thrombocytopenia, and DIC?

• Instruct patient/family on bleeding precautions

• Instruct patient to avoid sexual intercourse when the platelet count is less

than 50,000/mm3

• Explain the necessity of frequent CBCs, laboratory tests

Connective tissue disorder characterized by fibrotic, degenerative, and sometimes inflammatory changes in the blood vessels, skin, synovium, skeletal muscles, and internal organs

Scleroderma