Blood and blood disorders stuff

BLOOD

Serum = plasma minus fibrinogen

Albumin- provides osmotic pressure
(low albumin----edema)
-binds hormones and drugs

γ-globulin-antibodies (=humoral immunity)


BLOOD CELLS

Erythrocytosis -lack of oxygen: high altitude, COPD
Leukocytosis -infections
-leukemia

Neutrophils -infection, acute stress
Eosinophils -allergies, parasites
Lymphocytes –viral infections, TBC

Leucopenia -radiation
-bone marrow suppression

Thrombocytopenia -DIC
-bone marrow suppression


RED BLOOD CELLS

Hematocrit -percentage of RBCs in blood volume
-male adult: 40-50%
-female adult: 35-45%

Hemoglobin -oxygen carrying protein in red blood cells
-male adult: 14-16 g/dl
-female adult: 12-15 g/dl

Hematocrit may be false low if blood is obtained with capillary fingerstick (“milking”)

Direct Coombs’ test -test for antibodies on patient’s erythrocytes
Positive if: -hemolytic transfusion reaction (mismatch)
-autoimmune hemolytic` anemia
-erythroblastosis fetalis (maternal antibodies bind to fetal RBCs→hemolysis)

Indirect Coombs’ test -test for antibodies in patient’s serum
Positive if: -sensitization from previous transfusion
-Rh sensitization from previous pregnancy




ANEMIAS

Acute blood loss -hematocrit remains normal in acute phase!

Chronic blood loss -may lead to iron deficiency

Iron deficiency -search for occult bleeding, especially in elderly

Vit. B12 or folate deficiency -required for RBC maturation in bone marrow


-Alcoholics:
B12 and folate deficiency common

-Pregnancy:
Folate deficiency common (supplementation!)

Pernicious anemia -chronic gastritis type A (autoimmune disease)
-antibodies against intrinsic factor from stomach→reduced vit. B12 absorption in small bowels

Sickle cell anemia -abnormal hemoglobin (electrophoresis)
-“sickle cells” seen on blood smear
-painful crises, leg ulcers

Thalassemias -abnormal hemoglobin (electrophoresis)
-“target cells” on blood smear

Hemolysis -antibodies against RBCs
-fragile RBCs

Blood hemolysis→increased serum bilirubin!

ASSESSMENT
Fatigue
Pale skin and mucosa
ALL ANEMIAS:
-Check CBC, blood smear, hematocrit, hemoglobin and iron.
APLASTIC ANEMIA:
-Low reticulocyte count indicates decreased production of RBCs.
THALSSEMIA, SICKLE CELL ANEMIA:
-Hemoglobin electrophoresis shows abnormal hemoglobins
PERNICIOUS ANEMIA:
-Schilling test: to evaluate vitamin B12 absorption

ANALYSIS
-Skin integrity?
-Delayed wound healing?
-Effective gas exchange



IMPLEMENTATION
-Provide rest periods to avoid fatigue
-Assist with blood transfusions
-Monitor for transfusion reactions: pruritus, chills, fever, shock


BLOOD PRODUCTS
USED FOR:
Whole blood -active bleeding, massive transfusions (packed RBC are preferred; less volume load)
Fresh frozen plasma -bleeding patient with coagulation deficiency:
-chronic lever disease
-alcoholics
-DIC
Cryoprecipitate -DIC (to replace fibrinogen)

Factor VIII or IX concentrate -hemophilia (to replace missing factors)

TRANSFUSION RISKS:

ABO incompatibility 1:33,000
Febrile reaction 1:200
HIV 1:500,000
Hepatitis B or C 1:60,000

Most fatal transfusion reactions are due to a mismatch caused by clerical error!!!!


TRANSFUSION REACTIONS

ASSESSMENT
HEMOLYTIC REACTION (10-20 minutes):
-chills, headache
-lower back pain
-flushing, “head feels full”
-signs of shock
-acute renal failure (oliguria)
FEBRILE REACTION (30 minutes):
-chills headache
-elevated temperature
ALLERGIC REACTION:
-Pruritus
-Hives, wheezes
-Anaphylaxis


IMPLEMENTATION
HEMOLYTIC OR FEBRILE REACTION:
-immediately stop transfusion
-keep IV access-replace transfusion with normal saline
-call physician
ALLERGIC REACTION:
-if hives are the only sign: proceed at slower rate (check institutional guidelines!)
-prepare epinephrine if signs of anaphylaxis occur

Start infusion slowly (2ml/min) to observe for early reaction. Stay at bedside.

ACUTE LEUKEMIA

ALL-acute lymphoblastic leukemia (3-7 years)
(prognosis is fair)
-fatigue, weakness, anorexia
-fever
-petechiae (numerous tiny bruises (size of pinheads)
-ecchymoses (large area bruises)

AML-acute myeloblasic leukemia (all ages)
(prognosis is poor)
-fatigue, weakness, anorexia
-fever
-petechiae
-ecchymoses
-lymphadenopathy, splenomegaly
-Auer rods in myeloblasts

ANALYSIS
-risk of injury and infections
-effective coping?

IMPLEMENTATION
-monitor vital signs
-watch for bleeding
-watch for signs of infection
-minimize side effects of chemotherapy

Bleeding and infection are the major causes of death.


CHRONIC LEUKEMIA
CML-chronic myelocytic leukemia (50 years)
(prognosis is poor)
-fatigue, weakness, anorexia
-fever
-night sweats
-splenomegaly
-Philadelphia chromosome

CLL-chronic lymphocytic leukemia (70 years)
(prognosis is fair)
-insidious onset
-few symptoms
-low Ig levels
-infections



ANALYSIS
-risk of injury and infections
-effective coping?

IMPLEMENTATION
-monitor vital signs
-watch for bleeding
-watch for signs of infection
-minimize side effects of chemotherapy

BLEEDING DISORDERS
Platelet defect -bleeding into skin and mucous membranes
-males and females
Coagulation defect -bleeding into joints, muscle, viscera
-mainly males
Vascular defect -purpura (bleeding into skin)
-gastrointestinal bleeding
-mainly females

Prolonged use of a tourniquet before drawing blood sample may falsely increase PTT and PT!


THROMBOCYTOPENIA

Platelets<100,000/μL increased bleeding risk
Platelets<20,000/ μL spontaneous bleeding
Platelets<10,000/ μL CNS bleeding

CAUSES
Bone marrow failure -viral infections
-drugs
-radiation
Platelet destruction -ITP
Platelet consumption -TTP
-DIC
Platelet sequestration -splenomegaly

ASSESSMENT
-history of drugs/medication intake
-fatigue
-easy bruising: petechiae, ecchymoses
-bleeding from mucosal surfaces

IMPLEMENTATION
-prevent avoidable injury
-avoid invasive procedures if possible
-apply pressure to venipuncture site until bleeding stops

CLIENT EDUCATION
-avoid aspirin (suppresses platelet activity)
-avoid coughing and straining (increased BP increases risk of CNS bleeding)

HEMOPHILIA

Hemophilia A -factor VIII deficiency
-X-linked recessive
Hemophilia B -factor IX deficiency
-X-linked recessive
von Willebrand’s -deficiency of von Willebrand Factor
-autosomal recessive or dominant

ASSESSMENT
• Excessive bruising
• Prolonged bleeding after minor injuries
• Nosebleeds
• Hemarthrosis (bleeding into joints: elbows, knees, ankles)
IMPLEMENTATION
• When bleeding: Elevate and apply pressure for 10-20 minutes
• Avoid taking rectal temperatures
• Treat hemarthroses early to preserve mobility of joint (elevation, ice packs, may require splinting)
MEDICATIONS:
• Replace coagulation factors VIII or IX (heat-treated to inactivate viruses, or recombinant)
CLIENT EDUCATION
• Avoid trauma whenever possible
• Never use aspirin (inhibits platelets and worsens bleeding)
GENETIC COUNSELING:
• X-linked recessive disease: -transmitted by asymptomatic female carriers
-50% of male offspring will have disease
-50% of female offspring are carriers

DIC
(Disseminated Intravascular Coagulation)

Intravascular activation of coagulation causes consumption of coagulation factors. This results in thrombosis or bleeding tendency or both and is a very serious condition.

CAUSES OF DIC:
Gram negative sepsis
Adenocarcinomas
Crash injury
Amniotic fluid embolism

ASSESSMENT
LAB:
• PT and PTT prolonged
• Thrombocytopenia
• Decreased fibrinogen
• Increased fibrin split products
IMPLEMENTATION
• Identify and treat underlying disorder!
• Observe fro bleeding
• Monitor platelet count
• Assist with platelet transfusion (increases platelet count)
cryopreciptate (increases fibrinogen)
fresh frozen plasma(increases clotting factors)


LEUKEMIA

Leukemia is a general term used to describe a group of malignant disorders affecting the blood and blood-forming tissues of the bone marrow, lymph system, and spleen. It results in an accumulation of dysfunctional cells because of a loss of regulation in cell division. Although often thought of as a disease of children, the number of adults affected with leukemia is 10 times that of children.
Regardless of the specific type, there is generally no single causative agent in the development of leukemia. Most leukemias result from a combination of factors including genetic and environmental influences.

Acute lymphocytic leukemia (ALL) is the most common type of leukemia in children and accounts for 15% of acute leukemia in adults. In ALL< immature lymphocytes proliferate in the bone marrow. Fever is present in the majority of patients at time of diagnosis. Signs and symptoms may appear abruptly with bleeding or fever, or they may be insidious with progressive weakness, fatigue, and bleeding tendencies.

Acute myelogenous leukemia (AML) represents only one fourth of all leukemias, but it makes up approximately 85% of the acute leukemias in adults. Its onset is often abrupt and dramatic. A patient may have serious infections and abnormal bleeding from the onset of the disease.

Chronic lymphocytic leukemia (CLL) is characterized by the production and accumulation of functionally inactive but long-lived, mature-appearing lymphocytes. The lymphocyte involved is usually the B cell. Lymph node enlargement (lymphadenopathy) throughout the body is present and there is an increased incidence of infection. Because CLL is usually a disease of older adults, treatment decisions must be made by considering disease progression and treatment of side effects. Many individuals in the early stages of CLL require no treatment.

Chronic myelogenous leukemia (CML) is a clonal stem cell disorder characterized by excessive neoplastic granulocytes in the bone marrow and the presence of the Philadelphia chromosome (a cytogenetic abnormality). The chronic phase of CML can last for several years and can usually be well controlled with treatment.

Hodgkin’s disease, which makes up 15% of all lymphomas, is a malignant condition characterized by proliferation of abnormal, giant, multinucleated cells called Reed-Sternberg cells, which are located in the lymph nodes. The disease has a bimodal age-specific incidence, occurring most frequently in persons from 15-35 years old and over 50 years old. In adults, it is twice as prevalent in men as in women.

Non-Hodgkin’s lymphomas are a heterogeneous group of malignant neoplasms of the immune system that affect all ages. They are classified according to the different cellular and lymph node characteristics. NHLs can originate outside the lymph nodes, and the method of spread can be unpredictable. The majority of patients have widely disseminated disease at the time of diagnosis. (AKA; Burkitt’s lymphoma, reticulum cell sarcoma and lymphosarcoma.)

Human immunodeficiency virus (HIV) infection follows a highly individualized course from the time of infection to the clinical manifestations of acquired immunodeficiency syndrome (AIDS). More than 34 million people worldwide are infected with HIV; AIDS is the final phase of a chronic, progressive immune function disorder caused by HIV. HIV is an RNA virus. It is called a retrovirus because it replicates in a “backward” manner, going from RNA to DNA. Like all viruses, HIV cannot replicate unless it is in a living cell. HIV infects human cells that have CD4 receptors on their surfaces. These include lymphocytes, monocytes/macrophages, astrocytes, and oligodendrocytes. Immune dysfunction in HIV disease is caused predominantly by destruction of CD4+Tcells (known as T-helper cells or CD4+lymphocytes). The major concern related to immune suppression is the development of opportunistic diseases (infections and cancers that occur in immunosuppressed patients that can lead to disability, disease, and death).