NUR424: Chapter 34: Management of Patients with Hematologic Neoplasms

Essential Thrombocythemia

A stem cell disorder within the bone marrow that causes a marked increase in platelet production. Exact cause is unknown, and rarely evolves into acute leukemia. Affects women twice as often as men, and occurs later in life.

Chronic Myeloid Leukemia

Arises from a mutation in the myeloid stem cell. Patients with CML have an abnormal chromosome called the Philadelphia chromosome (Ph Chromosome).

Myelodysplastic Syndrome

MDS is a group of clonal disorders of the myeloid stem cell that causes dysplasia (abnormal development) in one or more of the cell lines. Primary MDS tends to be a disease of people older than 70: 30% of cases evolve into AML.

Acute Lymphocytic Leukemia (cont.)

Medical Management: Goal of treatment is to obtain remission without excess toxicity and with a rapid hematologic recovery so that additional therapy can be administered to promote full recovery. Nursing Management- do chemo first, support of chemo patient.

Lymphoma

Neoplasms of lymphoid origin: Usually start in the nodes, but can involve lymphoid tissue in the spleen, GI tract, liver or bone marrow. Hodgkin Lymphoma Non-Hodgkin Lymphoma.

Chronic Lymphocytic Leukemia (cont.)

Medical Management: Treatment now initiated early, in hopes to achieve complete remission. If it does not start early, treatment is initiated when symptoms are severe or when disease progresses. Nursing Management- education on treatment.

Non-Hodgkin Lymphoma (MORE)

Most involve B lymphocytes, only 5% involve T lymphocytes. In contrast to Hodgkin's, the lymphoid tissue involved are largely infiltrated with malignant cells in the entire body.

Assessment and Diagnostic Findings: Acute Myeloid Leukemia

Presenting CBC- blast cells hallmark, early wbc might be normal. Blast cells are immature cells that don't work the right way. Blood work is important. Clinical Manifestations.

Polycythemia Vera

Proliferative disorder of the myeloid stem cells. Bone marrow is hypercellular, and erythrocyte, leukocyte and platelet counts in peripheral blood are often elevated. Hematocrit can often exceed 60%.

Hodgkin's Lymphoma

Rare malignancy with high cure rate. More common in men. Two peaks of incidence: Early 20s and then again after age 55. Pathophysiology: Unicentric in origin- infiltrates a single node. Malignant cell is Reed Sternberg. Viral etiology is suspected.

Primary Myelofibrosis (cont,)

Rarest of the classic myeloproliferative disease. Average survival ranges from 3-10 years.

Acute Myeloid Leukemia

Results from a defect in the hematopoietic stem cell that differentiates into the myeloid cells. Variable prognosis, although patient age is a significant factor.

Acute Lymphocytic Leukemia

Results from an uncontrolled proliferation of immature cells, lymphoblasts) derived from the lymphoid stem cell. ALL most common in young children Boys > Girls. Peak incidence is 4 years old. Extremely responsive to treatment, 5 year event free- 80%.

Polycythemia Vera (comt.)

Risks include thrombosis complications (CVA, MI), and bleeding from dysfunctional platelets. Treatment includes phlebotomy, chemotherapeutic agents, platelet aggregation inhibitors, Interferon. Nursing Management: Education: risk factors for clots.

Secondary Myelodysplastic Syndrome

Secondary MDS can occur at any age and results from prior toxic exposure to chemicals, such as chemotherapy. Breast cancer treatment. Robin Roberts. Hallmark is lower CBC.

leukocyte:

one of several cellular components of blood involved in defense of the body; subtypes include neutrophils, eosinophils, basophils, monocytes, and lymphocytes (synonym: white blood cell [WBC]).

lymphoid:

pertaining to lymphocytes.

myeloid:

pertaining to nonlymphoid blood cells that differentiate into RBCs, platelets, macrophages, mast cells, and various WBCs.

stem cell:

primitive cell, capable of self-replication and differentiation into myeloid or lymphoid stem cell.

blast cell:

primitive leukocyte.

phagocytosis:

process of cellular ingestion and digestion of foreign bodies.

apoptosis

programmed cell death.

clonality (clone):

proliferation from same cell of origin so that descendent cells are identical to the cell of origin.

cytokines

proteins produced by leukocytes that are vital to regulation of hematopoiesis, apoptosis, and immune responses.

white blood cell (WBC):

synonym: leukocyte.

petechiae:

tiny capillary hemorrhages.

lymphocyte:

type of WBC involved in immune functions.

leukemia:

uncontrolled proliferation of WBCs, often immature.

Polycythemia Vera (cont.)

Symptoms include ruddy complexion and splenomegaly from increased blood volume and viscosity of the blood. Diagnostics: elevated erythrocyte mass, normal oxygen saturation level, enlarged spleen: JAK2 mutation.

Primary Myelofibrosis (cont.)

Symptoms may result from an often massively enlarged spleen, causing discomfort and early satiety. Other signs and symptoms include fatigue, pruritus, bone pain, weight loss, infection, bleeding, cachexia. Medical Management. Nursing Management- blood product and symptom managment.

HYDREA

TO REDUCE THE AMOUNT OF CELLS.

Multiple Myeloma (cont.)

The 5 year survival rate is 39% for newly diagnosed patients.The M protein is the marker used to check the disease progress.

Chronic Myeloid Leukemia cont.

The BCR-ABL oncogene gives stem cells instructions to make a dysfunctional protein called BCR-ABL tyrosine kinase, which leads to the development of CML.. Treatment-Tyrosine Kinase Inhibitor.

Chronic Myeloid Leukemia (cont)

The Ph chromosome is the result of a translocation of a cancer causing gene (oncogene) called the BCR-ABL gene- not in normal people but cancer patient do.

Chronic Myeloid Leukemia (cont.)

Tyrosine kinase inhibitor- Imatinib. Nursing Management. Patient adhering to medication regimen.

absolute neutrophil count:

a calculation of the number of circulating neutrophils, derived from the total white blood cells (WBCs) and the percentage of neutrophils counted in a microscope's visual field.

indolent neoplasm:

a slow-growing cancer that often remains localized or causes few symptoms.

monocyte:

large WBC that becomes a macrophage when it leaves the circulation and moves into body tissues.

leukopenia:

less-than-normal amount of WBCs in circulation.

neutropenia

lower-than-normal number of neutrophils.

thrombocytopenia:

lower-than-normal platelet count.

symptoms of Chronic Myeloid Leukemia

Doesn't generally interfere with the development of bone marrow. Low RBC- SOB, fatigue. Enlarged spleen- not as hungry because less space in the abdomen.

Multiple Myeloma

A malignant disease of the most mature form of B lymphocyte- the plasma cell. Myeloma is the second most common hematologic malignancy in the US.

Primary Myelofibrosis

Chronic myeloproliferative disorders that arises from neoplastic transformation of an early hematopoietic stem cell. Fibrous tissue replaces the normal bone marrow cells.

Stages of Chronic Myeloid Leukemia

Chronic- reduce symptoms of CML. Transformational- drugs stop working, this is were transplant is possible. Blast crisis leads to AML- see a lot of blast cells in CBC.

Myelodysplastic Syndrome (cont.)

Clinical Manifestations: Can range from asymptomatic to profound. Assessment and Diagnostic Findings. Medical Management: Only cure is bone marrow transplant. Nursing Management- teaching. Early the patient is asymptomatic. Infection and anemia is what brings them to hospital.

Clinical Manifestations: Multiple Myeloma

Clinical Manifestations: Classic presenting symptom is bone pain, Hypercalcemia and Anemia.

Hodgkin's Lymphoma

Clinical Manifestations: One sided and B symptoms. Diagnostic Findings: Rule out infection, Reed Sternberg. Medical Management CURE. Nursing Management: Care of the Chemotherapy Patient. Care of the Radiation Patient.

Chronic Lymphocytic Leukemia

Common malignancy of older adults (av. age 72); most common form of leukemia in the US and Europe. Family history is the may be the most important risk factor and Exposure to Agent Orange.

Acute Myeloid Leukemia: Nursing implications and complications

Complications- bleeding, infection, tumor lysis (high risk), GI problems. Nursing management- symptoms, IV, pain.

DIC

DIC- bleeding and clotting at the same time.

Multiple Myeloma- OLD CARB

Diagnostic Findings: Elevated monoclonal protein level in the serum, urine, or light chain. CRAB. Medical.

Medical Management: Acute Myeloid Leukemia

Goal is to achieve complete remission, where there is no evidence of residual leukemia in the bone marrow. If remission is achieved, next step is Bone Marrow Transplant.

What are Hematopoietic Malignancies?

Hematopoiesis: characterized by a rapid, continuous turnover of blood cells. Hematologic malignancies are often classified by the cells involved: Leukemia, Myelodysplastic Syndrome, Lymphoma, Thrombocytopenia, Myeloma.

Non-Hodgkin Lymphoma

Heterogeneous group of cancers that originate from the neoplastic growth of lymphoid tissues.

Essential Thrombocythemia (cont.)

Nursing Management: Education about the risks of hemorrhage and thrombus. Risk factors. Signs and symptoms of Thrombus- pain edema, warmth, SOB, chest pain.

Chronic Lymphocytic Leukemia (cont)

On average, most patients with CLL survive more than 20 years. Typically derived from a malignant clone of B lymphocytes.

Multiple Myeloma (more)

Pathophysiology: The malignant plasma cells produce an increased amount of a specific immunoglobulin that are nonfunctional.

Myeloproliferative Neoplasms

Polycythemia Vera. Essential Thrombocythemia. Primary Myelofibrosis. Too much blood cells.

Multiple Myeloma (CONT.)

The specific immunoglobulin secreted by the tumor cells is detectable in the blood or urine, and is referred to as monoclonal protein, or M protein.

Non-Hodgkin Lymphoma (cont.)

The spread occurs unpredictably and true localized disease is uncommon. The 7th most common type of cancer diagnosis in the US; incidence increases with each decade of life. Prognosis varies greatly among the more than 30 subtypes.

pancytopenia:

abnormal decrease in WBCs, RBCs, and platelets.

erythrocyte sedimentation rate (Sed rate)

laboratory test that measures the rate of settling of red blood cells (RBCs); elevation is indicative of inflammation; also called the sed rate.

hematopoiesis:

complex process of the formation and maturation of blood cells.

lysis:

destruction of cells.

lymphadenopathy:

enlargement of a lymph node or lymph nodes.

splenomegaly:

enlargement of the spleen.

polycythemia:

excess RBCs.

angiogenesis:

formation of new blood vessels.

neutrophil:

fully mature WBC capable of phagocytosis; primary defense against bacterial infection.

granulocyte

granulated WBC (neutrophil, eosinophil, basophil); term sometimes used as synonymous with neutrophil.

thrombocytosis:

higher-than-normal platelet count.

nucleated red blood cell (RBC):

immature form of RBC; portion of nucleus remains within the RBC.