Advanced Pathophysiology
Case Study 6

Case Study 6
Scenario: John is a 4 year-old boy who was admitted for chemotherapy following diagnosis of acute lymphoblastic leukemia (ALL). He had a white blood cell count of 250,000. Clinical presentation included loss of appetite, easily bruised, gum bleeding, and fatigue. Physical examination revealed marked splenomegaly, pale skin color, temperature of 102°F, and upper abdomen tenderness along with nonspecific arthralgia. Pathophysiology signs and symptoms
The primary pathophysiological etiology for signs, symptoms and laboratory findings in 4 year-old John’s case study are secondary splenomegaly as a result of primary Acute Lymphoblastic Leukemia (ALL). Because of its proximity to the diaphragm, splenomegaly results in pain or fullness in the left upper abdomen (subsequent tenderness and loss of appetite) that may spread to the left shoulder (non-specific arthralgia). Splenomegaly may result in anemia (bruising, bleeding gums, decrease in pallor) fatigue and frequent infections (4 year old John’s WBC 250, 000 count and fever 102°F). However, in John’s case, some of the symptoms related to splenomegaly can also overlap with those of his diagnosis of ALL such as fever and abdominal distention. Additionally, anorexia, headache, seizures, lethargy, arthralgia, infection, and bruising are also potential side effects (Ghazi et al., 2010).
Pathophysiology of acute lymphoblastic leukemia-AML comparison

According to Gedaly et al., 2006, acute lymphoblastic leukemia is the most “common form of childhood cancer and accounts for 1/3 of cancer diagnoses in children younger than 15 and 28% of cancer diagnoses in those younger than 20, page 641.” The pathogenesis of acute lymphoblastic leukemia (ALL) is a form of leukemia in which there is a neoplasm of the immature white blood cells (lymphoblast) produced by the bone marrow. This subsequently interferes with the body’s natural ability to compensate. The pathophysiology of ALL involves its monoclonal origin, uncontrolled cell proliferation by sustained self-stimulation of their receptors for growth, no response to inhibitory signals, and cellular longevity conditioned by decreased apoptosis (page 43, Gallegos-Arreola et al., 2013).”
In comparison, the symptoms of AML are caused by the replacement of normal bone marrow with leukemic cells. This subsequently causes a drop in red blood cells, platelets and normal white blood cell production. As an acute form of leukemia, AML progresses rapidly and is typically fatal within weeks or months if left untreated (Nazki et al., 2012).
Chemotherapy in ALL
The defining goal is to eliminate all evidence of the cancer cells with the use of cytotoxic agents. Chemotherapeutic agents are chosen because of their ability to interfere with oncologic cell division. The treatment of ALL involves 3 phases: remission induction, consolidation, and maintenance therapy. Pediatric patients with ALL are often given 8 or more months of intensive chemotherapy. This is often followed by maintenance therapy of two or more years (Gedaly-Duff et al., 2006). Most treatment protocols use systemic chemotherapy with or without radiotherapy (McCance & Huether, 2010). According to a study conducted by McDonald & Mc Carthy (2005), success of chemotherapy and remission depends on “aggressive induction chemotherapy followed by delayed intensification and maintenance therapy, p. 809.”
Side effects of chemotherapy
Chemotherapeutic agents eradicate the rapidly reproductive cancer cells. However they also damage normal cells that proliferate at a rapid rate (i.e. skin cells). The side effects of chemotherapy depend upon the type and dose of the agent given and the length of use. Common side effects might include: alopecia, stomatitis, and increased risk of infection (decreased white blood cells), thrombocytopenia, bruising, lethargy, anemia, anorexia, nausea, vomiting and diarrhea. Additionally, in a study by Gedaly-Duff et al., (2006), the authors surmised that sleep disturbances, pain and fatigue experienced by that of the pediatric patient, also affected the quality of life of the parents, as they often suffered similar symptoms throughout the course of child’s therapy. The most serious side effects of chemo for ALL, is the increased risk of acquiring acute myelogenous leukemia (AML), Non-Hodgkins lymphoma, and tumor lysis (Gallegos-Arreola et al., 2013).
Etiology and complications of ALL
Without chemotherapy intervention, ALL can lead to a decrease in red blood cell production (anemia). Additionally, due to the risk of developing neutropenia, the body’s natural immune response is compromised (Marchesi & Girardi, 2011). Reproductive infertility is also a risk of ALL due to the use of radiation and stem cell transplantation. Although rare, ALL patients are at risk for developing Richter’s Syndrome which is an aggressive chronic form of b-cell lymphocytic carcinoma. Subsequently, patients with chronic lymphocytic leukemia also are at risk for developing other types of carcinomas such as melanoma, cancers of the lung and digestive tract. A small number of patients with chronic lymphocytic leukemia although rare, pose the risk of immune disorders that cause red blood cell and or platelet destruction (Lee et al. 2011; Ghazi et al., 2010). Prognostically, for the purpose of this case study, 4 year old John with an intensive course of chemotherapy and associated therapeutic modalities would have about a 70% chance of going into remission. Survival rates for children younger than 15 years of age are on average 1.5% per year with chemotherapeutic treatment, symptom management, and maintenance therapy (McCance & Huether, 2010).
The Advanced Practice Nurse
In this case study, the role of the advanced practice nurse for the initial treatment would address symptom management to include IV therapy to correct dehydration; broad spectrum antibiotics; platelet transfusion; acetaminophen (fever) therapy unless contraindicated; nutrition intervention, providing a single patient room (risk of cross contamination), and pain management. Immediate follow up treatment to address splenomegaly concerns and subsequent treatment. Additional surveillance methods would include constant laboratory and radiographic monitoring. Therapy may also include chemotherapy and adjunct treatment induction (McCance & Huether, 2010). The APRN may also encourage patient and family centered care with the use of cancer teaching (avoid fresh fruit/flowers), holistic and behavioral health support therapy. Additionally, the success of remission and recovery depends upon interdisciplinary team involvement often through planning, and patient and family involvement

References

Gallegos-Arreola, M.P.; Borjas-Gutierrez, Gonzalez, G.M., Zuniga-Gonzalez,
L.E. Figuera, A.M. Puebla-Perez, & J.R, Garcia-Gonzalez (2013) Pathophysiology of
Acute Lymphoblastic Leukemia. http://dx.doi.org/10.5772/546652
Gedaly-Duff, V.; Lee, K.; Nail, L.; Nicholson, S.; Johnson, K. (2006). Pain, sleep disturbance, andFatigue in children with leukemia and their parents: A pilot study. Oncology Nursing Forum, (33)3.
Ghazi, A.A., Attarian, H., Attarian, S., Abasahl, A., Daryani, E., Farasat, E., …Kovacs, K.
(2010). Hypercalcemia and huge splenomegaly presenting in an elderly patient with B- cell non-Hodgkins Lymphoma: a case report. Journal of medical case reports, (4)330. from http://www.jmedicalcasereports.com/4/1/330
Lee H.J, Thompson JE, Wang ES, Wetzler M. (2011) Philadelphia Chromosome-Positive

cute Lymphoblastic Leukemia Current Treatment and Future Perspectives. Cancer.

117(8):1583-94
Marchesi F, Girardi K, Avvisati G. (2011) Pathogenetic, Clinical, and Prognostic Features

of Adult t(4;11)(q21;q23)/MLL-AF4Positive B-Cell Acute Lymphoblastic Leukemia.

AdvHematol. 2011;2011:62162.
McCance, K.L. & Huether, S.E. (Eds). 2010. Pathophysiology: The biological basis of disease in adults and children (6th ed.) Mosby: St. Louis
McDonald, L.R. & McCarthy, C.H. (2005). Nursing Considerations for Clofarabine. Clinical Journal of Oncology Nursing, (10)6.
Nazki F.H, Masood A, BandayMA,Bhat A, GanaiBA. (2012)Thymidylate synthase enhancer

region polymorphism not related to susceptibility to acute lymphoblastic leukemia

in the Kashmir population.Genet. Mol. Res. 11 (2): 906-17