Journal of Neuroscience Nursing:
Intracerebral Granulocytic Sarcoma
Questions or comments about this article may be directed to Margaret Alvarez, MSN RN APN, at email@example.com. She is a neurosurgery inpatient nurse practitioner at the University of Chicago Medical Center, Chicago, IL.
Granulocytic sarcomas, also known as chloromas, are rare extramedullary tumors of myeloid or myelocytic origin. They are usually associated with both acute and chronic myelogenous leukemia and myeloproliferative disorders. Leukemia involvement of the central nervous system most commonly presents as meningeal leukemia; intracerebral granulocytic sarcoma (IGS) is rare. Signs and symptoms depend on the brain structures involved. Magnetic resonance imaging with and without gadolinium is the imaging of choice to evaluate the tumor; however, tissue biopsy is essential for definitive diagnosis. Treatment usually involves radiation followed by chemotherapy, depending on the previous systemic treatment. Because medical literature about IGS is scarce, optimal treatment is unclear. With the number of leukemia patients in remission, the incidence of IGS is expected to rise. This is because most chemotherapeutic agents do not cross the blood‐brain barrier, making the brain a target for leukemia recurrence. Nurses play a vital role in helping patients and families understand the disease process, the treatments involved, and the necessary adjustments, such as performing mundane activities of daily living, especially when neurocognitive impairments are present.
Granulocytic sarcomas are solid tumors of myelocytic origin associated with both acute and chronic myelocytic leukemia or other myeloproliferative disorders (Krishnamurthy, Nusbacher, Elguezabal, & Seligman, 1976). The tumors are composed of myeloblasts, which are immature cells of granulocytic origin. Also known as chloroma, myeloblastoma, or extramedullary leukemia, granulocytic sarcomas can be found in almost every part of the body, such as the bones, orbit, paranasal sinuses, para‐ and intraspinal spaces, brain, skin, stomach, colon, kidney, breast, cervix, and vagina (Nishimura, Kyuma, Kamijo, & Maruta, 2004). Leptomeningeal involvement—the presence of tumor cells in the leptomeninges or cerebrospinal fluid (CSF)—is the usual path of involvement after leukemia has invaded the central nervous system (CNS). Intraparenchymal infiltration of leukemia in a solid, localized form is rare (Lee, Park, & Hwang, 2006).
Because of its rarity, screening for granulocytic sarcoma among patients with acute myeloid leukemia is not routinely done (Meltzer & Jubinsky, 2005). This article presents the case of a patient who developed intracerebral granulocytic sarcoma (IGS) after being diagnosed with chronic myeloid leukemia 7 years earlier. Pathophysiology, clinical features, diagnosis, medical and surgical treatments involved, and nursing implications associated with the patient with intracerebral granulocytic sarcoma are discussed.
GR is a 52‐year‐old male with a history of chronic myeloid leukemia (CML) diagnosed 7 years ago. He presented to the emergency department (ED) with a new onset of drooling of saliva on the left side of his mouth and slurring of speech while talking on the phone. He denied any fever but admitted to having photophobia and night sweats. He also complained of a frontal headache. He was confused and could not remember the details that brought him to the ED.
His medications were 30 mg lansoprazole daily, 300 mg allopurinol daily, 100 mg docusate sodium twice a day, and 1‐2 tablets acetaminophen with codeine every 4‐6 hours as needed. He was also taking 1,500 mg hydroxyurea orally twice a day for a recent leukocytosis episode. His social history revealed that he was a 30 pack‐year smoker, was divorced, and lived alone. He had no children.
Past history revealed that GR had been treated with interferon alpha and hydroxyurea upon diagnosis of CML 7 years earlier; however, his treatment was complicated by pericardial and pleural effusion. As a result, interferon was stopped. He was then placed on oral imatinib mesylate (Gleevec) without achieving remission. His last admission was 1 month earlier for leukocytosis and renal insufficiency. His white blood cell count was 300,000/mm3, which was successfully lowered and maintained with hydration and hydroxyurea, respectively, to 20,000/mm3. Furthermore, his creatinine was 1.6 mg/dl (normal: 0.8‐1.2 mg/dl), lactate dehydrogenase was 5,700 IU/L (normal: 340‐670 IU/L), and uric acid 9 mg/dl (normal: 4‐8.6 mg/dl); all were elevated and were successfully treated with hydration and allopurinol.
On examination in the ED, GR was alert and oriented to person and place but not to year. His pupils were both 4 to 3 mm round and equally reactive to light, and extraocular movements were intact. A left facial droop was noted. He had a strong bilateral shoulder shrug; reflexes were 2+ in both upper extremities and 1+ in both lower extremities. His strength was 5/5 in both upper and lower extremities, with a slight arm drift on the left. His gait was slightly unsteady.
A noncontrast computed tomography (CT) scan of the head was ordered, which showed a large nonenhancing right frontal lesion with edema. There was no hemorrhage or hydrocephalus. Magnetic resonance imaging (MRI) of the brain was ordered for further evaluation. It revealed a right frontal mass that measured 3.2 × 3.4 × 2.5 cm (Fig 1), with high signal intensity on T2‐weighted images and mild peripheral enhancement following contrast administration. There was vasogenic edema around the mass but no midline shift. No meningeal enhancement was seen on either CT or MRI. His complete blood count (CBC), including his white blood cell (WBC) count, were normal on admission and during the entire hospital stay. His creatinine was also normal during his entire hospitalization.
GR was admitted to the intensive care unit (ICU) and started on 10 mg intravenous (IV) dexamethasone every 6 hours for the brain edema. He was also given a loading dose of 500 mg phenytoin intravenously and started on 100 mg orally every 8 hours to prevent seizures. CT scans of the chest, abdomen, and pelvis were done to rule out a primary source for the brain mass; they were all negative. The oncology service was consulted; a brain biopsy was recommended because the mass was thought unlikely to be leukemic in etiology. GR's leukemia was currently in the chronic phase, meaning his WBC counts were adequately controlled. He was continued on hydroxyurea to maintain his WBC and platelet counts as normal as possible.
On day 2, GR showed improvement. His drift was gone, and his gait was steady. Slow weaning of his dexamethasone was started.
On day 3, GR was taken for a stereotactic brain biopsy. Tissue examination showed granulocytic cells in different stages of maturation, but with a distinct and prominent shift toward immature forms at the promyelocyte and myelocyte stages. The tissue was positive for myeloperoxidase, which confirmed its myeloid or myelocytic lineage, which is compatible with IGS. GR was kept in the ICU overnight and transferred to the acute care unit after a postbiopsy head CT was found to be negative for hemorrhage.
After the biopsy, GR continued to be neurologically stable. His phenytoin levels were monitored and maintained at therapeutic levels. He did not have any seizure events or any adverse effects from his medications. A lumbar puncture was done to evaluate for presence of leptomeningeal involvement of leukemia. The cytology results were negative.
Upon review of his case by the tumor board, radiation therapy was recommended for treatment of his IGS, since IGS usually responds well to radiation, and it is usually well tolerated. Further chemotherapy was not recommended because GR had not responded to oral imatinib in the past. The recommendation was presented to the patient and his friends, and the patient agreed to proceed with radiation therapy after hospital discharge.
On day 4, GR was discharged home, and follow‐up appointments were scheduled with the neurosurgeon, radiation oncologist, and hematologist. His oral medications on discharge were 300 mg allopurinol daily, 1,500 mg hydroxyurea twice a day, 1‐2 tablets acetaminophen with codeine every 4‐6 hours as needed, 30 mg omeprazole daily, and a scheduled tapered dose of dexamethasone.
Two weeks after discharge from the hospital, GR's WBC count again went up to 200,000/mm3; this was successfully lowered with hydroxyurea and hydration, but his radiation therapy was delayed. GR finally received 4,000 centi‐Gray (cGy) intensity‐modulated radiation therapy. This was given in fractionated doses for more than 4 weeks. GR tolerated the procedure well. He was maintained on both hydroxyurea and allopurinol during the entire course of his radiation to maintain normal WBC and platelets counts and to prevent uric acid nephropathy, respectively.
At the time of submission of this case study, 3 months from the time of his diagnosis, GR was still living and was continuing his follow‐up with his hematologist and radiation oncologist. His most recent MRI of the brain showed complete resolution of right frontal mass (Fig 2).
Background: Central Nervous System Leukemia
Chronic myeloid leukemia is a disease characterized by excessive production of granulocytic cells, especially neutrophils, leading to marked splenomegaly and very high WBC counts. Associated basophilia and thrombocytosis are common. The Philadelphia (Ph1) chromosome, a feature cytogenetic abnormality in CML, is present in the bone marrow cells in more than 95% of cases (Keating & Kantarjian, 2004).
The following is a brief review of how the WBC evolves. The pluripotent stem cells are a group of bone marrow stem cells from which all blood cells originated (Vander, Sherman, & Luciano, 1994). The pluripotent stem cells divide into myeloid stem cells and lymphoid stem cells. The myeloid stem cells begin with myeloblasts or myelocytes, which are immature blood cells found only in the bone marrow, then further differentiate into more mature cells (such as the erythrocytes or red blood cells [RBCs]) and polymorphonuclear granulocytes (such as neutrophils, eosinophils, and basophils), monocytes, and platelets (Guyton & Hall, 2000). Note that rapid proliferation of immature blood cells either in the bone marrow or in the circulation is abnormal and usually signifies a cancerous process.
Oral imatinib mesylate (Gleevec) is considered the first choice of treatment for patients with CML, especially those patients who are not candidates for allogenic stem cell transplantation. Other treatment modalities include oral hydroxyurea and allopurinol for symptomatic relief of CML, to decrease leukocyte counts and prevent urate nephropathy, respectively (Ferri, 2007).
With the increased use of advanced and efficacious chemotherapeutic agents, the number of leukemia patients in remission is increasing. Because systemic treatments for leukemia often do not cross the blood‐brain barrier, CNS invasion by leukemia can occur. With this disadvantage, the brain becomes a perfect target for future leukemia relapse.
According to Vidal, Baer, and Bloomfield (1999), granulocytic sarcoma can present in three clinical scenarios. First, granulocytic sarcoma can present in patients with known acute myelogenous leukemia (AML), either with or without bone marrow involvement. Second, it may occur as an isolated event without a history of leukemia and without bone marrow involvement at the time of diagnosis. Finally, it may occur in patients with CML or other myeloproliferative disorders such as myelodysplastic syndromes or polycythemia vera.
The manner in which IGS forms is unknown. One possible mechanism is that leukemic cells from the bone marrow of the skull travel to the adjacent dura, and then to the subarachnoid space via the Virchow‐Robin spaces (VRS; Woo et al., 1986). Virchow‐Robin spaces are extensions of the subarachnoid space and contain CSF; these spaces are located along blood vessels entering the brain parenchyma and are usually seen in the basal ganglia, hippocampus, or anywhere in the white matter (Barkhof, 2004). After leukemic cells are in the VRS, cells can infiltrate the brain parenchyma. However, this theory does not support the development of IGS in the absence of obvious meningeal or skull involvement (Suzer et al., 2004).
The other mechanism that Woo and colleagues (1986) suggested is that IGS is formed by hematogenous spread of blast cells. Simpson, Anderson, Garcia, and Barton (1989) agreed with Woo and colleagues that hematogenous spread is the likely route in the formation of IGS. This theory is supported by the formation of leukemic nodules in the brain during an episode of severe leukocytosis. Leukostasis can occur as a result of increased WBC counts >100,000/mm3, which can lead to destruction of vascular walls and result in invasion of cerebral blood vessel by leukemic cells (Rogers, 2003). This could result in hematogenous implantation of leukemic cells and formation of IGS lesions.
The signs and symptoms of IGS depend on the affected brain structure or anatomy involved. Symptoms usually are related to edema of surrounding structures. Signs and symptoms could range from mental status changes, headache, focal deficits, visual changes, nausea, vomiting, cranial nerve palsies, seizures, and possible paraplegia.
Although CT of the head is often the initial diagnostic approach for patients with suspected CNS leukemic involvement, the best imaging modality for characterizing IGS is MRI with and without gadolinium. The MRI in IGS shows the lesions as hypointense or isointense on both T1‐ and T2‐weighted images with marked homogeneous enhancement after contrast administration (Parker, Hardjasudarma, McClellan, Fowler, & Milner, 1996), but T2 hyperintensity is not unusual (Fitoz et al., 2002). MRI may also identify meningeal enhancement, which indicates involvement or spread of tumor cells in the meninges. CSF analysis is recommended to rule out leptomeningeal spread if the MRI is negative for leptomeningeal enhancement. MRI has the same sensitivity as lumbar puncture in diagnosing leptomeningeal metastases but has only 70% specificity (Kesari & Batchelor, 2003). Lumbar puncture should be deferred in the presence of a brain lesion with mass effect to prevent brain herniation.
Brain tissue biopsy is the gold standard in diagnosing IGS. The tissue obtained is often green in color, hence the term chloroma. However, not all chloromas are green; therefore, the term granulocytic sarcoma is more appropriate (Neiman et al., 1981).
A CT scan of chest, abdomen, and pelvis is usually done prior to proceeding to brain biopsy to rule out a primary cause for the intracerebral lesion. Presence of other lesions in the body may direct the biopsy to a less risky area than the brain.
After surgical pathology confirms the diagnosis of IGS, radiation combined with chemotherapy is considered to be the treatment of choice (Smidt, De Bruin, Van't Veer, & Van Den Bent, 2005). However, treatment strategies can vary depending on the manner of presentation, tumor location, history of leukemia therapy, and patient factors such as age and performance status (Fruauff, Barasch, & Rosenthal, 1988; Vidal et al., 1999).
Chemotherapy and radiation therapy are usually given in the setting of isolated granulocytic sarcoma (GS) without evidence of leukemia. Subsequent conversion to systemic leukemia with bone marrow involvement can occur in the setting of isolated GS (Tsimberidou et al., 2003). The mean interval time for an isolated GS to progress to AML is 5 months (Tsimberidou et al.) to 49 months (Neiman et al., 1981). Furthermore, GS is an indication of disease progression or conversion of chronic bone marrow disorders to AML (Neiman et al.; Vidal et al., 1999).
In a retrospective study done by Imrie and colleagues (1995) on patients with isolated GS, radiation therapy or surgical resection alone were found to be effective in locally controlling the tumor but did not affect survival or the conversion to AML. Chemotherapy significantly lowered the probability of developing leukemia from 71% to 41%, and survival was significantly improved for those who had chemotherapy. It is interesting to note that in a study done by Tsimberidou and colleagues (2003), patients with isolated GS who were treated with combined radiotherapy and chemotherapy did not develop AML.
In the case of our patient, chemotherapy was not given because GR was a known leukemia patient and had previously undergone chemotherapy (alphainterferon and imatinib) without success. Surgical debulking is indicated only in the presence of progressive neurological deficit due to increasing intracranial pressure that is not responsive to aggressive medical management (Nishimura et al., 2004).
Granulocytic sarcomas are radiosensitive (Woo et al., 1986). The mode of radiation therapy depends on the size, location, and number of tumors, patient's anticipated length of survival, and extent of extracranial disease. Brain tumor lesions that are less than 3 cm may be treated with stereotactic radiosurgery (SRS) or fractionated SRS, because it offers adequate local control of the tumor and the convenience of a single treatment with SRS, and it minimizes the neurocognitive consequences, especially if lengthy survival is expected. The use of SRS for larger brain tumors can result in a higher risk of radiation‐induced toxicity (Hickey, 2003) because a higher dose of radiation is given at one time. Because our patient's tumor was greater than 3 cm, intensitymodulated radiation therapy (IMRT) was used.
IMRT is an advanced form of radiation therapy that uses 3D images of the tumor in combination with a sophisticated computerized dose calculation (Teh et al., 2002). Intensity‐modulated radiation therapy allows the radiation oncologist, with the help of a modern computerized optimization program, to modulate or adjust the dose across the treatment field. The computer software generates an accurate treatment plan, allowing the radiation dose to be delivered across the treatment field in multiple beams with different doses and intensities rather than as a single‐beam dose (Cash, 2006). These features allow the radiation oncologist to deliver the accurate maximum amount of radiation to the target tumor while decreasing the radiation dose delivered to nearby critical structures (Teh et al.), resulting in less radiation toxicity.
Because more cancer patients with brain metastases have good functional outcome upon diagnosis, the use of either up‐front whole‐brain radiation therapy (WBRT) plus radiosurgery (RS) versus RS alone is highly controversial. In their evidence‐based review, Mehta and colleagues (2005) concluded that the WBRT plus RS should be the recommended treatment for patients with multiple brain metastases. They concluded that combination of WBRT plus RS offers the advantage of lower brain tumor recurrence and better local control. Furthermore, WBRT has the advantage of addressing the micrometastases not visible by standard imaging techniques (Kuo & Recht, 2006).
Although case reports from Yamamoto and colleagues (1999) and Binder, Tiemann, Haase, Humpe, & Kneba (2000) showed two patients in complete remission for 21 and 20 months, respectively, after being diagnosed with IGS, the prognosis is generally poor. This is probably due to the fact that GS could indicate the progression of chronic leukemia to a blast crisis, characterized by an increased number of immature blood cells in the peripheral blood or bone marrow (Ferri, 2007). Patients in blast crisis develop complications such as bleeding or sepsis, eventually leading the patient to succumb to the systemic disease. The median survival for patients in blast crisis is 3‐12 months (Giles, Cortes, Kantarjian, & O'Brien, 2004).
Potential for Decreased Cerebral Perfusion Secondary to Cerebral Edema
Vasogenic edema due to tumor cell invasion can cause alteration of fluid flow into the extracellular spaces of the brain parenchyma as a result of an ineffective blood‐brain barrier (Wen et al., 2006). Signs and symptoms of cerebral edema can range from headache to nausea, vomiting, seizures, and decreased level of consciousness. Dexamethasone, a corticosteroid, is used to relieve symptomatic brain edema. The mechanism of action is unclear, but Wen and colleagues claimed that corticosteroids reduce vasogenic edema by reducing tumor capillary permeability. Corticosteroids usually have an impressive effect, which can allow time to determine definitive therapy (Lassman & DeAngelis, 2003); the effect, however, is temporary.
Nurses should be aware of possible side effects associated with corticosteroid use and educate patients accordingly. Side effects can range from gastrointestinal bleeding, steroid myopathy, and mood and behavioral changes to hyperglycemia. Patients should be educated not to stop their corticosteroids without telling their healthcare providers. Stopping corticosteroids abruptly can cause adrenal crisis, especially for patients who have been on corticosteroids for long periods. Furthermore, rapid tapering of steroids can also exacerbate brain edema (Taillibert & Delattre, 2005). Patients who are on high doses of steroids, such as those on a total of 40 mg daily, should be instructed on how to taper off the steroids slowly.
The signs and symptoms of intracerebral granulocytic sarcoma depend on the affected brain structure or anatomy involved.
Steroid myopathy can occur in any patient taking corticosteroids. It is characterized by subacute onset of weakness and proximal muscle weakness, especially in the lower extremities (Lassman & DeAngelis, 2003). Steroid myopathy should be suspected in patients who are unable to lift their arms over their head or climb stairs. Treatment consists of physical therapy to improve muscle strength while reducing the corticosteroid dose to the lowest possible dose.
Taking steroids with food can prevent gastrointestinal upset; histamine blockers or proton pump inhibitors are used to prevent gastrointestinal ulceration. Patients should be told that they may experience mood and behavioral complications. If these changes become problematic, consideration should be given to reducing the steroids to the lowest possible dose. If neuroleptics are considered, a psychiatrist should be consulted (Wen et al., 2006).
Patients are at risk for hyperglycemia while they are on corticosteroids and should have their blood glucose closely monitored and treated as necessary. This is especially important in patients with IGS because elevated blood glucose favors the development of infection, and patients with IGS on corticosteroids are further immunosuppressed.
Seizures can be a presenting symptom in patients with IGS, depending on tumor location. Patients whose tumors are located in frontal, parietal, and temporal cortical areas are at high risk for seizures (Sperling & Ko, 2006). Those who present with seizures should be treated with standard antiepileptic drugs (AEDs; Lassman & DeAngelis, 2003). The use of prophylactic AEDs for patients with a newly diagnosed brain tumor without seizure is not recommended because AEDs carry potential serious side effects. The use of prophylactic AEDs continues to be controversial (Glantz et al., 2000). The controversy could be related to the clinical judgment that the danger of seizure occurrence, such as a convulsive seizure causing temporary increase in intracranial pressure which may lead to a potential dangerous herniation syndrome, outweighs the risk of AEDs and its side effects (Sirven, Wingerchuk, Drazskowski, Lyons, & Zimmerman, 2004).
Pharmacologic seizure management remains challenging because most patients are on multiple medications, which could have significant interactions. Many AEDs can induce or upregulate the cytochrome P450 enzyme system, resulting in increased metabolism or elimination of chemotherapeutic drugs. This renders the chemotherapeutic agent less efficacious (Vecht, Wagner, & Wilms, 2003). Dexamethasone can decrease the phenytoin level; patients on dexamethasone may require a higher dose of phenytoin to maintain therapeutic levels. Last, phenytoin can decrease the half‐life of dexamethasone, causing steroids to be less effective in controlling brain edema (Chalk, Ridgeway, Brophy, Yelland, & Eadie, 1984).
Patients who are taking AEDs should be instructed not to stop their antiseizure medications abruptly because this may cause seizures. Patients also should be instructed to call their healthcare providers immediately if they experience any increase in seizure frequency while on AEDs, for this needs to be further investigated. Causes of increased seizure frequency could be tumor progression or edema, subtherapeutic AED levels, or medical causes such as electrolyte abnormalities, infection, or changes in plasma protein such as albumin. Patients taking certain antiepileptic drugs (e.g., phenytoin and carbamazepine) are at high risk of developing erythema multiforme (EM) when the drugs are used in conjunction with radiation therapy (Lassman & DeAngelis, 2003). An acute self‐limited disease of skin and mucous membranes, EM can range from a mild condition, with few lesions, to a widespread vesiculobullous form known as Stevens‐Johnson syndrome (Shaw, 2001). Nurses should monitor patient's skin condition for any changes and heighten both patients' and families' awareness that it is imperative to call their healthcare providers for any new rash at any point during their treatment.
Headache can occur in 50% of brain tumor patients, and about 60% of brain tumor patients will have a headache during their illness (Silberstein & Young, 2003). Headache in brain tumor patients is usually described as a severe early morning headache, often accompanied with nausea and vomiting.
Certain areas in the brain are more pain sensitive than others (Purdy & Kirby, 2004). These areas include the venous sinuses, some of the dura at the base of the brain, the dural arteries, and the cerebral arteries at the base of the brain. Beyond location, headaches in brain tumor patients could be due to increased intracranial pressure (ICP) secondary to mass effect; hydrocephalus; visual dysfunction secondary to optic nerve or extraocular motor nerve compression leading to difficulty focusing and diplopia, respectively; and hypertension due to increased ICP (Greenberg, 2001).
Headaches can be very debilitating, resulting in lack of enthusiasm to participate in activity or therapy, thus leading to further deconditioning. Nurses have an important role in alleviating headache. Treatment usually starts with obtaining history about the headache. Interventions consist of offering pain medications to patients every 4 hours around the clock. This is crucial because some patients may be too tired or may lack the ability to watch time or reach the call light, or they may be afraid to become dependent on pain medications. Nurses can offer pain medications before the patient participates in physical therapy or undergoes a procedure.
Cognitive function refers to the ability to develop, think through, and follow a course of action depending on the information presented. Cognitive function has several domains, including attention, learning and memory, psychomotor efficiency and manual dexterity, visual spatial ability, and general intelligence (Baumgartner, 2004). Cognitive impairments depend on tumor location. Nurses should explain reasons for cognitive impairments to patients and families, while guiding families in patient safety measures and should tell patients what to anticipate upon discharge from the hospital. Assessing whether the patient with cognitive impairment lives alone, has someone to provide observation, has transportation for tests or treatments, or is capable of taking care of mundane activities of life, such as paying bills, shopping for groceries, cooking meals, and working, is imperative. Knowledge about the patient's neurocognitive deficits will not only help patients and families to understand the impact of the disease but also will help families plan for the future. Even if the prognosis of patients with IGS is poor, nurses have an important role in helping them live the highest quality of life.
Associated Complications of Hematologic Malignancies and Treatment
Patients with metastatic leukemic brain lesions are highly prone to infection due to improperly functioning cell‐mediated immunity and frequent use of steroid medications (Recht & Mrugala, 2003). Nurses should report any signs of mental status changes or new or constant headache not relieved by pain medications because these might be symptoms of an insidious infection. A high index of suspicion is needed to prevent mortality related to infection. Furthermore, patients may or may not have a fever. Fever should always be investigated and treated immediately, as if an infection exists, until proven otherwise (Ballen & Stewart, 2000). Hand‐washing and avoidance of prolonged use of central catheters and indwelling catheters may help lower the incidence of infection among patients with leukemia.
Patients with IGS whose leukocyte counts are greater than 100,000/mm3 and include predominantly blast cells are at high risk of developing intracranial hemorrhage (ICH; Ballen & Stewart, 2000). Hyperviscosity as a result of leukocytosis can cause blockage of thin‐walled cerebral vessels or thrombosis, resulting in rupture of cerebral vessels (Hess & Sztajnkrycer, 2005). Any trending up of WBC should be reported immediately, and the hematology‐oncology service should be consulted immediately. Treatments usually include emergent leukapheresis, hydration, and hydroxyurea.
Tumor Lysis Syndrome
Patients who have hematologic malignancies are prone to develop tumor lysis syndrome (TLS). TLS is a hematologic emergency that is characterized by metabolic imbalance such as hyperuricemia, hyperkalemia, and hypocalcemia secondary to hyperphosphatemia (Davidson et al., 2004). Risk factors for TLS are those who have elevated lactate dehydrogenase (LDH) prior to initial therapy, large tumor burden, renal insufficiency, dehydration prior to chemotherapy or radiation, or bulky, rapidly proliferating tumors (Cantril & Haylock, 2004).
TLS can occur 1‐3 days after initiation of cytotoxic treatment but may occur spontaneously (Davidson et al., 2004). The destruction of large numbers of rapidly dividing malignant cells can overwhelm the kidney's ability to excrete uric acid, phosphorus, and potassium. Increased uric acid can cause precipitation of calcium crystals into the kidney. Depending on the hydration and renal status of the patient, these calcium crystals can block the renal tubules, resulting in acute renal failure (ARF; Davidson et al.).
Signs and symptoms usually depend on the severity of metabolic derangement. Both hypocalcemia and hyperkalemia can cause cardiac dysrhythmias, and hyperuricemia can cause urate nephropathy leading to ARF. Hypocalcemia can also cause muscle cramps, seizures, and changes in mental status. Treatment of TLS usually includes hydration, lowering of uric acid with allopurinol, and hemodialysis as needed. Any worsening laboratory values while a patient is undergoing chemotherapy or radiation should be reported.
Because our patient had a history of increased creatinine and LDH together with increased WBC from his previous admission, laboratory values were monitored cautiously, with close observation of levels of electrolytes, creatinine, LDH, uric acid, and WBC especially while radiation or chemotherapy was given. Prevention, early detection, and prompt intervention are needed to avoid the devastating complications associated with hematologic malignancies such as IGS.
Intracerebral granulocytic sarcoma is rare. Its occurrence usually signifies the transition from the chronic phase of CML into an accelerated phase or, at worst, into a blast crisis. The occurrence of IGS is expected to rise because of the increased survival rates of those with leukemia. The prognosis for IGS is generally poor, but nurses play a crucial role in improving patients' quality of life by helping patients and families understand the mechanism of the disease and its treatments, side effects, and possible complications.
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