CHAPTER Hematologic-Oncologic 85 Emergencies in Children · CHAPTER 85: Hematologic-Oncologic...

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Hematologic-Oncologic Emergencies in Children Ilene Claudius

Childhood cancer is a leading cause of death in children, but with improve-ments in management and outcomes, many patients with new, active, or treated malignancies present to the ED. This chapter will cover the most common pediatric malignancies and hematologic issues. More information on malignancy-related complications is provided in Chapter 139 and hemo-philia and Von Willebrand Disease are discussed in detail in Chapter 135 .

■ CHILDHOOD LEUKEMIA Acute Lymphoblastic Leukemia (ALL) is the most common pediatric malignancy, with a peak incidence between 3 to 5 years of age and a 75% to 80% 5-year survival.

Clinical Features

Patients can present with any of the following signs or symptoms of bone marrow infiltration: pallor, fatigue, easy bruising, fever, or bone pain. Many have hepatomegaly or splenomegaly. Rarely, acute myelogenous leukemia (AML) can present with gingival hyperplasia or subcutaneous masses (chloromas).

Diagnosis and Differential

The complete blood count (CBC) with manual differential is the most use-ful test, though leukocytosis and blasts may be absent early in the disease process, requiring close follow-up of patients with insidious complaints such as bone pain. WBC counts below 4000/mL 3 , mild anemia, and mild thrombocytopenia should raise suspicion in these cases. Abnormalities of 2 or more cell lines make leukemia more likely. If the CBC is concerning for acute leukemia, obtain a chest radiograph (for mediastinal mass); elec-trolytes with creatinine, uric acid, and phosphate (for evidence of tumor lysis); liver function tests and lactate dehydrogenase, PT/PTT (looking for disseminated intravascular coagulation); type and screen if anemic; and blood and urine cultures if febrile.

The differential diagnosis is extensive depending on the patient’s pre-senting symptom. Aplastic anemia and viral infections can cause bone marrow suppression; rheumatologic diseases can overlap with symptoms and findings of leukemia; and idiopathic immune thrombocytopenia can be difficult to differentiate, though classically involves isolated destruction of the platelets without affecting other cell lines.

Emergency Department Care and Disposition

Chemotherapy need not be initiated immediately in most cases. ED care is directed at potential complications and symptoms.

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410 SECTION 9: Pediatrics

1. Anemia: a. Irradiated, leukodepleted packed red blood cells (PRBCs) (10 mL/

kg) for life-threatening hemorrhage or hemolysis. b. If no hemorrhage or hemolysis, nonemergent transfusions can be

given to keep hemoglobin (Hb) > 8 grams/dL. This should be done in coordination with oncologist.

2. Thrombocytopenia: a. Platelets (10 mL/kg) for life-threatening hemorrhage, consumption,

or urgent need for invasive procedure (eg, lumbar puncture). b. If no urgent indication exists, nonemergent transfusions can given to

keep platelets >10 000/mL 3 . This does not need to be done in the ED. 3. Infection: Fever and neutropenia typically become an issue after the

initiation of chemotherapy. However, granulocyte function is impaired in newly presenting leukemics, and fever or suspicion of infection in a new leukemic should be treated emergently with broad spectrum antibi-otics. In children with known neutropenia from treatment, consider unusual infections such as perirectal abscess/cellulitis and typhlitis (an appendicitis-like syndrome, treated nonsurgically) as well as bactere-mia. Common choices include:

a. Cefepime (50 milligrams/kilogram) or ceftazidime (50 milligrams/kilogram)

b. If ill-appearing, add gentamycin (2.5 milligrams/kilogram) c. If suspicion of gram positive infection, add vancomycin (15 milligrams/

kilogram) d. If anaerobic source (eg, typhlitis), add clindamycin (10 milligrams/

kilogram) or metronidazole (7.5 milligrams/kilogram) 4. Tumor lysis describes the release of intracellular potassium, phosphate,

and uric acid, and subsequent decline in serum calcium that occurs from turnover of tumor cells. It typically occurs with chemotherapy, but can occur prior to treatment, particularly in patients with a high tumor burden. Recognition and treatment of this life and kidney threatening condition must begin in the ED, and prevention should be considered in patients with a particularly high white blood cell (WBC) count. Specifics of manage-ment are discussed in Chapter 139 .

5. Hyperleukocytosis typically requires treatment if symptoms of stasis (eg, stroke, dyspnea) occur or presenting WBC > 200,000/mL for AML and >300,000/mL for ALL in the absence of symptoms. Treatment includes:

a. Aggressive hydration with normal saline bolus of 20 mL/kg repeated as tolerated .

b. If patient is symptomatic after hydration, arrange for leukapheresis . c. Avoid PRBC transfusions and diuretics if possible . d. Anticipate and initiate treatment for tumor lysis syndrome . e. If asymptomatic with high levels, consider hydroxyurea which will

half WBCs in 24 to 48 hours.

■ LYMPHOMA Hodgkin lymphoma is a lymphoid neoplasm preferentially affecting ado-lescents. Most cases present in the cervical or supraclavicular lymph nodes causing nontender, nonerythematous, rubbery lymphadenopathy. Systemic


CHAPTER 85: Hematologic-Oncologic Emergencies in Children 411

symptoms (eg, fever, night sweats, weight loss) occur in less than one-third of teens. A chest radiograph may demonstrate an anterior mediastinal mass. Non-Hodgkin’s lymphoma can originate in or outside of the lymphatic system, and occurs in older children, particularly those with a history of immunosuppression. Because the tumor can occur in any organ, presenting signs and symptoms differ by location. A CBC, electrolytes and creatinine (looking for tumor lysis), and chest radiograph (looking for mediastinal mass) should be performed in the ED. ED care involves management of acute complications such as superior vena cava syndrome (see Chapter 139 for details), avoidance of steroid therapy except in life threatening situa-tions, and consultation with an oncologist.

■ CENTRAL NERVOUS SYSTEM TUMORS Brain tumors are common pediatric malignancies, and typically present with headaches related to increased intracranial pressure. In infants, overt signs of increased pressure (eg, bulging fontanel) can sometimes be appreciated. Vom-iting, ataxia, cranial nerve palsies, or vague neurologic signs and symptoms can occur as well. CT scan or MRI are acceptable imaging studies in the ED. Seizures should be treated if present, and dexamethasone (1 milligrams/year of age up to 10 milligrams) can be given to reduce vasogenic edema. Further management should be determined by oncology and neurosurgery.

■ EXTRACRANIAL SOLID TUMORS Neuroblastoma is a primitive ganglion tumor that can arise in the adrenal, other abdominal location, chest, or neck. Patients can present with a painless mass, hepatomegaly, or symptoms of mass effect from compression of the bowel, bladder, lymphatics, spinal cord, trachea, or superior vena cava. Occa-sionally, retrobulbar metastasis can cause raccoon eyes and proptosis. Para-neoplastic manifestations can include hypertension, watery diarrhea, and opsoclonus-myoclonus syndrome (rapid, multidirectional eye movements and jerking of the extremities). CBC for evidence of bone marrow infiltration and chest radiograph for mediastinal mass should be obtained in the ED.

Wilms tumor , or nephroblastoma, primarily affects young children (<10 years of age), and typically presents with an abdominal mass with few symptoms other than those explained by local compression. Many patients present with lung metastasis.

Germ cell tumors present as masses in the ovary or testicle, and are particularly common in boys with a history of an undescended testicle. Diagnosis is by ultrasound.

Retinoblastoma is a white-grey intraocular malignancy, that typically presents in children less than 2 years of age with loss of the normal red reflex ( Fig. 85-1 ). Strabismus, decreased visual acuity, a fixed pupil, or an injected, painful eye are less common presentations. One-quarter are bilateral, and diagnosis is made through ophthalmology consultation and CT scan.

Bone and tissue sarcomas include rhabdomyosarcoma (a painless tis-sue mass), osteosarcoma (a bony tumor around the metaphysis of the knee, proximal humerus, pelvis, or mandible), and Ewing sarcoma (a bony tumor of the long bones and axial skeleton). Osteosarcoma and Ewing sarcoma can present as a dull, aching pain, particularly at night, a tender



mass that seems to appear following a minor trauma, or with systemic symptoms. Diagnosis of rhabdomyosarcoma is by CT scan, while osteosar-coma and Ewing often are seen on plain radiograph, with osteosarcoma demonstrating a mixed sclerotic/lytic picture and Ewing having a destruc-tive, “moth-eaten” appearance.

■ ANEMIA Clinical Features

Anemia may be asymptomatic or accompanied by pallor and fatigue or heart failure.


Iron deficiency anemia due primarily to excessive cow’s milk intake typi-cally affects children aged 6 months to 3 years of age. Children outside of this age range should be assessed for occult GI bleeding with stool guaiac testing. Laboratory studies demonstrate anemia with a low reticulocyte

FIGURE 85-1. Typical appearance of leukocoria in a patient with retinoblastoma . (Reproduced with permission from Shah BR, Lucchesi M. Atlas of Pediatric Emergency Medicine, © 2006, McGraw-Hill Companies, Inc. All rights reserved.)


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count and low mean corpuscular volume (MCV). Hemolytic anemia can be primary or secondary to an underlying infection or intrinsic disorder. Patients have isolated anemia with high MCV, spherocytes and schistocytes on periph-eral smear, elevated indirect bilirubin, and a positive direct antibody test.


Most asymptomatic or minimally symptomatic patients with iron defi-ciency anemia can be safely discharged with careful follow-up, reduc-tion of milk intake below 24 oz/d, and initiation of iron therapy (2 to 3 milligrams/kilogram/dose of elemental iron 3 times daily). Hemolytic anemia may require more careful observation or transfusion, based on the patient’s clinical status and hemoglobin level. Steroids are indicated in the treatment of autoimmune hemolytic anemia. If transfusion is required, patients with very low hemoglobin levels (< 6 grams/dL) should receive 3 to 5 mL/kg of PRBCs over 3 hours, while patients with a pretransfusion Hb > 6 grams/dL can receive 10 mL/kg.

■ IDIOPATHIC THROMBOCYTOPENIC PURPURA (ITP) ITP is an autoimmune disorder of platelet destruction.

Clinical Features

While often an isolated condition in preschool-aged children, ITP can be a feature of rheumatologic disorders (eg, lupus) or infections (eg, HIV, hepatitis C), particularly in teens. Patients can present with petechiae or bleeding (frequently epistaxis or gingiva) and, in the younger child without a coexisting condition, ITP often follows a viral infection by days to weeks.


Other autoimmune and infectious disorders should be considered, particu-larly in the teen. On CBC, other cell lines are not affected and platelets are often below 20,000/mL 3 with a large platelet volume. CBC and blood type should be sent.


ITP in children carries an excellent prognosis, with 70% resolving sponta-neously within 6 months, regardless of treatment. Children with minimal symptoms and platelets >10,000/mL 3 are often followed without interven-tion. For children with significant bleeding of platelet counts <10,000/mL 3 , the following options exist:

1. Prednisone 1 to 2 milligrams/kilogram/d for 2 to 4 weeks. Steroids should only be started in conjunction with a hematologist once leukemia has been excluded.

2. IVIG 1 gram/kilogram. This infusion typically runs over 4 to 6 hours and requires admission.

3. Anti-Rh (D) immunoglobulin (WinRho®) 75 micrograms/kilogram over 1 hour can be used in patients whose blood type is Rh+. Because these antibodies bind the RBCs, hemolysis ensues, dropping the Hb 1 to 2 grams/dL over the week following treatment. In April 2010, a boxed



warning was issued by the FDA in response to hemolysis-related deaths of primarily older adults following use of WinRho ® for ITP, recom-mending 8 hours of monitoring for signs of hemolysis (back pain, chills, fever, discolored urine). Premedication with acetaminophen and diphen-hydramine is recommended.

4. In the case of a life-threatening bleed, single-donor platelet transfusion (20 to 30 mL/kg), IVIG (1 gram/kilogram over 30 min), and high-dose methylprednisolone (30 milligrams/kilogram) should be considered. This platelet dose is much higher than the normal dose of 5 to 10 mL/kg of a single donor pack or 1 U/10 kilogram of the pooled or “random” donor packs. In a patient without antibodies, this lower dose will increase the platelet level by 30,000 to 50,000.

■ NEUTROPENIA Neutropenia implies a neutrophil count below 1000/mL 3 , but risk of infec-tions increases significantly when the count falls below 500/mL 3 .

Clinical Features

Neutropenia can be asymptomatic or associated with serious bacterial illness.


Benign forms of neutropenia include: benign transient neutropenia (from viral infections or medications), autoimmune neutropenia, and cyclic neu-tropenia. More serious forms of neutropenia are chronic and persistent, such as congenital agranulocytosis, or chemotherapy related.


Patients with benign forms of neutropenia and evidence of infection can typically be discharged home, though consultation with the patient’s hema-tologist and a single dose of ceftriaxone (50 milligrams/kilogram) may be considered depending on the clinical situation. Patients with fever and a more serious neutropenia variant should have blood cultures sent and broad spectrum antibiotics initiated.

For further reading in Emergency Medicine: A Comprehensive Study Guide, 7th ed.,

see Chapter 136, “Oncology and Hematology Emergencies in Children,” by Rick

Place, Anne Marie T. Lagoc, Thomas A. Mayer, and Christopher J. Lawlor


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