Testicle Swelling and Fever
Answer: C
This boy presents with vague symptoms that overlap with the common viral syndrome. However, careful re-framing of the details of the history and physical examination reveals a different presentation: prolonged symptoms, sustained musculoskeletal pain, palatal petechiae, and an enlarged hemiscrotum. Any one of these symptoms in the proper time frame or in isolation may be associated with a viral illness; together, and over a longer than typical time period suggest a more insidious process – in this case, malignancy.
Acute lymphoblastic leukemia (ALL) is the most common cancer of childhood. The typical age range in diagnosis is two to five years of age, but can present throughout life. Symptoms are all non-specific: fever, fatigue, bone pain; the key here is in the abundance or persistence of symptoms, triggering a work-up. Signs include bleeding, petechiae or purpura, lymphadenopathy (non-tender, firm, matted), mediastinal mass on chest film, and testicular enlargement.
Our patient’s complete blood count (CBC) revealed: WBC 40,000/mm3; Hb 9.2 g/dL; Hct 26.9%; Platelets 15,000/mm3
CBC abnormalities in children presenting with ALL may be misleading: 50% of children have a WBC < 10,000/mm3 and only 20% will present with a WBC > 50,000/mm3. Half will present with bleeding or petechiae.
Pay close attention to a depressed platelet count with at least one other cell line abnormality. If in doubt or your suspicion is more than low, obtain a peripheral smear.
Although the pediatric hematologist-oncologist will play a major role in counseling, preparing, and guiding the patient and family through the process, the emergency physician is often the first to broach the subject. A general knowledge of what the patient and his family will face may help the EP to counsel and prepare them.
BASICS TO KNOW:
There are three phases of treatment after the diagnosis is made: induction, consolidation (also called intensification), and maintenance, each with its own set of complications. The three phases are typically completed within two to three years. Therapy is tailored according to risk factors for treatment failure and/or recurrence.
Induction includes weekly parenteral chemotherapy (e.g. vincristine), daily oral steroids, and scheduled asparaginase. The major complications that will bring the child with ALL to the ED during induction include:
During induction, children are often treated with intrathecal chemotherapy (CNS preventive therapy with cytarabine/methotrexate/hydrocortisone).
Consolidation therapy typically starts a month after induction, and lasts for 4 to 6 months. The goal is to prevent regrowth; children are often on experimental or tailored regimens. Patients undergo a series of bone marrow biopsies to track progress. Major complications during consolidation include:
Maintenance therapy continues until 2 to 3 years post-diagnosis. It is a less intensive regimen, and the child may have periodic intrathecal therapy. Fevers are still an issue as before. Up to this point, the hematologist-oncologist is essentially the child’s primary care provider. Once maintenance is established, the general pediatrician may play a larger role. Good communication between the emergency physician, the pediatrician, and the hematologist-oncologist is important in outpatient follow up after the ED visit.
The Other Answers:
Without symptoms of cough, shortness of breath, or evidence of fever or abnormal lung findings, a chest film for pneumonia would be very low yield (A). Although recommended in the evaluation of the initial presentation of ALL, it is not a good screening test in isolation.
A urinalysis (UA) may be considered in the evaluation of possible epididymo-orchitis, but recent studies suggest that a UA in that context is low yield (D).
A viral respiratory panel (B) may be helpful in the patient who will be hospitalized to augment the work-up of fever without a source or to cohort patients in the hospital. It is not recommended in the routine evaluation of outpatients with a suspected viral illness.
References
Margolin JF, Steuber CP, Poplack DG. Acute Lymphoblastic Leukemia. In: Principles and Practice of Pediatric Oncology, 4th ed, Pizzo PA, Poplack DG (Eds), Lippincott-Raven, Philadelphia 2001.
Sinigaglia R, Gigante C, Bisinella G, Varotto S, Zanesco L, Turra S. Musculoskeletal manifestations in pediatric acute leukemia. J Pediatr Orthop. 2008;28(1):20.
This boy presents with vague symptoms that overlap with the common viral syndrome. However, careful re-framing of the details of the history and physical examination reveals a different presentation: prolonged symptoms, sustained musculoskeletal pain, palatal petechiae, and an enlarged hemiscrotum. Any one of these symptoms in the proper time frame or in isolation may be associated with a viral illness; together, and over a longer than typical time period suggest a more insidious process – in this case, malignancy.
Acute lymphoblastic leukemia (ALL) is the most common cancer of childhood. The typical age range in diagnosis is two to five years of age, but can present throughout life. Symptoms are all non-specific: fever, fatigue, bone pain; the key here is in the abundance or persistence of symptoms, triggering a work-up. Signs include bleeding, petechiae or purpura, lymphadenopathy (non-tender, firm, matted), mediastinal mass on chest film, and testicular enlargement.
Our patient’s complete blood count (CBC) revealed: WBC 40,000/mm3; Hb 9.2 g/dL; Hct 26.9%; Platelets 15,000/mm3
CBC abnormalities in children presenting with ALL may be misleading: 50% of children have a WBC < 10,000/mm3 and only 20% will present with a WBC > 50,000/mm3. Half will present with bleeding or petechiae.
Pay close attention to a depressed platelet count with at least one other cell line abnormality. If in doubt or your suspicion is more than low, obtain a peripheral smear.
Although the pediatric hematologist-oncologist will play a major role in counseling, preparing, and guiding the patient and family through the process, the emergency physician is often the first to broach the subject. A general knowledge of what the patient and his family will face may help the EP to counsel and prepare them.
BASICS TO KNOW:
There are three phases of treatment after the diagnosis is made: induction, consolidation (also called intensification), and maintenance, each with its own set of complications. The three phases are typically completed within two to three years. Therapy is tailored according to risk factors for treatment failure and/or recurrence.
Induction includes weekly parenteral chemotherapy (e.g. vincristine), daily oral steroids, and scheduled asparaginase. The major complications that will bring the child with ALL to the ED during induction include:
- Tumor lysis syndrome: hyperphosphatemia, hypocalcemia, hyperuricemia, hyperkalemia, and acute renal failure
- Infection (any)
- Chemotherapy-related adverse effects: bleeding (thrombocytopenia), mucositis, pancreatitis, anaphylaxis
- Thrombosis: central sinus thrombosis, deep vein thrombosis, and pulmonary embolism
During induction, children are often treated with intrathecal chemotherapy (CNS preventive therapy with cytarabine/methotrexate/hydrocortisone).
Consolidation therapy typically starts a month after induction, and lasts for 4 to 6 months. The goal is to prevent regrowth; children are often on experimental or tailored regimens. Patients undergo a series of bone marrow biopsies to track progress. Major complications during consolidation include:
- Infection (bacterial, viral, fungal) – any fever must be aggressively investigated
- Secondary cancer development (previously astrocytoma, now mostly AML)
Maintenance therapy continues until 2 to 3 years post-diagnosis. It is a less intensive regimen, and the child may have periodic intrathecal therapy. Fevers are still an issue as before. Up to this point, the hematologist-oncologist is essentially the child’s primary care provider. Once maintenance is established, the general pediatrician may play a larger role. Good communication between the emergency physician, the pediatrician, and the hematologist-oncologist is important in outpatient follow up after the ED visit.
The Other Answers:
Without symptoms of cough, shortness of breath, or evidence of fever or abnormal lung findings, a chest film for pneumonia would be very low yield (A). Although recommended in the evaluation of the initial presentation of ALL, it is not a good screening test in isolation.
A urinalysis (UA) may be considered in the evaluation of possible epididymo-orchitis, but recent studies suggest that a UA in that context is low yield (D).
A viral respiratory panel (B) may be helpful in the patient who will be hospitalized to augment the work-up of fever without a source or to cohort patients in the hospital. It is not recommended in the routine evaluation of outpatients with a suspected viral illness.
References
Margolin JF, Steuber CP, Poplack DG. Acute Lymphoblastic Leukemia. In: Principles and Practice of Pediatric Oncology, 4th ed, Pizzo PA, Poplack DG (Eds), Lippincott-Raven, Philadelphia 2001.
Sinigaglia R, Gigante C, Bisinella G, Varotto S, Zanesco L, Turra S. Musculoskeletal manifestations in pediatric acute leukemia. J Pediatr Orthop. 2008;28(1):20.