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From Pizzo PA, Poplack DG. Principles and Practice of Pediatric Oncology. 3rd ed. Philadelphia: Lippincott-Raven, 1997, with permission.

Clinical Manifestations Arising from Lymphoid System Invasion

1. Lymphadenopathy—sometimes mediastinal lymphadenopathy causing superior vena cava syndrome

2. Splenomegaly

3. Hepatomegaly.

Clinical Manifestations of Extramedullary Invasion

Central nervous system (CNS) involvement occurs in less than 5% of children with

ALL at initial diagnosis. It may present with the following:

1. Signs and symptoms of raised intracranial pressure (e.g., morning headache, vomiting, papilledema, bilateral sixth-nerve palsy).

2. Signs and symptoms of parenchymal involvement (e.g., focal neurologic signs such as hemiparesis, cranial nerve palsies, convulsions, cerebellar involve-ment—ataxia, dysmetria, hypotonia, hyperflexia).

3. Hypothalamic syndrome (polyphagia with excessive weight gain, hirsutism, and behavioral disturbances).

4. Diabetes insipidus (posterior pituitary involvement).

5. Chloromas of the spinal cord—(very infrequent in ALL) may present with back pain, leg pain, numbness, weakness, Brown-Sequard syndrome, and bladder and bowel sphincter problems.

6. CNS hemorrhage—complication that occurs more frequently in patients with AML than in those with ALL. It is caused by:

a. Leukostasis in cerebral blood vessels, leading to leukothrombi, infarcts, and hemorrhage b. Thrombocytopenia and coagulopathy, contributing to CNS hemorrhage.

Genitourinary Tract Involvement

Testicular Involvement

1. Usually presents with painless enlargement of the testis.

2. Occurs in 10-23% of boys during the course of the disease at a median time of 13 months from diagnosis.

3. Occult testicular involvement recognized in 10-33% of boys undergoing bilateral wedge biopsies.

4. Risk factors for the development of testicular involvement:

b. Leukocytosis at diagnosis (>20,000/mm3)

c. Presence of a mediastinal mass d. Moderate to severe hepatosplenomegaly and lymphadenopathy e. Thrombocytopenia (<30,000/mm3).

Ovarian Involvement Occurs very rarely.

Priapism

Occurs rarely. It is due to involvement of sacral nerve roots or mechanical obstruction of the corpora cavernosa and dorsal veins by leukemic infiltrates or by the coagulation of the platelet-rich leukemic blood in the corpora cavernosa.

Renal Involvement

1. Occasionally may present with hematuria, hypertension, and renal failure.

2. Evaluated in many patients by ultrasonography; more common in T-cell ALL or mature B-cell ALL.

Gastrointestinal Involvement

1. The gastrointestinal (GI) tract is frequently involved in ALL. The most common manifestation is bleeding.

2. Leukemic infiltrates in the GI tract are usually clinically silent until terminal stages when necrotizing enteropathy might occur. The most common site for this is the cecum, giving rise to a syndrome known as typhlitis (Chapter 26).

Bone and Joint Involvement

Bone pain is one of the initial symptoms in 25% of patients. It may result from direct leukemic infiltration of the periosteum, bone infarction, or expansion of marrow cavity by leukemic cells. Radiologic changes seen most frequently include:

1. Osteolytic lesions involving medullary cavity and cortex

2. Transverse metaphyseal radiolucent bands

3. Transverse metaphyseal lines of increased density (growth arrest lines)

4. Subperiosteal new bone formation.

Skin Involvement

Skin involvement occurs most commonly in neonatal leukemia or AML. Cardiac Involvement

One half to two thirds of patients have demonstrated cardiac involvement at autopsy, although symptomatic heart disease occurs in less than 5% of cases. Pathologic findings include leukemic infiltrates and hemorrhage of the myocardium or the pericardium.

Lung Involvement

This may be due to leukemic infiltrates or hemorrhage.

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