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lead to life-threatening airway compromise and/or superior vena cava (SVC)
syndrome. The approach to the presentation and management of AMM is discussed
in more detail in the section on thoracic tumors (see “Tumors of the Thorax”
section). Chloroma, a mass of leukemic blasts in the soft tissue, can occur in any
body part and is much more common with AML compared to ALL. When this mass
develops and rapidly expands near the spinal cord, compression of the cord can
result (see “Tumors in and Around the Spinal Cord” section). Leukemic
involvement in the spinal fluid can cause meningeal symptoms, cranial nerve palsy,
headache, seizure, increased intracranial pressure (ICP), or visual disturbances (from
retinal infiltrates). Boys can present with testicular enlargement. There may also be
skin lesions due to leukemia cutis (more common in monocytic leukemias and infant
leukemia) and gingival hypertrophy (with AML) due to leukemic infiltration.
It is not uncommon for patients with leukemia to be febrile at presentation.
Although the fever may be an inflammatory reaction driven by the leukemia itself,
serious infection must be explored. It is essential to determine if the febrile patient is
neutropenic. If the absolute neutrophil count (ANC) is less than 500/μL, broadspectrum antibiotics covering gram-positive and gram-negative bacteria (including
pseudomonas) should be administered in the ED. If localizing signs of a bacterial
infection are evident, or if high fevers are present (>39°C), empiric therapy should
be initiated even if the patient is not neutropenic. Management is summarized in
Table 98.3 . Patients may present with sepsis at the time of diagnosis or relapse. The
increased risk of sepsis may be because of neutropenia and/or immune dysfunction
caused by the underlying malignancy. Management of sepsis in the setting of
leukemia is not unique and the principles are similar to those described more
thoroughly in the Children’s Hospital of Philadelphia Severe Sepsis Clinical
Pathway 1 and Chapter 94 Infectious Disease Emergencies .
Pain. For some patients, limp or a refusal to walk or bear weight is one of the first
signs of leukemia. In fact, it is not uncommon for a patient to be treated for
diagnoses such as osteomyelitis or septic hip before the diagnosis of leukemia is
uncovered. It is essential for the emergency clinician to ensure that a refusal to walk
is not because of cord compression from chloroma as discussed above. Bone pain is
usually due to replacement of the bone marrow with rapidly proliferating leukemic

cells causing strain on the marrow spaces. Pathologic fractures may develop as the
expanding marrow compartments put strain on and weaken the bony cortex.

HISTIOCYTIC DISEASES
Goal of Treatment