Page 1534 - Williams Hematology ( PDFDrive )

1508 Part XI: Malignant Lymphoid Diseases Chapter 91: Acute Lymphoblastic Leukemia 1509

TABLE 91–2. Presenting Clinical Features in Children and
Adults with Acute Lymphoblastic Leukemia
Feature Children (%) Adult (%)
Age (years)
<1 2 —
1–9 72–78 —
10–19 20–26 —
20–39 — 40
40–59 — 40
≥60 — 20
Male 56–57 62
Symptoms
Fever 57 33–56
Fatigue 50 Common
Bleeding 43 33
Bone or joint pain 25 25
Lymphadenopathy
None 30 51
Marked (>3 cm) 15 11 Figure 91–3. Chest radiograph of a 12-year-old black male with T-cell
Hepatomegaly acute lymphoblastic leukemia (ALL) and an anterior mediastinal mass.
None 34 65
Marked (below umbilicus) 17 Rare PHYSICAL FINDINGS
Splenomegaly Among frequent findings are pallor, petechiae, and ecchymosis in the
None 41 56 skin and mucous membranes, and bone tenderness as a result of leu-
Marked (below umbilicus) 17 Uncommon kemic infiltration or hemorrhage that stretches the periosteum. Liver,
Mediastinal mass 8–10 15 spleen, and lymph nodes are the most common sites of extramedullary
involvement, and the degree of organomegaly is more pronounced in
CNS leukemia 3 8
children than in adults. An anterior mediastinal (thymic) mass is pres-
Testicular leukemia 1 0.3 ent in 8 to 10 percent of childhood cases and in 15 percent of adult
cases (Fig. 91–3). A bulky, anterior mediastinal mass can compress the
Data from Pui CH: Acute lymphoblastic leukemia, in Childhood Leukemias, great vessels and trachea and possibly lead to the superior vena cava
2nd ed, edited by CH Pui, p 439. Cambridge University Press, New
York, 2006 and Larson RA, Dodge RK, Burns CP, et al: A five-drug syndrome. Patients with this syndrome present with cough, dyspnea,
remission induction regimen with intensive consolidation for adults orthopnea, stridor, cyanosis, dysphagia, facial edema, increased intra-
with acute lymphoblastic leukemia: Cancer and Leukemia Group B cranial pressure, and sometimes syncope. Painless enlargement of the
study 8811. Blood 85:2025, 1995. scrotum can be a sign of testicular leukemia cell infiltration or hydro-
cele, the latter resulting from lymphatic obstruction. Both conditions
can be readily diagnosed by ultrasonography. Overt testicular disease is
relatively rare, is generally seen in infants or adolescents with T-cell
leukemia and/or hyperleukocytosis, and does not require radiation ther-
Fatigue and lethargy are common manifestations of anemia in apy. Other uncommon presenting features include ocular involvement
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patients with ALL. In older patients, anemia-related dyspnea and (leukemic infiltration of the orbit, optic nerve, retina, iris, cornea, or
lightheadedness may be the dominant presenting features. More than conjunctiva), subcutaneous nodules (leukemia cutis), enlarged salivary
25 percent of patients, especially young children, may have a limp glands (Mikulicz syndrome), cranial nerve palsy, and priapism (result-
from bone pain or arthralgia, or an unwillingness to walk because ing from leukostasis of the corpora cavernosa and dorsal veins or sacral
of leukemic infiltration of the periosteum, bone, or joint or because nerve involvement). Epidural spinal cord compression at presentation is
of expansion of the marrow cavity by leukemia cells. Children with a rare but serious finding that requires immediate treatment to prevent
prominent bone pain often have nearly normal blood counts, which permanent paraparesis or paraplegia. In some pediatric patients, infil-
can contribute to delayed diagnosis. In a small proportion of patients, tration of tonsils, adenoids, appendix, or mesenteric lymph nodes leads
marrow necrosis can result in severe bone pain and tenderness, fever, to surgical intervention before leukemia is diagnosed.
and a very high level of serum lactate dehydrogenase. 68,69 Arthral-
gia and bone pain are less severe in adults. Less common signs and
symptoms include headache, vomiting, altered mental function, olig- LABORATORY FEATURES
uria, and anuria. Occasionally, patients present with a life-threaten-
ing infection or bleeding (e.g., intracranial hematoma). Intracranial Anemia, neutropenia, and thrombocytopenia are common in patients
hemorrhage occurs mainly in patients with an initial leukocyte count with newly diagnosed ALL. The severity reflects the degree of marrow
greater than 400 × 10 /L. Very rarely, ALL produces no signs or replacement by leukemic lymphoblasts (Table 91–3). 18,65–67 Presenting
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symptoms and is detected during routine examination. leukocyte counts range widely, from 0.1 to 1500 × 10 /L (median:
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