Acute Lymphoblastic Leukemia Clinical Presentation
- Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD more...
Patients with acute lymphoblastic leukemia (ALL) present with either symptoms relating to direct infiltration of the marrow or other organs by leukemic cells, or symptoms relating to the decreased production of normal marrow elements.
Fever is one of the most common symptoms of ALL, and patients with ALL often have fever without any other evidence of infection. However, in these patients, one must assume that all fevers are from infections until proved otherwise, because a failure to treat infections promptly and aggressively can be fatal. Infections are still the most common cause of death in patients undergoing treatment for ALL.
Patients with ALL often have decreased neutrophil counts, regardless of whether their total white blood cell (WBC) count is low, normal, or elevated. As a result, these individuals are at an increased risk of infection. The prevalence and severity of infections are inversely correlated with the absolute neutrophil count (ANC), which is defined as the number of mature neutrophils plus bands per unit of volume. Infections are common when the absolute neutrophil count is less than 500/µL, and they are especially severe when it is less than 100/µL. See the Absolute Neutrophil Count calculator.
Symptoms of anemia are common and include fatigue, dizziness, palpitations, and dyspnea upon even mild exertion. Other patients present with signs of bleeding. Bleeding can be the result of thrombocytopenia due to marrow replacement. Additionally, approximately 10% of patients with ALL have disseminated intravascular coagulation (DIC) at the time of diagnosis. These patients may present with hemorrhagic or thrombotic complications.
Some patients present with palpable lymphadenopathy. Others, particularly those with T-cell ALL, present with symptoms related to a large mediastinal mass, such as shortness of breath.
Infiltration of the marrow by massive numbers of leukemic cells frequently manifests as bone pain. This pain can be severe and is often atypical in distribution.
About 10-20% of ALL patients may present with left upper quadrant fullness and early satiety due to splenomegaly.
Although patients may present with symptoms of leukostasis (eg, respiratory distress, altered mental status) because of the presence of large numbers of lymphoblasts in the peripheral circulation, leukostasis is much less common in people with ALL than those with acute myelogenous leukemia (AML), and it occurs only in patients with the highest WBC counts (ie, several hundred thousand per μL).
Patients with a high tumor burden, particularly those with severe hyperuricemia, can present in renal failure.
Patients with acute lymphoblastic leukemia (ALL) commonly have physical signs of anemia, including pallor and a cardiac flow murmur. Fever and other signs of infection, including lung findings of pneumonia, can also occur. Fever should be interpreted as evidence of infection, even in the absence of other signs.
Patients with thrombocytopenia usually demonstrate petechiae, particularly on the lower extremities. A large number of ecchymoses is usually an indicator of a coexistent coagulation disorder such as disseminated intravascular coagulation (DIC).
Signs relating to organ infiltration with leukemic cells and, to a lesser degree, lymphadenopathy may be present.
Occasionally, patients have rashes that result from infiltration of the skin with leukemic cells.
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|Chromosome Number||3-Year Event-Free Survival|
|Abnormality||Genes Involved||3-Year Event-Free Survival|
|* Traditional regimens.
† Hyper-CVAD (cyclophosphamide, vincristine, doxorubicin [Adriamycin], dexamethasone) with rituxan.
‡ Hyper-CVAD with imatinib.
|Early B-precursor ALL||+||+||-||-||-|
|ALL = acute lymphoblastic leukemia; Cylg = Cytoplasmic immunoglobulin; SIg =Surface immunoglobulin; TdT = terminal deoxynucleotidyl transferase.|
|ALL Cells||TdT||Surface CD3||CD4/CD8|
|Early T-precursor ALL||+||-||+/+ or -/-|
|T-cell ALL||+||+||+/- or -/+|