Leukemia: An Overview for Primary Care



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Am Fam Physician. 2014 May 1;89(9):731-738.

This clinical content conforms to AAFP criteria for continuing medical education (CME). See CME Quiz Questions.

  Patient information: See related handout on leukemia, written by the authors of this article.

Author disclosure: No relevant financial affiliations.

Leukemia is a clonal proliferation of hematopoietic stem cells in the bone marrow. The four broad subtypes most likely to be encountered by primary care physicians are acute lymphoblastic, acute myelogenous, chronic lymphocytic, and chronic myelogenous. Acute lymphoblastic leukemia occurs more often in children, whereas the other subtypes are more common in adults. Risk factors include a genetic predisposition as well as environmental factors, such as exposure to ionizing radiation. Symptoms are nonspecific and include fever, fatigue, weight loss, bone pain, bruising, or bleeding. A complete blood count usually reveals leukocytosis and other abnormally elevated or depressed cell lines. Patients with suspected leukemia should be referred promptly to a hematologist-oncologist. The diagnosis is confirmed by further examination of the bone marrow or peripheral blood. Treatment may include chemotherapy, radiation, monoclonal antibodies, or hematopoietic stem cell transplantation. Complications of treatment include tumor lysis syndrome and serious infections from immunosuppression. Leukemia survivors should be monitored closely for secondary malignancies, cardiac complications, and endocrine disturbances such as metabolic syndrome, hypothyroidism, and hypogonadism. Five-year survival rates are highest in younger patients and in patients with chronic myelogenous leukemia or chronic lymphocytic leukemia.

Leukemia is a common malignancy in children and adults that occurs when alterations in normal cell regulatory processes cause uncontrolled proliferation of hematopoietic stem cells in the bone marrow. The age-adjusted incidence rate of leukemia in the United States is 12.8 per 100,000 persons each year.1 The prevalence of leukemia is generally higher in whites and in males, and increases with age.1 Approximately one in 70 persons develops leukemia in his or her lifetime.1 The four subtypes of leukemia most often encountered by primary care physicians are acute lymphoblastic, acute myelogenous, chronic lymphocytic, and chronic myelogenous. Family physicians should be able to recognize the common presentations of leukemia, perform the initial diagnostic evaluation, and understand how to care for leukemia survivors.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Chronic lymphocytic leukemia should be suspected in older adults with a markedly elevated white blood cell count and an enlarged liver or spleen.

C

14

When evaluating a patient with leukocytosis, common causes such as infection, inflammation, and stress response should be considered. A peripheral blood smear should be obtained in patients with a white blood cell count greater than 20,000 per μL (20.0 × 109 per L) and with associated anemia; thrombocytopenia; thrombocytosis; enlarged liver, spleen, or lymph nodes; or constitutional symptoms.

C

914

Patients with early-stage chronic lymphocytic leukemia (i.e., no anemia or thrombocytopenia and with less than three areas of nodal involvement) may be monitored without treatment if they are asymptomatic.

C

17

In patients with leukemia, fever with neutropenia (fewer than 500 neutrophils per μL [0.5 × 109 per L]) warrants evaluation for infection source and initiation of empiric broad-spectrum antibiotics.

C

22

Leukemia survivors are at increased risk of secondary malignancies. They require serial complete blood count monitoring, as well as age- and sex-specific cancer screening.

C

8, 2325


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

View Table

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Chronic lymphocytic leukemia should be suspected in older adults with a markedly elevated white blood cell count and an enlarged liver or spleen.

C

14

When evaluating a patient with leukocytosis, common causes such as infection, inflammation, and stress response should be considered. A peripheral blood smear should be obtained in patients with a white blood cell count greater than 20,000 per μL (20.0 × 109 per L) and with associated anemia; thrombocytopenia; thrombocytosis; enlarged liver, spleen, or lymph nodes; or constitutional symptoms.

C

914

Patients with early-stage chronic lymphocytic leukemia (i.e., no anemia or thrombocytopenia and with less than three areas of nodal involvement) may be monitored without treatment if they are asymptomatic.

C

17

In patients with leukemia, fever with neutropenia (fewer than 500 neutrophils per μL [0.5 × 109 per L]) warrants evaluation for infection source and initiation of empiric broad-spectrum antibiotics.

C

22

Leukemia

The Authors

AMANDA S. DAVIS, MD, is an assistant professor at the AnMed Health Family Medicine Residency Program in Anderson, S.C. At the time the manuscript was written, Dr. Davis was a fellow in the Faculty Development Fellowship in the Department of Family Medicine at the University of North Carolina at Chapel Hill School of Medicine.

ANTHONY J. VIERA, MD, MPH, is an associate professor in the Department of Family Medicine at the University of North Carolina at Chapel Hill School of Medicine.

MONICA D. MEAD, MD, is a hematology/oncology fellow at the University of California–Los Angeles David Geffen School of Medicine.

Address correspondence to Amanda S. Davis, MD, AnMed Health Family Medicine Residency Program, 2000 East Greenville St., Ste. 3600, Anderson, SC 29621 (e-mail: Amanda.davis1@anmedhealth.org). Reprints are not available from the authors.

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This article is one in a series from the Faculty Development Fellowship of the Department of Family Medicine at the University of North Carolina at Chapel Hill. Guest editor of the series is Anthony J. Viera, MD, MPH.



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