Chronic Myelogenous Leukemia (CML) Clinical Presentation

Updated: Jun 23, 2017
  • Author: Emmanuel C Besa, MD; Chief Editor: Koyamangalath Krishnan, MD, FRCP, FACP  more...
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Presentation

History

The clinical manifestations of chronic myelogenous leukemia (CML) are insidious. The disease is often discovered incidentally in the chronic phase, when an elevated white blood cell (WBC) count is revealed by a routine blood count or when an enlarged spleen is found on a general physical examination.

Nonspecific symptoms of fatigue and weight loss may occur long after the onset of the disease. Loss of energy and decreased exercise tolerance may occur during the chronic phase after several months.

Patients often have symptoms related to enlargement of the spleen, liver, or both. The large spleen may encroach on the stomach and cause early satiety and decreased food intake. Left upper quadrant abdominal pain described as "gripping" may occur from spleen infarction. The enlarged spleen may also be associated with a hypermetabolic state, fever, weight loss, and chronic fatigue. The enlarged liver may contribute to the patient's weight loss.

Some patients with CML have low-grade fever and excessive sweating related to hypermetabolism.

In some patients who present in the accelerated, or acute, leukemia phase of the disease (skipping the chronic phase), bleeding, petechiae, and ecchymoses may be the prominent symptoms. In these situations, fever is usually associated with infections. Bone pain and fever, as well as an increase in bone marrow fibrosis, are harbingers of the blast phase.

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Physical Examination

Splenomegaly is the most common physical finding in patients with chronic myelogenous leukemia (CML). In more than 50% of the patients with CML, the spleen extends more than 5 cm below the left costal margin at time of discovery.

The size of the spleen correlates with the peripheral blood granulocyte counts, with the largest spleens being observed in patients with high WBC counts. A very large spleen is usually a harbinger of the transformation into an acute blast crisis form of the disease.

Hepatomegaly also occurs, although less commonly than splenomegaly. Hepatomegaly is usually part of the extramedullary hematopoiesis occurring in the spleen.

Physical findings of leukostasis and hyperviscosity can occur in some patients, with extraordinary elevation of their WBC counts, exceeding 300,000-600,000 cells/μL. Upon funduscopy, the retina may show papilledema, venous obstruction, and hemorrhages.

The blast crisis is marked by an increase in the bone marrow or peripheral blood blast count or by the development of soft-tissue or skin leukemic infiltrates. Typical symptoms are due to increasing anemia, thrombocytopenia, basophilia, a rapidly enlarging spleen, and failure of the usual medications to control leukocytosis and splenomegaly.

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