Pediatric Acute Myelocytic Leukemia Clinical Presentation

Updated: Sep 12, 2017
  • Author: Mark E Weinblatt, MD; Chief Editor: Jennifer Reikes Willert, MD  more...
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Presentation

History

Symptoms of acute myeloid leukemia can be divided into those caused by a deficiency of normally functioning cells, those due to the proliferation and infiltration of the abnormal leukemic cell population, and constitutional symptoms.

Symptoms due to a deficiency of normally functioning cells

Cytopenias can result from a deficiency of normally functioning cells.

Anemia, a common finding, is characterized by pallor, fatigue, tachycardia, and headache. The major pathophysiologic mechanism is related to decreased production in the infiltrated bone marrow. Bleeding, hemolysis, and sequestration and destruction in an enlarged spleen or liver may all contribute to anemia.

Another symptom, hemorrhage due to thrombocytopenia, is in part due to decreased production of megakaryocytes in the bone marrow. The most common findings are easy bruising, petechiae, epistaxis, gingival bleeding, and, sometimes, GI or CNS hemorrhage. The patient with disseminated intravascular coagulation might also have symptoms of hemorrhage or thrombosis, including painful swelling and sharp, colored demarcation of an extremity.

Fever is a common presenting complaint in patients with acute leukemia. In this context, fever should initially always be attributed to infection. Depending on the site of infection, symptoms may vary. Symptoms may be pulmonary (eg, cough, dyspnea, hypoxia, chest pain), as in patients with pneumonias; neurologic (eg, lethargy, emesis, headache), as in patients with meningitis; or other (eg, pain or changes in bladder and bowel function due to colitis or urinary tract infection).

Symptoms due to the proliferation and infiltration of the abnormal leukemic cell mass and infiltrative disease

The most common extramedullary infiltration due to leukemic cells occurs in the reticuloendothelial system. This infiltration may manifest as adenopathy, hepatomegaly, or splenomegaly.

In rare cases, a mediastinal mass may cause symptoms of respiratory insufficiency or superior vena cava syndrome.

Abdominal masses may cause pain or obstruct the GI or urogenital tracts. Nodules of myeloblasts, called chloromas, can be found in the skin, CNS or any other organ.

Monoblastic leukemia is often associated with gingival hyperplasia (seen in the image below) and CNS infiltration.

Gingival hyperplasia in a patient with monoblastic Gingival hyperplasia in a patient with monoblastic leukemia.

Constitutional and miscellaneous symptoms

Unexplained, persistent fevers are sometimes the only presenting symptom of patients with leukemia. Weight loss and cachexia are unusual findings in children with leukemia but not in adults. These effects can result from an increased catabolic nutritional state combined with decreased caloric intake from anorexia.

Bone pain is less common in patients with acute myelocytic leukemia than in patients with ALL. Its cause may be periosteal elevation due to leukemic cell infiltrates or bone infarctions. On occasion, weakened bony cortex permits pathologic fractures of the extremity, which result in pain and decreased mobility, or vertebral compression fractures after minimal trauma. Such compression fractures cause back pain and dysfunction of the lower extremity (eg, weakness, loss of bladder and bowel function).

CNS symptoms, although uncommon initially, can appear during follow-up with various findings. The most common signs and symptoms are related to elevated intracranial pressure, including headache, nausea and emesis, lethargy, irritability, and visual complaints.

Involvement of cranial nerves, most often the facial nerve (resulting in Bell palsy) and the abducens nerve (resulting in esotropia), may be isolated or may occur in combination with other manifestations.

In addition to infiltration and proliferation of leukemic cells with mass effect, intracranial hemorrhage and CNS infections can cause similar devastating CNS complications.

Spinal lesions are rare.

In acute myeloid leukemia, blast cells periodically form large aggregates called chloromas or granulocytic sarcomas, leading to epidural compression. Extreme leukocytosis with WBC counts of more than 200 X 109/L is often associated with hyperviscosity, intracerebral leukostasis, and intracerebral hemorrhage early in the course.

In rare cases, leukemic cells infiltrate all parts of the eye. The retina and iris are the sites most commonly affected. Iritis often causes photophobia, pain, and increased lacrimation, whereas retinal involvement is often accompanied by hemorrhage and can lead to a loss of vision.

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Physical Examination

Pancytopenia

Pallor with tachycardia is observed to different degrees proportional to the severity of anemia. With severe anemia, patients may have lethargy, a heart murmur, and signs of congestive heart failure.

Bleeding manifestations are most commonly observed in the skin and include petechiae, purpuric lesions, and ecchymoses.

GI bleeding may indicate erosions or perforation.

Signs of infection include fever, gingivitis, hypotension, or respiratory distress, depending on the site of infection.

Signs of leukemic infiltration and proliferation

Adenopathy, at times generalized, is less common in acute myeloid leukemia than in ALL.

Splenomegaly is sometimes massive, particularly in young children.

Pronounced organomegaly occasionally results in respiratory embarrassment in infants due to decreased diaphragmatic excursion.

CNS findings include lethargy, cranial nerve dysfunction (particularly esotropia and facial palsy), and papilledema.

Typhlitis can lead to acute pain in the lower quadrants that mimic signs of appendicitis.

Signs of perforation include hypotension, abdominal distension, and decreased bowel sounds. Clinical deterioration is rapid if the condition is not recognized.

Skin nodules are occasionally found in patients with acute myeloid leukemia. They are typically firm, raised, and often bluish-purple in color. (See the image below.)

Leukemia cutis (a skin nodule) in a patient with l Leukemia cutis (a skin nodule) in a patient with leukemia.
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