Acute Promyelocytic Leukemia Clinical Presentation: History, Physical

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Presentation

History

Most of the signs and symptoms of acute promyelocytic leukemia (APL) are also seen in cases of acute myelogenous leukemia (AML). These include the following:

Fatigue, weakness, and dyspnea related to anemia
Easy bruising or bleeding caused by thrombocytopenia or coagulopathy
Fever and infection related to leukopenia

Most patients with APL present with pancytopenia. About 10-30% of patients present with leukocytosis.^[7]

APL differs from AML in that most patients present with coagulopathy. The coagulopathy has been described as disseminated intravascular coagulation (DIC) with associated hyperfibrinolysis. APL has been associated with low levels of plasminogen, alpha2-plasmin inhibitor, and plasminogen activator inhibitor 1 found in fibrinolytic states. There is increased expression of annexin II, a receptor for plasminogen and plasminogen-activating factor, on the surface of leukemic promyelocytes.^[8]This leads to overproduction of plasmin and fibrinolysis.

It is important to treat the coagulopathy as a medical emergency. In 40% of untreated patients, pulmonary and cerebral hemorrhages can occur. It takes 5-8 days for coagulopathy to improve with treatment.

Physical

The physical examination can reveal pallor, petechiae, and areas of ecchymoses. Bleeding from the gums may be evident. A flow murmur can be heard in patients with severe anemia. Neurologic deficits or headaches may be present if central nervous system (CNS) involvement has occurred.

Differential Diagnoses

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Media Gallery

Bone marrow aspirate hybridized with the RARA dual color break-apart probe set (Abbott-Vysis). The cell to the left shows a normal cell with 2 fusion signals, as the 5'RARA probe (orange) and the 3'RARA probe (green) are not separated. The cell on the right shows split signals for one RARA gene, indicating a chromosomal rearrangement disrupting the RARA gene. Image courtesy of Dr. Tina Edmonston from the Department of Pathology at Thomas Jefferson University Hospital.
Bone marrow aspirate hybridized with PML/RARA dual color translocation probe set (Abbott-Vysis). The cell to the right shows a normal cell with 2 separate PML (orange) and RARA (green) signals each. The cell to the left shows an abnormal cell characterized by a single fusion signal juxtaposing the PML and RARA signals, suggestive of a translocation of the 2 genes. Images courtesy of Dr. Tina Edmonston from the Department of Pathology at Thomas Jefferson University Hospital.
Hypogranular subtype of acute promyelocytic leukemia. Image courtesy of Dr. William Kocher.
Regularly hypergranular subtype of acute promyelocytic leukemia. Image courtesy of Dr. William Kocher.

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Author

Sandy D Kotiah, MD Director of the Neuroendocrine Center, Hematology and Medical Oncology, Mercy Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ronald A Sacher, MB, BCh, FRCPC, DTM&H Professor, Internal Medicine and Pathology, Director, Hoxworth Blood Center, University of Cincinnati Academic Health Center

Ronald A Sacher, MB, BCh, FRCPC, DTM&H is a member of the following medical societies: American Association for the Advancement of Science, American Association of Blood Banks, American Clinical and Climatological Association, American Society for Clinical Pathology, American Society of Hematology, College of American Pathologists, International Society of Blood Transfusion, International Society on Thrombosis and Haemostasis, Royal College of Physicians and Surgeons of Canada

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: GSK Pharmaceuticals,Alexion,Johnson & Johnson Talecris,Grifols, BMS<br/>May receive honoraria from the above companies for speaking and teaching.

Chief Editor

Additional Contributors

Clarence Sarkodee Adoo, MD, FACP Consulting Staff, Department of Bone Marrow Transplantation, City of Hope Samaritan BMT Program

Clarence Sarkodee Adoo, MD, FACP is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

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