Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Acute Myelocytic Leukemia Clinical Presentation

  • Author: Mark E Weinblatt, MD; Chief Editor: Jennifer Reikes Willert, MD  more...
 
Updated: Mar 31, 2016
 

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.

Next

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.
Previous
 
 
Contributor Information and Disclosures
Author

Mark E Weinblatt, MD Chief, Division of Pediatric Hematology/Oncology, Professor of Clinical Pediatrics, Department of Pediatrics, Winthrop University Hospital

Mark E Weinblatt, MD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD, FAAP Chief, Division of Hematology/Oncology/BMT, Gordon Teter Endowed Chair in Pediatric Cancer, Nationwide Children's Hospital; Professor of Pediatrics, Ohio State University College of Medicine

Timothy P Cripe, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Gene and Cell Therapy, American Society of Pediatric Hematology/Oncology, Connective Tissue Oncology Society, Society for Pediatric Research, Children's Oncology Group

Disclosure: Nothing to disclose.

Chief Editor

Jennifer Reikes Willert, MD Associate Clinical Professor, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Section of Stem Cell Transplantation, Stanford University Medical Center, Lucile Packard Children's Hospital

Jennifer Reikes Willert, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society for Blood and Marrow Transplantation, Children's Oncology Group, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Gurney JG, Ross JA, Wall DA, Bleyer WA, Severson RK, Robison LL. Infant cancer in the U.S.: histology-specific incidence and trends, 1973 to 1992. J Pediatr Hematol Oncol. 1997 Sep-Oct. 19(5):428-32. [Medline].

  2. Bhatia S, Neglia JP. Epidemiology of childhood acute myelogenous leukemia. J Pediatr Hematol Oncol. 1995 May. 17(2):94-100. [Medline].

  3. Matasar MJ, Ritchie EK, Consedine N, Magai C, Neugut AI. Incidence rates of acute promyelocytic leukemia among Hispanics, blacks, Asians, and non-Hispanic whites in the United States. Eur J Cancer Prev. 2006 Aug. 15(4):367-70. [Medline].

  4. [Guideline] Tomizawa D, Tabuchi K, Kinoshita A, Hanada R, Kigasawa H, Tsukimoto I, et al. Repetitive cycles of high-dose cytarabine are effective for childhood acute myeloid leukemia: long-term outcome of the children with AML treated on two consecutive trials of Tokyo Children's Cancer Study Group. Pediatr Blood Cancer. 2007 Aug. 49(2):127-32. [Medline].

  5. Imamura T, Iwamoto S, Kanai R, Shimada A, Terui K, Osugi Y, et al. Outcome in 146 patients with paediatric acute myeloid leukaemia treated according to the AML99 protocol in the period 2003-06 from the Japan Association of Childhood Leukaemia Study. Br J Haematol. 2012 Aug 28. [Medline].

  6. Gamis AS, Woods WG, Alonzo TA, Buxton A, Lange B, Barnard DR, et al. Increased age at diagnosis has a significantly negative effect on outcome in children with Down syndrome and acute myeloid leukemia: a report from the Children's Cancer Group Study 2891. J Clin Oncol. 2003 Sep 15. 21(18):3415-22. [Medline].

  7. Ortega JJ, Madero L, Martín G, Verdeguer A, García P, Parody R, et al. Treatment with all-trans retinoic acid and anthracycline monochemotherapy for children with acute promyelocytic leukemia: a multicenter study by the PETHEMA Group. J Clin Oncol. 2005 Oct 20. 23(30):7632-40. [Medline].

  8. Klco JM, Miller CA, Griffith M, et al. Association Between Mutation Clearance After Induction Therapy and Outcomes in Acute Myeloid Leukemia. JAMA. 2015 Aug 25. 314 (8):811-22. [Medline].

  9. [Guideline] Children's Oncology Group. Chemotherapy. Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers. Mar 2006.

  10. Harding A. One-third cure rate in first-ever pediatric relapsed AML trial. Medscape Medical News. January 18, 2013. Available at http://www.medscape.com/viewarticle/777872. Accessed: January 28, 2013.

  11. Kaspers GJ, Zimmermann M, Reinhardt D, et al. Improved Outcome in Pediatric Relapsed Acute Myeloid Leukemia: Results of a Randomized Trial on Liposomal Daunorubicin by the International BFM Study Group. J Clin Oncol. 2013 Jan 14. [Medline].

  12. Lie SO, Abrahamsson J, Clausen N, Forestier E, Hasle H, Hovi L, et al. Treatment stratification based on initial in vivo response in acute myeloid leukaemia in children without Down's syndrome: results of NOPHO-AML trials. Br J Haematol. 2003 Jul. 122(2):217-25. [Medline].

  13. Sanz MA, Grimwade D, Tallman MS, Lowenberg B, Fenaux P, Estey EH, et al. Management of acute promyelocytic leukemia: recommendations from an expert panel on behalf of the European LeukemiaNet. Blood. 2009 Feb 26. 113(9):1875-91. [Medline].

  14. [Guideline] Children's Oncology Group. Hematopoetic cell transplant. Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers. Mar 2006.

  15. Klingebiel T, Reinhardt D, Bader P. Place of HSCT in treatment of childhood AML. Bone Marrow Transplant. 2008 Oct. 42 Suppl 2:S7-9. [Medline].

  16. Richardson PG, Riches ML, Kernan NA, Brochstein JA, Mineishi S, Termuhlen AM, et al. Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure. Blood. 2016 Jan 29. [Medline]. [Full Text].

  17. Bucaneve G, Micozzi A, Menichetti F, Martino P, Dionisi MS, Martinelli G, et al. Levofloxacin to prevent bacterial infection in patients with cancer and neutropenia. N Engl J Med. 2005 Sep 8. 353(10):977-87. [Medline].

  18. Creutzig U, Zimmermann M, Bourquin JP, Dworzak MN, Fleischhack G, Graf N, et al. Randomized trial comparing liposomal daunorubicin with idarubicin in induction for pediatric acute myeloid leukemia: results from Study AML-BFM 2004. Blood. 2013 May 23. [Medline].

  19. Johnson K. Less cardiotoxicity in pediatric AML. Medscape Medical News. May 30, 2013. [Full Text].

 
Previous
Next
 
Gingival hyperplasia in a patient with monoblastic leukemia.
Leukemia cutis (a skin nodule) in a patient with leukemia.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.