Know the differences in treatment for aplastic crisis and aplastic anemia

Know the differences in treatment for aplastic crisis and aplastic anemia: Excerpt from Avoiding Common Pediatric Errors

Author: Heidi Herrera, MD

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Anemia can present in children who are otherwise healthy, or who have systemic disease or a known hematologic disorder. Anemia is defined as a reduction in red blood cell (RBC) mass or blood hemoglobin concentration, resultinginreducedoxygen-carryingcapacity.Anemiacanpresentasasingle (infectioussuchasparvovirusB19)ormultiplecelllinedefect,includingbone marrowinvolvement(aplasticanemia).Therefore,childrenwhopresentwith anemia with associated symptoms should be carefully evaluated to promptly formulate a differential diagnosis, anticipate a life-threatening condition, confirm the diagnosis, and start appropriate therapies.

An aplastic crisis is an infection caused by human parvovirus B19 transmitted through exposure to infected respiratory droplets or other viruses that including cytomegalovirus, Epstein-Barr virus, and human immunodeficiency virus. The virus targets rapidly proliferating erythroid progenitor cells in the bone marrow and attaches to the P antigen receptor, resulting in reticulocytopenia and erythropoietic arrest. The RBC production is "turned off" for approximately 10 days. In patients with sickle cell disease, the RBCs only live for 10 to 15 days. Therefore, these patients are severely affected and will experience significant reductions in blood counts, with resulting congestive heart failure, cerebrovascular accident, and acute splenic sequestration. Confirmation of parvovirus B19 is performed with DNA testing. Patients with transient aplastic anemia or immunodeficiency will not test positive for immunoglobulin (Ig)M or IgG with the B19-specific antibody and remain contagious. Treatment includes simple blood transfusion to correct the severe anemia and prevent other organ complication or failure. After viral infection, patients will gain lifelong immunity with parvovirus B19, a one-time event for the patient.

Aplastic anemia is a condition of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia. Aplastic anemia can be congenital (Fanconi anemia, Shwachman-Diamond, paroxysmal nocturnal hemoglobinuria, amegakaryocytic thrombocytopenia) or acquired (idiopathic, transfusional graft-versus-host disease, pregnancy, infectious: hepatitis, Epstein-Barr virus, human immunodeficiency virus, Parvovirus, mycobacteria).Aplasticanemiaisalsoassociatedwithexposuretosubstances such as benzene, and radiation, drugs (chloramphenicol, carbamazepine, phenytoin, quinine, phenylbutazone). Diagnosis can be confirmed with a bone marrow biopsy. The treatment includes suppression of the immune system or bone marrow transplantation for more severe cases. A short course of antithymocyte globulin and several months of cyclosporine to help modulate the immune system. Mild chemotherapy (cyclophosphamide and vincristine) may be effective. Steroids, although often used, are ineffective. Untreated aplastic anemia leads to rapid deterioration and death within 6 months. If promptly diagnosed and immediate therapy started, the survival rate increases dramatically over the next 5 to 10 years. A relapse of aplastic anemia is common.

Pediatricians should have a high index of suspicion with patients presentingwith anemiaas theprimary event.Apromptandthoroughevaluation of the acutely ill-appearing patient is imperative, with the aid of the physical and laboratory exams. Patients with aplastic crisis require simple blood transfusion until the marrow recovers. Children with aplastic anemia will require a bone marrow biopsy and aspirate to confirm diagnosis followed by prompt appropriate medical therapy.

Suggested Readings

Blackman SC, Gonzalez del Rey JA. Hematologic emergencies: acute anemia. Clin Ped Emerg Med. 2005;6:124–137.
Guinan EC. Clinical aspects of aplastic anemia. Aplastic anemia and stem cell biology. Hematol Oncol Clin North Am. 1997;1:1025–1044.
Wanki SO, Telen MJ. Transfusion management in sickle cell disease. Hematol Oncol Clin North Am. 2005;19:803–826.

Book Source Details

• Book Title: Avoiding Common Pediatric Errors
• Author(s): Anthony D Slonim MD, DrPH; Lisa Marcucci MD
• Year of Publication: 2008
• Copyright Details: Avoiding Common Pediatric Errors, Copyright © 2008 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: Avoiding Common Pediatric Errors Authors: Anthony D Slonim MD, DrPH; Lisa Marcucci MD Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7489-6

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