MANAGEMENT OF SICKLE CELL DISEASE

NIH Publication No. 02-2117. Revised May28, 2002 (Forth Edition) National Institutes of Health, National Heart, Lung, and Blood Institute. Download the entire PDF  file for Adobe   Click Here to return to Contents

Chapter 12 TRANSIENT RED CELL APLASIA

Because the life span of red blood cells is greatly shortened in sickle cell disease (SCD), temporary suppression of erythropoiesis can result in severe anemia. Transient red cell aplasia (TRCA) typically is preceded by or associated with a febrile illness. The infectious nature of TRCA is apparent from the fact that several members of families with congenital hemolytic anemia may be affected within a period of several days.

Between 70 and 100 percent of episodes of TRCA are due to infection by human parvovirus B19, also the cause of erythema infectiosum ("fifth disease") (1). Aplasia is the result of direct cytotoxicity of the parvovirus to erythroid precursors, although other progenitors may be affected in some conditions. Patients may present with increased headache, fatigue, dyspnea, more severe anemia than usual, and a severe decrease in reticulocytes (usually <1 percent or 10,000/µL). Patients may have fever, signs of upper respiratory infection, and/or gastrointestinal symptoms. Skin rashes are characteristically absent. Reticulocytopenia begins about 5 days postexposure and continues for 7 to 10 days. Exacerbation of anemia develops shortly after reticulocytopenia.

Hemoglobin levels reached a mean nadir of 3.9 g/dL in one series (2). Patients who present in the convalescent phase may be thought mistakenly to have a hyperhemolytic process because of severe anemia and high reticulocyte levels. However, the diagnosis of TRCA is supported by increased B19 parvovirus IgM levels. In at least 20 percent of patients with serologic evidence of past B19 parvovirus infection, there was no acute severe anemia. Following B19 infection, parvovirus-specific IgG concentrations are increased in most patients and protective immunity appears to be life-long; no cases of recurrent disease have been reported in children with SCD (1,3). Recovery is often heralded by a massive outpouring of nucleated red blood cells (>100/100 white blood cells).

Although the majority of adults have acquired immunity to B19 parvovirus, hospital workers who are susceptible and are exposed to patients with TRCA are at high risk of contracting nosocomial erythema infectiosum (4). Because infection during the mid-trimester of pregnancy may result in hydrops fetalis and stillbirth, isolation precautions for pregnant staff are a necessity if a parvovirus problem is suspected (5).

No experimental trials have been reported regarding the management of TRCA. Although many patients recover spontaneously, red cell transfusions should be considered for those who become symptomatic (see chapter 25, Transfusion, Iron Overload, and Chelation).

If patients are beginning to show evidence for red cell production, as determined by the reticulocyte count, they may not need transfusions. Transfusion was required in 87 percent of children with SCD-SS and TRCA in a large Jamaican series (2), but it is much less commonly needed for SCD-SC. Because parvovirus is so contagious, siblings and close contacts with SCD should be monitored for the development of aplastic events.

A single case report describes an alternative to transfusion in a child with Hb SD whose mother was a Jehovah’s Witness and refused transfusion (6). This patient was treated with a single dose of intravenous immune globulin (1 g/kg) and daily infusions of erythropoietin (100 units/kg) and exhibited a reticulocytosis beginning on day 4 after onset of this treatment. Intravenous immune globulin is now the treatment of choice for parvovirus and aplasia since it will clear the parvovirus infection (7).

In the past decade, it has become apparent that a number of complications of B19 parvovirus infection besides TRCA can occur in patients with SCD. Complications reportedat single centers or in small series include  bone marrow necrosis with pancytopenia (8), glomerulonephritis (9), stroke (10), acute chest syndrome (11), and splenic or hepatic sequestration (12,13). Treatment must then be based on these manifestations.

1. Serjeant GR, Serjeant BE, Thomas PW, et al. Human parvovirus infection in homozygous sickle cell disease. Lancet 1993;341:1237-40.

2. Goldstein AR, Anderson MJ, Serjeant GR. Parvovirus associated aplastic crisis in homozygous sickle cell disease. Arch Dis Child 1987;62:585-8.

3. Rao SP, Miller ST, Cohen BJ. Transient aplastic crisis in patients with sickle cell disease. Am J Dis Child 1992;146:1328-30.

4. Bell LM. Human parvovirus B19 infection among hospital staff members after contact with infected patients. N Engl J Med 1989;321:485-91.

5. Anand A, Gray ES, Brown T, et al. Human parvovirus infection in pregnancy and hydrops fetalis. N Engl J Med 1987;316:183-6.

6. Lascari AD, Pearce JM. Use of gamma globulin and erythropoietin in a sickle cell aplastic crisis. Clin Pediatr 1994;33:117-9.

7. Brown KE, Young NS, Barbosa LH. Parvovirus B19: Implications for transfusion medicine. Summary of a workshop. Transfusion 2001;41:130-5.

8. Eichhorn RF, Buurke EJ, Blok P, et al. Sickle celllike crisis and bone marrow necrosis associated with parvovirus B19 infection and heterozygosity for hemoglobins S and E. J Intern Med 1999;245:103-6.

9. Tolaymat A, Mousily FA, MacWilliam K, et al. Parvovirus glomerulonephritis in a patient with sickle cell disease. Pediatr Nephrol 1999;13:340-2.

10. Balkaran B, Char G, Morris JS, et al. Stroke in a cohort of patients with homozygous sickle cell disease. J Pediatr 1992;120:360-6.

11. Lowenthal EA, Wells A, Emanuel PD, et al. Sickle cell acute chest syndrome associated with parvovirus B19 infection: Case series and review. Am J Hematol 1996;51:207-13.

12. Mallouh AA, Qudah A. Acute splenic sequestration together with aplastic crisis caused by human parvovirus B19 in patients with sickle cell disease. J Pediatr 1993;122:593-5.

13. Koduri PR, Patel AR, Pinar H. Acute hepatic sequestration caused by parvovirus B19 infection in a patient with sickle cell anemia. Am J Hematol 1994;47:250-1.

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Last modified: November 05, 2002