Page 1956 - Hematology_ Basic Principles and Practice ( PDFDrive )

1736 Part XI Transfusion Medicine

The clinical course is quite variable. An occasional infant is have rapid resolution of pulmonary infiltrates and return of arte-
asymptomatic, but almost all affected children have an infection. The rial blood gas values to normal within 96 hours after the initial
most common infections are umbilicus infections, skin infections, respiratory insult. However, pulmonary infiltrates have persisted for
abscesses, and respiratory tract infections. Less commonly, infants at least 7 days after the transfusion reaction in 17% of TRALI
experience otitis media, urinary tract infections (UTIs), and gastro- patients.
enteritis. Serious infections such as sepsis, pneumonia, and meningitis TRALI has been associated with both neutrophil and HLA anti-
can occur. The duration of the neutropenia may be as short as a few bodies. Antibodies reported in these reactions include HNA-1a,
261
days or as long as 28 weeks. The mean duration of neutropenia is HNA-1b, HNA-2, HNA-3a, and HLA class I and II antibodies.
about 11 weeks. 261 Most of these cases involve the passive transfusion of the offending
For an asymptomatic child, no immediate treatment may be antibody in donor plasma, as contrasted with the reactivity of the
required. Prompt and aggressive antibiotic treatment of children with recipient’s antibody with donor leukocytes to cause febrile nonhemo-
fevers or other signs of infections is indicated. Intravenous immuno- lytic reactions. Retrospective studies involving antibodies to HNA-3a
globulin has a limited role in the treatment of neonatal alloimmune have implicated blood components from single donors with anti-
neutropenia. Approximately half the patients treated have a transient HNA-3a in several TRALI cases. 258
increase in count lasting only a few days. The use of G-CSF to treat
alloimmune neutropenia has also had mixed results. The administra-
tion of G-CSF elevates the neutrophil count in some but not all NEUTROPHIL ANTIGENS SUMMARY
neonates. 261
Five neutrophil antigen systems, HNA-1, HNA-2, HNA-3, HNA-4
and HNA-5 have been well described. HNA-1 antigens are located
AUTOIMMUNE NEUTROPENIA OF CHILDHOOD on FcγRIIIb, and antibodies to these antigens are frequently
implicated in autoimmune and alloimmune neutropenia. HNA-2
Autoimmune neutropenia has been well described in children. 262–265 is located on CD177 glycoprotein, and antibodies to HNA-2 are
Typically the onset of the autoimmune neutropenia of children found in patients with alloimmune and autoimmune neutropenia.
begins at 8 months of age, but children between 1 and 36 months Antibodies to HNA-3a are rare; but relative to other neutrophil
of age can be affected. Most studies found that neutrophil counts antibodies, may be more frequently associated with cases of TRALI.
recover spontaneously by the age of 5 years, with a median of 13 to The significance, if any, of HNA-4a and HNA-5a antibodies is
20 months of neutropenia. 262–265 uncertain.
In most cases, children presented with severe neutropenia, having
9
neutrophil counts less than 0.5 × 10 /L. Monocytosis has been
reported to occur in up to 38% of patients. Results of bone marrow REFERENCES
biopsies in affected patients usually show normal to hypercellular
marrow with a decreased number of mature granulocytes. Febrile For the complete list of references, log on to www.expertconsult.com.
episodes and infections, including bacterial skin infections, otitis
media, respiratory tract infections, and UTIs, are common. Life-
threatening complications are rare. SUGGESTED READINGS
Antibodies to neutrophils can be detected in up to 98% of affected
patients. If antibody specificity is identified, the antibodies are almost Braun WE: Update in kidney transplantation: Increasing clinical success,
always specific to epitopes located on FcγRIIIb. The antibodies are expanding waiting lists. Cleve Clin J Med 69:501, 2002.
directed to HNA-1a in 10% to 46% of patients, to HNA-1b in 2% Bux J, Behrens G, Jaeger G, et al: Diagnosis and clinical course of autoimmune
to 3% of patients, and rarely to FcγRIIIb epitopes expressed by neutropenia in infancy: Analysis of 240 cases. Blood 91:181, 1998.
granulocytes from all donors. 262,265 Bux J, Jung KD, Kauth T, et al: Serological and clinical aspects of granulocyte
Autoimmune neutropenia has been treated with corticosteroids, antibodies leading to alloimmune neonatal neutropenia. Transfus Med
intravenous immunoglobulin, and G-CSF. Approximately half the 2:143, 1992.
patients responded to intravenous immunoglobulin, but neutrophil Childs R, Srinivasan R: Advances in allogeneic stem cell transplantation:
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responded to G-CSF and 75% to corticosteroids, and neutrophil 2002.
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TRANSFUSION REACTIONS Dawkins RL, Degli-Esposti MP, Abraham LJ, et al: Conservation versus poly-
morphism of the MHC in relation to transplantation, immune responses
Antibodies to neutrophil and HLA antigens can cause febrile nonhe- and autoimmune disease. In Klein J, Klein D, editors: Molecular evolution
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transfusion of leukocyte-reduced blood components, approximately 391.
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TRALI is approximately 5%. Of patients with TRALI, 80% neutropenias and transfusion reactions. Eur J Immunol 31:1301, 2001.