Persistent lymphadenopathy associated with hypertransfusion in sickle-cell disease

Area of Science:

• Immunology
• Hematology
• Virology

Background:

• Sickle-cell disease (SCD) patients, particularly those receiving hypertransfusion (HT), may exhibit altered immune profiles.
• Persistent generalized lymphadenopathy in HT SCD patients warrants investigation into underlying immunologic dysfunction.

Observation:

• Immunologic evaluation of two HT SCD patients with lymphadenopathy revealed decreased T-lymphocyte percentages (including helper and suppressor subsets) and decreased lymphocyte proliferation.
• These patients exhibited higher spontaneous suppressor cell activity and specific antibodies for human T cell lymphotropic virus type III (HTLV-III).

Findings:

• HT SCD patients with lymphadenopathy showed distinct immunologic abnormalities compared to other HT SCD patients.
• These abnormalities included decreased T4+ lymphocytes, reduced T helper/suppressor ratios, and diminished mitogen responses.
• Antibody to HTLV-III was detected in the serum of the affected patients.

Implications:

• The findings suggest transfusion-acquired HTLV-III infection as the cause of acquired immunodeficiency syndrome-related lymphadenopathy in these patients.
• Hypertransfusion therapy in SCD, potentially combined with phenytoin, may pose a risk for developing HTLV-III-associated syndromes.
• This highlights the importance of screening blood products for HTLV-III and monitoring immune status in transfused SCD patients.