Scholarlink

Background <p>Combined immunodeficiency (CID) involves profound defects in B and T lymphocyte development and function. This study examined clinical and immunological phenotypes of CID patients with and without pulmonary manifestations.</p> Methods <p>This retrospective multicenter study included 53 CID patients diagnosed between 2009 and 2022 with available thoracic computed tomography scans. Patients were categorized based on pulmonary manifestations presence. Demographic, clinical, and laboratory characteristics were compared using conservative statistical thresholds (<i>P</i> &lt; 0.01). All laboratory parameters were interpreted using age-adjusted pediatric reference ranges.</p> Results <p>Among 53 patients (56.6% male), 43 had pulmonary abnormalities on HRCT. Common clinical features included skin lesions (43.4%), failure to thrive (34%), and autoimmunity (32.1%). HRCT revealed pneumonia (28.3%), bronchiectasis (18.9%), interstitial lung disease with BOOP-like pattern (3.8%), and other findings. Using age-adjusted pediatric reference ranges, profound immunological defects were confirmed: absolute lymphocyte count below the 5th percentile in 92% (49/53), CD3 + T cells below the 5th percentile in 94% (47/50 tested), CD4 + T cells below the 5th percentile in 96% (51/53), CD19 + B cells below the 5th percentile in 94% (50/53), and hypogammaglobulinaemia (IgG below the 5th percentile) in 98% (52/53). Patients with abnormal HRCT had significantly lower CD4 + T-cell counts (178 vs. 498 cells/µL; <i>P</i> = 0.008) and CD19 + B-cell counts (42 vs. 189 cells/µL; <i>P</i> = 0.009). Bronchoscopy identified Aspergillus fumigatus, Streptococcus pneumoniae, and multidrug-resistantAcinetobacter baumannii. Deceased patients showed significantly lower baseline platelets (183,000 vs. 266,000 cells/µL; <i>P</i> = 0.009), IgG (380 vs. 720&#xa0;mg/dL; <i>P</i> = 0.007), and IgE (0.8 vs. 12 IU/mL; <i>P</i> = 0.008).</p> Conclusion <p>Pulmonary manifestations affect 81.1% of Iranian CID patients. Low baseline platelets, IgG, and IgE constitute a robust prognostic triad for mortality (<i>P</i> = 0.009, <i>P</i> = 0.007, <i>P</i> = 0.008 respectively). Application of age-adjusted reference ranges revealed profound immunological defects. Systematic HRCT surveillance using low-dose protocols and distinguishing infectious sequelae from immune-mediated lung disease guides targeted management in resource-limited settings.</p>

错误：搜索内容不能为空，请输入英文关键词
错误：关键词超出字数限制，请精简
高级检索

Demographic, clinical, and immunological features in combined immunodeficiency patients: a comparative analysis of those with and without pulmonary manifestations – a multicenter study from Iran

  • 期刊论文

  • 出版模式：

    开放获取

  • 发表日期：

    2026年1月31日

查看全文
  • Ghamartaj Khanbabaee,
  • Matin Pourghasem,
  • Mahnaz Jamee,
  • Seyed Ahmad Tabatabaii,
  • Mitra Khalili,
  • Samin Sharafian,
  • Mehrnaz Mesdaghi,
  • Mahnaz Sadeghi-Shabestari,
  • Armin Shirvani,
  • Saeid Sadr,
  • Arefeh Zahmatkesh,
  • Samaneh Delavari,
  • Narges Eslami,
  • Nazanin Farahbakhsh,
  • Mahboubeh Mansouri,
  • Ebrahim Tabiei,
  • Seyedeh Zalfa Modarresi,
  • Abdolhamid Taghizadeh Behbahani,
  • Golnaz Eslamian,
  • Mazdak Fallahi,
  • Javad Enayat,
  • Shahrzad Fallah,
  • Mahsa Pourghasem,
  • Asghar Aghamohammadi,
  • Zahra Chavoshzadeh

摘要

Background

Combined immunodeficiency (CID) involves profound defects in B and T lymphocyte development and function. This study examined clinical and immunological phenotypes of CID patients with and without pulmonary manifestations.

Methods

This retrospective multicenter study included 53 CID patients diagnosed between 2009 and 2022 with available thoracic computed tomography scans. Patients were categorized based on pulmonary manifestations presence. Demographic, clinical, and laboratory characteristics were compared using conservative statistical thresholds (P < 0.01). All laboratory parameters were interpreted using age-adjusted pediatric reference ranges.

Results

Among 53 patients (56.6% male), 43 had pulmonary abnormalities on HRCT. Common clinical features included skin lesions (43.4%), failure to thrive (34%), and autoimmunity (32.1%). HRCT revealed pneumonia (28.3%), bronchiectasis (18.9%), interstitial lung disease with BOOP-like pattern (3.8%), and other findings. Using age-adjusted pediatric reference ranges, profound immunological defects were confirmed: absolute lymphocyte count below the 5th percentile in 92% (49/53), CD3 + T cells below the 5th percentile in 94% (47/50 tested), CD4 + T cells below the 5th percentile in 96% (51/53), CD19 + B cells below the 5th percentile in 94% (50/53), and hypogammaglobulinaemia (IgG below the 5th percentile) in 98% (52/53). Patients with abnormal HRCT had significantly lower CD4 + T-cell counts (178 vs. 498 cells/µL; P = 0.008) and CD19 + B-cell counts (42 vs. 189 cells/µL; P = 0.009). Bronchoscopy identified Aspergillus fumigatus, Streptococcus pneumoniae, and multidrug-resistantAcinetobacter baumannii. Deceased patients showed significantly lower baseline platelets (183,000 vs. 266,000 cells/µL; P = 0.009), IgG (380 vs. 720 mg/dL; P = 0.007), and IgE (0.8 vs. 12 IU/mL; P = 0.008).

Conclusion

Pulmonary manifestations affect 81.1% of Iranian CID patients. Low baseline platelets, IgG, and IgE constitute a robust prognostic triad for mortality (P = 0.009, P = 0.007, P = 0.008 respectively). Application of age-adjusted reference ranges revealed profound immunological defects. Systematic HRCT surveillance using low-dose protocols and distinguishing infectious sequelae from immune-mediated lung disease guides targeted management in resource-limited settings.