Solunum Sıkıntısı ile Beraber Anemi ve Retikülosit Yüksekliği: İdiopatik Pulmoner Hemosideroz İçin Uyarıcı
Abstract
Amaç: İdiopatik pulmoner hemosideroz (İPH), tekrarlayan hemoptizi, demir eksikliği anemisi ve pulmoner infiltrasyonlar ile giden nadir bir hastalıktır. Bu çalışmada başlangıçta anemi ve/veya retikülosit yüksekliği bulunan ve İPH tanısı alan hastaların özelliklerinin incelenmesi amaçlanmıştır.
Gereç ve Yöntemler: Anemi ve/veya retikülosit yüksekliği ile başvurup İPH tanısı almış ve Hacettepe Çocuk Göğüs Hastalıkları bölümünde takipte olan hastalar retrospektif olarak incelendi.
Bulgular: Çalışmaya dahil edilen 14 hastanın dokuzu (%64.3) kızdı. Ortanca tanı yaşı 60 ay (IQR: 42)’di. Şikayetler ile tanı arasında geçen ortanca süre 12 ay (IQR:15.5)’di. Ortanca başvuru hemoglobin (Hb) düzeyleri 6.7 g/dL (IQR: 4.07), ortanca retikülosit yüzdesi 5.1 (IQR: 3.2)’di. Tanı anında hastaların sadece 5’sinde (%35.71) hastalığın klasik triadı olan hemoptizi, demir eksikliği anemisi ve pulmoner infiltrasyon bulguları saptandı.
Oniki (%85.7) hastada tanı anında anormal akciğer grafisi saptandı. On (71.4%) hastaya başlangıçta pnömoni tanısı konulduğu kaydedildi. Tanı öncesinde 12 (%85.7) hastada demir eksikliği anemisi saptanarak tedavi verildiği saptandı. Bu hastaların tümünde demir eksikliği anemisinin tedaviye dirençli seyrettiği öğrenildi. Hastaların 13’üne (%92.9) tanı anına kadar en az bir kez kan trasfüzyonu yapıldığı saptandı.
Sonuç: İPH her zaman klasik triad şeklinde izlenmeyebilir. Demir eksikliği anemisi ile birlikte solunumsal bulguları olan ve görüntülemede pulmoner infiltrasyon bulunan hastalarda, tedavilere beklenen yanıt alınamaması veya tekrarlayan ataklar olması durumunda İPH akla gelmelidir.
Keywords
References
- 1. de Silva C, Mukherjee A, Jat KR, Lodha R, Kabra SK. Pulmonary Hemorrhage in Children: Etiology, Clinical Profile and Outcome. Indian J Pediatr. 2019;86(1):7-11.
- 2. Zhang Y, Luo F, Wang N, Song Y, Tao Y. Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients. J Int Med Res. 2019;47(1):293-302.
- 3. Le Clainche L, Le Bourgeois M, Fauroux B, et al. Long-term outcome of idiopathic pulmonary hemosiderosis in children. Medicine (Baltimore). 2000;79(5):318-326.
- 4. Taytard J, Nathan N, de Blic J, et al. New insights into pediatric idiopathic pulmonary hemosiderosis: the French RespiRare (R) cohort. Orphanet Journal of Rare Diseases. 2013;8.
- 5. Bakalli I, Kota L, Sala D, et al. Idiopathic pulmonary hemosiderosis - a diagnostic challenge. Ital J Pediatr. 2014;40:35.
- 6. Kiper N, Gocmen A, Ozcelik U, Dilber E, Anadol D. Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979-1994): prolonged survival with low-dose corticosteroid therapy. Pediatr Pulmonol. 1999;27(3):180-184.
- 7. Zhang X, Wang L, Lu A, Zhang M. Clinical study of 28 cases of paediatric idiopathic pulmonary haemosiderosis. J Trop Pediatr. 2010;56(6):386-390.
- 8. Castellazzi L, Patria MF, Frati G, Esposito AA, Esposito S. Idiopathic pulmonary haemosiderosis in paediatric patients: how to make an early diagnosis. Ital J Pediatr. 2016;42(1):86.
- 9. Ahmed M, Raj D, Kumar A, Kumar A. Anaemia and respiratory failure in a child: can it be idiopathic pulmonary haemosiderosis? BMJ Case Rep. 2017;2017.
- 10. Blanco A, Solis P, Gomez S, Valbuena C, Telleria JJ. Antineutrophil cytoplasmic antibodies (ANCA) in idiopathic pulmonary hemosiderosis. Pediatr Allergy Immunol. 1994;5(4):235-239.
- 11. Kurland G, Deterding RR, Hagood JS, et al. An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med. 2013;188(3):376-394.
- 12. Poggi V, Lo Vecchio A, Menna F, Menna G. Idiopathic pulmonary hemosiderosis: a rare cause of iron-deficiency anemia in childhood. J Pediatr Hematol Oncol. 2011;33(4):e160-162.
- 13. Kabra SK, Bhargava S, Lodha R, Satyavani A, Walia M. Idiopathic pulmonary hemosiderosis: clinical profile and follow up of 26 children. Indian Pediatr. 2007;44(5):333-338.
Anemia and Reticulocyte Elevation with Respiratory Symptoms: Warning For Idiopathic Pulmonary Hemosiderosis
Abstract
Objective: Idiopathic pulmonary hemosiderosis (IPH) is a rare disease with recurrent hemoptysis, iron deficiency anemia, and pulmonary infiltrates. We aimed to examine the characteristics of patients who had anemia and/or elevation of reticulocyte and were diagnosed with IPH.
Material and Methods: 14 patients with a diagnosis of idiopathic pulmonary hemosiderosis who had anemia and/or elevation of reticulocyte at initial presentation were retrospectively analyzed.
Results: A total of 14 children were included in the study. Nine (64.3%) were female. The median age at the diagnosis was 60 months (IQR: 42). The median time from onset to diagnosis was 12 months (IQR: 15.5). The median hemoglobin levels were 6.7 g/dL (IQR:4.07). At the time of initial diagnosis, only 5 (35.71%) patients had a classical triad of hemoptysis, iron deficiency anemia, and pulmonary infiltration. Abnormal chest X-ray was detected in 12 (85.7%) of patients at the time of diagnosis. Ten (71.4%) patients were diagnosed with pneumonia at the initial presentation. Iron deficiency anemia was detected in 12 (85.7%) patients before diagnosis and all were resistant to treatment. Thirteen (92.9%) patients had a history of blood transfusion at least once before diagnosis.
Conclusion: IPH may not always present with the classical triad. In patients with respiratory findings and pulmonary infiltration with iron deficiency anemia, IPH should be considered in unresponsive patients to treatments or recurrent attacks.
References
- 1. de Silva C, Mukherjee A, Jat KR, Lodha R, Kabra SK. Pulmonary Hemorrhage in Children: Etiology, Clinical Profile and Outcome. Indian J Pediatr. 2019;86(1):7-11.
- 2. Zhang Y, Luo F, Wang N, Song Y, Tao Y. Clinical characteristics and prognosis of idiopathic pulmonary hemosiderosis in pediatric patients. J Int Med Res. 2019;47(1):293-302.
- 3. Le Clainche L, Le Bourgeois M, Fauroux B, et al. Long-term outcome of idiopathic pulmonary hemosiderosis in children. Medicine (Baltimore). 2000;79(5):318-326.
- 4. Taytard J, Nathan N, de Blic J, et al. New insights into pediatric idiopathic pulmonary hemosiderosis: the French RespiRare (R) cohort. Orphanet Journal of Rare Diseases. 2013;8.
- 5. Bakalli I, Kota L, Sala D, et al. Idiopathic pulmonary hemosiderosis - a diagnostic challenge. Ital J Pediatr. 2014;40:35.
- 6. Kiper N, Gocmen A, Ozcelik U, Dilber E, Anadol D. Long-term clinical course of patients with idiopathic pulmonary hemosiderosis (1979-1994): prolonged survival with low-dose corticosteroid therapy. Pediatr Pulmonol. 1999;27(3):180-184.
- 7. Zhang X, Wang L, Lu A, Zhang M. Clinical study of 28 cases of paediatric idiopathic pulmonary haemosiderosis. J Trop Pediatr. 2010;56(6):386-390.
- 8. Castellazzi L, Patria MF, Frati G, Esposito AA, Esposito S. Idiopathic pulmonary haemosiderosis in paediatric patients: how to make an early diagnosis. Ital J Pediatr. 2016;42(1):86.
- 9. Ahmed M, Raj D, Kumar A, Kumar A. Anaemia and respiratory failure in a child: can it be idiopathic pulmonary haemosiderosis? BMJ Case Rep. 2017;2017.
- 10. Blanco A, Solis P, Gomez S, Valbuena C, Telleria JJ. Antineutrophil cytoplasmic antibodies (ANCA) in idiopathic pulmonary hemosiderosis. Pediatr Allergy Immunol. 1994;5(4):235-239.
- 11. Kurland G, Deterding RR, Hagood JS, et al. An official American Thoracic Society clinical practice guideline: classification, evaluation, and management of childhood interstitial lung disease in infancy. Am J Respir Crit Care Med. 2013;188(3):376-394.
- 12. Poggi V, Lo Vecchio A, Menna F, Menna G. Idiopathic pulmonary hemosiderosis: a rare cause of iron-deficiency anemia in childhood. J Pediatr Hematol Oncol. 2011;33(4):e160-162.
- 13. Kabra SK, Bhargava S, Lodha R, Satyavani A, Walia M. Idiopathic pulmonary hemosiderosis: clinical profile and follow up of 26 children. Indian Pediatr. 2007;44(5):333-338.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Internal Diseases |
| Journal Section | ORIGINAL ARTICLES |
| Authors | |
| Publication Date | May 25, 2021 |
| Submission Date | March 13, 2021 |
| Published in Issue | Year 2021 Volume: 15 Issue: 3 |
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