Rapid Resolution of Multiple Liver Abscesses in a Chronic Granulomatous Disease Patient with Granulocyte Transfusions

Yıl 2022, Cilt: 16 Sayı: 1, 70 - 74, 28.01.2022

Ayşe Metin Azize Pınar Metbulut Ömer Güneş Gülsüm İclal Bayhan Güzin Cinel Gülşah Bayram Ilıkan Abdurrahman Kara

https://doi.org/10.12956/tchd.858827

Cited By: 1

Öz

Chronic granulomatous disease (CGD) is a genetically heterogeneous primary immune deficiency of phagocyte function characterized by recurrent, life-threatening bacterial and fungal infections that lead to granuloma formation. Early diagnosis is possible by the awareness of the clinician about early infectious clues of the disease. Aggressive treatment of infectious complications is very important in CGD patients and subsequently antimicrobial (antibiotic and antifungal) and immunomodulatory (interferon-gamma) prophylaxis until hematopoietic stem cell transplantation. Despite improved mortality, morbidities due to complications associated with CGD remain significant. One of these is a hepatic abscess in CGD patients which is seen in more than one-quarter of patients and also very refractory and frequently requires multiple surgeries with frequent morbidities. Therefore, the most optimal and beneficial treatments are still being investigated in the world. We present a 3 y old CGD patient with multiple liver abscesses due to S.aureus and Aspergillus spp who treated by several percutaneous liver-directed interventional radiological treatment along with granulocyte transfusions

Anahtar Kelimeler

chronic granulomatous disease, child, hepatic abcess

Çoklu Karaciğer Apsesi Olan Bir Kronik Granülomatöz Hastasının Granülosit Transfüzyonu İle Hızlı Tedavisi

Öz

Kronik granülomatöz hastalık (CGD), tekrarlayan, yaşamı tehdit eden bakteri ve mantar enfeksiyonları ile karakterize olan, granülom oluşumuna yol açan genetik olarak heterojen bir primer immün yetmezlik olan fagosit fonksiyon yetersizliğidir. Erken teşhis, klinisyenin hastalığın erken enfeksiyon ipuçları konusunda bilinçlenmesi ile mümkündür. CGD hastalarında Enfeksiyöz komplikasyonların agresif tedavisi çok önemlidir ve hematopoietik kök hücre nakline kadar antimikrobiyal (antibiyotik ve antifungal) ve immünomodülatör (interferon-gama) profilaksileri de çok önemlidir. Mortalitenin azalmasına rağmen, CGD ile ilişkili komplikasyonlara bağlı morbiditeler önemini korumaktadır. Bunlardan biri, CGD hastasında, hastaların dörtte birinden fazlasında görülen ve aynı zamanda çok dirençli ve sıklıkla sık morbiditeli birden fazla ameliyat gerektiren karaciğer apsesidir. Bu nedenle dünyada halen en uygun ve faydalı tedaviler araştırılmaktadır. Granülosit transfüzyonları ile birlikte perkütan karaciğere yönelik girişimsel radyolojik tedavi ile tedavi edilen S.aureus ve Aspergillus spp’ye bağlı çoklu karaciğer apsesi olan 3 yaşında bir CGD hastasını sunuyoruz.

Anahtar Kelimeler

Kronik granulomatöz hastalık, çocuk, hepatik abse

Kaynakça

• Segal BH,Leto TL, Gallin JI, malech HI, holland SM. genetic, biochemical, and clinical features of chronic granulomatous disease. Medicine 2000; 79:170-200.
• Ahlin A, Fasth A. Chronic granulomatous disease- conventional treatment vs. hematopoietic stem cell transplantation: an update. Curr Opin Hematol 2015; 22:41–5.
• Winkelstein JA, Marino MC, Johnston RB Jr, Boyle J, Curnutte J, Gallin JI, et al. Chronic granulomatous disease. Report on a national registry of 368 patients. Medicine. 2000; 79:155–69.
• T Turul-Özgür, G Türkkani-Asal, I Tezcan, MY Köker, A Metin, L Yel, et al. Clinical features of chronic granulomatous disease: a series of 26 patients from a single center. Turk J Pediatr 2010;52: 576-81.
• Köker MY, Camcıoğlu Y, van Leeuwen K, Kılıç SŞ, Barlan I, Yılmaz M, et al. Clinical, functional, and genetic characterization of chronic granulomatous disease in 89 Turkish patients. J Allergy Clin Immunol 2013;132;1156-63.
• Köker MY, Sanal O, De Boer M, Tezcan I, Metin A, Ersoy F, Roos D. Mutations of chronic granulomatous disease in Turkish families. Eur J Clin Invest 2007; 37: 589-95.
• MY Köker, Ö Sanal, M De Boer, I Tezcan, A Metin, C Tan, F Ersoy, D Roos. Skewing of X‐chromosome inactivation in three generations of carriers with X‐linked chronic granulomatous disease within one family. Eur J Clin Invest 2006; 36: 257-64.
• Lublin M, Bartlett DL, Danforth DN, Kauffman H, Gallin JI,Malech HL et al. Hepatic abscess in patient with chronic granulomatous disease. Annals of Surgery 2002;235:383-91.
• Straughan DM, McLoughlin KC, Mullinax JE, Marciano BE, Freeman AF, Anderson VL et al. The changing paradigm of management of liver abscesses in chronic granulomatous disease. Clin Infect Dis 2018;66:1427-34.
• Price TH, Boeckh M, Harrison RW, McCullough J, Ness PM,Strauss RG, et al .Efficacy of transfusion with granulocytes from G-CSF/dexamethasone treated donors in neutropenic patients with infection. Blood 2015;126: 2153-61.
• Gea-Banacloche. Granulocyte transfusions: A concise review for practitioners. Cytotherapy 2017;19: 1256-69. Stroncek DF, Leonard K, Eiber G, Malech HL, Gallin JI, Leitman SF. Alloimmunization after granulocyte transfusions. Transfusion 1996;36:1009-15.