Brucellosis presenting with pancytopenia due to hemophagocytic syndrome

Abstract

Reactive hemophagocytic syndrome is clinically characterized by fever, hepatosplenomegaly, pancytopenia, and coagulopathy, and is histologically characterized by excessive proliferation and activation of histiocytes or macrophages. It can occur with systemic infections, immunodeficiency, or underlying malignancy. Brucellosis is one of the rare causes of hemophagocytosis. Herein we report an 11-year-old male with pancytopenia due to hemophagocytosis during the course of brucellosis that responded favorably to therapy. Although rare, hemophagocytosis should be considered as a possible cause of pancytopenia in patients with brucellosis, especially in regions where brucellosis is frequently encountered. (Turk J Hematol 2011; 28: 68-71 ÖzetReaktif hemofagositik sendrom klinik olarak ateş, karaciğer ve dalak boyutlarında artış, pansitopeni ve pıhtılaşma bozuklukları; histolojik olarak da histiyosit ya da makrofajların artmış proliferasyon ve aktivasyonları ile karakterizedir. Sistemik hastalıklar, immun yetmezlikler ve altta yatan malignitelerle birlikte olabilir. Brusella, hemofagositozun nadir nedenlerinden biridir. Brusellanın sık görüldüğü ülkelerde pansitopenisi olan hastalarda hemofagositozu hatırlatmak amacı ile, brusella seyri sırasında hemofagositoza bağlı pansitopenisi olan, uygun tedavi sonrası iyileşen 11 yaşında bir erkek hasta sunulmaktadır. (Turk J Hematol 2011; 28: 68-71) Anahtar kelimeler: Brusella, pansitopeni, hemofagositoz

Pancitopenia secundaria a infección por Brucella abortus

“…Las alteraciones paraclínicas más comunes son a nivel de las líneas celulares dado por neutropenia y anemia, siendo más raros los hallazgos de trombocitopenia y pancitopenia con una posible frecuencia entre 2 y 14% de los individuos afectados (10). La patogénesis de la pancitopenia no ha sido claramente establecida, posibles mecanismos asociados son la hemofagocitosis histiocítica, condición caracterizada por la fagocitosis de todos los tipos de células sanguíneas por histiocitos activados, otras condiciones incluyen hiperesplenismo, granulomas o hipoplasia de médula ósea y destrucción inmune (13)(14)(15). La brucelosis también se ha relacionado con alteración de enzimas hepáticas y elevación de marcadores inflamatorios (2).…”

Hemophagocytic Lymphohistiocytosis Disease Mimic Due to Brucellosis

“…The umbrella term would include: 6 HLH disease: Patients who would benefit from HLH-specific therapies HLH disease mimics: Patients in whom distinctive immune dysregulation is not the core problem and thus would not benefit from immune suppression but rather treatment of the underlying condition A PubMed search and review of case reports of pediatric brucellosis and HLH in the literature mainly occur in areas of the world where brucella is endemic or in patients who recently visited such areas (Table 2). [7][8][9][10][11][12][13][14][15][16][17] Many of these patients underwent invasive procedures (such as bone marrow biopsy) to complete HLH evaluation, and some received HLH-directed treatment, despite most patients improving quickly on antibiotics alone. We believe that in those cases where brucella and HLH disease mimics are suspected, a cautious approach should be taken, and invasive procedures and HLH-directed treatment should be deferred if the patient remains stable.…”

An Unusual Cause of Febrile Neutropenia: Brucellosis

“…Hematolojik komplikasyonlar brusellozda sık görülmekte ve birçok hematolojik hastalığı taklit edebilmektedir [11][12][13] . Brusellozun hematolojik bulguları arasında anemi, lökopeni, trombositopeni, pansitopeni, kanama diyatezi, yaygın damar içi pıhtılaşma ve hemofagositik sendrom yer almaktadır 1,[12][13][14][15][16][17][18] .…”