Información del artículo
La hemofagocitosis es un cuadro clínico caracterizado por la activación de los macrófagos y histiocitos, con intensa actividad fagocítica en la médula ósea e otras localizaciones del sistema reticuloendotelial, lo que provoca la fagocitosis de los eritrocitos, leucocitos, plaquetas y sus precursores. Su presencia puede estar asociada con infecciones, neoplasias, enfermedades autoinmunitarias, drogas y una variedad de otras condiciones médicas. En este caso clínico, presentamos a una mujer de 36 años, previamente sana, que desarrolló hemofagocitosis, al mismo tiempo que completó los criterios de diagnóstico de lupus eritematoso sistémico. La hemofagocitosis asociada con el lupus es una entidad rara, potencialmente mortal, de diagnóstico diferencial complicado y, que requiere una intervención terapéutica urgente. Hay muy pocos casos comunicados en la literatura, y es necesaria una mejor comprensión de los aspectos clínicos, causas, fisiopatología, criterios de diagnóstico y tratamiento de este síndrome.
Palabras clave:
Hemofagocitosis
Palabras clave:
Lupus eritematoso sistémico
Hemophagocytic Syndrome is a clinical condition characterized by the activation of either macrophages or histiocytes with a prominent hemophagocytosis feature in the bone marrow and other reticuloendothelial systems. It leads to the phagocytosis of erythrocytes, leukocytes, platelets and their precursors. The presence of hemophagocytosis can be associated to infections, malignancies, autoimmune diseases, drugs and a variety of other medical conditions. We report a case of a previously healthy 36 year-old woman that developed hemophagocytosis at the same time that fulfilled diagnostic criteria for systemic lupus erythematosus. Lupus related hemophagocytic syndrome is a rare and potentially fatal entity. It offers significant differential diagnosis challenges and requires urgent therapeutic intervention. There are only few cases reported in the literature. However, much is still needed in order to better understand its causes, all the immunopathogenic mechanisms, as well as its clinical and therapeutic aspects.
Keywords:
Hemophagocytic síndrome
Keywords:
Systemic lupus erythematosus
El Texto completo está disponible en PDF
Bibliografía
[1]
Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis. Int J Hematol. 2000;72:1-11. [Pubmed]
[2]
Papo T, Andre MH, Amoura Z, et al. The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus. J Rheumatol 1999;26:927-30. [Pubmed]
[3]
Billiau AD, Roskams T, Van Damme-Lombaerts R, Matthys P, Wouters C. Macrophage activation syndrome: characteristic findings on liver biopsy illustrating the key role of activated, IFNgamma-producing lymphocytes and IL-6 and TNF-alpha-producing macrophages. Blood 2005;105:1648-51. [Pubmed]
[5]
Osugi Y, Hara J, Tagawa S, et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood 1997;89:4100-3. [Pubmed]
[6]
Wong KF, Hui PK, Chan JK, Chan YW, Ha SY. The acute lupus hemophagocytic syndrome. Ann Intern Med 1991;114:387-90. [Pubmed]
[7]
Takahashi K, Kumakura S, Ishikura H, et al. Reactive hemophagocytosis in systemic lupus erythematosus. Intern Med 1998;37:550-3. [Pubmed]
[8]
Mouy R, Stephan JL, Pillet P, Haddad E, Hubert P, Prieur AM. Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: report of five cases. J Pediatr 1996;129:750-4. [Pubmed]
[9]
Emmenegger U, Frey U, Reimers A, Fux C, Semela D, Cottagnoud P, Spaeth PJ, Neftel KA. Hyperferritinema as indicator for intravenous immunoglobulin treatment in reactive macrophage activation syndromes. Am J Hematol 2001;68:4-10. [Pubmed]
[10]
Takahashi N, Naniwa T, Banno, S. Successful use of etanercept in the treatment of acute lupus hemophagocytic syndrome. Mod Rheumatol 2008;18:72-5. [Pubmed]
[11]
Buyon JP. Systemic lupus erythematosus. Clinical and laboratory features. Primer on the Rheumatic Diseases. GA: Arthritis Foundation; 2001:335-45.
[12]
Romanou V, Hatzinikolaou P, Mavragani KI, Meletis J, Vaiopoulos G. Lupus Erythematosus complicated by Hemophagocytic Syndrome. J Clin Rheumatol 2006;12:301-3. [Pubmed]
[13]
KumakuraS, Ishikura H, Endo J, C Autoimmune-associated hemophagocytosis. Am J Hematol 1995;50:148-9. [Pubmed]
[14]
KumakuraS, Ishikura H, Umagae N, Yamagata S, Kobayashi S. Autoimmune-associated hemophagocytosis syndrome. Am J Med 1997;102:113-5. [Pubmed]
[15]
Kumakura S, Kondo M, Murakawa Y, et al. Autoimmune-associated hemophagocytic syndrome (Review article). Mod Rheumatol 2004;14:205-15. [Pubmed]
[16]
Lou YJ, Jin J, Mai WY. Ankylosing spondylitis presenting with macrophage activation syndrome. Clin Rheumatol 2007;26:1929-1930. [Pubmed]
[17]
Ken-ichi H, Masayuki M, MarikoI, et al. Successul treatment of fulminant pulmonary hemorrhage associated with systemic lupus erythematosus. Clin Rheumatol 2004;23:252-5. [Pubmed]
[18]
Mori Y, Sugiyama T, Chiba R, et al. A case of systemic lupus erythematosus with hemophagocytic syndrome and cytophagic histiocytic paniculitis. Ryumachi 2001;41:31-6. [Pubmed]
[19]
Nomura S, Koshikawa K, Hamamoto K, et al. Steroid and gamma globulin therapy against virus associated hemophagocytic syndrome. Rinsho Ketsueki 1992;33:1242-7. [Pubmed]
[20]
Dhate R, Simon J, Papo T, et al. Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Arthritis Rheum 2003; 49:633-9. [Pubmed]
[21]
Aoki A, Hagiwara E, Ohno S, et al. Case report of systemic lupus erythematosus patient with hemophagocytic syndrome treated with plasma exchange, with specific reference to clinical profile and serum cytokine levels. Ryumachi 2001;41:945-50. [Pubmed]
[22]
Matsumoto Y, Naniwa D, Banno S, et al. The efficacy of therapeutic plasmapheresis for the treatment of fatal hemophagocytic syndrome: two case reports. Ther Apher 1998;2:300-4. [Pubmed]
[23]
Godfrey T, Khmashta MA, Hughes GRV. Therapeutic advances in systemic lupus erythematosus. Curr Opin Rheum 1998;10:435-41.
