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http://www.revistanefrologia.com © 2010 Revista <strong>Nefrología</strong>. Órgano Oficial de la Sociedad Española de <strong>Nefrología</strong> Acute lupus hemophagocytic syndrome: report of a case C. Botelho 1 , F. Ferrer 1 , L. Francisco 2 , P. Maia 1 , T. Mendes 1 , A. Carreira 1 1 Departments of Nephrology and 2 Hematology. Centro Hospitalar de Coimbra. Coimbra. Portugal Nefrologia 2010;30(2):247-51 ABSTRACT 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. Key words: Hemophagocytic síndrome. Systemic lupus erythematosus. INTRODUCTION Hemophagocytic Syndrome (HS) is a clinicopathologic entity characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system. 1 HS may develop as a rare but potentially fatal complication of several disorders including malignancies, drugs, infections and autoimmune disorders. Patients usually present with an acute febrile illness, sometimes fulminant and lethal. Common manifestations Correspondencia: Carlos Botelho Rua São Teotónio Lote 17, 1.º D. 3000 Coimbra. Portugal. medmigui@hotmail.com casos clínicos Hemofagocitosis asociada con el lupus: caso clínico RESUMEN 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. Lupus eritematoso sistémico. include high fever, pancytopenia, hepatosplenomegaly, weight loss, lymphadenopathy, abnormal liver function tests (LFTs), hyperferritinemia and high blood triglyceride levels 2 . Disseminated intravascular coagulation and central nervous system dysfunction often ensue, and less frequently the lungs and cardiac tissues are involved. Although the pathogenic mechanisms still remain unknown, overproduction of pro-inflammatory cytokines, uncontrolled activation of T cells and macrophages, associated with decreased natural killer cell and cytotoxic cell functions, seem to be the hallmarks of the immunologic abnormalities in HS 3-5 . HS has been reported in patients with various connective tissue diseases, such as systemic juvenile idiopathic arthritis, 247
casos clínicos adult-onset Still’s diseases, scleroderma, dermatomyositis, and systemic lupus erythematosus (SLE). HS developed in active SLE patients without evidences of other underlying causes of HS, such as infections or malignancies, has been called «acute lupus hemophagocytic syndrome (ALHS)» 6 . The occurrence of ALHS was closely related with the presence of high titers of antinuclear antibodies (ANA) and hypocomplementemia, indicating that the immune complex-mediated mechanisms might be responsible for the pathogenesis of ALHS 7 . The first line therapy for the treatment of HS associated with autoimmune diseases includes high-dose corticosteroids and immunosuppressives, such as cyclophosphamide and cyclosporine 8 . High-dose intravenous immunoglobulin, 9 anticancer drugs and TNF-α inhibitor (etanercept) have been used in the refractory cases 10 . We report a case of SLE with an unusual presentation. The patient presented with acute febrile illness along with progressive pancytopenia. Concomitant polyarthritis, myositis, nephritis, high titer of antinuclear factor and positive test for anti-DNA antibody, made her fit the diagnostic criteria of SLE. No definitive evidence of associated infections was confirmed by the bacteriologic, serologic and viral studies. ALHS was diagnosed and the patient was treated with highdose steroids and cyclophosphamide with an excellent outcome. The case is intriguing because ALHS is a rare and lifethreatening entity, challenging in differential diagnosis that requires urgent therapeutic intervention. Making a systematic review of the Medline database, we found only a few cases reported in the literature. Possible pathogenetic mechanisms and currently applied therapeutic modalities are also briefly reviewed. CASE REPORT A 36-year-old, caucasian woman, with no previous serious illnesses, was admitted to our hospital because of continuous fever lasting for 4 weeks and thrombocytopenia. She had no family history of HS, and had been well until 2 months before admission, when she began increased muscle weakness, fever, weight loss, photosensitivity and symmetric polyarthritis involving the small joints of the hands and wrists. On clinical examination, she appeared unwell, was febrile at 39 ºC, with a marked photosensitive rash on her face and an extensive confluent psoriasiform rash on both shoulder areas. A vasculitic rash most prominent on the tips of the fingers with periungual erythema and alopecia was present. The patient also had multiple painful oral 248 C. Botelho et al. Acute lupus hemophagocytic syndrome lesions with necrotizing ulceration on the buccal mucosa, upper palate, and lips. No evidence of lymphadenopathy was observed and the rest of the examination was unremarkable. Laboratory evaluation on admission revealed normochromic normocytic anemia (hematocrit 24.5%, haemoglobin 8,1 g/dL, red blood cell count 2,9 x 10 12 /L), leukopenia (white cell count 2,92 x 10 3 /µL with normal differential) and low platelet count (PLT 21 x 10 3 /µL). Biochemistry was abnormal for aspartate transaminase 84 U/L (normal range, 15-46 U/L), alanine transaminase 200 U/L (normal range, 10-66 U/L), gGT 144 U/L (normal range 12-58 U/L), lactic dehydrogenase 1,182 U/L (normal range 313-618 U/L) and albumin 21 g/L (normal range 35-50 g/L). Urea and creatinine were normal. Erythrocyte sedimentation rate (ESR) was markedly elevated at 115 mm/ 1 st h (normal range
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