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and less frequently the lungs and cardiac tissues are involved&#46;</p><p class="elsevierStylePara">Although the pathogenic mechanisms still remain unknown&#44; overproduction of pro-inflammatory cytokines&#44; uncontrolled activation of T cells and macrophages&#44; associated with decreased natural killer cell and cytotoxic cell functions&#44; seem to be the hallmarks of the immunologic abnormalities in HS<span class="elsevierStyleSup">3-5</span>&#46;</p><p class="elsevierStylePara">HS has been reported in patients with various connective tissue diseases&#44; such as systemic juvenile idiopathic arthritis&#44; adult-onset Still&#8217;s diseases&#44; scleroderma&#44; dermatomyositis&#44; and systemic lupus erythematosus &#40;SLE&#41;&#46; HS developed in active SLE patients without evidences of other underlying causes of HS&#44; such as infections or malignancies&#44; has been called &#171;acute lupus hemophagocytic syndrome &#40;ALHS&#41;&#187;<span class="elsevierStyleSup">6</span>&#46; The occurrence of ALHS was closely related with the presence of high titers of antinuclear antibodies &#40;ANA&#41; and hypocomplementemia&#44; indicating that the immune complex-mediated mechanisms might be responsible for the pathogenesis of ALHS<span class="elsevierStyleSup">7</span>&#46;</p><p class="elsevierStylePara">The first line therapy for the treatment of HS associated with autoimmune diseases includes high-dose corticosteroids and immunosuppressives&#44; such as cyclophosphamide and cyclosporine<span class="elsevierStyleSup">8</span>&#46; High-dose intravenous immunoglobulin&#44;<span class="elsevierStyleSup">9</span> anticancer drugs and TNF-&#945; inhibitor &#40;etanercept&#41; have been used in the refractory cases<span class="elsevierStyleSup">10</span>&#46;</p><p class="elsevierStylePara">We report a case of SLE with an unusual presentation&#46; The patient presented with acute febrile illness along with progressive pancytopenia&#46; Concomitant polyarthritis&#44; myositis&#44; nephritis&#44; high titer of antinuclear factor and positive test for anti-DNA antibody&#44; made her fit the diagnostic criteria of SLE&#46; No definitive evidence of associated infections was confirmed by the bacteriologic&#44; serologic and viral studies&#46; ALHS was diagnosed and the patient was treated with highdose steroids and cyclophosphamide with an excellent outcome&#46;</p><p class="elsevierStylePara">The case is intriguing because ALHS is a rare and lifethreatening entity&#44; challenging in differential diagnosis that requires urgent therapeutic intervention&#46; Making a systematic review of the Medline database&#44; we found only a few cases reported in the literature&#46;</p><p class="elsevierStylePara">Possible pathogenetic mechanisms and currently applied therapeutic modalities are also briefly reviewed&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE REPORT</span></p><p class="elsevierStylePara">A 36-year-old&#44; caucasian woman&#44; with no previous serious illnesses&#44; 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haemoglobin 8&#44;1 g&#47;dL&#44; red blood cell count 2&#44;9 x 1012&#47;L&#41;&#44; leukopenia &#40;white cell count 2&#44;92 x 103&#47;&#956;L with normal differential&#41; and low platelet count &#40;PLT 21 x 103&#47;&#956;L&#41;&#46; Biochemistry was abnormal for aspartate transaminase 84 U&#47;L &#40;normal range&#44; 15-46 U&#47;L&#41;&#44; alanine transaminase 200 U&#47;L &#40;normal range&#44; 10-66 U&#47;L&#41;&#44; gGT 144 U&#47;L &#40;normal range 12-58 U&#47;L&#41;&#44; lactic dehydrogenase 1&#44;182 U&#47;L &#40;normal range 313-618 U&#47;L&#41; and albumin 21 g&#47;L &#40;normal range 35-50 g&#47;L&#41;&#46; Urea and creatinine were normal&#46;</p><p class="elsevierStylePara">Erythrocyte sedimentation rate &#40;ESR&#41; was markedly elevated at 115 mm&#47; 1sth &#40;normal range &#60;12 mm&#47;1sth&#41; and C-reactive protein &#40;CRP&#41; was negative&#46; Coagulation screen was normal&#46; Ferritin and triglyceride blood levels were markedly elevated to 820 ng&#47;ml and 7&#44;3 mmol&#47;L &#40;normal ranges 9-120 ng&#47;ml and &#60;2&#44;3 mmol&#47;L respectively&#41;&#46;</p><p class="elsevierStylePara">Urinalysis showed proteinuria and microscopic haematuria&#46; Urine microscopy showed 10 erythrocytes and 2-4 granular casts per high power field&#46; 24-hour urine protein excretion was 12 g&#46;</p><p class="elsevierStylePara">The autoimmune profile revealed ANA &#62;1&#44;280&#44; homogeneous&#44; Anti-Ro&#47;SSA antibodies and anti-RNP strongly positive&#46; Serum complement C3 and C4 levels were both decreased to 450 mg&#47;l and 5 mg&#47;l &#40;normal ranges 900-1&#44;800 and 100-400 respectively&#41;&#46; Direct Coombs test was positive&#46; Circulating immune complexes were not detected&#44; and other autoantibodies including anti-neutrophil antibody&#44; antibodies against cyclic citrullinated peptide and rheumatoid factor were negative&#46;</p><p class="elsevierStylePara">Repeated urine and blood cultures were negative&#46; Sputum was negative for bacterial and fungal cultures&#46; A thorough infection screen&#44; including a viral panel for Herpes Simplex&#44; Herpes Zoster&#44; Epstein-Barr&#44; Cytomegalovirus&#44; Hepatitis B&#44; Hepatitis C&#44; HIV&#44; Echo&#44; Coxsackie and Parvo B19 viruses was negative&#46; A malignancy survey excluded any significant pathology&#46;</p><p class="elsevierStylePara">Chest X-ray&#44; electrocardiogram&#44; and echocardiography had no abnormal findings&#46; Thoracoabdominal computed tomography showed no abnormalities other than mild hepatosplenomegaly&#46;</p><p class="elsevierStylePara">A bone marrow aspiration revealed hemophagocytosis with activated histiocytes &#40;figure 1&#41;&#44; normal hematopoiesis and no malignant cell invasion&#46;</p><p class="elsevierStylePara">A percutaneous needle kidney biopsy was performed&#46;</p><p class="elsevierStylePara">Histological examination at light microscopy showed sclerosis in 6 of the 9 glomeruli&#44; modest mesangial proliferation&#44; tubular atrophy and diffuse interstitial fibrosis &#40;figure 2&#41;&#46;</p><p class="elsevierStylePara">Immunofluorescence staining of 6 glomeruli revealed mesangial deposits of IgM and C3 &#40;figure 3&#41;&#46; The diagnosis of Focal Proliferative Lupus Nephritis &#40;class III WHO&#41; was established&#46;</p><p class="elsevierStylePara">No causative disorder for HS could be detected other than active SLE&#46; The diagnosis of acute lupus hemophagocytic syndrome was established&#46;</p><p class="elsevierStylePara">The patient was treated with high-dose corticosteroid therapy with intravenous administration of methylprednisolone&#44; 1 g&#47;day for 3 days followed by 1 mg&#47;Kg&#47;day p&#46;o&#46; Because of a partial response&#44; five days after the initiation of the therapeutic scheme&#44; 1 g of intravenous cyclophosphamide was added to her treatment&#46;</p><p class="elsevierStylePara">As the consequence of these treatments the patient improved clinically&#44; the cytopenias reversed concomitantly with the suppression of hemophagocytic histiocytes&#44; and the inflammatory indices&#44; LFTs&#44; and ferritin levels all gradually returned to normal&#46; Two weeks later she was discharged in good condition on a tapered dose of oral steroids&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">Hematologic involvement in SLE is a very common finding&#44; but usually only one or 2 cell lines are affected&#46; Pancytopenia occurs in fewer than 10&#37; of patients and&#44; in the absence of offending medication &#40;ie&#44; azathioprine&#44; cyclophosphamide&#44; and so on&#41;&#44; is usually the result of peripheral destruction<span class="elsevierStyleSup">6&#44;11</span>&#46;</p><p class="elsevierStylePara">In our patient&#44; all laboratory findings pointed to central marrow suppression&#46; The extremely high ferritin&#44; with normal CRP could not be explained by any other factor &#40;ie&#44; infection&#41; than HS&#44; because our patient also did not have evidence of malignancy or hemochromatosis&#46; Liver dysfunction and excessively high ferritin fit well in HS and are caused by high levels of circulating cytokine &#40;IL-1&#44; IL-6&#44; interferon-gamma &#91;INF-gamma&#93;&#44; TNF &#945;&#41;<span class="elsevierStyleSup">12</span>&#46;</p><p class="elsevierStylePara">HS is a rare entity characterized by the proliferation of nonneoplastic cells of the histiocyte lineage that phagocytose the hematopoietic cells&#46; It is usually aggressive&#44; the outcome varies&#44; and some cases are lethal<span class="elsevierStyleSup">2&#44;6</span>&#46;</p><p class="elsevierStylePara">HS can be associated with infection&#44; malignancies&#44; autoimmune diseases&#44; drugs and immunosuppression&#46; The infective agents include bacteria&#44; viruses&#44; most commonly Epstein-Barr virus and cytomegalovirus&#44; or fungi&#46; Causative malignancies are usually hematologic&#44; especially lymphomas&#44; but solid organ tumors have also been implicated&#46; Many autoimmune disorders are related with HS&#44; including SLE&#44; Sj&#246;gren syndrome&#44; mixed connective tissue disease&#44; progressive systemic sclerosis&#44; rheumatoid arthritis&#44; Crohn disease&#44; vasculitis and sarcoidosis<span class="elsevierStyleSup">2</span>&#46; According to historical and current findings&#44; HS is now classified as shown in table 1&#46;</p><p class="elsevierStylePara">HS can complicate both active and inactive SLE&#46; When HS is diagnosed in inactive SLE&#44; the cause can be an infection&#44; usually a viral one&#44; or the immunosuppressive treatment&#46; In 1991&#44; Wong&#44; et al reported patients with active SLE who demonstrated reactive hemophagocytosis in bone marrow<span class="elsevierStyleSup">6</span>&#46; The occurrence of hemophagocytosis was associated with activity of SLE itself&#44; and they proposed the disease entity of acute lupus hemophagocytic syndrome &#40;ALHS&#41;&#46; In 1995 and 1997&#44; Kumakura&#44; et al reported cases of reactive HS&#44; which were associated with autoimmune diseases other than SLE&#44; and proposed a new disease entity&#44; autoimmune associate hemophagocytic syndrome &#40;AAHS&#41;<span class="elsevierStyleSup">13&#44;14</span>&#46; Diagnostic criteria for AAHS have been proposed by Kumakura&#44; et al &#40;table 2&#41;<span class="elsevierStyleSup">15</span>&#46; To diagnose secondary HS&#44; it is necessary to detect the underlying disorder causing HS&#46;</p><p class="elsevierStylePara">In our patient the clinical and biochemical findings met the American College of Rheumatology diagnostic criteria for SLE&#44; and the kidney biopsy established the diagnosis of Lupus Nephritis class III&#46; At the same time she fulfilled diagnostic criteria for AAHS&#46; An extensive study showed no evidence of infection&#44; drugs or malignancy&#44; but she currently had symptoms&#44; signs and serologic findings of active SLE&#46; To the best of our knowledge&#44; our patient had no family history suggestive of familial hemophagocytic lymphohistiocytosis &#40;FHL&#41;&#46; Therefore&#44; no causative disorder for HS could be detected other than active SLE&#44; and the diagnosis of ALHS was established&#46;</p><p class="elsevierStylePara">The proposed pathogenetic mechanisms of HS in SLE&#44; which are not mutually exclusive&#44; include&#58; 1&#41; autoantibodymediated phagocytosis of hematopoietic cells&#44; 2&#41; deposition of immune complexes on the bone marrow hematopoietic cells&#44; and 3&#41; oversecretion of cytokines &#40;ie&#44; IL-1&#44; IL-6&#44; INF-gamma&#44; TNF&#41; by primary uncontrolled T-cell activation<span class="elsevierStyleSup">2&#44;6</span>&#46; Autoantibodies and&#47;or immune-complexes account for the sensitization of marrow hematopoietic cells to macrophages&#44; which are then engaged into uncontrolled phagocytosis<span class="elsevierStyleSup">12</span>&#46; Cytokines secreted by T cells involved in the previously mentioned process or acting independently enhance the inappropriate activation of macrophages&#46; Central to the uncontrolled phagocytosis in lupus-related HS is the intrinsic lupus-related macrophage dysfunction per se accentuated by auto-reactive lymphocytes or concurrent infection<span class="elsevierStyleSup">2&#44;6</span>&#46;</p><p class="elsevierStylePara">Another interesting point is that our patient presented high titers of ANA and hypocomplementemia&#44; indicating that immune complex-mediated mechanisms might be responsible for the pathogenesis of ALHS&#46; In our patient triglyceride blood levels were markedly elevated&#44; which may be explained by the decrease in lipolytic activity induced by IL-1 and TNF-&#945; cytokines<span class="elsevierStyleSup">16</span>&#46;</p><p class="elsevierStylePara">The strategies of treatment for secondary HS include therapy for the underlying disease and that for hypercytokinemia&#44; which plays a major role and causes hemophagocytosis&#46;</p><p class="elsevierStylePara">When the trigger event is disease flare&#44; pulse steroids and cyclophosphamide are indicated<span class="elsevierStyleSup">2&#44;6&#44;12</span>&#46;</p><p class="elsevierStylePara">In one case report tacrolimus was considered beneficial<span class="elsevierStyleSup">17</span>&#46;</p><p class="elsevierStylePara">IVIG has also been used extensively as an immunomodulator&#44; both in infection related and in lupus related<span class="elsevierStyleSup">2&#44;17-20</span>&#46; Because the pathogenetic mechanisms are not elucidated&#44; therapeutic interventions are not clearcut either&#59; immunosuppressants have been used to counteract the activation of macrophages&#44; whereas IVIG has been given in infection-related HS&#44; presumably acting by binding cytokines and inhibiting their synthesis<span class="elsevierStyleSup">2</span>&#46;</p><p class="elsevierStylePara">Plasmapheresis has been reported as a therapeutic option in a few cases only<span class="elsevierStyleSup">17&#44;21&#44;22</span>&#46; However&#44; this procedure alone has not been shown to benefit&#44; probably because of resultant antibody rebound<span class="elsevierStyleSup">23</span>&#46; Takahashi&#44; et al reported a case of ALHS who was refractory to high-dose corticosteroids&#44; cyclosporine&#44; and IVIG&#44; but was successfully treated with additional administration of TNF-&#945; inhibitor&#44; etanercept<span class="elsevierStyleSup">10</span>&#46;</p><p class="elsevierStylePara">In conclusion&#44; we describe a rare case of ALHS that responded well to pulse intravenous steroids and pulse cyclophosphamide&#46; In febrile patients with SLE pancytopenia and unduly high ferritin levels&#44; infections should be readily exclude and a bone marrow aspiration&#44; which is crucial for diagnosis&#44; carried out promptly&#46; If hemophagocytosis is confirmed&#44; early treatment should be initiated&#46;</p><p class="elsevierStylePara"><a href="grande&#47;10121127&#95;figura1&#46;jpg" class="elsevierStyleCrossRefs"><img src="10121127_figura1.jpg"></img></a></p><p class="elsevierStylePara">Figure 1&#46; </p><p class="elsevierStylePara"><a href="grande&#47;10121127&#95;figura2&#46;jpg" class="elsevierStyleCrossRefs"><img src="10121127_figura2.jpg"></img></a></p><p class="elsevierStylePara">Figure 2&#46; </p><p class="elsevierStylePara"><a href="grande&#47;10121127&#95;figura3&#46;jpg" class="elsevierStyleCrossRefs"><img src="10121127_figura3.jpg"></img></a></p><p class="elsevierStylePara">Figure 3&#46; </p><p class="elsevierStylePara"><a href="grande&#47;1012118078&#95;tripav30&#95;n2&#95;2010&#95;t1&#95;pag250&#46;jpg" class="elsevierStyleCrossRefs"><img src="1012118078_tripav30_n2_2010_t1_pag250.jpg" alt="Classification of HS"></img></a></p><p class="elsevierStylePara">Table 1&#46; Classification of HS</p><p class="elsevierStylePara"><a href="grande&#47;1012118078&#95;tripav30&#95;n2&#95;2010&#95;t2&#95;pag250&#46;jpg" class="elsevierStyleCrossRefs"><img src="1012118078_tripav30_n2_2010_t2_pag250.jpg" alt="Diagnostic criteria for AAHS proposed by Kumakura"></img></a></p><p class="elsevierStylePara">Table 2&#46; Diagnostic criteria for AAHS proposed by Kumakura</p>"
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        "resumen" => "<p class="elsevierStylePara">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&#46; It leads to the phagocytosis of erythrocytes&#44; leukocytes&#44; platelets and their precursors&#46; The presence of hemophagocytosis can be associated to infections&#44; malignancies&#44; autoimmune diseases&#44; drugs and a variety of other medical conditions&#46; 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&#46; Lupus related hemophagocytic syndrome is a rare and potentially fatal entity&#46; It offers significant differential diagnosis challenges and requires urgent therapeutic intervention&#46; There are only few cases reported in the literature&#46; However&#44; much is still needed in order to better understand its causes&#44; all the immunopathogenic mechanisms&#44; as well as its clinical and therapeutic aspects&#46;</p>"
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        "resumen" => "La hemofagocitosis es un cuadro cl&#237;nico caracterizado por la activaci&#243;n de los macr&#243;fagos y histiocitos&#44; con intensa actividad fagoc&#237;tica en la m&#233;dula &#243;sea e otras localizaciones del sistema reticuloendotelial&#44; lo que provoca la fagocitosis de los eritrocitos&#44; leucocitos&#44; plaquetas y sus precursores&#46; Su presencia puede estar asociada con infecciones&#44; neoplasias&#44; enfermedades autoinmunitarias&#44; drogas y una variedad de otras condiciones m&#233;dicas&#46; En este caso cl&#237;nico&#44; presentamos a una mujer de 36 a&#241;os&#44; previamente sana&#44; que desarroll&#243; hemofagocitosis&#44; al mismo tiempo que complet&#243; los criterios de diagn&#243;stico de lupus eritematoso sist&#233;mico&#46; La hemofagocitosis asociada con el lupus es una entidad rara&#44; potencialmente mortal&#44; de diagn&#243;stico diferencial complicado y&#44; que requiere una intervenci&#243;n terap&#233;utica urgente&#46; Hay muy pocos casos comunicados en la literatura&#44; y es necesaria una mejor comprensi&#243;n de los aspectos cl&#237;nicos&#44; causas&#44; fisiopatolog&#237;a&#44; criterios de diagn&#243;stico y tratamiento de este s&#237;ndrome&#46;"
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Acute lupus hemophagocytic syndrome: report of a case
Hemofagocitosis asociada con el lupus: caso clínico
Carlos Botelhoa, F.. Ferrera, L.. Franciscob, P.. Maiaa, T.. Mendesa, A.. Carreiraa
a Department of Nephrology, Centro Hospitalar de Coimbra, Coimbra, Portugal,
b Department Hematology, Centro Hospitalar de Coimbra, Coimbra, Portugal,
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        "titulo" => "Hemofagocitosis asociada con el lupus&#58; caso cl&#237;nico"
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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">INTRODUCTION</span></p><p class="elsevierStylePara">Hemophagocytic Syndrome &#40;HS&#41; is a clinicopathologic entity characterized by increased proliferation and activation of benign macrophages with hemophagocytosis throughout the reticuloendothelial system&#46;<span class="elsevierStyleSup">1</span> HS may develop as a rare but potentially fatal complication of several disorders including malignancies&#44; drugs&#44; infections and autoimmune disorders&#46;</p><p class="elsevierStylePara">Patients usually present with an acute febrile illness&#44; sometimes fulminant and lethal&#46; Common manifestations include high fever&#44; pancytopenia&#44; hepatosplenomegaly&#44; weight loss&#44; lymphadenopathy&#44; abnormal liver function tests &#40;LFTs&#41;&#44; hyperferritinemia and high blood triglyceride levels<span class="elsevierStyleSup">2</span>&#46; Disseminated intravascular coagulation and central nervous system dysfunction often ensue&#44; and less frequently the lungs and cardiac tissues are involved&#46;</p><p class="elsevierStylePara">Although the pathogenic mechanisms still remain unknown&#44; overproduction of pro-inflammatory cytokines&#44; uncontrolled activation of T cells and macrophages&#44; associated with decreased natural killer cell and cytotoxic cell functions&#44; seem to be the hallmarks of the immunologic abnormalities in HS<span class="elsevierStyleSup">3-5</span>&#46;</p><p class="elsevierStylePara">HS has been reported in patients with various connective tissue diseases&#44; such as systemic juvenile idiopathic arthritis&#44; adult-onset Still&#8217;s diseases&#44; scleroderma&#44; dermatomyositis&#44; and systemic lupus erythematosus &#40;SLE&#41;&#46; HS developed in active SLE patients without evidences of other underlying causes of HS&#44; such as infections or malignancies&#44; has been called &#171;acute lupus hemophagocytic syndrome &#40;ALHS&#41;&#187;<span class="elsevierStyleSup">6</span>&#46; The occurrence of ALHS was closely related with the presence of high titers of antinuclear antibodies &#40;ANA&#41; and hypocomplementemia&#44; indicating that the immune complex-mediated mechanisms might be responsible for the pathogenesis of ALHS<span class="elsevierStyleSup">7</span>&#46;</p><p class="elsevierStylePara">The first line therapy for the treatment of HS associated with autoimmune diseases includes high-dose corticosteroids and immunosuppressives&#44; such as cyclophosphamide and cyclosporine<span class="elsevierStyleSup">8</span>&#46; High-dose intravenous immunoglobulin&#44;<span class="elsevierStyleSup">9</span> anticancer drugs and TNF-&#945; inhibitor &#40;etanercept&#41; have been used in the refractory cases<span class="elsevierStyleSup">10</span>&#46;</p><p class="elsevierStylePara">We report a case of SLE with an unusual presentation&#46; The patient presented with acute febrile illness along with progressive pancytopenia&#46; Concomitant polyarthritis&#44; myositis&#44; nephritis&#44; high titer of antinuclear factor and positive test for anti-DNA antibody&#44; made her fit the diagnostic criteria of SLE&#46; No definitive evidence of associated infections was confirmed by the bacteriologic&#44; serologic and viral studies&#46; ALHS was diagnosed and the patient was treated with highdose steroids and cyclophosphamide with an excellent outcome&#46;</p><p class="elsevierStylePara">The case is intriguing because ALHS is a rare and lifethreatening entity&#44; challenging in differential diagnosis that requires urgent therapeutic intervention&#46; Making a systematic review of the Medline database&#44; we found only a few cases reported in the literature&#46;</p><p class="elsevierStylePara">Possible pathogenetic mechanisms and currently applied therapeutic modalities are also briefly reviewed&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">CASE REPORT</span></p><p class="elsevierStylePara">A 36-year-old&#44; caucasian woman&#44; with no previous serious illnesses&#44; was admitted to our hospital because of continuous fever lasting for 4 weeks and thrombocytopenia&#46; She had no family history of HS&#44; and had been well until 2 months before admission&#44; when she began increased muscle weakness&#44; fever&#44; weight loss&#44; photosensitivity and symmetric polyarthritis involving the small joints of the hands and wrists&#46;</p><p class="elsevierStylePara">On clinical examination&#44; she appeared unwell&#44; was febrile at 39 &#186;C&#44; with a marked photosensitive rash on her face and an extensive confluent psoriasiform rash on both shoulder areas&#46; A vasculitic rash most prominent on the tips of the fingers with periungual erythema and alopecia was present&#46; The patient also had multiple painful oral lesions with necrotizing ulceration on the buccal mucosa&#44; upper palate&#44; and lips&#46; No evidence of lymphadenopathy was observed and the rest of the examination was unremarkable&#46;</p><p class="elsevierStylePara">Laboratory evaluation on admission revealed normochromic normocytic anemia &#40;hematocrit 24&#46;5&#37;&#44; haemoglobin 8&#44;1 g&#47;dL&#44; red blood cell count 2&#44;9 x 1012&#47;L&#41;&#44; leukopenia &#40;white cell count 2&#44;92 x 103&#47;&#956;L with normal differential&#41; and low platelet count &#40;PLT 21 x 103&#47;&#956;L&#41;&#46; Biochemistry was abnormal for aspartate transaminase 84 U&#47;L &#40;normal range&#44; 15-46 U&#47;L&#41;&#44; alanine transaminase 200 U&#47;L &#40;normal range&#44; 10-66 U&#47;L&#41;&#44; gGT 144 U&#47;L &#40;normal range 12-58 U&#47;L&#41;&#44; lactic dehydrogenase 1&#44;182 U&#47;L &#40;normal range 313-618 U&#47;L&#41; and albumin 21 g&#47;L &#40;normal range 35-50 g&#47;L&#41;&#46; Urea and creatinine were normal&#46;</p><p class="elsevierStylePara">Erythrocyte sedimentation rate &#40;ESR&#41; was markedly elevated at 115 mm&#47; 1sth &#40;normal range &#60;12 mm&#47;1sth&#41; and C-reactive protein &#40;CRP&#41; was negative&#46; Coagulation screen was normal&#46; Ferritin and triglyceride blood levels were markedly elevated to 820 ng&#47;ml and 7&#44;3 mmol&#47;L &#40;normal ranges 9-120 ng&#47;ml and &#60;2&#44;3 mmol&#47;L respectively&#41;&#46;</p><p class="elsevierStylePara">Urinalysis showed proteinuria and microscopic haematuria&#46; Urine microscopy showed 10 erythrocytes and 2-4 granular casts per high power field&#46; 24-hour urine protein excretion was 12 g&#46;</p><p class="elsevierStylePara">The autoimmune profile revealed ANA &#62;1&#44;280&#44; homogeneous&#44; Anti-Ro&#47;SSA antibodies and anti-RNP strongly positive&#46; Serum complement C3 and C4 levels were both decreased to 450 mg&#47;l and 5 mg&#47;l &#40;normal ranges 900-1&#44;800 and 100-400 respectively&#41;&#46; Direct Coombs test was positive&#46; Circulating immune complexes were not detected&#44; and other autoantibodies including anti-neutrophil antibody&#44; antibodies against cyclic citrullinated peptide and rheumatoid factor were negative&#46;</p><p class="elsevierStylePara">Repeated urine and blood cultures were negative&#46; Sputum was negative for bacterial and fungal cultures&#46; A thorough infection screen&#44; including a viral panel for Herpes Simplex&#44; Herpes Zoster&#44; Epstein-Barr&#44; Cytomegalovirus&#44; Hepatitis B&#44; Hepatitis C&#44; HIV&#44; Echo&#44; Coxsackie and Parvo B19 viruses was negative&#46; A malignancy survey excluded any significant pathology&#46;</p><p class="elsevierStylePara">Chest X-ray&#44; electrocardiogram&#44; and echocardiography had no abnormal findings&#46; Thoracoabdominal computed tomography showed no abnormalities other than mild hepatosplenomegaly&#46;</p><p class="elsevierStylePara">A bone marrow aspiration revealed hemophagocytosis with activated histiocytes &#40;figure 1&#41;&#44; normal hematopoiesis and no malignant cell invasion&#46;</p><p class="elsevierStylePara">A percutaneous needle kidney biopsy was performed&#46;</p><p class="elsevierStylePara">Histological examination at light microscopy showed sclerosis in 6 of the 9 glomeruli&#44; modest mesangial proliferation&#44; tubular atrophy and diffuse interstitial fibrosis &#40;figure 2&#41;&#46;</p><p class="elsevierStylePara">Immunofluorescence staining of 6 glomeruli revealed mesangial deposits of IgM and C3 &#40;figure 3&#41;&#46; The diagnosis of Focal Proliferative Lupus Nephritis &#40;class III WHO&#41; was established&#46;</p><p class="elsevierStylePara">No causative disorder for HS could be detected other than active SLE&#46; The diagnosis of acute lupus hemophagocytic syndrome was established&#46;</p><p class="elsevierStylePara">The patient was treated with high-dose corticosteroid therapy with intravenous administration of methylprednisolone&#44; 1 g&#47;day for 3 days followed by 1 mg&#47;Kg&#47;day p&#46;o&#46; Because of a partial response&#44; five days after the initiation of the therapeutic scheme&#44; 1 g of intravenous cyclophosphamide was added to her treatment&#46;</p><p class="elsevierStylePara">As the consequence of these treatments the patient improved clinically&#44; the cytopenias reversed concomitantly with the suppression of hemophagocytic histiocytes&#44; and the inflammatory indices&#44; LFTs&#44; and ferritin levels all gradually returned to normal&#46; Two weeks later she was discharged in good condition on a tapered dose of oral steroids&#46;</p><p class="elsevierStylePara"><span class="elsevierStyleBold">DISCUSSION</span></p><p class="elsevierStylePara">Hematologic involvement in SLE is a very common finding&#44; but usually only one or 2 cell lines are affected&#46; Pancytopenia occurs in fewer than 10&#37; of patients and&#44; in the absence of offending medication &#40;ie&#44; azathioprine&#44; cyclophosphamide&#44; and so on&#41;&#44; is usually the result of peripheral destruction<span class="elsevierStyleSup">6&#44;11</span>&#46;</p><p class="elsevierStylePara">In our patient&#44; all laboratory findings pointed to central marrow suppression&#46; The extremely high ferritin&#44; with normal CRP could not be explained by any other factor &#40;ie&#44; infection&#41; than HS&#44; because our patient also did not have evidence of malignancy or hemochromatosis&#46; Liver dysfunction and excessively high ferritin fit well in HS and are caused by high levels of circulating cytokine &#40;IL-1&#44; IL-6&#44; interferon-gamma &#91;INF-gamma&#93;&#44; TNF &#945;&#41;<span class="elsevierStyleSup">12</span>&#46;</p><p class="elsevierStylePara">HS is a rare entity characterized by the proliferation of nonneoplastic cells of the histiocyte lineage that phagocytose the hematopoietic cells&#46; It is usually aggressive&#44; the outcome varies&#44; and some cases are lethal<span class="elsevierStyleSup">2&#44;6</span>&#46;</p><p class="elsevierStylePara">HS can be associated with infection&#44; malignancies&#44; autoimmune diseases&#44; drugs and immunosuppression&#46; The infective agents include bacteria&#44; viruses&#44; most commonly Epstein-Barr virus and cytomegalovirus&#44; or fungi&#46; Causative malignancies are usually hematologic&#44; especially lymphomas&#44; but solid organ tumors have also been implicated&#46; Many autoimmune disorders are related with HS&#44; including SLE&#44; Sj&#246;gren syndrome&#44; mixed connective tissue disease&#44; progressive systemic sclerosis&#44; rheumatoid arthritis&#44; Crohn disease&#44; vasculitis and sarcoidosis<span class="elsevierStyleSup">2</span>&#46; According to historical and current findings&#44; HS is now classified as shown in table 1&#46;</p><p class="elsevierStylePara">HS can complicate both active and inactive SLE&#46; When HS is diagnosed in inactive SLE&#44; the cause can be an infection&#44; usually a viral one&#44; or the immunosuppressive treatment&#46; In 1991&#44; Wong&#44; et al reported patients with active SLE who demonstrated reactive hemophagocytosis in bone marrow<span class="elsevierStyleSup">6</span>&#46; The occurrence of hemophagocytosis was associated with activity of SLE itself&#44; and they proposed the disease entity of acute lupus hemophagocytic syndrome &#40;ALHS&#41;&#46; In 1995 and 1997&#44; Kumakura&#44; et al reported cases of reactive HS&#44; which were associated with autoimmune diseases other than SLE&#44; and proposed a new disease entity&#44; autoimmune associate hemophagocytic syndrome &#40;AAHS&#41;<span class="elsevierStyleSup">13&#44;14</span>&#46; Diagnostic criteria for AAHS have been proposed by Kumakura&#44; et al &#40;table 2&#41;<span class="elsevierStyleSup">15</span>&#46; To diagnose secondary HS&#44; it is necessary to detect the underlying disorder causing HS&#46;</p><p class="elsevierStylePara">In our patient the clinical and biochemical findings met the American College of Rheumatology diagnostic criteria for SLE&#44; and the kidney biopsy established the diagnosis of Lupus Nephritis class III&#46; At the same time she fulfilled diagnostic criteria for AAHS&#46; An extensive study showed no evidence of infection&#44; drugs or malignancy&#44; but she currently had symptoms&#44; signs and serologic findings of active SLE&#46; To the best of our knowledge&#44; our patient had no family history suggestive of familial hemophagocytic lymphohistiocytosis &#40;FHL&#41;&#46; Therefore&#44; no causative disorder for HS could be detected other than active SLE&#44; and the diagnosis of ALHS was established&#46;</p><p class="elsevierStylePara">The proposed pathogenetic mechanisms of HS in SLE&#44; which are not mutually exclusive&#44; include&#58; 1&#41; autoantibodymediated phagocytosis of hematopoietic cells&#44; 2&#41; deposition of immune complexes on the bone marrow hematopoietic cells&#44; and 3&#41; oversecretion of cytokines &#40;ie&#44; IL-1&#44; IL-6&#44; INF-gamma&#44; TNF&#41; by primary uncontrolled T-cell activation<span class="elsevierStyleSup">2&#44;6</span>&#46; Autoantibodies and&#47;or immune-complexes account for the sensitization of marrow hematopoietic cells to macrophages&#44; which are then engaged into uncontrolled phagocytosis<span class="elsevierStyleSup">12</span>&#46; Cytokines secreted by T cells involved in the previously mentioned process or acting independently enhance the inappropriate activation of macrophages&#46; Central to the uncontrolled phagocytosis in lupus-related HS is the intrinsic lupus-related macrophage dysfunction per se accentuated by auto-reactive lymphocytes or concurrent infection<span class="elsevierStyleSup">2&#44;6</span>&#46;</p><p class="elsevierStylePara">Another interesting point is that our patient presented high titers of ANA and hypocomplementemia&#44; indicating that immune complex-mediated mechanisms might be responsible for the pathogenesis of ALHS&#46; In our patient triglyceride blood levels were markedly elevated&#44; which may be explained by the decrease in lipolytic activity induced by IL-1 and TNF-&#945; cytokines<span class="elsevierStyleSup">16</span>&#46;</p><p class="elsevierStylePara">The strategies of treatment for secondary HS include therapy for the underlying disease and that for hypercytokinemia&#44; which plays a major role and causes hemophagocytosis&#46;</p><p class="elsevierStylePara">When the trigger event is disease flare&#44; pulse steroids and cyclophosphamide are indicated<span class="elsevierStyleSup">2&#44;6&#44;12</span>&#46;</p><p class="elsevierStylePara">In one case report tacrolimus was considered beneficial<span class="elsevierStyleSup">17</span>&#46;</p><p class="elsevierStylePara">IVIG has also been used extensively as an immunomodulator&#44; both in infection related and in lupus related<span class="elsevierStyleSup">2&#44;17-20</span>&#46; Because the pathogenetic mechanisms are not elucidated&#44; therapeutic interventions are not clearcut either&#59; immunosuppressants have been used to counteract the activation of macrophages&#44; whereas IVIG has been given in infection-related HS&#44; presumably acting by binding cytokines and inhibiting their synthesis<span class="elsevierStyleSup">2</span>&#46;</p><p class="elsevierStylePara">Plasmapheresis has been reported as a therapeutic option in a few cases only<span class="elsevierStyleSup">17&#44;21&#44;22</span>&#46; However&#44; this procedure alone has not been shown to benefit&#44; probably because of resultant antibody rebound<span class="elsevierStyleSup">23</span>&#46; Takahashi&#44; et al reported a case of ALHS who was refractory to high-dose corticosteroids&#44; cyclosporine&#44; and IVIG&#44; but was successfully treated with additional administration of TNF-&#945; inhibitor&#44; etanercept<span class="elsevierStyleSup">10</span>&#46;</p><p class="elsevierStylePara">In conclusion&#44; we describe a rare case of ALHS that responded well to pulse intravenous steroids and pulse cyclophosphamide&#46; In febrile patients with SLE pancytopenia and unduly high ferritin levels&#44; infections should be readily exclude and a bone marrow aspiration&#44; which is crucial for diagnosis&#44; carried out promptly&#46; If hemophagocytosis is confirmed&#44; early treatment should be initiated&#46;</p><p class="elsevierStylePara"><a href="grande&#47;10121127&#95;figura1&#46;jpg" class="elsevierStyleCrossRefs"><img src="10121127_figura1.jpg"></img></a></p><p class="elsevierStylePara">Figure 1&#46; </p><p class="elsevierStylePara"><a href="grande&#47;10121127&#95;figura2&#46;jpg" class="elsevierStyleCrossRefs"><img src="10121127_figura2.jpg"></img></a></p><p class="elsevierStylePara">Figure 2&#46; </p><p class="elsevierStylePara"><a href="grande&#47;10121127&#95;figura3&#46;jpg" class="elsevierStyleCrossRefs"><img src="10121127_figura3.jpg"></img></a></p><p class="elsevierStylePara">Figure 3&#46; </p><p class="elsevierStylePara"><a href="grande&#47;1012118078&#95;tripav30&#95;n2&#95;2010&#95;t1&#95;pag250&#46;jpg" class="elsevierStyleCrossRefs"><img src="1012118078_tripav30_n2_2010_t1_pag250.jpg" alt="Classification of HS"></img></a></p><p class="elsevierStylePara">Table 1&#46; Classification of HS</p><p class="elsevierStylePara"><a href="grande&#47;1012118078&#95;tripav30&#95;n2&#95;2010&#95;t2&#95;pag250&#46;jpg" class="elsevierStyleCrossRefs"><img src="1012118078_tripav30_n2_2010_t2_pag250.jpg" alt="Diagnostic criteria for AAHS proposed by Kumakura"></img></a></p><p class="elsevierStylePara">Table 2&#46; Diagnostic criteria for AAHS proposed by Kumakura</p>"
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        "resumen" => "<p class="elsevierStylePara">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&#46; It leads to the phagocytosis of erythrocytes&#44; leukocytes&#44; platelets and their precursors&#46; The presence of hemophagocytosis can be associated to infections&#44; malignancies&#44; autoimmune diseases&#44; drugs and a variety of other medical conditions&#46; 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&#46; Lupus related hemophagocytic syndrome is a rare and potentially fatal entity&#46; It offers significant differential diagnosis challenges and requires urgent therapeutic intervention&#46; There are only few cases reported in the literature&#46; However&#44; much is still needed in order to better understand its causes&#44; all the immunopathogenic mechanisms&#44; as well as its clinical and therapeutic aspects&#46;</p>"
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        "resumen" => "La hemofagocitosis es un cuadro cl&#237;nico caracterizado por la activaci&#243;n de los macr&#243;fagos y histiocitos&#44; con intensa actividad fagoc&#237;tica en la m&#233;dula &#243;sea e otras localizaciones del sistema reticuloendotelial&#44; lo que provoca la fagocitosis de los eritrocitos&#44; leucocitos&#44; plaquetas y sus precursores&#46; Su presencia puede estar asociada con infecciones&#44; neoplasias&#44; enfermedades autoinmunitarias&#44; drogas y una variedad de otras condiciones m&#233;dicas&#46; En este caso cl&#237;nico&#44; presentamos a una mujer de 36 a&#241;os&#44; previamente sana&#44; que desarroll&#243; hemofagocitosis&#44; al mismo tiempo que complet&#243; los criterios de diagn&#243;stico de lupus eritematoso sist&#233;mico&#46; La hemofagocitosis asociada con el lupus es una entidad rara&#44; potencialmente mortal&#44; de diagn&#243;stico diferencial complicado y&#44; que requiere una intervenci&#243;n terap&#233;utica urgente&#46; Hay muy pocos casos comunicados en la literatura&#44; y es necesaria una mejor comprensi&#243;n de los aspectos cl&#237;nicos&#44; causas&#44; fisiopatolog&#237;a&#44; criterios de diagn&#243;stico y tratamiento de este s&#237;ndrome&#46;"
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                  "referenciaCompleta" => "Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis. Int J Hematol. 2000;72:1-11. <a href="http://www.ncbi.nlm.nih.gov/pubmed/10979202" target="_blank">[Pubmed]</a>"
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