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    "textoCompleto" => "<p class="elsevierStylePara"><span class="elsevierStyleBold">To the Editor&#44;</span></p><p class="elsevierStylePara">Reactive haemophagocytic syndrome or secondary haemophagocytic lymphohistiocytosis &#40;HLH&#41; is a disorder of the mononuclear phagocyte system characterised by generalised&#44; ineffective and uncontrolled histiocytic proliferation that leads to cell damage and multiple organ dysfunction with haemophagocytosis&#46; The first description of secondary forms of this disease was by Risdall et al&#44;<span class="elsevierStyleSup">1</span> who in 1979 described a syndrome characterised by a proliferation of histiocytes with haemophagocytic activity&#44; associated with a viral infection&#46; This syndrome was later described in association with infections of all types and with non-infectious diseases such as rheumatoid arthritis&#44; lupus&#44; leukaemia&#44; lymphomas&#44; myelodysplastic syndromes and carcinomas&#46;</p><p class="elsevierStylePara">Its pathogenesis is still unclear&#44; although there are several hypotheses&#46; The development of this syndrome is likely to be due to an immunological disorder that results in uncontrolled T-lymphocyte activation&#44;<span class="elsevierStyleSup">2</span> causing hypercytokinaemia&#44; and consequently&#44; excessive macrophage activation&#46;</p><p class="elsevierStylePara">It is diagnosed according to the criteria in HLH-2004<span class="elsevierStyleSup">3</span> and the treatment focuses on the infectious process&#44; as well as on the use of gamma globulin and immunosuppression&#46;<span class="elsevierStyleSup">4</span></p><p class="elsevierStylePara">We describe the case of a patient aged 42 years with a history of type 1 diabetes&#44; diabetic nephropathy&#44; and chronic renal failure who underwent a kidney-pancreas transplant &#40;October 2010&#41;&#46; The patient&#8217;s maintenance immunosuppressants are deltisone B&#44; everolimus&#44; and tacrolimus &#40;FK&#41; in addition to prophylaxis with valgancyclovir and trimethoprim&#47;sulfamethoxazole &#40;TMS&#41;&#46; The patient experienced fever&#44; vomiting and odynophagia for 2 weeks&#44; and was treated with oral antibiotics&#46; Fever&#44; asthenia and dehydration persisted&#44; so the patient was hospitalised&#46; Laboratory analyses revealed pancytopoenia and renal and pancreatic dysfunction&#59; the patient received subcutaneous insulin but not haemodialysis&#46; Blood and urine cultures were performed&#44; as well as a PCR &#40;polymerase chain reaction&#41; test for cytomegalovirus &#40;CMV&#41;&#44; and empirical treatment with ceftriaxone and ciprofloxacin was administered&#46; Twenty-four hours after admission&#44; the haemodynamic state had deteriorated severely and the patient was moved to intensive care&#44; where all immunosuppressants except for corticosteroids were discontinued&#46; Antibiotic coverage was increased through vancomycin&#44; imipenem&#44; fluconazole and ganciclovir&#46; Seven days after admission&#44; the patient was still feverish with positive cultures for common microbes and fungi and a negative PCR for CMV&#46; We ordered PCR for parvovirus B19 due to the persistent pancytopoenia&#46; The physical examination showed cutaneous and mucosal pallor&#44; asthenia&#44; adynamia and splenomegaly&#46; The laboratory results were as follows&#58; Hb&#58; 8&#46;6mg&#47;dl&#44; leukocytes&#58; 900mm<span class="elsevierStyleSup">3</span>&#44; triglycerides&#58; 317mg&#47;dl&#59; ferritin &#62;1500mcg&#47;l&#46; In light of suspected haemophagocytic syndrome &#40;5 criteria met&#41;&#44; we performed a bone marrow biopsy&#44; which revealed histiocytes with haemophagocytosis&#46; The patient was treated with high doses of gamma globulin &#40;400mg&#47;kg&#41; during 5 days&#46; In the end&#44; PCR was positive for parvovirus B19&#46;</p><p class="elsevierStylePara">All of the patient&#8217;s low values improved &#40;haematocrit 28&#37;&#59; Hb 9&#46;4g&#47;dl&#59; leukocytes 1900mm<span class="elsevierStyleSup">3</span>&#59; platelets 203&#160;000mm<span class="elsevierStyleSup">3</span>&#41; and immunosuppressant treatment was resumed&#46; Pancreatic function remained weak&#44; and the nephrology department found the renal function to be so severely affected that the patient needed haemodialysis&#46; A kidney biopsy puncture was performed which yielded insufficient material&#46;</p><p class="elsevierStylePara">At 30 days of hospitalisation&#44; the patient was once again feverish with a headache&#59; lumbar puncture revealed normal cerebrospinal fluid&#44; acid-alcohol resistant bacilli &#40;AARB&#41; negative&#59; PCR for CMV&#44; herpes simplex virus&#44; Epstein-Barr virus&#44; cryptococcal antigenaemia all negative&#59; adenosine deaminase at the upper cut-off level&#59; cerebral MRI showed no lesions&#46; The thoracic radiography showed bilateral interstitial and alveolar infiltrates&#44; which was confirmed by thoracic CT as bilateral radiodense infiltrates&#59; fibrobronchoscopy with bronchoalveolar lavage was performed&#59; negative for AARB and positive for pneumocystitis carinii &#40;PCP&#41; when TMS treatment began&#46; Due to the persistent fever and the lack of culture isolation in a case with pulmonary lesions&#44; empirical treatment with isoniazid&#44; rifampicin&#44; ethambutol and liposomal amphotericin was administered&#46; Another kidney biopsy puncture was performed&#44; but graft bleeding ensued and the patient had to go to the surgical ward&#46; Doctors decided to extirpate both grafts&#44; and observed mesenteric adenopathies and abundant purulent matter&#46; This matter tested AARB &#40;&#43;&#41; under direct examination&#44; and therefore antibiotic and antifungal treatments were suspended&#44; with the patient continuing tuberculosis treatment&#46; Final culture was positive for tuberculosis&#46; Patient&#8217;s fever subsided and overall condition improved&#59; he returned to his home city and is monitored by his local haemodialysis centre&#46;</p><p class="elsevierStylePara">Haemophagocytic syndrome that reacts to associated infections is a severe and potentially fatal condition&#46; Immunosuppressed patients who present with a fever and haematological abnormalities &#40;cytopoenias&#41; should be screened for haemophagocytosis as early diagnosis enables proper treatment and a favourable prognosis&#46;</p><p class="elsevierStylePara">&#160;<a href="modules&#46;php&#63;name&#61;traducciones&#38;op&#61;manuscrito&#38;idarticulo&#61;11179&#38;idversion&#61;11279&#38;idlangread&#61;EN&#35;&#95;msoanchor&#95;1" class="elsevierStyleCrossRefs">&#91;P1&#93;</a>Las unidades son diferentes en word y pdf</p>"
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