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Objective: In order to study patients with a diagnosis of xanthogranulomatous cholecystitis (XGC), we analyzed their demographics, epidemiology and clinical data., Material and Methods: We analyzed the clinical records of XGC during a period of 6 years, obtaining demographic, epidemiologic and clinical data., Results: Of a total of 1425 cholecystectomies performed between January 1991 and December 1996, we found 35 cases of XGC (2.4%). Twenty six (74%) were women (median age: 44 years), 60% were from a low socioeconomic group, 34% has a history of alcoholism and smoking, and 25 patients (71%) had a blood type of O positive. Thirteen patients (37%) presented obstructive jaundice, 11 had dilatation of the choledocus and were treated with ERCP. Of the 35 cholecystectomies, 15 were urgent and 20 elective. Eight were operated laparoscopically and two were converted because of firm adhesions. We had 5 transoperative complications., Discussion: Pre-operative XGC diagnosis is difficult, often mistaken for gall bladder cancer. The incidence in our study (2.4%) is higher than reports in industrialized countries (0.7-1.8%), with a female predominance. The most frequent clinical presentation is that of chronic cholecystitis, but we found a high percentage of patients with obstructive jaundice. We had 0% mortality and 26% morbidity, and no association was found between XGC and gallbladder cancer.

Haemophagocytic syndrome is a rare disorder of immune regulation. Clinical features are fever, haemophagocytosis, hepatosplenomegaly, pancytopenia, lymph nodes enlargement, hypertriglyceridaemia and coagulopathy. Systemic findings may also include pulmonary infiltrates, renal failure and an inappropriate antidiuretic state. It has been described associated to many diseases, like lymphomas, most of them T-cell lymphomas, infections and systemic diseases, among other entities. Haemopoietic cells are actively ingested by monocytes and macrophages in lymph nodes, bone marrow, liver and spleen. This is a diagnostic criteria for haemophagocytic syndrome. The treatment of haemophagocytic syndrome is difficult and requires intensive supportive therapy, treatment of coagulopathy disorders, high-dose corticosteroids and immunosuppressive treatment, although in most cases there is no response to treatment and the evolution is fatal. The T-cell lymphomas that involve subcutaneous tissue and simulate panniculitis are classified recently as primary cutaneous T-cell lymphomas, and their presentation as inflammatory nodules of the legs are uncommon.

Introduction: Haemophagocyte lymphohistiocytosis (HLH) is a hematological disorder, autosomal recessive and in which there is benign proliferation of histiocytes with intense phagocytic activity of hematopoietic cells. The clinical features include fever, pancytopenia, coagulation disorders, liver dysfunction, the presence of histiocytes and haemophagocytes in the bone marrow, lymph nodes, spleen and liver. The nervous system is always involved and sooner or later patients develop a nervous system disorder with variable symptoms which may include irritability, disorders of consciousness, convulsions, ataxia, nystagmus or signs of intracranial hypertension., Clinical Case: Onset of the disease showing purely neurological features is rare. We therefore describe the case of an 8 month old baby with HLH with a purely neurological condition involving irritability, horizontal rapid eye movements and vertical saccadic movements of both eyes and focal convulsive seizures. Initial complementary examinations were normal, except for study of the CSF with a lowered protein level and cells (monocytes). Finding hepatosplenomegaly and pallor, together with the laboratory investigations, made it advisable to do a bone marrow punch biopsy to detect haemophagocytes which would be diagnostic of HLH. In spite of chemotherapy there was rapid neurological deterioration, with alterations of the white matter and hydrocephaly which required insertion of a ventriculo-peritoneal shunt. The patient died when he was 10 months old., Conclusions: The cases of HLH in which cerebromeningeal disorders alone precede systemic symptoms are extremely rare. Hence the interest in reporting this case, so that it may be borne in mind in other cases of acute neurological onset. In this case initially there was encephalitis alone, but this was rapidly followed by systemic complications.

Three cases of haemophagocytic syndrome (HPS) due to histoplasmosis in patients with the acquired immunodeficiency syndrome (AIDS) are described. At admission all had pancytopenia and the bone marrow aspirate showed typical haemophagocytosis and the presence of intracellular inclusions compatible with Histoplasma capsulatum. One patient had also cerebral toxoplasmosis. One patient died shortly after admission from multiple organ failure. Two patients survived, one treated with fluconazole and another with amphotericin-B with good response and progressive increase in their blood cell counts. We suggest that haemophagocytic syndrome due to disseminated histoplasmosis should be included in the causes of pancytopenia in patients with AIDS. Bone marrow aspirate is the best method for diagnosis.

We report a series of seven patients with reactive hemophagocytic syndrome, which was quite characteristic of its etiological spectrum. Infections were the leading cause, among them a case associated with HIV and another one with Salmonella enteritidis (a hitherto unreported association). The clinical findings consisted of fever, hepatomegaly, splenomegaly, lymphadenopathy, rash and pancytopenia. The diagnosis was carried out by bone marrow aspiration-biopsy except in two patients who were diagnosed at autopsy. The difficulty of the differentiation from malignant histiocytosis is discussed: one case of hemophagocytic syndrome due to diphenylhydantoin toxicity (the second reported one in the literature) was histologically undistinguishable from it. We think that, in any etiology, hemophagocytic syndrome is a reactive syndrome with variable intensity. The need for extensive microbiological investigation even in cases of histiocytosis of neoplastic appearance is emphasized.