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Multicentric reticulohistiocytosis is a rare non-Langerhans cell histiocytosis of unknown etiology. It is classified as multicentric because of multisystem involvement. The disease predominantly affects the skin and joints, but visceral involvement is possible. Multiple erythematous-brownish, pruritic nodules and papules on the face, hands, neck, and trunk are characteristic. It is associated with autoimmune diseases, or malignant neoplasms are seen in 20% to 30% of patients with multicentric reticulohistiocytosis. The diagnosis is based on histopathology of affected tissues. As it is an underreported disease, there is no standardized treatment. A case of multicentric reticulohistiocytosis is reported as a paraneoplastic manifestation of ductal breast cancer, being successfully treated with no recurrence after two years of follow-up. Few cases of multicentric reticulohistiocytosis associated with breast cancer have been reported in the literature., Competing Interests: The authors declare that there are no conflicts of interest in this manuscript., (This work is licensed under a Creative Commons Attribution 4.0 International License.)

Sarcoidosis is a chronic multisystemic granulomatous disease of unknown origin. Generalised eruptive histiocytosis is a rare, benign, self-healing, non-Langerhans' cell histiocytosis (non-LCH). We report the case of an 8-year-old girl with sarcoidosis who was misdiagnosed as non-LCH. She was treated with oral corticosteroids, methotrexate and adalimumab, but there was insufficient control of ocular disease. The introduction of infliximab achieved a control of the uveitis and enabled the corticosteroid dose to be tapered. In some cases of sarcoidosis the lack of well-organised granuloma formation at the beginning of the disease, and the presence of prominent giant cells may suggest alternative diagnoses, such as non-LCH. Although the experience of tumour necrosis factor-α antagonists use in children with sarcoidosis is limited, these drugs may be helpful for those patients experiencing a severe and refractory disease., (Copyright © 2011 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.)

The reticulohistiocytoses make up a heterogeneous group of diseases whose origin lies in an accumulation of cells of histiocytic lineage in different tissues and primarily in the skin. Three main clinical forms have been described (multicentric, solitary, diffuse cutaneous), which present with identical histological, ultrastructural and immunohistochemical characteristics. We present a case of diffuse cutaneous reticulohistiocytosis, which is the least common clinical pattern in the spectrum of this disease.

The haemophagocytic syndrome is characterised by systemic proliferation of non-neoplastic histiocytes showing haemophagocytosis resulting in blood cytopenia. It has been described in relation to several viruses earlier. We present three patients with haemophagocytic syndrome (HFS) secondary to dengue haemorrhagic fever (DHF) confirmed by standard laboratory tests. The patients were hospitalized at the University Hospital (Hospital Universitario Ramón González Valencia-HURGV) in Bucaramanga, Colombia, during the past two years. They were all school-aged patients who presented DHF with intense abdominal pain, prolonged fever, hypotension and painful hepatomegaly. Laboratory tests showed thrombocytopenia, anaemia and leukopenia. A calculous cholecystitis was observed in the abdominal ultrasonography, and all bone marrow aspirations showed that platelets, red and white blood cells were phagocyted by histiocytes. According to the International Society of Histiocytosis, SHF is defined and classified in three major categories; the reported cases corresponded to histiocytosis class II, specifically to secondary SHF. Diverse associations of this syndrome correspond to viral infections and some other non-infectious diseases. A difference has been established between primary SHF and secondary SHF. Finally, we emphasize that these three patients had an atypical evolution of FHD, being prolonged fever and persistent abdominal pain the most important symptoms. The authors recommend that a bone marrow aspiration should be carried out as part of the differential diagnosis study in prolonged fever associated with dengue, as there is a possibility that this complication could be secondary SHF.

Familial hemophagocytic lymphohistiocytosis (FHL) is characterized by proliferation and non-malignant activation of histiocytes and T lymphocytes in the reticuloendothelial system. Diagnostic guidelines include fever, splenomegaly, cytopenia, hypertriglyceridemia and/or hypofibrinogenemia with hemophagocytosis in the bone marrow, spleen or lymph nodes. In many patients diagnosis is difficult due to the lack of diagnostic criteria, hemophagocytosis, variability of clinical presentation, spontaneous improvement and the absence of a specific marker of the disease. When there is strong clinical suspicion of FHL, chemotherapy and immunosuppressor treatment should be started early to achieve complete cure and should be followed by hematopoietic stem cell transplantation. We present the case of a 2-month-old girl who presented fever, anemia and thrombocytopenia, enlarged liver and spleen, hyperferritinemia, hypertriglyceridemia, and hypertransaminasemia without the finding of hemophagocytosis in bone marrow. Two of the girl's relatives had died of fulminant hepatic failure of unknown etiology. The patient improved spontaneously but presented reactivation of the disease 3 weeks later and died after splenic biopsy.

Macrophage activation syndrome, or hemophagocytic syndrome, is a rare disease with high morbidity and mortality. It is a disorder of the mononuclear phagocyte system. Two forms have been described; primary or familial hemophagocytic lymphohistiocytosis and secondary or sporadic hemophagocytic syndrome. Diagnosis of macrophage activation syndrome poses a real challenge for the pediatrician and in many cases is only made at autopsy. We describe two patients with hemophagocytic syndrome associated with parvovirus B19 and Epstein-Barr virus infection.

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.

The reactive hemophagocytic syndrome is a rare clinical-pathological entity which usually has a benign evolution and which is characterized by the systemic proliferation of mature histiocytes, with a great phagocytosis ability and which occurs in a secondary form in some infections and neoplasias and after the administration of certain drugs. We present a case of reactive hemophagocytic syndrome in a patient presenting a non-Hodgkin lymphoma, of crest malignancy, B immunoblastic and kappa monoclonal, with fatal evolution and in which the diagnosis was obtained after necropsy.

Reactive hemophagocytic syndrome (RHS) or hemophagocytic histiocytosis is a disease with anatomo-pathological features of systemic proliferation of non-neoplastic histiocytes, with prominent hemophagocytosis, associated to infection of other diseases. The cases of three patients afflicted with RHS are presented. 2 of them secondary to a brucellosis and the other of unknown origin. The clinical features were similar: high fever, wasting, and splenomegaly. Pancytopenia existed together with liver disfunction, CID and hyperferremia. Marrow infiltration of reactive histiocytes with important hemophagocytic phenomenon, demonstrated by aspirated and bone marrow biopsies, were observed in all cases. Studies of the immunology system were performed, showing changes in two of them. All of them fully recovered after antibiotic treatment.