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The incidence of the haemophagocytic syndrome in the ICU patients with multiple organ failure seems to be high. The haemophagocytic syndrome can be considered as the consequence of the initial aggression leading to multiple organ failure. On the contrary the haemophagocytic syndrome could be the cause of multiple organ failure. A case of haemophagocytic syndrome is presented which led to rapidly fatal multiple organ failure.

Haemophagocytic syndrome corresponds to an unconnected macrophagic activity with haemophagocytosis. We report the case of a haemophagocytic syndrome in a 49-year-old woman with initially a severe acute hepatic failure. This syndrome is probably underestimated in ICU patients. Haemophagocytic syndrome should be suspected in patients with fever and jaundice without infection.

Introduction: Necrobiotic xanthogranuloma is a rare disease, usually associated with a monoclonal gammapathy. We report a case with pericardial and pulmonary involvement that preceded the appearance of the cutaneous lesions., Observation: A 58 year-old woman was hospitalized for cardiac tamponade. She has a past history of a ductal carcinoma of the right breast that had been completely cured. Pericardial and pulmonary samples showed a predominantly histiocyte monomorphous infiltrate. Twelve months later, indurated papular-like cutaneous lesions appeared around the eye orbits and on the thorax. The histological examination of the skin revealed a necrobiotic xanthogranuloma and the diagnosis of cutaneous and visceral necrobiotic xanthogranuloma was retained. The patient exhibited an IgG Kappa monoclonal gammapathy of undetermined significance. Sequential treatment with melphalan and general corticosteroids moderately improved the cutaneous lesions but did not modify the monoclonal peak., Discussion: To our knowledge, this is the first case of pericardial involvement of a necrobiotic xanthogranuloma. The discovery of the disease, revealed by a visceral manifestation at the onset is uncommon. The subsequent appearance of typical cutaneous lesions permits the retrospective diagnosis of systemic necrobiotic xanthogranuloma. Because its diagnosis is difficult in the absence of concomitant cutaneous involvement, visceral localizations of the disease must be recognized.

Introduction: Multicentric reticulo-histiocytosis also known as lipoid dermoarthritis is a rare systemic disease leading to a massive osteoarticular destruction and systemic complications., Exegesis: This case report is a 44 year old black woman who was first seen with a rheumatoid arthritis clinical presentation associated with the presence of rheumatoïd factor. Five years later the diagnosis has been reconsidered after skin nodules histological examination. After that the patient has been lost from the follow up clinic. After a twenty years evolution she presented a complex clinical picture including: a cutaneous syndrome with a non pruriginous and hyperchromic papulonodular rash on the arms and fore-arms; a very destructive polyarthritis with major handicap; and systemic manifestations like cardiomyopathy with heart failure. The heart failure treatment associated first corticosteroids and secondary chloroquine was successful., Conclusion: The rheumatoid factor presence should not avoid to consider the possibility of multicentric reticulohistiocytosis in case of polyarthritis associated with a papulonodular rash. Then skin biopsy must be performed. The severity of osteoarticular and systemic lesions require an early prescription of a treatment for which there is so far no compromise.

Erdheim-Chester's Disease is a very uncommon variety of non-Langerhans histiocytosis of unknown etiology, which characteristically affects long bones bilaterally and symmetrically in adults. It may be accompanied by visceral foci of variable localization and extension determining prognosis. Bone scintigraphy is characteristic enough to evoke the disease but histologic examination of a peripheral specimen is required to confirm the diagnosis: spumous histiocytes CD68+, PS100+/-, CD1a-. We describe a case revealed by a severe lung disease with detailed autopsy.

Erdheim-Chester disease is a non-langerhans cell histiocytosis. It is a rare and plurifocal disease. Retroperitoneal involvement manifests as a mass associated with fibrosis, which is well visualized on CT scan and MRI. This disease is characterized by its potential to involve the whole retroperitoneum. We report a case of this disease that developed over twenty years, consisting of renal arteries stenosis, bilateral ureteral stenosis and evolutive adhesive capsulitis.

Between december 1996 and february 1998, rhinopharyngeal and tracheal aspirates from 165 children exhibiting symptoms compatible with M. pneumoniae infection were tested by a PCR method using in the same tube primers specific for M. pneumoniae P1 adhesin gene and for a human control gene. The positive cases were controlled using culture and/or serology. PCR was positive in 22 out of 165 samples (13.3%); an evaluation of the clinical and biological data was possible in 20 of these infected children. From 17 PCR positive respiratory samples tested by culture, 13 (76.5%) grew M. pneumoniae. From 14 serum specimens tested by ELISA, 12 exhibited specific IgM (3 of them with low titers); cold agglutinins were detected in all 7 tested sera. Only one case was not confirmed by any of the 3 former markers. The mean age of patients was 8.1 years. The main clinical symptoms included fever > 38 degrees C, cough, clinical and radiological pneumonia in 90, 95, 50 and 85% of cases, respectively. Neurological symptoms were the main clinical manifestation in 3 patients; another child exhibited a pneumonia associated to an hemophagocytic syndrome and a bone marrow failure which needed a graft. These results emphasize the value of PCR for the rapid diagnosis of M. pneumoniae infection in children.

Haemophagocytic syndrome (HM) is a severe disorder with characteristic clinical and biological features. The case of a patient with malignant lymphoma who experienced HM during Herpes virus I pulmonary infection is reported. Main clinical, biologial and pathological findings are described. The risk of underestimation of this disease in patients requiring intensive care as well as the absolute necessity of an early diagnosis are emphasised.

Background: Mycosis fungoides can mimic pigmented purpuric dermatitis. We report such a case which progressed to peripheral T-cell lymphoma; progression was revealed by reactive hemophagocytic syndrome (RHS)., Case Report: A 65-year old male patient was hospitalized for a pigmented and purpuric eruption. The skin lesions appeared 2 years earlier and at that time biopsy had shown pigmented and purpuric dermatitis. One month before hospitalization, general signs appeared. On admission, he had papular and purpuric rash, mainly on the trunk, hepatosplenomegaly, enlarged axillar and inguinal lymph nodes, and fever at 38.2 degrees. A skin biopsy showed histologic changes typical of mycosis fungoides. He also had bicytopenia, hepatitis, and increased triglyceride and ferritin levels suggesting RHS which was proved by means of bone marrow biopsy. These tests also evidenced peripheral T-cell lymphoma. The patient was treated with two courses of chemotherapy (CHOP) but the disease progressed and he deceased., Discussion: Mycosis fungoides can occasionally begin with an eruption very closely resembling pigmented purpuric dermatitis. Therefore, repeated biopsies should be done in case of widespread permanent pigmented purpuric dermatitis of no apparent origin. RHS is a life-threatening disease. The diagnosis should be suspected in any cytopenic patient with fever, increased triglyceride levels and abnormal liver tests. A search for an etiology must then be undertaken a prompt treatment is needed.

Background: Hemophagocytosis has already been reported in cases of visceral leishmaniasis and thus may complicate search for diagnosis., Case Report: A previously healthy 2 year-old boy was referred for fever and splenomegaly with pancytopenia. An initial diagnosis of kala-azar was refuted because of absence of biological inflammatory syndrome, negativity of bone-marrow aspiration and splenic ponction and of specific serology. After three months of clinical deterioration and apparition of active hemophagocytosis, both bone marrow aspiration and specific serology for visceral leishmaniasis became positive. The boy was given sodium stibogluconate for 20 days; he improved gradually with complete and definitive remission., Conclusion: Diagnosis of visceral leishmaniasis may be difficult, even in countries where this condition is relatively frequent; the association with hemophagocytosis is possible and does not constitute a poor factor of prognosis if specific therapy is proposed.

Introduction: The aim of this work was to present a case of multicentric histiocytosis with haematologic involvement., Case Report: A 68-year-old man presented with poor general health and a nodular eruption of the skin and larynx. On clinical examination there was an enlarged spleen and laboratory results revealed an inflammatory syndrome, platelet count 60,000 and myelemia with 10 p. 100 immature elements in a white cell count of 14,000. Pathology and ultrastructure examinations led to the diagnosis of multicentric histiocytosis. Bone marrow aspiration was normal. Pancytopenia then developed with bone marrow hypoplasia without infiltration. Corticosteroids then cyclophosphamide were uneffective for either the skin disease or the hematologic disorder. The patient developed severe buccal aphthosis which responded well to thalidomide. This treatment reduced the size and the number of skin nodules. Finally, renal failure of unknown origin was observed., Discussion: Electron microscopy is essential for positive and differential diagnosis of atypical multicentric histiocytosis. Hematological disorders associated with multicentric histiocytosis may either be specific or totally independent.

Thrombocytopenia is a common feature in ICU patients which occurs usually in case of infection or septic shock. Its mechanisms, which are often unclear, include the haemophagocytic syndrome initially linked with histiocytic proliferation but probably also associated with infectious diseases. This syndrome is characterized by a phagocytosis of medullar blood cells. Reactive haemophagocytic syndrome can probably lead to thrombocytopenia in ICU patients as in this case report of a E. Coli infection.

Background: Clinical onset of familial lymphohistiocytosis is non-specific so that the diagnosis of this rare and severe disease is difficult., Case Report: An 8 week-old girl was admitted suffering from fever and rash. She had hepatosplenomegaly. She developed pancytopenia (Hb: 6.6 g/100 ml; WBC: 4500/mm3; platelets: 25,000/mm3) impaired liver function tests (prothrombin: 15%, blood bilirubin: 40 mg/l; SGOT: 160 mU/ml) and hypofibrinogenemia (0.3 g/l) within a few days. Bone marrow examination showed diffuse histiocytic infiltration and erythrophagocytosis, suggesting a syndrome of inappropriate macrophage activation. The age of the patient, parental consanguinity and absence of specific infection led to diagnosis of familial erythrophagocytic lymphohistiocytosis. The patient died 18 days after clinical onset., Conclusion: The diagnosis of this unusual syndrome in infants is strongly supported by parental consanguinity as seen in our case or a positive family history. In this condition, erythrophagocytosis is often a marked feature.

Erdheim-Chester's disease is a rare form of visceral xanthogranulomatosis. We report a case of a patient aged 50 presenting with a diffuse interstitial pneumonia which revealed Erdheim-Chester's disease with localisation in the bones, peri-aortic region and also with neurological involvement. The diffuse interstitial pneumonia which progressed chronically was characterised by a diffuse thickening of the septa with subpleural cysts and bilateral apical bullae with thickening of the pleura. Respiratory function tests showed a restrictive ventilatory defect with resting hypoxaemia which was aggravating by exercise. Broncho-alveolar lavage showed a lymphocytosis (26%) also with polymorpho neutrophils (11%). The pathological diagnosis was confirmed by transbronchial lung biopsy showing an excess of foamy histiocytes in the interstitium which are characteristic of the disease. The progress of this interstitial pneumonia is stabilised without immunosuppressive drugs.

The authors report the case of a patient who had both multicentric reticulohistiocytosis--a systemic disease characterized by symmetric polyarthritis and papulonodular skin lesions--and an ovarian adenocarcinoma. Synovial fluid analysis found histiocytes with a ground-glass cytoplasm, binucleate giant cells, and undifferentiated cells undergoing mitosis. PAS-positive, diastase-resistant cells similar to the synovial fluid histiocytes were seen in the synovial and cutaneous biopsy specimens. These findings established the diagnosis of multicentric reticulohistiocytosis and suggested an underlying malignancy. Whether or not multicentric reticulohistiocytosis is a paraneoplastic syndrome remains unsettled. However, our report provides evidence that synovial fluid analysis can be helpful not only for the early diagnosis of multicentric reticulohistiocytosis but also for suggesting the presence of an underlying tumor, especially when arthritis is the presenting symptom.

An 11-year-old girl presented with a typical serologically proven infectious mononucleosis with persistent fever, jaundice and hepatosplenomegaly in spite of steroid therapy. Laboratory tests showed pancytopenia, fibrinopenia and hypertriglyceridemia. The liver biopsy revealed an infiltration with hyperbasophilic cells. One month later, a slight improvement was noted and fever disappeared after 4 days on acyclovir therapy. The authors recall the spectrum of the macrophagic activation syndrome.

Xanthosiderohistiocytosis is a rare non-Langerhans histiocytosis (4 reported cases). The clinical characteristics include diffuse, sometimes deep, dark-brown infiltrations of the skin. Histological examination reveals abundant deposits of haemosiderin within the histiocyte proliferation. This entity is a clinical form of Montgomery's disease (xanthoma disseminatum) which has been reported in over 100 cases. Our case of disseminated xanthosiderohistiocytosis was particular because it involved the heart and was associated with a monoclonal gammapathy. Five cases have been reported associating xanthoma disseminatum and monoclonal gammapathy, including one case of xanthosiderohistiocytosis. In our case, rapidly increasing levels of monoclonal immunoglobulin suggested an evolution towards a myeloma. A monoclonal gammapathy should be looked for and monitored in cases of xanthosiderohistiocytosis, and more generally xanthoma disseminatum because of the risk of developing lymphoma or myeloma.