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The different patterns of histiocytosis X are usually classified into three forms according to their clinical and pathological expression. One form is acute and disseminated ; the two others are chronic, unifocal or multifocal. Borderline cases and intermediate forms can be seen. For instance, onset
Langerhan's cell histiocytosis (LCH) is a rare disorder in which granulomatous deposits occur at multiple sites within the body. The aetiology is unknown. Is more frequent in children. Presenting symptoms are polyuria and polydipsia, due to diabetes insipidus, other symptoms are skin rash, dyspnea
Langerhans cell histiocytosis (LCH) occurs as a clonal disease with enigmatic immune responses. LCH patients occasionally present with fever, although the significance remains elusive. We investigated the predicting factors for developing intractable disease of refractory and/or reactivated LCH. In
Systemic histiocytosis was found in a patient who presented with chronic, nodular, and ulcerative skin lesions. The patient's hospital course was complicated by persistent fever, thrombocytopenia, severe neutropenia, and coagulation abnormalities. Treatment consisted only of splenectomy and
Hematophagocytic histiocytosis is a clinicopathologic syndrome associating fever, liver dysfunction, blood cytopenia and coagulation abnormalities with hematophagocytosis in bone marrow and lymphoïd organs. This syndrome is found in immunocompromized patients and is triggered by infection. We
BACKGROUND
Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial lung disease characterized by bilateral nodular and cystic lesions. Clinically it seems to be a reactive process related to cigarette smoking.
METHODS
In 2 cases of PLCH, cytologic and immunocytochemical evaluation of
OBJECTIVE
To improve the recognition of the clinical presentation and radiologic manifestation of children with Langerhans cell histiocytosis (LCH) with pulmonary involvement.
METHODS
A retrospective analysis was conducted on children who presented with respiratory symptoms or abnormal lung
Malignant histiocytosis, as defined by Rappaport, is now known as a manifestation of malignant lymphoma, the majority of which is the T-cell type. However, unlike the typical presentation of most non-Hodgkin lymphomas, this condition presents with atypical features mimicking acute hepatitis or
A 70-year-old woman was admitted for evaluation of hepatosplenomegaly, fever and elevated serum LDH levels. A biopsy specimen of the liver revealed histiocytic proliferation at the portal triad, and a mild degree of hepatitis. A bone marrow biopsy specimen showed proliferation of histiocytes with
Clinicopathological features of this female patient described here, showing unusual and extremely marked sinus histiocytosis of lymph nodes with considerable systemic lymphadenopathy, accompanying fever as well as acceleration of erythrocyte sedimentation rate fairly well coincided with the symptoms
The patient is 11-year-old girl who was diagnosed as having hybrid acute leukemia (myeloid and lymphoid) from morphological (cytochemistry) findings, immunophenotype and genotype. During reinduction therapy after a second relapse, she presented hepatosplenomegaly and the remittent fever unresponsive
BACKGROUND--Histiocytosis of Langerhans cells includes a range of clinical manifestations that have been described as bone eosinophilic granuloma, Hand-Schüller-Christian syndrome, Letterer-Siwe syndrome and Hashimoto-Pritzker histiocytosis. These syndromes represent a spectrum of severity and